tag:blogger.com,1999:blog-34953233789088774162024-02-19T07:21:58.717-08:00Life As A Pediatric NurseLindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comBlogger23125tag:blogger.com,1999:blog-3495323378908877416.post-78022149786188147182010-07-13T04:28:00.000-07:002010-07-13T04:33:22.321-07:00Sweet's Syndrome<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgaBNQWxgJ9YkS1ZXaimOHrbtswV0sJi8UMFKNwzQ7x8tboGsTeVkpBHls66ScEYUZ7YwlwNqhkDoVqt8f2XDuwAwYmNrUspuf2DC_Hk2_a4bCnyylCIU_A0a0F8vPGWAUOBtluniUdjFal/s1600/r7_sweetssyndrome.jpg"><img id="BLOGGER_PHOTO_ID_5493352406819890418" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 400px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgaBNQWxgJ9YkS1ZXaimOHrbtswV0sJi8UMFKNwzQ7x8tboGsTeVkpBHls66ScEYUZ7YwlwNqhkDoVqt8f2XDuwAwYmNrUspuf2DC_Hk2_a4bCnyylCIU_A0a0F8vPGWAUOBtluniUdjFal/s400/r7_sweetssyndrome.jpg" border="0" /></a><br /><div><strong>Sweet's syndrome</strong> — also known as acute febrile neutrophilic dermatosis — is a skin condition marked by fever and painful skin lesions that appear mainly on your arms, face and neck.<br />The exact cause of Sweet's syndrome isn't always known. In some people, it's triggered by an infection, illness or certain medications. Sweet's syndrome can also occur with some types of cancer. </div><br /><div><br />Most often, Sweet's syndrome will clear on its own in a few months or once the underlying cause is resolved or eliminated. Healing is much more rapid with treatment.<br /></div><br /><div><strong>Symptoms</strong></div><br /><div>The most obvious signs of Sweet's syndrome are distinctive skin lesions that usually develop according to this pattern: A series of small red bumps appear suddenly on your arms, neck, face or back, often after a fever or upper respiratory infection. The bumps grow quickly in size, spreading into clusters called plaques that may be up to an inch or so in diameter. The eruptions are tender or painful and may develop blisters, pustules or even ulcers. Lesions may persist for weeks to months and then disappear on their own, without medication. With medical treatment, you're likely to be free of skin lesions in just a few days. </div><br /><p>Other signs and symptoms of Sweet's syndrome may include:<br />- Moderate to high fever preceding the skin lesions<br />- Pink eye (conjunctivitis) or sore eyes<br />- Tiredness<br />- Aching joints and headache<br />- Mouth ulcers </p><br /><p>When to see a doctorSweet's syndrome is rare. When it occurs, it often develops after an upper respiratory tract infection. If you develop a red rash that quickly grows in size soon after a bout with strep throat or another upper respiratory infection, see your doctor for appropriate treatment. Although the rash may eventually disappear without treatment, the right medication can make the rash go away in just a few days. </p><br /><p>Sweet's syndrome may also be a reaction to a more serious condition, such as leukemia, or to certain medications. </p><br /><p><strong>Causes<br /></strong>Although Sweet's syndrome may be associated with infections, the condition itself isn't infectious. Sweet's syndrome is typically divided into three categories, depending on its associations:<br />Idiopathic (classical). In most cases, the cause of Sweet's syndrome isn't known (idiopathic). Idiopathic Sweet's syndrome predominantly affects women between the ages of 30 and 50, and is often preceded by an upper respiratory or gastrointestinal infection. It's also been associated with pregnancy and inflammatory bowel disease. </p><br /><p><em>Malignancy-associated</em>. In about 20 percent of cases, Sweet's syndrome is associated with cancer (malignancy), most often acute leukemia. A few cases may be associated with a solid tumor, such as breast or colon cancer. Sweet's syndrome can occur as an early sign of a cancer, after diagnosis or as a sign of a recurrence. Fever is often present but skin lesions typically aren't preceded by an upper respiratory infection, as is the case with idiopathic Sweet's syndrome. Malignancy-associated Sweet's syndrome appears to affect men and women equally but among older adults, it's more likely to occur in women. </p><br /><p><em>Drug-induced</em>. Although uncommon, Sweet's syndrome may occur as a reaction to a medication, most commonly to granulocyte colony-stimulating factor, a hormone preparation designed to increase your white blood cell count. Other medications associated with Sweet's syndrome include certain antibiotics, oral contraceptives, diuretics and anti-epileptic drugs, among others. Once the offending drug is discontinued, Sweet's syndrome usually goes away.<br /><strong></strong></p><br /><p><strong>Risk factors</strong><br />Sweet's syndrome is uncommon, but certain factors increase your risk, including:<br />Being a woman. Women are far more likely to have idiopathic Sweet's syndrome than men are.<br />Being between 30 and 50 years of age. Though older adults and even infants can develop Sweet's syndrome, the condition mainly affects women between the ages of 30 and 50.<br />Having other health problems. Sweet's syndrome often follows an upper respiratory infection, and many people report having flu-like symptoms before the rash appears. Sweet's syndrome can also be associated with a number of other illnesses, including inflammatory bowel disease, certain systemic infections and cancer. </p><br /><p><br /><em>Being pregnant</em>. Some pregnant women develop Sweet's syndrome during their first or second trimester. In these cases, the condition usually clears without treatment.<br />A previous history of the condition. Sweet's syndrome tends to recur. About one-third of people who have had Sweet's syndrome once get it again. </p><br /><p><strong>Complications</strong><br />There is a risk of the skin lesions becoming infected. Follow your doctor's recommendations for caring for the affected skin. The most challenging aspect of Sweet's syndrome may be in dealing with recurrences, which occur in about a third of cases. Signs and symptoms may reappear, especially if treatment is tapered off too quickly. Be certain to follow your treatment plan exactly as your doctor recommends. </p><br /><p><br /><strong>Preparing for your appointment:<br /></strong>Your family doctor or general practitioner is likely to refer you to a dermatologist for diagnosis and treatment of Sweet's syndrome. If tests reveal an underlying condition, you'll also be referred to the appropriate specialist, such as a gastroenterologist or oncologist.<br />Because appointments can be brief and there's often a lot of ground to cover, it can help to be well prepared. Here are some tips to help you get ready for your appointment and what to expect from your doctor. </p><br /><p><br /><em>What you can do...</em>Write down all your signs and symptoms — even those that seem unrelated to your rash. Sweet's syndrome can be a sign of several illnesses, so it's important that your doctor know all of your symptoms. Include key personal information, such as major stresses or recent life changes. Make a list of all medications, including vitamins, herbs and over-the-counter drugs, that you're taking. Even better, take the original bottles and a written list of the dosages and directions. If possible, take along a family member or friend. It can be difficult to absorb all the information provided to you during an appointment. The person who accompanies you may remember something that you forgot or missed.<br />Write down questions that you want to ask your doctor. Don't be afraid to ask questions or to speak up when you don't understand something your doctor says. Start with the problems that concern you most. If you run out of time, ask to speak with a nurse or physician's assistant or leave a message for your doctor. </p><br /><p>If you have symptoms of Sweet's syndrome, questions you may want to ask include:<br />What might be causing my rash?<br />What tests do I need to confirm the diagnosis?<br />Is this condition temporary or chronic?<br />What is the best course of action?<br />What are the alternatives to the primary treatment approach that you're suggesting?<br />I don't like the idea of taking steroids. Are there other medications you can prescribe?<br />Is there a generic alternative to the medicine you're prescribing me?<br />What if I just wait to see if my signs and symptoms go away on their own?<br />What to expect from your doctorYour doctor is likely to ask you a number of questions, such as:<br />When did your symptoms start?<br />Did they come on suddenly or gradually?<br />What did the rash look like when it first appeared?<br />Is the rash painful?<br />What, if anything, makes it better?<br />What, if anything, makes it worse?<br />Were you sick before the rash started?<br />Do you have other symptoms that started about the same time?<br />What medications do you take?<br /></p><br /><p><strong>Tests and diagnosis</strong><br />Your dermatologist can usually diagnose Sweet's syndrome simply by looking at the lesions. But you're likely to have certain tests to rule out conditions that have similar symptoms and to search for the underlying cause. </p><br /><p>These tests include:<br />Blood tests. A small sample of your blood may be sent to a laboratory where it's checked for an unusually large number of white blood cells and certain blood disorders.<br />Tissue sample. Your doctor may remove a small piece of affected tissue (biopsy) for examination under a microscope. The tissue is analyzed to determine whether it has the characteristic abnormalities of Sweet's syndrome. The area where the sample is taken is numbed, and a small piece of skin is removed with an instrument that looks like a small cookie cutter — a procedure called a punch biopsy. You're not likely to need stitches, and the incision should heal without scarring. </p><br /><p><br /><strong>Treatments and drugs<br /></strong>Left untreated, Sweet's syndrome not associated with a more serious condition may disappear on its own within one to three months. Medications can improve skin lesions and associated symptoms in just two or three days, with the worst of the lesions disappearing within one to four weeks. This is true even for malignancy-associated Sweet's syndrome, although treatment or remission of the associated cancer will help, too. </p><br /><p>With or without treatment, the lesions rarely leave a mark or scar when they eventually disappear. Your doctor may advise continuing treatment because recurrence of the condition is common. </p><br /><p>Medications Systemic corticosteroids (prednisone or prednisolone) are generally very effective in treating Sweet's syndrome. You typically take these oral anti-inflammatory medications for about four to six weeks. Topical corticosteroids may be used to provide immediate relief of swelling. </p><br /><p>Other first line medications your doctor may use include potassium iodide therapy, which you take as an oral tablet or as drops, and colchicine, which has anti-inflammatory properties. Follow your doctor's instructions exactly when taking these medications and be sure your doctor knows about any other medications you're taking, to avoid harmful drug interactions.<br />Lifestyle and home remedies</p><br /><p>If you have Sweet's syndrome, it's important to treat your skin gently. These steps can help reduce additional injury to the skin:<br />Avoid injury to your skin. Wear protective clothing if you think you might injure or damage your skin. </p><br /><p>Apply sunscreen. Use sunscreen with a sun protection factor (SPF) of 15 or greater before you head outdoors. </p><br /><p><a id="link_references" href="javascript:toggleDivSlide("><span style="font-size:78%;">References</span></a><span style="font-size:78%;"><br />Farhi D, et al. The neutrophilic dermatoses. Dermatology Nursing. 2008;20:274.<br />Moschella SL. Neutrophilic dermatoses. http://www.uptodate.com/home/index.html/. Accessed April 5, 2010.<br />Sweet's syndrome: A dermatologic condition associated with fever and frequently confused with an infectious process. In: Mandell GL, et al. Mandell, Douglas, and Bennet's Principles and Practice of Infectious Diseases. 7th ed. Philadelphia, Pa.: Churchill Livingstone Elsevier; 2010. http://www.mdconsult.com/book/player/book.do?method=display&type=bookPage&decorator=header&eid=4-u1.0-B978-0-443-06839-3..00052-7--s0060&uniq=193269557&isbn=978-0-443-06839-3&sid=978680989#lpState=open&lpTab=contentsTab&content=4-u1.0-B978-0-443-06839-3..00052-7--s0060%3Bfrom%3Dtoc%3Btype%3DbookPage%3Bisbn%3D978-0-443-06839-3. Accessed April 5, 2010.<br />Cohen PR. Sweet's syndrome — A comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet Journal of Rare Diseases. 2007;2:34.<br />Franks AG Jr. Skin manifestations of internal disease. Medical Clinics of North America. 2009;93:1265.<br />Colchicine: Drugdex Evaluations. Micromedex Healthcare Series. http://www.micromedex.com. Accessed April 5, 2010.<br />DS00752<br />June 24, 2010<br />© 1998-2010 Mayo Foundation for Medical Education and Research (MFMER). All rights reserved. A single copy of these materials may be reprinted for noncommercial personal use only. "Mayo," "Mayo Clinic," "MayoClinic.com," "EmbodyHealth," "Enhance your life," and the triple-shield Mayo Clinic logo are trademarks of Mayo Foundation for Medical Education and Research. </span></p>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-63375412860804623682010-05-11T20:39:00.000-07:002010-05-11T20:43:25.523-07:00Total Anomalous Pulmonary Venous Return<div align="center">Total anomalous pulmonary venous return (TAPVR) is a rare congenital heart defect that causes cyanosis or blueness. The basic problem is that the blood flow coming back from the lungs (which is full of Oxygen) through pulmonary veins is diverted to the right atrium so that there is complete mixing of blood (red and Blue) within the heart. These abnormalities are divided into two major groups - obstructed and unobstructed. Unobstructed is the most common and this frequently involves the common pulmonary vein being connected by an abnormal vertical vein to the superior vena cava and emptying into the right atrium. There can also be a direct connection of the common pulmonary vein to the right atrium. Either way this causes complete mixing of the blood within the right atrium. These babies are usually a little blue at birth, but not necessarily very sick. Many times they will go home from the nursery and be found to be breathing fast by the pediatrician. This is because the mixing of blood causes the heart to work harder than normal. This is usually relatively easy to fix. It does involve open-heart surgery. Once fixed, the heart is for the most part normal. </div><div align="center"> </div><div align="center">Obstructive veins are another matter. These babies are usually very ill at birth. In this situation the blood flow leaving the lungs through the pulmonary veins is not only going to the wrong place but is at least partially blocked, this makes it harder for blood to enter the lungs. Most of the time these abnormal connections occur in the liver. They can occur in the upper part of the chest as well. When the veins are obstructed It also makes the pressure in the right ventricle higher than normal. When blood cannot easily enter the lungs the babies are very blue because not enough blood goes to the lungs and there is a lot of mixing of blood (blue blood with red blood) at the level of the PDA and Foramen Ovale (opening in wall between the upper chambers of the heart). Because not enough oxygen is being circulated in the body the baby becomes acidotic and the muscle tissue and kidneys, etc. do not work well. When the oxygen level is low, the arteries in the lungs tend to tighten up, making it harder for blood to be pumped through the lungs and thus the situation even worse. When the lungs are full of blood they become stiff, making it harder to breathe. Very frequently in this condition the actual veins draining back from the lungs are smaller than normal. This is because they never developed normally in the first place. This condition is lethal unless it can be fixed quickly after diagnosis. </div><div align="center"> </div><div align="center">Most of the time we are able to diagnose this condition by echocardiogram. One can see the abnormal flow patterns of blood coming from the lungs into the SVC or we can see abnormal patterns in the liver, which is where most of the obstructed veins return. We also look to see if we can see the normal pulmonary veins returning to the left atrium. We can measure oxygen levels in the right atrium with an umbilical catheter and this will be abnormally high. </div><div align="center"> </div><div align="center">TAPVR can closely mimic a condition called persistent fetal circulation. In this case the pulmonary veins are normal but very little blood flow is going to the lungs. This can sometimes occur with stressed or infected babies and can be very difficult to treat. The pressures in the lungs can be very high and little blood flow goes through the lungs. As with TAPVR this is a lot of mixing of blood within the heart and the oxygen level can be quite low. This condition can be treated with a ventilator, pulmonary vasodilators, nitric oxide and sometimes ECMO. It can be very difficult to distinguish these two conditions as they have many similar clinical features. </div><div align="center"> </div><div align="center">TAPVR is not something that can be treated medically. The longer the delay in surgery, the worse the outcome. This condition is frequently associated with other rare conditions such as dextrocardia, and ambiguous situs. In this situation a baby's internal organs can have either two right sides or two left sides. </div><div align="center"> </div><div align="center">The outcome for unobstructive TAPVR is usually quite good. The outcome of obstructive TAPVR can vary and ultimately depends on the overall size of the pulmonary veins. Many attempts have been made to enlarge these surgically and with balloons and stents but the results are seldom satisfactory. </div><div align="center"> </div><div align="center">If you have any questions, please ask one of the doctors. </div><div align="center"></div><div align="center"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgtcjcw7pXCfewdg3LCjFUXJ20ciPOtPpwE1ZT2QEIaqVWdLGOFhwT-SRUfrToZOXdIQdlpeueOi8ZYaMz8S2yuDSOH89DseJEMm-ko0Bok1lpfwLT-GtfwiFfkYS_5xYiwhZ_gV-BploUd/s1600/Picture%20125.jpg"><img id="BLOGGER_PHOTO_ID_5470223440706187634" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 377px; CURSOR: hand; HEIGHT: 510px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgtcjcw7pXCfewdg3LCjFUXJ20ciPOtPpwE1ZT2QEIaqVWdLGOFhwT-SRUfrToZOXdIQdlpeueOi8ZYaMz8S2yuDSOH89DseJEMm-ko0Bok1lpfwLT-GtfwiFfkYS_5xYiwhZ_gV-BploUd/s400/Picture%2520125.jpg" border="0" /></a><span style="font-size:78%;"><br />Click </span><a href="http://www.carson-appleton.com/total_anomalous_pulmonary_venous_return.htm"><span style="font-size:78%;">here</span></a><span style="font-size:78%;"> for source.</span></div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-33137422472181746572010-04-14T00:30:00.000-07:002010-04-14T00:36:46.194-07:00Excercise-Intuced Anaphylaxis (EIA)<strong>Background</strong><a id="IntroductionBackground" name="IntroductionBackground"></a><br />Exercise-induced anaphylaxis (EIA) is a syndrome in which patients experience the symptoms of anaphylaxis, which occur only after increased physical activity. The symptoms include pruritus and urticaria (typically with giant hives), and, without emergency intervention, the patient may develop hypotension and collapse. Now increasingly recognized as more children and teenagers participate in physical activities and sports, exercise-induced anaphylaxis may become more common in the future. Those affected by the syndrome are typically accomplished athletes and have a history of atopy, but anyone can be affected.<br />The types of physical activities that have triggered episodes of exercise-induced anaphylaxis have included walking, dancing, racquet sports, swimming, jogging, bicycling, skiing, basketball, and sprinting. Hot humid weather and cold weather can precipitate episodes in some patients. If a patient has recurrent exercise-induced anaphylaxis, the episodes tend to be worse in the summer months. The first reported case of exercise-induced anaphylaxis was in 1979 by Maulitz and coworkers and was food-related, occurring in a 31-year-old patient who had ingested shellfish prior to long-distance running.<a href="javascript:showcontent("><a href="javascript:showcontent(">1</a> </a>Since then, many different allergens have been reported in the literature to have caused exercise-induced anaphylaxis, including shrimp, oyster, celery, cheese sandwiches, pizza, wheat gliadin,<a href="javascript:showcontent("><a href="javascript:showcontent(">2</a> </a>eggs, peaches, grapes, pomegranites,<a href="javascript:showcontent("><a href="javascript:showcontent(">3</a> </a>chick peas,<a href="javascript:showcontent("><a href="javascript:showcontent(">4</a> </a>pears, poppy seeds, soybean,<a href="javascript:showcontent("><a href="javascript:showcontent(">5</a> </a>and snails (which have been reported to have cross-reactivity with dust mites).<br />In 1980, Sheffer and Austen provided the first report of patients with exercise-induced anaphylaxis.<a href="javascript:showcontent("><a href="javascript:showcontent(">6</a> </a>Sixteen patients, aged 12-54 years, experienced exercise-induced anaphylaxis without a specific allergen exposure. Ten of these patients had onset of exercise-induced anaphylaxis in their teenage years, indicating that those who care for pediatric patients should be aware of this syndrome.<br />Exercise-induced anaphylaxis has been categorized in a few different ways in the literature. Classic exercise-induced anaphylaxis is the most common type. Sheffer and Austen (1980) originally described 4 phases in the sequence of symptomatology of classic exercise-induced anaphylaxis.<a href="javascript:showcontent("><a href="javascript:showcontent(">6</a> </a>A prodromal phase is characterized by fatigue, warmth, pruritus, and cutaneous erythema. The early phase follows, with the urticarial eruption that progresses from giant hives (about 10-15 mm in diameter) to become confluent and may include angioedema of the face, palms, and soles. Then, the fully established phase occurs, which can include hypotension, syncope, loss of consciousness, choking, stridor, nausea, and vomiting and can last 30 minutes to 4 hours. The final phase is the late or postexertional phase, which is characterized by prolonged urticaria and headache persisting for 24-74 hours.<br />Another type of exercise-induced anaphylaxis is variant-type exercise-induced anaphylaxis, which is similar to classic exercise-induced anaphylaxis, except the typical giant hives are not observed. In their place are small punctate skin lesions, more typical of cholinergic urticaria, but the syndrome does lead to hypotension and collapse if allowed to progress. The variant type of exercise-induced anaphylaxis accounts for approximately 10% of cases.<br />Familial exercise-induced anaphylaxis has been described involving patients with a family history of exercise-induced anaphylaxis and atopy. No inheritance pattern has been established.<br />Two forms of food-dependent exercise-induced anaphylaxis have been described. Inherent in the definition of food-dependent exercise-induced anaphylaxis is that the food or exercise alone does not produce symptoms. First, specific-food exercise-induced anaphylaxis in which a specific food is known to be the offending allergen is recognized. Second, nonspecific-food exercise-induced anaphylaxis in which no specific food is known, but eating any food prior to exercise causes symptoms of exercise-induced anaphylaxis is also recognized.<a href="javascript:showcontent("><a href="javascript:showcontent(">7</a> </a><br />The last type of exercise-induced anaphylaxis described is medication-dependent or drug-dependent exercise-induced anaphylaxis. This category includes patients who develop the syndrome only after ingesting a specific medication and then exercising. The offending medications that have been reported include nonsteroidal anti-inflammatory drugs (NSAIDs), aspirin, antibiotics, and cold remedies.<br /><a name="0104"></a><br /><strong>Pathophysiology</strong><a id="IntroductionPathophysiology" name="IntroductionPathophysiology"></a><br />In exercise-induced anaphylaxis, an exercise-induced lowering of the mast cell degranulation threshold occurs, which causes the release of histamine and other mediators and leads to the progression from pruritus and urticarial rash to the symptoms of anaphylaxis. In the food-dependent subset, this process is influenced by immunoglobulin E (IgE) mast cell sensitization by a known or unknown food. If the offending food is known, the amount of the specific food ingested has an effect on whether the patient has symptoms. The mechanism by which exercise lowers the mast cell degranulation threshold is unknown. Previous observations suggest that increased physical activity has a direct effect on mast cell releasability and does not result in an increased sensitivity to histamine.<br />Once the histamine and other mast cell mediators, including leukotrienes, are released, they cause the smooth muscle contraction responsible for the wheezing and GI symptoms. The histamine and other mast cell mediators also cause the vascular dilatation that leads to the escape of plasma into the tissues, causing urticaria and <a href="http://emedicine.medscape.com/article/885100-overview">angioedema</a>, and results in hypotension and shock.<a href="javascript:showcontent("><a href="javascript:showcontent(">8</a>,<a href="javascript:showcontent(">9</a> </a><br /><a name="0105"></a><br /><strong>Frequency</strong><a id="IntroductionFrequency" name="IntroductionFrequency"></a><br />United States<a id="IntroductionFrequencyUnitedStates" name="IntroductionFrequencyUnitedStates"></a><br />Prevalence is not well established. In one study, 9% of total episodes of childhood anaphylaxis and 20% of episodes in children older than 8 years were triggered by exercise.<br />International Case reports from Germany, Italy, Japan, United States, and Thailand are provided in the literature.<br /><a name="0108"></a><br /><strong>Mortality/Morbidity</strong><a id="IntroductionMortalityMorbidity" name="IntroductionMortalityMorbidity"></a><br />Deaths of children have been reported, but they are rare. Infrequently, patients must alter their lifestyle and physical activity significantly; in some patients, the syndrome causes them to be unable to perform daily activities without the risk of anaphylactic syndrome.<a name="0109"></a><br /><br /><strong>Race</strong><a id="IntroductionRace" name="IntroductionRace"></a><br />No racial predilection is known.<a name="0110"></a><br /><br /><strong>Sex</strong><a id="IntroductionSex" name="IntroductionSex"></a><br />One study showed a slight male predominance, but most other studies show no overwhelming difference between sexes.<a name="0111"></a><br /><br /><strong>Age</strong><a id="IntroductionAge" name="IntroductionAge"></a><br />Exercise-induced anaphylaxis has been reported from as young as 4 years into adulthood. In a study of 16 patients, 10 patients (63%) had onset in their teenage years.<a name="02"></a><br /><br /><strong>Clinical</strong><a id="Clinical" name="Clinical"></a><a name="0216"></a><strong> History</strong><a id="ClinicalHistory" name="ClinicalHistory"></a><br />Pediatric patients with exercise-induced anaphylaxis (EIA) typically are athletic or involved in school or otherwise organized sports, and they typically have a history of atopy and/or a family history of atopy or possibly of exercise-induced anaphylaxis.<br />Typical episodes occur after exercise on a particularly hot, humid, or cold day.<br />History of ingesting aspirin or other nonsteroidal anti-inflammatory drug (NSAID), a meal, or a specific food prior to exercising may be noted.<br /><br />In women, the episodes can be more frequent and more severe before and during menstrual cycles.<br /><br />The history of an episode most likely includes the initial pruritus and giant hives associated with the onset of the symptoms.<br /><br />As the syndrome progresses, the patient may report nausea, cramping, diarrhea, vomiting, tinnitus, vertigo, pruritus, difficulty breathing, chest tightness, and wheezing; a syncopal episode may occur.<br /><br />The history may be obtained from a paramedic who responded to the collapse of a child. In this case, the patient's history may include loss of consciousness or variable consciousness.<br />In several minutes or hours after the episode, the patient may report only a headache that can persist for as long as 3 days.<br /><a name="0217"></a><br /><strong>Physical</strong><a id="ClinicalPhysical" name="ClinicalPhysical"></a><br />The physical examination should start with the airway, breathing, and circulation (ABCs).<br />The most emergent assessments are those of airway maintenance and level of consciousness. One must rule out laryngeal obstruction.<br />Simultaneously assess for hypotension.<br />The rest of the physical examination should include looking for the typical features of exercise-induced anaphylaxis, including urticaria and giant hives, angioedema, wheezing, and stridor.<br /><a name="0218"></a><br /><strong>Causes</strong><a id="ClinicalCauses" name="ClinicalCauses"></a><br />Risk factors for exercise-induced anaphylaxis include personal or family history of exercise-induced anaphylaxis or atopy, male sex (in one study), exposure to food allergen, and extremes of weather.<br /><br />Beta-blocker medications can aggravate anaphylactic episodes.<br /><br /><span style="font-size:85%;">Click </span><a href="http://emedicine.medscape.com/article/886641-treatment"><span style="font-size:85%;">here</span></a><span style="font-size:85%;"> to read more...</span>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-72446549617990896852010-04-14T00:15:00.000-07:002010-04-14T00:30:21.369-07:00I-Cell Disease (Mucolipidosis II)<strong>Summary</strong><br />I-cell disease is an example of the mucolipidoses, a<br />group of diseases which show features of both the<br />mucopolysaccharidoses and the sphingolipidoses. A<br />clinical description is given of a child suffering from<br />this condition. The diagnostic criteria are discussed,<br />as well as some of the necropsy findings.<br />THE genetic mucolipidoses are a group of diseases<br />which show the symptoms and signs of both the<br />mucopolysaccharidoses and the sphingolipidoses<br />(Table 1). Some of them such as Gm2 gangliosidosis<br />and infantile sulphatidosis are related to known<br />enzyme defects, but in others the cause is unknown.<br />Among the latter some of the affected children have<br />been described as Hurler's variants as they show<br />many of the features of Hurler's syndrome, but<br />excrete normal amounts of urinary mucopolysaccharides.<br /><br /><strong>Case report</strong><br />M.W. born 29 May 1967.<br />The child was referred to Booth Hall Children's<br />Hospital, Manchester, at the age of 2- years. She<br />had been born at home and was the second child in<br />the family, the older sibling having developed<br />normally. The pregnancy and birth were normal.<br />Multiple deformities had been recognized from birth<br />and she had been treated for a dislocation of the<br />right hip. At the time of her referral she could not<br />sit up herself and made no effort to stand. In general<br />development was around 8-9 months. Three times<br />in the past year the child had lost consciousness and<br />become cyanotic. There was no other past history or<br />family history of note.<br /><br />On examination the unusual appearance of the<br />child was highly suggestive of gargoylism (Figs. 1<br />and 2). The bridge of the nose was broad and<br />flattened, the nostrils anteverted, and the tongue<br />was large. The eylids were puffy, the eybrows prominent,<br />and the cheeks were highly coloured. The<br />skin was coarsened. The abdomen was protuberant<br />and chest expansion was limited. There was flattening<br />of the left side of the skull and a fairly prominent<br />lumbo-dorsal kyphosis. There was fair movement in<br />the legs and feet, but the gluteal muscles appeared<br />to be weak. The right thumb was flexed in the palm<br />and could not be extended or abducted. Both hips<br />were held in about 45° of abduction by contractures,<br />probably in the abductor muscles. There was no<br />clouding of the cornea and the optic fundi appeared<br />normal. Muscle tone was slightly reduced, but the<br />tendon reflexes were present and equal. The liver<br />was enlarged one and a half finger's breadth, but the<br />spleen was not palpable, and there was no evidence<br />of cardiac involvement.<br /><br />Over the next 2 years the child was greatly<br />troubled by chest infections, sometimes severe<br />enough to be classified as broncho-pneumonia. She<br />showed some evidence of development, and began<br />to stand in splints and seemed to benefit from wearing<br />a spinal jacket. She started to say a few words.<br />At the age of 2 years 7 months the patient weighed<br />7-05 kg (third percentile at this age 10-4 kg), and her<br />height was 70-1 cm (third percentile at this age<br />83 cm). The GQ on the Griffiths Mental Development<br />Scale was 27-2. X-ray of the skull showed very<br />marked asymmetry. On X-ray of the spine and pelvis<br />there was scoliosis convex to the left, and widening<br />of the interpedicular spaces in the lumbar spine<br />(Fig. 3). The posterior borders of the vertebral<br />bodies in the lumbar spine were concave. The proximal<br />ends of both the femora were constricted (Fig. 4).<br />The X-rays of the hands showed that the metacarpal<br />and the phalangeal medullary cavities were widened.<br />The cortices were very narrow and thin. The lower<br />ends of both the ulna and radius were tapered. The<br />EEG was characterized by a generalized increase of<br />slow wave activity. There were no epileptic discharges.<br />There was no excess excretion of mucopolysaccharides<br />in the urine. Abnormal vacuoles were<br />found in approximately 30% of mononuclear cells.<br />No evidence of metachromasia was found. Some<br />granules in the monocytes were Sudan black positive.<br /><br /><br />Bone marrow aspirations yielded dry taps. The urine<br />amino acid chromatogram was normal, as were the<br />liver function tests. On one occasion the plasma true<br />glucose was 20 mg/100 ml, but the presence of hypoglycaemia<br />was not confirmed on a number of other<br />occasions. Fibroblast cultures were attempted but<br />were unfortunately unsuccessful. Plans had been<br />made to repeat these cultures when the child was<br />admitted to hospital with broncho-pneumonia and<br />died soon afterwards. Permission for necropsy was<br />refused.<br /><br /><strong>Discussion</strong><br />The name 'I-cell disease' was derived from the<br />striking granular inclusions seen in the cultured<br />fibroblasts from children suffering from this syndrome.<br />From the few reported cases it seems likely<br />that the condition is inherited as an autosomal<br />recessive. Slow development is recognized early in<br />life, as well as the hypotonia. Congenital dislocation<br />of the hips, herniae, and hyperplasia of the gums are<br />also a feature. Recurrent upper respiratory tract<br />infection seems to be a characteristic feature, and<br />often a cause of death when complicated by congestive<br />heart failure. Development does not seem to<br />proceed further than sitting and standing without<br />support, and a few social responses such as smiling<br />and early vocalization. Unaided walking is not<br />accomplished, nor is toilet-training or self-feeding.<br />The affected children do not seem to survive more<br />than a few years (Leroy et al., 1971).<br />The appearance of the child becomes strikingly<br />similar to children with Hurler's syndrome. The<br />tongue is large, the earlobes fleshy, the forehead<br />high, the epicanthic folds prominent, the bridge of<br />the nose flat, the nostrils anteverted, and the upper<br />lip elongated (Sprangler & Wiedemann, 1970a). In<br />fact this is the diagnosis likely to be made. Apart<br />from the facies, dwarfed stature and severe retardation,<br />there is kyphoscoliosis, limited joint mobility<br />with claw hands, and sometimes enlargement of the<br />liver and spleen; but no clouding of the corneae.<br />The X-ray findings are somewhat similar as well.<br />There is marked periostieal new bone formation.<br />The tubular bones of the arms are short and plump.<br />The metacarpals are irregular and expanded and the<br />phalanges are bullet-shaped. The distal ends of the<br />radius and ulna are tilted. The vertebral bodies are<br />short and rounded and there may be beaking of the<br />last dorsal and first lumbar vertebrae. The ribs are<br />broad and the cranial vault is thickened. However,<br />the mucopolysaccharide excretion in the urine is<br />normal.<br />The peripheral lymphocytes and monocytes are<br />vacuolated and finely vacuolated cells are present in<br />the bone marrow. Cultured fibroblasts contain<br />coarse, regular, refringent inclusions staining blue<br />with toluidine blue, which are PAS and Sudan black<br />positive (Sprangler & Wiedemann, 1970b). Special<br />staining may also reveal metachromasia, indicating<br />that they contain mucopolysaccharides as well as<br />lipids (Matalon et al., 1968).<br />At necropsy foam cells are found in the endocardium,<br />lungs, spleen, liver, kidneys, adrenals and<br />aorta. Electron microscopy does not reveal the lipid<br />inclusions (zebra bodies) typical of Hurler's syndrome.<br />The lipid content of the tissues is generally<br />normal, ecept for some increase in total values<br />(Leroy et al., 1971). Liver acid P-galactosidase<br />activity has been found to be decreased, with hyperactivity<br />of a number of other enzymes (Tondeur<br />et al., 1971). Although the findings so far suggest a<br />storage disease involving both lipids and mucopolysaccharides<br />no definite cause can yet be suggested.<br />The differentiation from Hurler's syndrome is<br />made by the normal urinary excretion of mucopolysaccharides.<br />A somewhat similar clinical picture<br />occurs in mucolipidosis I or lipomucopolysaccharidosis,<br />but the features of gargoylism are not so marked<br />and the course of the disease is much more protracted.<br />Gm,-gangliosidosis, type I, has also been<br />referred to as pseudo-Hurler's syndrome because of<br />the appearance of the affected child, but the diagnosis<br />of this disease is confirmed by the abnormal<br />ganglioside pattern on thin layer chromatography of<br />brain extracts.<br /><br /><br /><span style="font-size:78%;">References<br />LEROY, J.G., SPRANGLER, J.W., FEINGOLD, M., OPITZ, J.M.<br />& CROCKER, A.C. (1971) I-cell disease: a clinical picture.<br />Pediatrics, 79, 360.<br />MATALON, R., CIFONELLI, J.A., ZELLWEGER, H. & DORFMAN,<br />A. (1968) Lipid abnormalities in a variant of the Hurler's<br />syndrome. Proceedings of the National Academy of Science,<br />59, 1097.<br />SPRANGLER, J.W. & WIEDEMANN, H-R. (1970a) The genetic<br />mucolipidoses. Neuropddiatrie, 2, 3.<br />SPRANGLER, J.W. & WIEDERMANN, H-R. (1970b) The genetic<br />mucolipidoses. Humangenetik, 9, 113.<br />TONDEUR, M., VAMAS-HURWITZ, E., MOCKEL-POHL, S.,<br />DERENME, J.P., CREMER, N. & LOEB, H. (1971) Clinical,<br />biochemical, and ultrastructural studies in a case of<br />chondrodystrophy presenting the I-cell phenotype in<br />tissue culture. Pediatrics, 79, 366</span><br /><span style="font-size:78%;"></span><br /><span style="font-size:78%;">Information obtained from:</span><br /><a href="http://pmj.bmj.com/content/49/571/359.full.pdf"><span style="font-size:78%;">IINEIL GORDONM.D., F.R.C.P.</span></a>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-45536777580632728782009-10-30T03:38:00.000-07:002009-10-30T03:39:23.291-07:00H1N1 Flu ("Swine Flu")Click <a href="http://www.cdc.gov/h1n1flu/qa.htm">here</a> to read up on what the Center for Disease Control and Prevention (CDC) has reported about the H1N1 Flu.LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-56658397345152328322009-10-30T03:31:00.000-07:002009-10-30T03:34:33.571-07:00Hemophagocytic Lymphohistiocytosis (HLH)<strong>Hemophagocytic lymphohistiocytosis (HLH)</strong> is a rare disorder of the immune system primarily affecting young infants and children. Although physicians have written about the disorder over the years, it has been only in the last few years that it has received more widespread attention. The prevalence of HLH is 1.2 in every 1,000,000 children under the age of 15.<br /><br />In 1985, physicians from all over the world who were interested in studying the histiocyte and disorders related to this cell created the Histiocyte Society. Thanks to their research, in part financed by the Histiocytosis Association of America (HAA) and national subgroups, we now have a better understanding of the disease, as well as dramatically improved treatments. With growing knowledge, there is also increased awareness of the disease among nonspecialized physicians.<br /><br />The disease usually presents with fever and sometimes other symptoms of an infection. In many cases, a pathogen (viral, bacterial, etc.) can be identified. The human body contains many cells including T-cells and histiocytes that fight infection. The activation of these cells causes an inflammatory reaction in the body. Normally, when the pathogen has been eliminated, the inflammatory reaction is turned off, and the immune system returns to its steady state. In HLH patients, due to defect of the immune system, the inflammatory reaction persists and causes the symptoms of HLH.<br /><br /><strong>What is the cause of this severe immune dysregulation?</strong><br />We currently know that HLH occurs either on the basis of a genetic defect or as a secondary form with underlying diseases such as infections, cancer, or rheumatic diseases. In the primary form, also known as familial hemophagocytic lymphohistiocytosis (FHL or FHLH), defective genes are inherited from both the mother and the father (autosomal recessive inheritance). FHL is diagnosed if there is more than one affected child in the family and/or a gene defect has been determined. FHL should be suspected if the symptoms do not disappear with treatment or if symptoms recur when therapy has been stopped. The onset of FHL is usually early in life, and a persistent cure can only be achieved with BMT (bone marrow transplantation). It is important to know that infections can trigger both the familial and the secondary disease.<br /><br />So far, 3 gene defects have been identified, which account for approximately 50% to 80% of the familial cases, depending on the population that has been analyzed. Two of the genes, PRF1 and UNC13D, are responsible for the synthesis of proteins, perforin, and MUNC13-4 that are involved in the killing process of infectious pathogens. They are believed to also have a function in switching off immune responses. The precise mechanism, however, is not fully understood. A third defect affecting the Syntaxin 11 (STX11) gene has so far only been detected in patients of Turkish origin. The function of the mutated protein remains to be elucidated. There remains a considerable percentage of FHL patients with no known underlying gene defect.<br /><br />In cases of secondary HLH, a condition of temporary immunodeficiency seems to contribute to the development of the disease.<br /><br /><strong>Symptoms</strong><br />Typical symptoms of HLH besides persistent fever are pallor (paleness), jaundice, liver and spleen enlargement, and neurological symptoms, such as irritability or even seizures. The involvement of the bone marrow, the site of blood cell production, can lead to severe decline of the blood cell counts (red and white blood cells and platelets). On bone marrow examination, histiocytes that are “eating” other blood cells (also known as phagocytosis) can be detected. Although the disease was named after this phenomenon, it can be absent at the onset or even throughout the course of the disease.<br /><br />Because symptoms can vary widely, it is sometimes difficult for the physician to make a diagnosis of HLH early in the course of the disease without the help of specialized laboratory tests. To facilitate a rapid and accurate diagnosis, the Histiocyte Society has created diagnostic guidelines and recommendations concerning the treatment of HLH. This is known as the HLH-2004 treatment protocol.<br /><br /><strong>How is HLH diagnosed?</strong><br />It is sometimes difficult to establish the diagnosis of HLH, and the combination of the clinical picture and certain laboratory test criteria is required. A test that has been found very useful in substantiating a clinical diagnosis of HLH is absent or low NK (natural killer)-cell function. This is found in 90% of patients with FHL, as well as in many cases of secondary disease. Results of NK-cell function testing are generally reliable if the blood sample is properly shipped and tested in less than 24 hours. NK function cannot be determined prenatally, and it may not be reliably studied until a child is several weeks old. Notably, this test does not discriminate between familial and secondary disease.<br /><br />Detection of perforin by staining of lymphocytes and analysis by flow cytometry is a highly reliable method for predicting the likelihood of the PRF1 gene mutation as the cause of FHL in a given patient. This test can also be used with reasonable predictive potential to screen parents and siblings to determine whether they might be carriers of PRF1 mutations. This test is not available prenatally.<br /><br />Another test recently described analyzes the expression of a molecule on the surface of NK-cells (CD107) by flow cytometry that marks NK-cell degranulation. Reduced expression can predict mutations in the UNC13D gene. This test also requires specially prepared blood samples and cannot be used prenatally.<br /><br />Genetic testing is recommended in cases of suspected FHL and confirms the diagnosis. Usually a blood sample is used. Even in the event of death, salvaged tissue can be tested. Once the genetic defect of a patient is known, the parents and siblings can be easily tested to determine if they are carriers for this specific defect. In such cases, prenatal diagnosis is possible as well.<br /><br /><strong>How is HLH treated?</strong><br />Without treatment, FHL is usually rapidly fatal with a median survival of about 2 months. The current treatment protocol, HLH 2004, provides recommendations for HLH therapy with a combination of immunosuppressive drugs and chemotherapy. The protocol has been accepted internationally and is used in many countries worldwide. In order to prevent early death or severe persisting organ damage, therapy must be initiated in a timely manner. In FHL cases, only temporary remission will be achieved. For a definite cure, the patient must undergo BMT.<br /><br />With the former HLH-94 protocol and the now active HLH-2004 protocol, high remission rates and cure rates with BMT have been reported.<br /><br />Secondary HLH sometimes resolves spontaneously or after treatment of the underlying disease. In some cases, modified immunochemotherapy can be applied, while in others, full immunochemotherapy is required. <br /><br />Source: <a href="http://www.histio.org/site/c.kiKTL4PQLvF/b.1810505/k.F16D/Disease_Information.htm">Histiocytosis Association of America</a>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-76780557981200752982009-10-16T13:13:00.000-07:002009-10-16T13:14:33.873-07:00Sacred Work<div align="center">As I was packing up my scrapbooking room today - I came across a book that I had to read during my senior year of nursing school. In this book is a paragraph that I quoted in my presidential speech at graduation. But in reading it today, it once again reminded me of the main reason that chose nursing as a career - to care for others. You can read the rest of my speech <a href="http://lindseybarnes.blogspot.com/2008/05/pinning-ceremony-friday-night-may-2nd.html">here</a>, but below is the paragraph I am referring to. I comes from the book Sacred Work by Erie Chapman.<br />.....<br /><em>“In charitable care giving, your “boss” is the person you serve. The people you serve, by the way, are not customers. They don’t come in to buy a shirt or dress, they come to you in deep need. They have cancer, or heart disease, or have come for your help in delivering a baby. They are the raped, the abused, the homeless, the ignored. They are you mothers and brothers and sisters and children. They come to you because they are suffering in body and soul, and they are calling out to you for help. They are the voice of humanity in distress. They are not asking you to fill their shopping bags but to tend to the cry of their hearts. They need service far beyond customer-focused politeness. They need loving service from people who see their work as sacred.”<br /></em>.....<br />I pray that I never lose sight of that...<br /></div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-58266955209923078302009-08-26T00:35:00.000-07:002009-08-26T00:43:42.178-07:00Meningococcal Encephalitis<div><div><div><div><div><strong>What is meningitis? What is encephalitis?<br /></strong>Infections in the brain and spinal cord can cause dangerous inflammation. This inflammation can produce a wide range of symptoms, including fever, headache, or confusion and, in extreme cases, can cause brain damage, stroke, seizures, or even death.<br />Infection of the meninges, the membranes that surround the brain and spinal cord, is called meningitis and inflammation of the brain itself is called encephalitis. Myelitis is an infection of the spinal cord. When both the brain and the spinal cord become inflamed, the condition is called encephalomyelitis.<br /><br /><strong>What causes meningitis and encephalitis?<br /></strong>Meningitis and encephalitis are usually caused by viruses or bacteria. Most often, the body’s immune system is able to contain and defeat an infection. But if the infection passes into the blood stream and then into the cerebrospinal fluid that surrounds the brain and spinal cord, it can affect the nerves and travel to the brain and/or surrounding membranes, causing inflammation. This swelling can harm or destroy nerve cells and cause bleeding in the brain.<br /><br /><strong>Meningitis</strong><br />Meningitis is most often caused by a bacterial or viral infection. It also may be caused by a fungal infection, a reaction to certain medications or medical treatments, an inflammatory disease such as lupus, some types of cancer, or a traumatic injury to the head or spine.<br />Bacterial meningitis is a rare but potentially fatal disease. It can be caused by several types of bacteria that first cause an upper respiratory tract infection and then travel through the blood stream to the brain. The disease can also occur when certain bacteria invade the meninges directly. The disease can block blood vessels in the brain, causing stroke and permanent brain damage.<br /><br />Pneumococcal meningitis is the most common form of meningitis and is the most serious form of bacterial meningitis. Some 6,000 cases of pneumococcal meningitis are reported in the United States each year. The disease is caused by the bacterium Streptococcus pneumoniae, which also causes pneumonia, blood poisoning (septicemia), and ear and sinus infections. At particular risk are children under age 2 and adults with a weakened or depressed immune system. Persons who have had pneumococcal meningitis often suffer neurological damage ranging from deafness to severe brain damage.<br /><br />Meningococcal meningitis, which is caused by the bacterium Neisseria meningitides, is common in children ages 2-18. Each year in the United States about 2,600 people get this highly contagious disease. High-risk groups include infants under the age of 1 year, people with suppressed immune systems, travelers to foreign countries where the disease is endemic, and college students (freshmen in particular) who reside in dormitories. Between 10 and 15 percent of cases are fatal, with another 10-15 percent causing brain damage and other serious side effects.<br /><br />Haemophilus meningitis was at one time the most common form of bacterial meningitis. Fortunately, the Haemophilus influenzae b vaccine has greatly reduced the number of cases in the United States. Those most at risk of getting this disease are children in child-care settings and children who do not have access to the vaccine.<br /><br />Other forms of bacterial meningitis include Listeria monocytogenes meningitis, which can cross the placental barrier and cause a baby to be stillborn or die shortly after birth; Escherichia coli meningitis, which is most common in elderly adults and newborns and may be transmitted to a baby through the birth canal, and Mycobacterium tuberculosis meningitis, a rare disease that occurs when the bacterium that causes tuberculosis attacks the meninges.<br /><br />Viral, or aseptic, meningitis is the most common form of meningitis in the United States. This typically mild and non-lethal disease is usually caused by enteroviruses—common viruses that enter the body through the mouth and travel to the brain and surrounding tissues where they multiply. Enteroviruses are present in mucus, saliva, and feces and can be transmitted through direct contact with an infected person or an infected object or surface. Other viruses that cause meningitis include varicella zoster (the virus that causes chicken pox and can appear decades later as shingles), influenza, mumps, HIV, and herpes simplex type 2 (genital herpes).<br /><br />Many fungal infections can affect the brain. The most common form of fungal meningitis is caused by the fungus cryptococcus neoformans (found mainly in dirt and bird droppings). Cryptococcal meningitis is common in AIDS patients. Although treatable, fungal meningitis often recurs in nearly half of affected persons.<br /><br /><strong>Encephalitis</strong><br />Encephalitis can be caused by bacterial infection and, most often, viral infections. Several thousand cases of encephalitis are reported each year, but many more may actually occur since the symptoms may be mild to non-existent in most patients.<br /><br />There are two types of encephalitis. Primary encephalitis (also called acute viral encephalitis) is caused by a direct viral infection of the spinal cord and brain. The infection may be focal (located in only one area) or diffuse (located in many different areas). Secondary encephalitis, also known as post-infective encephalitis, can result from complications of a current viral infection. Secondary encephalitis that results from an immunization or earlier viral infection is known as acute disseminated encephalitis. This illness often occurs 2 to 3 weeks following the initial infection.<br /><br />Most cases of encephalitis in the United States are caused by enteroviruses, herpes simplex virus types 1 and 2, a bite from a rabid animal (rabies virus), or arboviruses, which are transmitted from infected animals to humans through the bite of an infected tick, mosquito, or other blood-sucking insect. Lyme disease, a bacterial infection spread by tick bite, can cause encephalitis.<br /><br />Herpes simplex encephalitis (HSE) is responsible for about 10 percent of all encephalitis cases, with a frequency of about 2 cases per million persons per year. More than half of untreated cases are fatal. About 30 percent of cases result from the initial infection with the herpes simplex virus; the majority of cases are caused by reactivation of an earlier infection.<br />HSE due to herpes simplex virus type 1 (which causes cold sores or blisters around the mouth or eyes) can affect any age group but is most often seen in persons under age 20 or over age 40. This rapidly progressing disease is the single most important cause of fatal sporadic encephalitis in the U.S. The virus is transmitted through contact with an infected person. Symptoms include headache and fever for up to 5 days, followed by personality and behavioral changes, seizures, partial paralysis, hallucinations, and altered levels of consciousness. Brain damage in adults and in children beyond the neonatal period is usually seen in the frontal and temporal lobes and can be severe.<br /><br />Type 2 virus (genital herpes) is most often transmitted through sexual contact. An infected mother can transmit the disease to her child at birth, through contact with genital secretions, but this is uncommon. In newborns, symptoms such as lethargy, irritability, tremors, seizures, and poor feeding generally develop between 4 and 11 days after delivery.<br /><br />Powassan encephalitis is the only well-documented tick-borne arbovirus in the United States and Canada. Symptoms are noticed 7-10 days following the bite and may include headache, fever, nausea, confusion, partial paralysis, and coma. Permanent neurologic damage occurs in about half of all cases and death in about 10-15 percent of all cases.<br /><br />Four common forms of mosquito-transmitted viral encephalitis are seen in the United States:<br />Equine encephalitis affects horses and humans. Eastern equine encephalitis also infects birds that live in freshwater swamps of the eastern U.S. seaboard and along the Gulf Coast. In humans, symptoms are seen 4-10 days following transmission and include sudden fever, general flu-like muscle pains, and headache of increasing severity, followed by coma and death in severe cases. About half of infected patients die from the disorder. Fewer than 10 human cases are seen annually in the United States. Western equine encephalitis is seen in farming areas in the western and central plains states. Symptoms begin 5-10 days following infection. Children, particularly those under 12 months of age, are affected more severely than adults and may have permanent neurologic damage. Death occurs in about 3 percent of cases. Venezuelan equine encephalitis is very rare in this country. Children are at greatest risk of developing severe complications, while adults generally develop flu-like symptoms. Epidemics in South and Central America have killed thousands of persons and left others with permanent, severe neurologic damage.<br /><br />LaCrosse encephalitis occurs most often in the upper midwestern states (Illinois, Wisconsin, Indiana, Ohio, Minnesota, and Iowa) but also has been reported in the southeastern and mid-Atlantic regions of the country. Most cases are seen in children under age 16. Symptoms such as vomiting, headache, fever, and lethargy appear 5-10 days following infection. Severe complications include seizure, coma, and permanent neurologic damage. About 100 cases of LaCrosse encephalitis are reported each year.<br /><br />St. Louis encephalitis is most prevalent in temperate regions of the United States but can occur throughout most of the country. The disease is generally milder in children than in adults, with elderly adults at highest risk of severe disease or death. Symptoms typically appear 7-10 days following infection and include headache and fever. In more severe cases, confusion and disorientation, tremors, convulsions (especially in the very young), and coma may occur.<br />West Nile encephalitis was first clinically diagnosed in the United States in 1999; 284 people are known to have died of the virus the following year. There were 9,862 reported cases of human West Nile disease in calendar year 2003, with a total of 560 deaths from this disorder over 5 years. The disease is usually transmitted by a bite from an infected mosquito, but can also occur after transplantation of an infected organ or transfusions of infected blood or blood products. Symptoms are flu-like and include fever, headache, and joint pain. Some patients may develop a skin rash and swollen lymph glands, while others may not show any symptoms. At highest risk are elderly adults and people with weakened immune systems.<br /><br /><strong>Who is at risk for encephalitis and meningitis?<br /></strong>Anyone can get encephalitis or meningitis. People with weakened immune systems, including those persons with HIV or those taking immunosuppressant drugs, are at the highest risk of contracting the diseases.<br /><br /><strong>How are these disorders transmitted?<br /></strong>Some forms of bacterial meningitis and encephalitis are contagious and can be spread through contact with saliva, nasal discharge, feces, or respiratory and throat secretions (often spread through kissing, coughing, or sharing drinking glasses, eating utensils, or such personal items as toothbrushes, lipstick, or cigarettes). For example, people sharing a household, at a day care center, or in a classroom with an infected person can become infected. College students living in dormitories—in particular, college freshmen—have a higher risk of contracting meningococcal meningitis than college students overall. Children who do not have access to childhood vaccines are at increased risk of developing certain types of bacterial meningitis. Because these diseases can occur suddenly, anyone who is suspected of having either meningitis or encephalitis should immediately contact a doctor or go to the hospital.<br /><br /><strong>What are the signs and symptoms?<br /></strong>The hallmark signs of meningitis are sudden fever, severe headache, and a stiff neck; encephalitis is characterized by seizures, stupor, coma, and related neurological signs. In more severe cases, neurological symptoms may include nausea and vomiting, confusion and disorientation, drowsiness, sensitivity to bright light, and poor appetite.<br /><br />Meningitis often appears with flu-like symptoms that develop over 1-2 days. Distinctive rashes are typically seen in some forms of the disease. Meningococcal meningitis may be associated with kidney and adrenal gland failure and shock.<br /><br />Patients with encephalitis often show mild flu-like symptoms. In more severe cases, patients may experience problems with speech or hearing, double vision, hallucinations, personality changes, loss of consciousness, loss of sensation in some parts of the body, muscle weakness, partial paralysis in the arms and legs, sudden severe dementia, impaired judgment, seizures, and memory loss.<br /><br />Important signs of encephalitis to watch for in an infant include vomiting, body stiffness, constant crying that may become worse when the child is picked up, and a full or bulging fontanel (the soft spot on the top of the head).<br /><br /><strong>How are meningitis and encephalitis diagnosed?<br /></strong>Following a physical exam and medical history to review activities of the past several days (such as recent exposure to insects or animals, any contact with ill persons, or recent travel), the doctor may order various diagnostic tests to confirm the presence of infection and inflammation. Early diagnosis is vital, as symptoms can appear suddenly and escalate to brain damage, hearing and/or speech loss, blindness, or even death.<br /><br />A neurological examination involves a series of tests designed to assess motor and sensory function, nerve function, hearing and speech, vision, coordination and balance, mental status, and changes in mood or behavior. Doctors may test the function of the nervous system through tests of strength and sensation, with the aid of items including a tuning fork, small light, reflex hammer, and pins.<br /><br />Laboratory screening of blood, urine, and body secretions can help detect and identify brain and/or spinal cord infection and determine the presence of antibodies and foreign proteins. Such tests can also rule out metabolic conditions that have similar symptoms. For example, a throat culture may be taken to check for viral or bacterial organisms that cause meningitis or encephalitis. In this procedure, the back of the throat is wiped with a sterile cotton swab, which is then placed on a culture medium. Viruses and bacteria are then allowed to grow on the medium. Samples are usually taken in the physician’s office or in a laboratory setting and sent out for analysis to state laboratories or to the U.S. Centers for Disease Control and Prevention. Results are usually available in 2 to 3 days.<br /><br />Analysis of the cerebrospinal fluid that surrounds and protects the brain and spinal cord can detect infections in the brain and/or spinal cord, acute and chronic inflammation, and other diseases. In a procedure known as a spinal tap (or lumbar puncture), a small amount of cerebrospinal fluid is removed by a special needle that is inserted into the lower back. The skin is anesthetized with a local anesthetic prior to the sampling. The fluid, which is completely clear in healthy people, is tested to detect the presence of bacteria or blood, as well as to measure glucose levels (a low glucose level is a sign of bacterial or fungal meningitis) and white blood cells (elevated white blood cell counts are also a sign of infection). The procedure is usually done in a hospital and takes about 45 minutes.<br /><br />Computer-assisted imaging can reveal signs of brain inflammation, internal bleeding or hemorrhage, or other brain abnormalities. Two painless, noninvasive imaging procedures are routinely used to diagnose meningitis and encephalitis.<br /><br />Computed tomography, also known as a CT scan, combines x-rays and computer technology to produce rapid, clear, two-dimensional images of organs, bones, and tissues. Occasionally a contrast dye is injected into the bloodstream to highlight the different tissues in the brain and to detect signs of encephalitis or inflammation of the meninges. CT scans can also detect bone and blood vessel irregularities, certain brain tumors and cysts, herniated discs, spinal stenosis (narrowing of the spinal canal), blood clots or intracranial bleeding in patients with stroke, brain damage from a head injury, and other disorders.<br /><br />Magnetic resonance imaging (MRI) uses computer-generated radio waves and a strong magnet to produce detailed images of body structures, including tissues, organs, bones, and nerves. The pictures, which are clearer than those produced by CT, can help identify brain and spinal cord inflammation, infection, tumors, eye disease, and blood vessel irregularities that may lead to stroke. A contrast dye may be injected prior to the test to reveal more detail.<br />Electroencephalography, or EEG, can identify abnormal brain waves by monitoring electrical activity in the brain through the skull. Among its many functions, EEG is used to help diagnose certain seizure disorders, brain damage from head injuries, specific viral infections such as herpes virus, and inflammation of the brain and/or spinal cord. This painless, risk-free test can be performed in a doctor’s office or at a hospital or testing facility.<br /><br /><strong>How are these infections treated?</strong><br />Persons who are suspected of having meningitis or encephalitis should receive immediate, aggressive medical treatment. Both diseases can progress quickly and have the potential to cause severe, irreversible neurological damage.<br /><br />Meningitis<br />Early treatment of bacterial meningitis is important to its outcome. Strong doses of general antibiotics may be prescribed first, followed by intravenous antibiotics in more severe cases. Antibiotics may also be given to prevent other bacterial infections. Appropriate antibiotic treatment for most types of meningitis can reduce the risk of dying from the disease to below 15 percent.<br /><br />Infected sinuses may need to be drained. Corticosteroids such as prednisone may be ordered to relieve brain pressure and swelling and to prevent hearing loss that is common in patients with Haemophilus influenza meningitis. Pain medicine and sedatives may be given to make patients more comfortable. Lyme disease is treated with intravenous antibiotics.<br /><br />Unlike bacteria, viruses cannot be killed by antibiotics (an exception is the herpes virus, which can be treated with the antiviral drug acyclovir). Patients with mild viral meningitis may be allowed to stay at home, while those who have a more serious infection may be hospitalized for supportive care. Patients with mild cases, which often cause only flu-like symptoms, may be treated with fluids, bed rest (preferably in a quiet, dark room), and analgesics for pain and fever. The physician may prescribe anticonvulsants such as dilantin or phenytoin to prevent seizures and corticosteroids to reduce brain inflammation. If inflammation is severe, pain medicine and sedatives may be prescribed to make the patient more comfortable.<br /><br />Acute disseminated encephalomyelitis is treated with steroids. Fungal meningitis is treated with intravenous antifungal medications.<br /><br />Encephalitis<br />Antiviral drugs used to treat viral encephalitis include acyclovir and ganciclovir.<br />Very mild cases of encephalitis may be monitored at home by the physician and a caregiver. Supportive care includes fluids, bed rest, and over-the-counter analgesics to reduce fever and headache. More severe cases may require hospitalization. Anticonvulsants may be prescribed to stop or prevent seizures, along with sedatives to calm more severely infected persons and drugs to counter nausea and vomiting. Corticosteroids and intravenous administration of carbohydrate solutions can reduce brain swelling. Patients with breathing difficulties may require artificial respiration.<br /><br />Patients who experience severe brain inflammation may need physical, speech, and occupational therapy once the acute illness is under control.<br /><br /><strong>Can meningitis and encephalitis be prevented?</strong><br />Good personal hygiene can reduce the risk of getting the disease from an infected person. Avoid sharing food, utensils, glasses, and other objects with a person who may be exposed to or have the infection. Wash hands often with soap and rinse under running water.<br /><br />Effective vaccines are available to prevent pneumonia, H. influenza, pneumococcal meningitis, and infection with other bacteria that can cause meningococcal meningitis.<br /><br />People who live, work, or go to school with someone who has been diagnosed with bacterial meningitis may be asked to take antibiotics for a few days as a preventive measure.<br />To lessen the risk of being bitten by an infected mosquito or other insect, people should limit outdoor activities at night, wear long-sleeved clothing when outdoors, use insect repellents that are most effective for that particular region of the country, and rid lawn and outdoor areas of free-standing pools of water, in which mosquitoes breed. Do not over-apply repellants, particularly on young children and especially infants, as chemicals may be absorbed through the skin.<br /><br /><strong>What is the prognosis for these infections?<br /></strong>Outcome generally depends on the particular infectious agent involved, the severity of the illness, and how quickly treatment is given. In most cases, people with very mild encephalitis or meningitis can make a full recovery, although the process may be slow.<br /><br />Patients who experience only headache, fever, and stiff neck may recover in 2-4 weeks. Patients receiving treatment for viral meningitis and encephalitis usually see some relief in 24-48 hours and recovery in about a month. Patients with bacterial meningitis typically show some relief 48-72 hours following initial treatment but are more likely to experience complications caused by the disease. In more serious cases, these diseases can cause hearing and/or speech loss, blindness, permanent brain and nerve damage, behavioral changes, cognitive disabilities, lack of muscle control, seizures, and memory loss. These patients may need long-term therapy, medication, and supportive care.<br /><br />Infomation obtained from <a href="http://www.ninds.nih.gov/disorders/encephalitis_meningitis/detail_encephalitis_meningitis.htm">National Institute of Neurological Disorders and Stroke</a></div></div></div></div></div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-12293760567041603672009-08-21T01:01:00.000-07:002009-08-21T01:09:18.477-07:00Treacher Collins Syndrome<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhHHLuRqClQJOu43ybdaou7HTm0O9TqXHCJZNqkashiKg3aQAyWZ9tMV24vBJLn-09DynHECv9P1E8kTGvvAjP_fgId76q2UWA0P1_Oo4Uua4kiqGulUzkn1wy2tLsQmNtvJoXHF1G-ApcV/s1600-h/100_4380.JPG"><img id="BLOGGER_PHOTO_ID_5372325884729038626" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 400px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhHHLuRqClQJOu43ybdaou7HTm0O9TqXHCJZNqkashiKg3aQAyWZ9tMV24vBJLn-09DynHECv9P1E8kTGvvAjP_fgId76q2UWA0P1_Oo4Uua4kiqGulUzkn1wy2tLsQmNtvJoXHF1G-ApcV/s400/100_4380.JPG" border="0" /></a><br /><div><strong>What is Treacher Collins syndrome?<br /></strong>Treacher Collins syndrome is a condition that affects the development of bones and other tissues in the face. The signs and symptoms of this disorder vary greatly, ranging from almost unnoticeable to severe. Most affected individuals have underdeveloped facial bones, particularly the cheek bones, and a very small jaw and chin (micrognathia). Some people with this condition are also born with an opening in the roof of the mouth called a cleft palate. In severe cases, underdevelopment of the facial bones may restrict an affected infant's airway, causing potentially life-threatening respiratory problems.</div><br /><div><br />People with Treacher Collins syndrome often have eyes that slant downward, sparse eyelashes, and a notch in the lower eyelids called a coloboma. Some affected individuals have additional eye abnormalities that can lead to vision loss. This condition is also characterized by absent, small, or unusually formed ears. Defects in the middle ear (which contains three small bones that transmit sound) cause hearing loss in about half of cases. People with Treacher Collins syndrome usually have normal intelligence.</div><a name="statistics"></a><br /><div><br /><strong>How common is Treacher Collins syndrome?<br /></strong>This condition affects an estimated 1 in 50,000 people.<a name="genes"></a></div><br /><div><br /><strong>What genes are related to Treacher Collins syndrome?<br /></strong>Mutations in the <a class="link-dapple" title="T C O F 1" href="http://ghr.nlm.nih.gov/gene=tcof1">TCOF1</a> gene cause Treacher Collins syndrome.<br />The TCOF1 gene provides instructions for making a protein called treacle. Although researchers have not determined the precise function of this protein, they believe that it plays a critical role before birth in the development of bones and other tissues in the face. Mutations in the TCOF1 gene reduce the amount of treacle that is produced in cells. Researchers believe that a loss of this protein signals cells that are important for the development of facial bones to self-destruct (undergo apoptosis). This abnormal cell death may lead to the specific problems with facial development found in Treacher Collins syndrome.<br />Read more about the <a title="T C O F 1" href="http://ghr.nlm.nih.gov/gene=tcof1">TCOF1</a> gene.</div><a name="inheritance"></a><br /><div><br /><strong>How do people inherit Treacher Collins syndrome?</strong><br />This condition has an autosomal dominant pattern of inheritance, which means one copy of the altered gene in each cell is sufficient to cause the disorder. About 60 percent of cases result from new mutations in the TCOF1 gene. These cases occur in people with no history of the disorder in their family. In the remaining cases, a person with Treacher Collins syndrome inherits the altered gene from an affected parent.</div><a name="treatment"></a><br /><div><br /><strong>Where can I find information about treatment for Treacher Collins syndrome?<br /></strong>These resources address the management of Treacher Collins syndrome and may include treatment providers.<br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=www.ncbi.nlm.nih.gov&uid=7bH4sIAAAAAAAAABXMMQ6AIAwAwK84OZbdxDg6uRh3IlihUVqCjSw-Xl1vOC-8kZKwXQqumpDVjsjYzHgTVjs9UTV3xtRagb0j4DMBU4Qgt3EixxXx3I0rsPtAwy99-II2r0V79dcLgltkmWIAAAA_', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=www.ncbi.nlm.nih.gov&uid=7bH4sIAAAAAAAAABXMMQ6AIAwAwK84OZbdxDg6uRh3IlihUVqCjSw-Xl1vOC-8kZKwXQqumpDVjsjYzHgTVjs9UTV3xtRagb0j4DMBU4Qgt3EixxXx3I0rsPtAwy99-II2r0V79dcLgltkmWIAAAA_">Gene Review: Treacher Collins Syndrome</a><br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=www.nlm.nih.gov&uid=7bH4sIAAAAAAAAACXKwQqAIAwA0D9ygtChfwg6dBfRkYM5xVYS9PEFvfOLVRIpVfFbx6AFRf2CiUlw5fPwy5NV2wwwxjDCxQhls9cLyp.alwAl3hC6UmQEa52zk8laXiB7RMhcAAAA', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=www.nlm.nih.gov&uid=7bH4sIAAAAAAAAACXKwQqAIAwA0D9ygtChfwg6dBfRkYM5xVYS9PEFvfOLVRIpVfFbx6AFRf2CiUlw5fPwy5NV2wwwxjDCxQhls9cLyp.alwAl3hC6UmQEa52zk8laXiB7RMhcAAAA">MedlinePlus Encyclopedia: Micrognathia</a><br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=www.nlm.nih.gov&uid=7bH4sIAAAAAAAAACXKwQqAIAwA0D.aBG.9g9Chu4iOHOgUW0nQxxf0zi82SazcxG-DglYS9Y5SYaG1nId3T1btC-KcE6RUEM6wtwvrn.qXkCTeGIZyLITGWGssZK0vUPSkAFwAAAA_', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=www.nlm.nih.gov&uid=7bH4sIAAAAAAAAACXKwQqAIAwA0D.aBG.9g9Chu4iOHOgUW0nQxxf0zi82SazcxG-DglYS9Y5SYaG1nId3T1btC-KcE6RUEM6wtwvrn.qXkCTeGIZyLITGWGssZK0vUPSkAFwAAAA_">MedlinePlus Encyclopedia: Pinna Abnormalities and Low-Set Ears</a><br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=www.nlm.nih.gov&uid=7bH4sIAAAAAAAAACXKQQqAIBAAwB-5diioPwgduovokgu6im1J0OMLmvP4woGECtutoZOMLNZgSMS4pvOw5okidQHovStOWTFFtZcL8p.qlwDZ3-CakE8IWg.TOKso-QWYVcQKXAAAAA__', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=www.nlm.nih.gov&uid=7bH4sIAAAAAAAAACXKQQqAIBAAwB-5diioPwgduovokgu6im1J0OMLmvP4woGECtutoZOMLNZgSMS4pvOw5okidQHovStOWTFFtZcL8p.qlwDZ3-CakE8IWg.TOKso-QWYVcQKXAAAAA__">MedlinePlus Encyclopedia: Treacher-Collins Syndrome</a> </div><br /><div><br />You might also find information on treatment of Treacher Collins syndrome in <a href="http://ghr.nlm.nih.gov/condition=treachercollinssyndrome/show/Educational+resources">Educational resources</a> and <a href="http://ghr.nlm.nih.gov/condition=treachercollinssyndrome/show/Patient+support">Patient support</a>.</div><a name="resources"></a><br /><div><br /><strong>Where can I find additional information about Treacher Collins syndrome?</strong><br />You may find the following resources about Treacher Collins syndrome helpful. These materials are written for the general public.</div><br /><div><br /><a href="http://ghr.nlm.nih.gov/condition=treachercollinssyndrome/show/MedlinePlus">MedlinePlus</a> - Health information (5 links)<br /><a href="http://ghr.nlm.nih.gov/condition=treachercollinssyndrome/show/Educational+resources">Educational resources</a> - Information pages (7 links)<br /><a href="http://ghr.nlm.nih.gov/condition=treachercollinssyndrome/show/Patient+support">Patient support</a> - For patients and families (10 links)<br />You may also be interested in these resources, which are designed for healthcare professionals and researchers.<br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=www.ncbi.nlm.nih.gov&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4t3T81LVQhKLctMLS9G5sT71mSUlBRY6euXl5fr5SUnZerl5eTq5WVm6KXnl-kn5ednF2ek5qTpJxXppSWnZ9qDRGzTgQaoFSQWldiWJBcDAO3nqgNlAAAA', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=www.ncbi.nlm.nih.gov&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4t3T81LVQhKLctMLS9G5sT71mSUlBRY6euXl5fr5SUnZerl5eTq5WVm6KXnl-kn5ednF2ek5qTpJxXppSWnZ9qDRGzTgQaoFSQWldiWJBcDAO3nqgNlAAAA">Gene Reviews</a> - Clinical summary<br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=www.genetests.org&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4t3T81LVQhJLS4pjnfxcwSz4n1rMkpKCqz09cvLy.XSgQpKQPJ6-UXp-oWlqUWV9iB-ZoqtkaGBIQDQS.mdSgAAAA__', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=www.genetests.org&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4t3T81LVQhJLS4pjnfxcwSz4n1rMkpKCqz09cvLy.XSgQpKQPJ6-UXp-oWlqUWV9iB-ZoqtkaGBIQDQS.mdSgAAAA__">Gene Tests</a> - DNA tests ordered by healthcare professionals<br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=clinicaltrials.gov&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4t3zsnMy0xOzAkpykzMKdZLzy.DJuRbk1FSUmClr58MlSuBy-kXpyYWJWfoJ8OMtFU1MiopSk1Mzkgt0k7OzwHqKNYursxLKcrPTQXKAQBaAEDQfAAAAA__', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=clinicaltrials.gov&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4t3zsnMy0xOzAkpykzMKdZLzy.DJuRbk1FSUmClr58MlSuBy-kXpyYWJWfoJ8OMtFU1MiopSk1Mzkgt0k7OzwHqKNYursxLKcrPTQXKAQBaAEDQfAAAAA__">ClinicalTrials.gov</a> - Linking patients to medical research<br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=www.ncbi.nlm.nih.gov&uid=7bH4sIAAAAAAAAADWNUUvDMBRG.02ZBNL5JkKRbtmDYqbI3kopaXrbXEhuZm5qqfjjHc49fRz4OMdGGjBjpO597jUMt9E.LufzY1kuyyLJ9ijJB0no5BS.SqCc4Lv8nCGtcrQTPu21qhhMsq5Qu-oqKTKkUG20uST62cfRWDReqJUj58jIja5fPto7UR-V2JwSGOsgiX30HokFrzSkGKA5Pde7.xfQ5JFd4037x24Ohprg2iImnLqhv6VH9Jc6V2.H4hyu0B2Uya8YMFf3D9utUGblX7117kb.AAAA', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=www.ncbi.nlm.nih.gov&uid=7bH4sIAAAAAAAAADWNUUvDMBRG.02ZBNL5JkKRbtmDYqbI3kopaXrbXEhuZm5qqfjjHc49fRz4OMdGGjBjpO597jUMt9E.LufzY1kuyyLJ9ijJB0no5BS.SqCc4Lv8nCGtcrQTPu21qhhMsq5Qu-oqKTKkUG20uST62cfRWDReqJUj58jIja5fPto7UR-V2JwSGOsgiX30HokFrzSkGKA5Pde7.xfQ5JFd4037x24Ohprg2iImnLqhv6VH9Jc6V2.H4hyu0B2Uya8YMFf3D9utUGblX7117kb.AAAA">PubMed</a> - Recent literature<br />Online Books - Medical and science texts<br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=books.mcgraw-hill.com&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4v3z8vJzEtVcMrPzy6O9.f1dfJ0ifetySgpKbDS108CierlJqcXJZbrZmTm5Ogl5-fqp6eW5OfmJmWm6BVkFNhnFifl2RoYmBuamFpamhmolaTmFuQklqTaQtSoJdsamZgBAFp1h1ZwAAAA', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=books.mcgraw-hill.com&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4v3z8vJzEtVcMrPzy6O9.f1dfJ0ifetySgpKbDS108CierlJqcXJZbrZmTm5Ogl5-fqp6eW5OfmJmWm6BVkFNhnFifl2RoYmBuamFpamhmolaTmFuQklqTaQtSoJdsamZgBAFp1h1ZwAAAA">Scriver's Online Metabolic and Molecular Bases of Inherited Disease (OMMBID): Treacher Collins Syndrome</a><br /><a onclick="openNewWindow('http://ghr.nlm.nih.gov/exit?to=www.ncbi.nlm.nih.gov&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4v39.X0hRC-NRklJQVW-vrl5eV6eclJmXp5Obl6eZkZeun5ZfqpeSVFqVX6KZnFBfm5mbl6yemZ9pkptoamJqYGBgAGaCEGTwAAAA__', '',''); return false;" href="http://ghr.nlm.nih.gov/exit?to=www.ncbi.nlm.nih.gov&uid=7bH4sIAAAAAAAAAEvOz0vJLMnMz4v39.X0hRC-NRklJQVW-vrl5eV6eclJmXp5Obl6eZkZeun5ZfqpeSVFqVX6KZnFBfm5mbl6yemZ9pkptoamJqYGBgAGaCEGTwAAAA__">OMIM</a> - Genetic disorder catalog <a name="synonyms"></a><br /></div><br /><div><strong>What other names do people use for Treacher Collins syndrome?</strong><br />Franceschetti-Zwahlen-Klein syndrome<br />Mandibulofacial dysostosis (MFD1)<br />Treacher Collins-Franceschetti syndrome<br />zygoauromandibular dysplasia </div><br /><div><br /><span style="font-size:78%;">The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. </span></div><br /><div><span style="font-size:78%;">Reviewed: December 2006<br />Published: August 14, 2009</span></div><div><span style="font-size:78%;"></span> </div><div><span style="font-size:78%;">Source: <a href="http://ghr.nlm.nih.gov/condition=treachercollinssyndrome">Genetics Home Reference</a></span></div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-45059649684658759032009-07-06T21:17:00.000-07:002009-07-06T21:24:18.747-07:00Shone's Complex<strong>Introduction</strong><br />Shone’s complex is a congenital heart disease, consisting of<br />multiple levels of left sided obstructive lesions including<br />supravalvar mitral ring, parachute mitral valve, subaortic<br />stenosis, and coarctation of aorta. This is a very rare malformation<br />and a very few cases have been reported in literature.<br /><br /><br /><strong>Discussion<br /></strong>Shone’s complex is a rare congenital heart disease described<br />by Shone et al initially in 1963. It typically consists of four<br />obstructive lesions of the left side of the heart and circulation<br />namely parachute like mitral valve, supravalvar mitral ring,<br />subaortic stenosis , and coarctation of aorta. There is a complete<br />form of Shone’s complex wherein all the four lesions are present;<br />however incomplete forms with two or three lesions are also<br />described. Other coexisting mitral valve anomalies have been<br />reported such as fused chordae, single papillary muscle and<br />“typical” (Ruckman & Van Praagh) congenital mitral stenosis.<br />The LVOT obstruction features may include subaortic stenosis,<br />valvar aortic stenosis, bicuspid aortic valve, and coarctation of<br />aorta.<br /><br />Supravalvar mitral ring is a circumferential ridge or<br />membrane, which arises from the left atrial wall overlying<br />the mitral valve and is frequently attached to the mitral valve.<br />The ring may range from a thin membrane to a thick discrete<br />fibrous ridge. It may vary in its extent. Adhesion to the valve<br />may impair opening of the leaflets causing mitral-valve inflow<br />obstruction in some patients. In other patients, the ring may<br />be large and protrude into the mitral-valve inflow thus causing<br />obstruction.<br /><br />Parachute mitral valve is defined as a unifocal attachment<br />of mitral valve chordae independent of the number of papillary<br />muscles. A true parachute mitral valve (PMV) is characterized by<br />attachment of the chordae to a single or fused papillary muscle;<br />however PMV also includes asymmetrical mitral valves having<br />two papillary muscles, one of which is dominant and elongated,<br />with its tip reaching to the valve leaflets. The unifocal attachment<br />of the chordae results in a restricted valve opening and subvalvar<br />obstruction and, rarely, valvar regurgitation. Oosthoek et<br />al suggested that these morphological features distinguish a<br />parachute-like mitral valve from a true PMV.<br /><br />Shone’s complex is a rare congenital anomaly. Fewer than 100<br />patients have been reported in the literature. It is mostly detected<br />in childhood as the patient becomes symptomatic by the age of<br />2 years. The usual symptoms are dyspnea, nocturnal cough,<br />tachypnea, poor feeding, failure to thrive, fatigue, and signs<br />and symptoms of heart failure and reduced cardiac output. The<br />child usually has recurrent episodes of wheezing and respiratory<br />tract infections due to pulmonary congestion and exudation of<br />fluid into the lungs. The patient may occasionally present with<br />acute pulmonary edema.<br /><br />It is extremely unusual for a patient to remain largely<br />asymptomatic throughout childhood and get incidentally<br />detected during adulthood while evaluating for some unrelated<br />illness. The present case therefore assumes significance. This<br />patient had been largely asymptomatic (except for NYHA class I<br />dyspnoea which he had ignored) in childhood. He had presented<br />to us for meningitis during which his clinical examination<br />revealed evidence of mitral stenosis along with left ventricular<br />outflow tract obstruction and aortic coarctation. This prompted<br />us to investigate the patient in detail. The echocardiographic<br />findings revealed the features of complete form of Shone’s<br />complex.<br /><br />A literature search revealed a few articles mostly case reports.<br />Goswami et al reported Shone’s anomaly in a young gravid<br />female mimicking preeclampsia at 25 weeks gestation. Most of<br />the other reports are in children. Most of these reports are from<br />foreign literature. To the best of our knowledge the present case<br />report is the first report of Shone’s anomaly from India.<br />A good outcome is possible in patients with Shone’s complex,<br />provided the surgical intervention is undertaken early before<br />the onset of pulmonary hypertension. Mitral valve repair<br />along with resection of supramitral ring is preferable over valve<br />replacement. Other surgical procedures depend upon existence<br />of associated cardiac anomalies, which ultimately define late<br />surgical outcome.<br /><br />Click here for <a href="http://www.japi.org/may_2009/article_12.pdf">Case Report </a>referenced.LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-8331819309301955882009-07-04T00:06:00.001-07:002009-07-04T00:19:18.707-07:00Ventricular Septal Defect (VSD)<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhk7wQdyA_nXTlOao10LQac3DQ0q7po3ZB9Wvve7MqIkiP-Z_TwKP_gt6h7qXtmTTOiJ9gsa_1VgAYq6E2pojdNnDB-E6LpFbuU0gN2uBe82zwkq2Appeozexj9XD8tZgU3PSLYvk9W2KCT/s1600-h/1.bmp"><img id="BLOGGER_PHOTO_ID_5354497947983020530" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 400px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhk7wQdyA_nXTlOao10LQac3DQ0q7po3ZB9Wvve7MqIkiP-Z_TwKP_gt6h7qXtmTTOiJ9gsa_1VgAYq6E2pojdNnDB-E6LpFbuU0gN2uBe82zwkq2Appeozexj9XD8tZgU3PSLYvk9W2KCT/s400/1.bmp" border="0" /></a><br /><div><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhyhuKnasupkZJi1EFpF3mVtB37uDk-uEHTBEzCqAjhKRmik6rsGRCoBHS-ol9inyOoTsE_aszToX2ACTlR5mVN-o-Fk7CgfdaCZnvv4TKkyOaeobXVt37y0YoKVddNZh16-k62yI0hfApD/s1600-h/2.bmp"><img id="BLOGGER_PHOTO_ID_5354497943557622050" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 400px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhyhuKnasupkZJi1EFpF3mVtB37uDk-uEHTBEzCqAjhKRmik6rsGRCoBHS-ol9inyOoTsE_aszToX2ACTlR5mVN-o-Fk7CgfdaCZnvv4TKkyOaeobXVt37y0YoKVddNZh16-k62yI0hfApD/s400/2.bmp" border="0" /></a><br /><br /><div><strong>What is a ventricular septal defect?</strong></div><div>A ventricular septal defect is an opening in the ventricular septum, or dividing wall between the two lower chambers of the heart known as the right and left ventricles. VSD is a congenital (present at birth) heart defect. As the fetus is growing, something occurs to affect heart development during the first 8 weeks of pregnancy, resulting in a VSD. </div><div><br />Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped out to the body through the aorta. </div><div><br />A ventricular septal defect allows oxygen-rich (red) blood to pass from the left ventricle, through the opening in the septum, and then mix with oxygen-poor (blue) blood in the right ventricle.</div><div><strong></strong> </div><div><strong>What are the different types of VSD?<br /></strong></div><div>Two basic types of VSD include the following:<br />1. <u>perimembranous VSD</u> - an opening in the upper section of the ventricular septum, near the valves, occurs in 75 percent of all VSD cases.<br />2. <u>muscular VSD</u> - an opening in the lower section of the ventricular septum occurs in up to 20 percent of all VSD cases. </div><div><br />Ventricular septal defects are the most commonly occurring type of congenital heart defect, occurring in 14 to 17 percent of babies born each year.</div><div><br /><strong>What causes ventricular septal defect?</strong></div><div>The heart is forming during the first 8 weeks of fetal development. It begins as a hollow tube, then partitions within the tube develop that eventually become the septa (or walls) dividing the right side of the heart from the left. Ventricular septal defects occur when the partitioning process does not occur completely, leaving an opening in the ventricular septum. </div><div><br />Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Most ventricular septal defects occur sporadically (by chance), with no clear reason for their development.</div><div><br /><strong>Why is ventricular septal defect a concern?</strong></div><div>If not treated, this heart defect can cause lung disease. When blood passes through the VSD from the left ventricle to the right ventricle, a larger volume of blood than normal must be handled by the right side of the heart. Extra blood then passes through the pulmonary artery into the lungs, causing higher pressure than normal in the blood vessels in the lungs. </div><div><br />A small opening in the ventricular septum allows a small amount of blood to pass through from the left ventricle to the right ventricle. A large opening allows more blood to pass through and mix with the normal blood flow in the right heart. Extra blood causes higher pressure in the blood vessels in the lungs. The larger the volume of blood that goes to the lungs, the higher the pressure.</div><div><br />The lungs are able to cope with this extra pressure for while, depending on exactly how high the pressure is. After a while, however, the blood vessels in the lungs become diseased by the extra pressure.</div><div><br />As pressure builds up in the lungs, the flow of blood from the left ventricle, through the VSD, into the right ventricle, and on to the lungs will diminish. This helps preserve the function of the lungs, but causes yet another problem. Blood flow within the heart goes from areas where the pressure is high to areas where the pressure is low. If a ventricular septal defect is not repaired, and lung disease begins to occur, pressure in the right side of the heart will eventually exceed pressure in the left. In this instance, it will be easier for oxygen-poor (blue) blood to flow from the right ventricle, through the VSD, into the left ventricle, and on to the body. When this happens, the body does not receive enough oxygen in the bloodstream to meet its needs.<br />Because blood is pumped at high pressure by the left ventricle through the VSD, tissue damage may eventually occur in the right ventricle. Bacteria in the bloodstream can easily infect this injured area, causing a serious illness known as bacterial endocarditis.</div><div><br />Some ventricular septal defects are found in combination with other heart defects (such as in transposition of the great arteries).</div><div><br /><strong>What are the symptoms of a ventricular septal defect?</strong></div><div>The size of the ventricular septal opening will affect the type of symptoms noted, the severity of symptoms, and the age at which they first occur. A VSD permits extra blood to pass from the left ventricle through to the right side of the heart, and the right ventricle and lungs become overworked as a result. The larger the opening, the greater the amount of blood that passes through and overloads the right ventricle and lungs. </div><div><br />Symptoms often occur in infancy. The following are the most common symptoms of VSD. However, each child may experience symptoms differently. </div><p>Symptoms may include:<br /></p><ul><li>fatigue </li><li>sweating </li><li>rapid breathing </li><li>heavy breathing </li><li>congested breathing </li><li>disinterest in feeding, or tiring while feeding </li><li>poor weight gain </li></ul><div>The symptoms of VSD may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.</div><div><br /><strong>How is a ventricular septal defect diagnosed?</strong></div><div>Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the opening from the left side of the heart to the right. </div><div><br />A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. The location within the chest where the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. </div><div> </div><div>However, other tests are needed to help with the diagnosis, and may include the following:</div><ul><li>chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. With a VSD, the heart may be enlarged because the right ventricle handles larger amounts of blood flow than normal. Also, there may be changes that take place in the lungs due to extra blood flow that can be seen on an x-ray.</li><li>electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress.</li><li>echocardiogram (echo) - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. An echo can show the pattern of blood flow through the septal opening, and determine how large the opening is, as well as much blood is passing through it.</li><li>cardiac catheterization - a cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart.<br /></li></ul><p><strong>Treatment for ventricular septal defect:</strong></p><p>Specific treatment for VSD will be determined by your child's physician based on:<br />your child's age, overall health, and medical history, extent of the disease,<br />your child's tolerance for specific medications, procedures, or therapies, expectations for the course of the disease , your opinion or preference. </p><p>Small ventricular septal defects may close spontaneously as your child grows. A larger VSD usually requires surgical repair. Regardless of the type, once a ventricular septal defect is diagnosed, your child's cardiologist will evaluate your child periodically to see whether it is closing on its own. A VSD will be repaired if it has not closed on its own - to prevent lung problems that will develop from long-time exposure to extra blood flow. Treatment may include:<br />medical managementSome children have no symptoms, and require no medication. However, most children may need to take medications to help the heart work better, since the right side is under strain from the extra blood passing through the VSD. </p><p>Medications that may be prescribed include the following:<br />1. digoxin - a medication that helps strengthen the heart muscle, enabling it to pump more efficiently.<br />2. diuretics - the body's water balance can be affected when the heart is not working as well as it could. These medications help the kidneys remove excess fluid from the body.</p><p><strong>Adequate Nutrition</strong> Infants with a larger VSD may become tired when feeding, and are not able to eat enough to gain weight. Options that can be used to ensure your baby will have adequate nutrition include the following:<br />1. high-calorie formula or breast milkSpecial nutritional supplements may be added to formula or pumped breast milk that increase the number of calories in each ounce, thereby allowing your baby to drink less and still consume enough calories to grow properly.<br />2. supplemental tube feedingsFeedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, can either supplement or take the place of bottle feedings. Infants who can drink part of their bottle, but not all, may be fed the remainder through the feeding tube. Infants who are too tired to bottle feed may receive their formula or breast milk through the feeding tube alone.</p><p><strong>Infection Control</strong> Children with certain heart defects are at risk for developing an infection of the inner surfaces of the heart known as bacterial endocarditis. A common procedure that puts your child at risk for this infection is a routine dental check-up and teeth cleaning. Other procedures may also increase the risk of the heart infection occurring. However, giving children with heart defects an antibiotic by mouth before these procedures can help prevent bacterial endocarditis. It is important that you inform all medical personnel that your child has a VSD so they may determine if the antibiotics are necessary before a procedure.</p><p><strong>Surgical Repair</strong> The goal is to repair the septal opening before the lungs become diseased from too much blood flow and pressure. Repair is indicated for defects that are causing symptoms, such as poor weight gain and rapid breathing. Your child's cardiologist will recommend when the repair should be performed based on echocardiogram and cardiac catheterization results.Your child's VSD may be repaired surgically in the operating room or by a cardiac catheterization procedure. One method currently being used to close some VSDs is the use of a device called a septal occluder. During this procedure, the child is sedated and a small, thin flexible tube is inserted into a blood vessel in the groin and guided into the heart. Once the catheter is in the heart, the cardiologist will pass the septal occluder into the VSD. The septal occluder closes the ventricular septal defect providing a permanent seal.The operation is performed under general anesthesia. Depending on the size of the heart defect and your physician's recommendations, the ventricular septal defect will be closed with stitches or a special patch. Consult your child's cardiologist for more information. </p><p><strong>Postoperative care for your child</strong>:<br />In most cases, children will spend time in the intensive care unit (ICU) after an VSD repair. During the first several hours after surgery, your child will most likely be drowsy from the anesthesia that was used during the operation, and from medications given to relax him/her and to help with pain. As time goes by, your child will become more alert.<br /></p><p>While your child is in the ICU<em>...which is where I come in...</em>special equipment will be used to help him/her recover, and may include the following:<br />1. ventilator - a machine that helps your child breathe while he/she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he/she is too sleepy to breathe effectively on his/her own. Many children have the ventilator tube removed right after surgery, but some other children will benefit from remaining on the ventilator for a few hours afterwards so they can rest.<br />intravenous (IV) catheters - small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medications that help your child recover from the operation.<br />arterial line - a specialized IV placed in the wrist, or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the ICU.<br />2. nasogastric (NG) tube - a small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.<br />3. urinary catheter - a small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and, therefore, the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys to remove excess fluids from the body.<br />chest tube - a drainage tube may be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.<br />4. heart monitor - a machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values. </p><p>Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.<br />Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff may also ask for your input as to how best to soothe and comfort your child.</p><p>After discharged from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you written instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.</p><p>Care for your child at home following VSD surgical repair:<br />Most infants and older children feel fairly comfortable when they go home. Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child's physician will discuss pain control before your child is discharged from the hospital.<br />Often, infants who fed poorly prior to surgery have more energy after the recuperation period, and begin to eat better and gain weight faster.</p><p>After surgery, older children usually have a fair tolerance for activity. Your child may become tired quicker than before surgery, but usually will be allowed to play with supervision, while avoiding blows to the chest that might cause injury to the incision or breastbone. Within a few weeks, your child should be fully recovered and able to participate in normal activity.<br />You may receive additional instructions from your child's physicians and the hospital staff.<br />Long-term outlook after VSD surgical repair:Most children who have had a ventricular septal defect repair will live healthy lives. Activity levels, appetite, and growth will return to normal in most children. Your child's cardiologist may recommend that antibiotics be given to prevent bacterial endocarditis for a specific time period after discharge from the hospital.<br />Consult your child's physician regarding the specific outlook for your child.</p><p><span style="font-size:78%;">Information obtained from </span><a href="http://www.rush.edu/rumc/page-P06622.html#"><span style="font-size:78%;">RUMC</span></a></p></div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-9419760692062553142009-07-03T23:55:00.000-07:002009-07-04T00:00:25.357-07:00Duodenal Atresia<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEilG2ayKJYOhZrBbGhMVwqoyasR-miAffrNI-ne1Lck5sEQPnZJlRgNDD5IECX__bknyxZ-W1JoURhSdOdiaZHdVpziVr51ynPXLMZq93MIg_bP6V1GL7styhXGDaYEnKxdF9FOkZdakqgn/s1600-h/duodenal-atresia.jpg"><img id="BLOGGER_PHOTO_ID_5354495562668614290" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 350px; CURSOR: hand; HEIGHT: 400px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEilG2ayKJYOhZrBbGhMVwqoyasR-miAffrNI-ne1Lck5sEQPnZJlRgNDD5IECX__bknyxZ-W1JoURhSdOdiaZHdVpziVr51ynPXLMZq93MIg_bP6V1GL7styhXGDaYEnKxdF9FOkZdakqgn/s400/duodenal-atresia.jpg" border="0" /></a><br /><div>Duodenal atresia represents complete obliteration of the duodenal lumen. A duodenal diaphragm (or duodenal web) is thought to represent a mild form of atresia. Duodenal stenosis (incomplete obstruction of the duodenal lumen) is discussed with duodenal atresia because the 2 disorders together represent a spectrum of similar intrauterine events. </div><div><br />Annular pancreas occurs when pancreatic tissue surrounds the second portion of the duodenum. If the encirclement is complete, it may be associated with complete or incomplete duodenal obstruction. Since duodenal atresia or duodenal stenosis occurs in all cases of annular pancreas, the anomalous pancreas should be considered a secondary change rather than a primary cause of duodenal obstruction.<br /><a name="0104"></a><br /><strong>Pathophysiology</strong><a id="IntroductionPathophysiology" name="IntroductionPathophysiology"></a><br />The etiology of duodenal atresia and stenosis is unknown. Failure of recanalization of the duodenal lumen remains the favored theory, compared with intrauterine vascular ischemia.<br />During the third week of embryonic development, the second portion of the duodenum, at the junction of the foregut and midgut, forms biliary and pancreatic buds, which are derived from endoderm. During the next 4 weeks, these buds differentiate into the hepatobiliary system, with the development and subsequent fusion of the 2 pancreatic anlagen. Concurrently, the epithelium of the duodenum undergoes active proliferation, which, at times, completely obliterates the duodenal lumen. Vacuolization, followed by recanalization, reestablishes the hollow viscus. </div><div><br />The second part of the duodenum is the last to recanalize. The early forming biliary system consists of 2 channels arising from the embryonic duodenum. This structure creates a narrow segment of bowel, approximately 0.125 mm in length, that is interposed between the 2 biliary channels. This narrow region is the area most prone to problems, with recanalization and with atresia formation. The ampulla of Vater usually is immediately adjacent to or traverses the medial wall of the diaphragm. The presence of a bifid biliary system, or the insertion of 1 duct above the atresia and 1 duct below it, is rare, occurring when both biliary duct anlagen remain patent. The presence of bile above and below the atresia indicates a bifid biliary system.<br /><a name="0105"></a><br /><strong>Frequency</strong><a id="IntroductionFrequency" name="IntroductionFrequency"></a><strong><br /></strong><em>United States</em><a id="IntroductionFrequencyUnitedStates" name="IntroductionFrequencyUnitedStates"></a> The incidence of duodenal atresia is 1 per 6000 births. Intrinsic congenital duodenal obstruction constitutes two thirds of all congenital duodenal obstructions (duodenal atresia, 40-60%; duodenal web, 35-45%; annular pancreas, 10-30%; duodenal stenosis, 7-20%).<br /><em>International</em><a id="IntroductionFrequencyInternational" name="IntroductionFrequencyInternational"></a> The incidence in Finland of congenital obstruction (intrinsic, extrinsic, combined) is 1 per 3400 live births.<a name="0108"></a> </div><div><br /><strong>Mortality/Morbidity</strong><a id="IntroductionMortalityMorbidity" name="IntroductionMortalityMorbidity"></a><br />If duodenal atresia or significant duodenal stenosis is left untreated, the condition rapidly becomes fatal as a result of electrolyte loss and fluid imbalance.<br />One half of the neonates with duodenal atresia or stenosis are born prematurely.<br />Hydramnios occurs in approximately 40% of neonates with duodenal obstruction.<br />Duodenal atresia or duodenal stenosis is most commonly associated with trisomy 21. About 22-30% of patients with duodenal obstruction have trisomy 21. Other problems associated with trisomy 21 include cardiac defects (most commonly ventricular septal defects and endocardial defects), as well as Hirschsprung disease.<br /><a name="0109"></a><br /><strong>Race</strong><a id="IntroductionRace" name="IntroductionRace"></a><strong><br /></strong>No racial predilection exists.<a name="0110"></a> </div><div><br /><strong>Sex</strong><a id="IntroductionSex" name="IntroductionSex"></a><br />The incidence of duodenal atresia and duodenal stenosis is approximately equal in males and females.<a name="0111"></a> </div><div><br /><strong>Age</strong><a id="IntroductionAge" name="IntroductionAge"></a><br />Infants with duodenal atresia present with vomiting in their first few hours of life, but patients with duodenal stenosis present at various ages. The clinical findings depend on the degree of stenosis. Occasionally, with duodenal web or duodenal stenosis, presentation occurs in adulthood.<a name="0106"></a><br /><strong>Anatomy</strong><a id="IntroductionAnatomy" name="IntroductionAnatomy"></a><br />In most cases, duodenal atresia occurs below the ampulla of Vater. In a very few cases, the atresia occurs proximal to the ampulla.<a name="0112"></a> </div><div><br /><strong>Presentation</strong><a id="IntroductionClinicalDetails" name="IntroductionClinicalDetails"></a><br />Bile-stained vomit in neonates aged 24 hours or younger is the typical presentation of atresia or severe stenosis. Minimal duodenal obstruction in mild stenosis or duodenal membrane may have few symptoms. In a few cases, the atresia is proximal to the ampulla of Vater and the vomit is free of bile.Both duodenal anomalies can be associated with other GI and biliary tract abnormalities (malrotation, esophageal atresia, ectopic anus, annular pancreas, gallbladder or biliary atresia, vertebral anomalies). In addition, duodenal atresia can be associated with a duodenal diaphragm, as well as with congenital abnormalities in other systems. Examples include VATER (vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, radial and renal anomalies) association and VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, limb) syndrome.Anomalies of the kidneys can occur in VATER association; the most common of these renal abnormalities include aplasia, dysplasia, hydronephrosis, ectopia, persistent urachus, vesicoureteral reflux, and ureteropelvic obstruction.<br />A few familial cases have been reported.<br /><a name="0113"></a><br /><strong>Preferred Examination</strong><a id="IntroductionPreferredExamination" name="IntroductionPreferredExamination"></a><strong><br /></strong>Plain radiographs that demonstrate a double-bubble appearance with no distal gas are characteristic of duodenal atresia. Distal bowel gas indicates stenosis, incomplete membrane, or a hepatopancreatic ductal anomaly. Occasionally, a radiograph must be obtained with the patient in the erect or the decubitus position to delineate the duodenal component. If a combination of esophageal atresia and duodenal atresia is present, ultrasonography is preferred.<a name="0114"></a> </div><div><br /><strong>Limitations of Techniques</strong><a id="IntroductionLimitationsofTechniques" name="IntroductionLimitationsofTechniques"></a><br />No oral contrast materials are necessary in the evaluation of complete duodenal obstruction. Occasionally, a small amount of positive contrast material can be instilled through a feeding tube into the distal stomach and duodenum to differentiate the diaphragm from a long stenosis.<br />Occasionally, barium enema examination is suggested as an adjunct study in the evaluation of duodenal atresia. Barium enema findings can demonstrate a malpositioned cecum, but this is not always diagnostic of malrotation and volvulus. In addition, if a microcolon is demonstrated, the presence of additional, more distal atresias can be suggested. Succus entericus may be prevented from reaching the colon because of the additional area of bowel obstruction. Multiple atresias are present in approximately 15% of patients. However, most surgeons can determine the presence of malrotation and additional atresias at the time of surgery. </div><div><br /> </div><div><span style="font-size:78%;"><em>Information Obtained from </em></span><a href="http://emedicine.medscape.com/article/408582-overview"><span style="font-size:78%;"><em>EMed</em></span></a><span style="font-size:78%;"><em> and </em></span><a href="http://www.lucinafoundation.org/assets/duodenal-atresia.jpg"><span style="font-size:78%;"><em>Lucina Foundation</em></span></a></div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-81731672153861172462009-07-03T22:01:00.000-07:002009-07-03T22:57:56.870-07:00Atrial Septal Defect (ASD)<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEguGWsERw6ghgyAXJ-IlT2iSA9S-Wo0mhxq-07qyRXE_EVc-GoCiS7wNP4lss3kg3gC-FxYcpHDzrqTmlKSo4hgZWG1aDBBj2mFqZa1vtPd3pPZ3617LcgBN2MDFdRIqXq0AhuMOF7Okd4s/s1600-h/asd.bmp"><img id="BLOGGER_PHOTO_ID_5354479995325846770" style="FLOAT: left; MARGIN: 0px 10px 10px 0px; WIDTH: 232px; CURSOR: hand; HEIGHT: 320px" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEguGWsERw6ghgyAXJ-IlT2iSA9S-Wo0mhxq-07qyRXE_EVc-GoCiS7wNP4lss3kg3gC-FxYcpHDzrqTmlKSo4hgZWG1aDBBj2mFqZa1vtPd3pPZ3617LcgBN2MDFdRIqXq0AhuMOF7Okd4s/s320/asd.bmp" border="0" /></a> <strong>What Is It?</strong><br />An Atrial Septal Defect (ASD) is a hole in the atrial septum, or muscle wall, that separates the right and left atria (singular = atrium), or upper chambers of the heart. Because of the lower pressure in the right atrium, this hole typically allows oxygenated blood from the lungs to move, or shunt, from the left into the right atrium. This blood proceeds into the right ventricle, which pumps it back to the lungs rather than to the body.ASDs vary in size and in the severity of symptoms they may cause. They account for between 5 and 10 per cent of all cases of congenital heart disease and are twice as prevalent among girls as boys.<br /><br /><br /><br /><br /><br />~~~~~~~~~<br /><br /><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEh2hhPvfuwfv8jVB1fB7rhtUZjpMF10YbnAUiEUIEoDIW3WGK4v7Y5ndZC0pDH0U5KsFwqUEFeYs7Ok3RjDoSQ9FCRVe5vQjoH6QfVEdcnEgPUbB4VntW07NGjjo8oX2KpmRCf66YK2aeho/s1600-h/2.bmp"><img id="BLOGGER_PHOTO_ID_5354465942371502274" style="FLOAT: left; MARGIN: 0px 10px 10px 0px; WIDTH: 223px; CURSOR: hand; HEIGHT: 376px" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEh2hhPvfuwfv8jVB1fB7rhtUZjpMF10YbnAUiEUIEoDIW3WGK4v7Y5ndZC0pDH0U5KsFwqUEFeYs7Ok3RjDoSQ9FCRVe5vQjoH6QfVEdcnEgPUbB4VntW07NGjjo8oX2KpmRCf66YK2aeho/s400/2.bmp" border="0" /></a><br /><strong>What are its effects? </strong>Children with significant ASDs are characteristically slender of build and have a heart murmur. The murmur is caused by the extra blood flow across the pulmonary valve. Some children may experience shortness of breath or heart palpitations. However, they are normally active and show no other outward symptoms. There are no exercise restrictions for these children.The larger the defect, the more likely children will have symptoms. Infants with a large ASD may develop congestive heart failure. However, if the defect is small (less than 2 millimeters), there is a very high probability that it will close on its own. Surgery is not usually performed in these cases.Larger ASDs, which are more likely to remain open, cause an excessive flow of blood into the right atrium, right ventricle and pulmonary artery (see animation). This enlarges the right atrium and right ventricle (dilatation) and causes high pressures in the pulmonary artery that will eventually distort its shape and may rarely damage the blood vessels in the lungs.The enlargement of the right atrium can result in abnormal heart rhythms. These effects are not reversed by closing the ASD after the damage has been done. Heart failure is likely when a person with an untreated ASD reaches young adulthood.<br /><div align="left"></div><div align="center">~~~~~~~</div><strong><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgQ7V9b43Wyu9d8euahxiIkwLdh0NnXpQx3-bUCE-xQfURh9YKhENzeOd9JcyMkCY_LHOVt3KaJ30qFZaeZvkyocXh-0bITxeHXN-DX3conlVlOJqzkMjrq5fos8stKDWxpDZcsl7qPXEIJ/s1600-h/untitled.bmp"><img id="BLOGGER_PHOTO_ID_5354475539043439730" style="FLOAT: left; MARGIN: 0px 10px 10px 0px; WIDTH: 234px; CURSOR: hand; HEIGHT: 400px" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgQ7V9b43Wyu9d8euahxiIkwLdh0NnXpQx3-bUCE-xQfURh9YKhENzeOd9JcyMkCY_LHOVt3KaJ30qFZaeZvkyocXh-0bITxeHXN-DX3conlVlOJqzkMjrq5fos8stKDWxpDZcsl7qPXEIJ/s400/untitled.bmp" border="0" /></a></strong><br /><strong>Variations of ASD </strong>Atrial Septal Defects are divided into three different types on the basis of the position of the hole (or holes) in the atrial septum.The first type of ASD is known as ostium primum defect, or simply, primum (number 1 in the diagram). In this kind of defect, the hole is located in the lower part of the atrial septum, near the tricuspid valve, which opens into the right ventricle.The most common type of ASD (accounting for 50-70% of all cases) is known as ostium secundum defect, or simply, secundum (2). In this case, the hole is located near the center of the atrial septum.The third type of ASD is known as a sinus venosus defect, in which the hole is located near one of the two places where the vena cava (the vein that carries blood from the body to the heart) enters the right atrium. The two kinds of sinus venosus defect are distinguished by whether the hole is near the entry point of the superior vena cava (SVC) (superior vena caval type - 3 in diagram) or of the inferior vena cava (IVC) (inferior vena caval type - 4 in diagram).<br /><br /><br /><br /><br /><br /><strong>How Is It Treated?</strong> Babies with congestive heart failure because of volume overload to the lungs may be treated with diuretics such as Lasix (Furosemide) and Aldactone (Spironolactone). These medications can help to reduce the volume of fluid in the lung, which makes it easier for the infant to breathe and eat.Digoxin may also be prescribed. It increases the squeeze (contraction) of the heart muscle and helps it to function more effectively.For those infants whose feeding is affected, nutritional additives may be used to fortify the baby's milk. In more severe cases, nourishment with a naso-gastric tube may be necessary.If slow growth and other symptoms continue despite treatment with medication, surgery may be required to close the ventricular septal defect. The benefits of this surgery are usually dramatic: paleness and rapid breathing are corrected and the rate of growth becomes normal. The mortality risk in this type of surgery is very low.VSDs may be closed by patching (see animation) or suturing during open heart surgery. Small defects may be closed with simple sutures using a monofilament thread made of Prolene or Polypropylene. Larger holes may be covered with patches made of pieces of pericardium (the membrane that covers the heart) or of silk or a synthetic material such as Dacron or Teflon.<br /><br /><br /><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgbClL2f7WrPVzEVjuB-OYHMOwFULd6V2LyqwSC4d0DqDK2qmmenTXdmsGDEs9rudblxyW8bEFLaNjDUCNuSy0ntQywVq6qhKxO29Cr1nmyf5p-A1dmqdljBQSk2GnMiKSHWCzX7Taf2CX8/s1600-h/3.bmp"><img id="BLOGGER_PHOTO_ID_5354465931964617810" style="FLOAT: left; MARGIN: 0px 10px 10px 0px; WIDTH: 183px; CURSOR: hand; HEIGHT: 306px" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgbClL2f7WrPVzEVjuB-OYHMOwFULd6V2LyqwSC4d0DqDK2qmmenTXdmsGDEs9rudblxyW8bEFLaNjDUCNuSy0ntQywVq6qhKxO29Cr1nmyf5p-A1dmqdljBQSk2GnMiKSHWCzX7Taf2CX8/s400/3.bmp" border="0" /></a><br /><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhdFKakqImGDvEqQnuMF_PchjEUkWLrUifuQSgNHMKYAkyc6kuHo6RTMk22svixYX7PU1ad-Y4Eofi9Q-M9BCu2qC1fcFElQEsISxOZEL2bqf49mCAoQ0BM5aAa_JdDvvZyTVVX2r7gbFCR/s1600-h/4.bmp"><img id="BLOGGER_PHOTO_ID_5354465924552941810" style="FLOAT: left; MARGIN: 0px 10px 10px 0px; WIDTH: 215px; CURSOR: hand; HEIGHT: 293px" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhdFKakqImGDvEqQnuMF_PchjEUkWLrUifuQSgNHMKYAkyc6kuHo6RTMk22svixYX7PU1ad-Y4Eofi9Q-M9BCu2qC1fcFElQEsISxOZEL2bqf49mCAoQ0BM5aAa_JdDvvZyTVVX2r7gbFCR/s400/4.bmp" border="0" /></a><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhFzlc_p-GLLjv4Lq_7VgKTdkFqJd2NIERI3EqjVtUmvTEVu6isSmbpHV9aXNGJnshXpYBT44y03A459G7vC7e6ouatjQi34Abtt8Mwqi0jxEGsbtt3DsIwVlcimGD_GLB27ateR4ojNrQi/s1600-h/5.bmp"><img id="BLOGGER_PHOTO_ID_5354465914653740146" style="FLOAT: left; MARGIN: 0px 10px 10px 0px; WIDTH: 200px; CURSOR: hand; HEIGHT: 292px" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhFzlc_p-GLLjv4Lq_7VgKTdkFqJd2NIERI3EqjVtUmvTEVu6isSmbpHV9aXNGJnshXpYBT44y03A459G7vC7e6ouatjQi34Abtt8Mwqi0jxEGsbtt3DsIwVlcimGD_GLB27ateR4ojNrQi/s400/5.bmp" border="0" /></a><br /><br /><br /><br /><br /><br /><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhdFKakqImGDvEqQnuMF_PchjEUkWLrUifuQSgNHMKYAkyc6kuHo6RTMk22svixYX7PU1ad-Y4Eofi9Q-M9BCu2qC1fcFElQEsISxOZEL2bqf49mCAoQ0BM5aAa_JdDvvZyTVVX2r7gbFCR/s1600-h/4.bmp"></a><br /><br /><br /><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhFzlc_p-GLLjv4Lq_7VgKTdkFqJd2NIERI3EqjVtUmvTEVu6isSmbpHV9aXNGJnshXpYBT44y03A459G7vC7e6ouatjQi34Abtt8Mwqi0jxEGsbtt3DsIwVlcimGD_GLB27ateR4ojNrQi/s1600-h/5.bmp"></a><br /><br /><br /><br /><br /><br /><br /><br /><br /><br /><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgGYHRuxhTQDOD_bwNBJd6l4b_m4k1CYgwXLpBrNjQdBccckkh6SS7XfRga_S7hMoENaHtukEGl17WMwhGuJ59x5GHQRLVxFwFmNp6p-jTDE01kC_dB5krhz16OlB6Z5wVanbK-AhRI9mvf/s1600-h/untitled.bmp"></a><br /><br /><em><span style="font-size:78%;">Information obtained from </span></em><a href="http://www.pted.org/?id=home"><em><span style="font-size:78%;">Cove Heart Foundation: Congenital Heart Disease</span></em></a>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-79368082930818879382009-07-02T20:33:00.000-07:002009-07-02T20:58:20.923-07:00Scaphocephaly with Craniofacial Surgery<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgugZwh5y52SyQ730OZzRVuBIyf8nezDpTlhyphenhyphen5wTA1CFmqQnu8FNUkdhk5lN0F7o9x6QitQFDqE1yRpVRr6CjMzD-eg9ajCYxn2ZlRiwmX9iPCblb5Jn3f53QJLudYwKNh7v6vmRWNSU2Y2/s1600-h/scaphocephaly-ct.jpg"><img id="BLOGGER_PHOTO_ID_5354076475207566482" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 371px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEgugZwh5y52SyQ730OZzRVuBIyf8nezDpTlhyphenhyphen5wTA1CFmqQnu8FNUkdhk5lN0F7o9x6QitQFDqE1yRpVRr6CjMzD-eg9ajCYxn2ZlRiwmX9iPCblb5Jn3f53QJLudYwKNh7v6vmRWNSU2Y2/s400/scaphocephaly-ct.jpg" border="0" /></a><strong>Definition:</strong> <a href="http://www.scaphocephaly.info/">Scaphocephaly</a> (also know as, <a href="http://www.dolichocephaly.info/" target="_top">dolichocephaly</a>) refers to the condition where the head is disproportionately long and narrow (see <a href="http://www.plagiocephaly.org/headshape/default.htm#ci">cranial index</a>. <a href="http://www.scaphocephaly.info/">Scaphocephaly</a> can result from the premature fusion of the <a href="http://www.plagiocephaly.org/headshape/default.htm#sagittal">sagittal suture</a> (see <a href="http://www.craniosynostosis.info/">craniosynostosis</a>) or from external deformation. <a href="http://www.scaphocephaly.info/">Scaphocephaly</a> is particularly common among infants who are born prematurely.<br /><strong></strong><br /><strong>Diagnosis</strong>: The diagnosis begins with an examination by a pediatrician, pediatric neurosurgeon or craniofacial surgeon. A primary objective of the examination is to rule out <a href="http://www.craniosynostosis.info/">craniosynostosis</a> (a condition that requires surgical correction). The initial examination involves questions about gestation, birth, in utero and post-natal positioning (for example, sleeping position). The physical examination includes inspection of the infant's head and may involve palpation (carefully feeling) of the child's skull for <a href="http://www.plagiocephaly.org/headshape/default.htm#ridges">suture ridges</a> and soft spots (the <a href="http://www.plagiocephaly.org/headshape/default.htm#fontanelles">fontanelles</a>). The physician may also request x-rays or computerized tomography (a CAT scan, a series of photographic images of the skull). These images provide the most reliable method for diagnosing premature fusion of the <a href="http://www.plagiocephaly.org/headshape/default.htm#sagittal">sagittal suture</a> (<a href="http://www.craniosynostosis.info/">craniosynostosis</a>). In addition, the physician may make (or order) a series of measurements from the child's face and head [more on <a href="http://www.plagiocephaly.org/headshape/anthropometry.htm" target="_top">cranial anthropometry</a>]. These measurements will be used to assess severity and monitor treatment.<br /><br /><strong>Treatment:</strong> The treatment of <a href="http://www.scaphocephaly.info/">scaphocephaly</a> depends upon the etiology (cause) of the condition: Scaphocephaly resulting from fusion of the <a href="http://www.plagiocephaly.org/headshape/default.htm#sagittal">sagittal suture</a> (<a href="http://www.craniosynostosis.info/">craniosynostosis</a>) must be treated surgically. Parents should consult a pediatric neurosurgeon or a craniofacial surgeon to discuss treatment option. Depending upon severity, <a href="http://www.scaphocephaly.info/">scaphocephaly</a> resulting from external/positional deformation can be treated with repositioning and/or head banding. Parents should consult a pediatrician, a pediatric neurosurgeon or a craniofacial surgeon for information on repositioning and/or for referral and a prescription for head banding.<br /><br />Support Groups:<br /><a href="http://www.scaphocephaly.info/support/">Plagiocephaly Parents Support</a><br /><a href="http://www.preemie-l.org/">Parents of Premature Babies Inc. (Preemie-L)</a><br /><a href="http://www.preemies.org/">Preemies.Org</a><br /><br />~~~~~~~~~~~~~~~~<br />Craniofacial Surgery:<br />The craniofacial area includes the base of the skull, the facial skeleton and underlying soft tissues, the skull vaults and the scalp. <a onclick="window.open('term_detail_popup.asp?TermID=198','Glossary','directory=no,menubar=no,width=500,height=200,top=100,left=100,scrollbars=yes')" href="http://www.ienhance.com/community/term_detail_popup.asp?TermID=198" target="Glossary">Craniofacial surgery</a> involves repairing damage caused by serious injuries as well as congenital deformities and abnormal growths such as tumors.<br /><br /><p>Congenital deformities include </p><ul><li>clefts of the lip and palate: In these conditions, all of the parts of the lip and roof of the mouth are present, but they have failed to fuse in a normal fashion. Clefts can appear with varying severity: a cleft lip can be incomplete with a fractional notching of the lip, or complete, extending through the lip and into the nose. ear deformities: In these conditions, the outer ear may be underdeveloped, misshaped, or completely absent. </li><li>premature fusing of the bones of the head in young children: In the normal infant skull, cracks or “sutures” appear in between bones of the head to allow for brain growth. When one of these sutures closes prematurely, the brain continues to grow, but pushes out toward the area of the skull where the sutures are still open. The result is a malformation of the skull and/or face. </li><li>misshapen jaws: often caused by misalignment of the teeth and jaws referred to as malocclusion, or mild hypoplasia (inadequate tissue development) which can appear as a recessed upper jaw or other underdeveloped bony area of the face. </li><li>facial asymmetries: or hemifacial microsomia, a condition wherein one side of the face is smaller than the other, due to underdevelopment of bone and/or cartilage.<br /></li></ul><p>After appropriate assessment, surgical treatment may be recommended and this will vary considerably depending on what the particular problem is. Sometimes craniofacial surgery for deformity can be carried out without making visible scars on the face. Craniofacial surgeries carry varying degrees of risk, depending on the particular problem. Sometimes bone or cartilage grafts need to be harvested from other areas of the body such as the ribs.<br /></p><p><em>***See pics below...note: none of these pictures are of any patients that I have cared for; they are all from the internet.</em></p><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEg5dWtV8gi4cFlnart9k3TCHqhJrPSoLP2BBLzTkYSWvYhyphenhyphenoCA6sXG9M9Hm01n-layDn_wetNjlYfpt2w0p1fxTi_d9NRUMV74TcmzinGRpWoVd9reqtBLiXv3y84ahgIDp6wIUqSXlm6TV/s1600-h/nf0903.02.fig2"><img id="BLOGGER_PHOTO_ID_5354076464583094450" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 275px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEg5dWtV8gi4cFlnart9k3TCHqhJrPSoLP2BBLzTkYSWvYhyphenhyphenoCA6sXG9M9Hm01n-layDn_wetNjlYfpt2w0p1fxTi_d9NRUMV74TcmzinGRpWoVd9reqtBLiXv3y84ahgIDp6wIUqSXlm6TV/s400/nf0903.02.fig2" border="0" /></a>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-4721617865138553732009-06-23T23:36:00.001-07:002009-06-24T01:49:57.590-07:00Coarctation of The Aorta<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjshWap9ppwoGFCK7GlSjGz7-sOlR-GRXjq-wHSAp9U-62wPuR2xf5fcH7Z4w2ls48ojefneEXfyfFkgsHm-vGItAT6Z_pgqPn3qDqPZKKR6mYbLelfz6ZoPitxAs4s3C_1bQtgC6VmfCWw/s1600-h/100_4062.JPG"><img id="BLOGGER_PHOTO_ID_5350779547823614546" style="FLOAT: left; MARGIN: 0px 10px 10px 0px; WIDTH: 260px; CURSOR: hand; HEIGHT: 400px" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjshWap9ppwoGFCK7GlSjGz7-sOlR-GRXjq-wHSAp9U-62wPuR2xf5fcH7Z4w2ls48ojefneEXfyfFkgsHm-vGItAT6Z_pgqPn3qDqPZKKR6mYbLelfz6ZoPitxAs4s3C_1bQtgC6VmfCWw/s400/100_4062.JPG" border="0" /></a> <strong>What It Is</strong><br />The aorta is the body's main artery. It distributes oxygen-rich blood to all parts of the body except the lungs. The first branches of the aorta go to the upper body (arms and head). After that, blood goes to the lower body (abdomen and legs). Coarctation of the aorta is a narrowing of the aorta between the upper-body artery branches and the branches to the lower body. This blockage can increase blood pressure in your arms and head, reduce pressure in your legs and seriously strain your heart. Aortic valve abnormalities often accompany coarctation.<br /><strong>Surgical Treatment</strong><br />The narrowing can be removed by surgery or sometimes by a nonsurgical balloon dilation in the cardiac catheterization lab. Aortic coarctation may return even after successful surgery or balloon dilation. This isn't uncommon if your repair was done when you were a newborn. (It's uncommon if it was repaired when you were a child.) If you've reached your full adult size and have no blood pressure difference between your arms and legs, it's highly unlikely that your aorta will become obstructed again.<br />Recurrent coarctation is usually treated with nonsurgical balloon dilation or by implanting a stent using cardiac catheterization.<br /><br /><strong>Ongoing Care</strong><br />Medical After the coarctation is repaired, you'll need your blood pressure checked every 1-2 years. The reason is that you're at higher risk of developing generalized high blood pressure or problems with your aortic valve. Both of these can be checked for during your routine cardiology visits.<br /><br /><strong>Activity Restrictions</strong> Depending on your blood pressure at rest or during exercise, you may be advised to avoid some forms of strenuous exercise. Heavy isometric exercise, such as power weightlifting, may be a particular concern if your pressure is elevated. In general, you don't need to restrict activity if your arm and leg blood pressures are normal. (See the <a href="http://www.americanheart.org/presenter.jhtml?identifier=11081">Physical Activity</a> section.) Ask your cardiologist if you should limit any activity.<br /><br /><strong>Endocarditis Prevention</strong><br />You may need antibiotics before certain dental or surgical procedures if you have an aortic obstruction or aortic valve abnormality. (See the section on <a href="http://www.americanheart.org/presenter.jhtml?identifier=11086">Endocarditis</a>.)<br />Pregnancy Most women with repaired coarctation shouldn't have any difficulties, unless there's residual aortic obstruction or generalized high blood pressure. However, if you have persistent coarctation or any associated problems that might affect you or your baby, check with your physician before considering getting pregnant. (See the section on <a href="http://www.americanheart.org/presenter.jhtml?identifier=11082">Pregnancy</a>.)<br /><br /><strong>Problems You May Hav</strong>e<br />Symptoms Coarctation of the aorta usually doesn't have symptoms. However, if the obstruction becomes severe, you may not tolerate exercise well. You could have a headache or leg pains after exertion. You also might have chest pain or palpitations. Tell your cardiologist promptly about any activity-related symptoms.<br /><br /><strong>Will You Need More Surgery? </strong><br />The need for surgery or catheterization depends mostly on the level of pressure in your arms and legs when you're resting and, under some circumstances, during exercise. If your arm and leg blood pressures are normal, you probably won't need more intervention.<br /><br /><span style="font-size:78%;"><a href="http://www.americanheart.org/presenter.jhtml?identifier=11069#">Information Obtained from The American Heart Association</a></span>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-73737389374105675192009-01-08T08:41:00.000-08:002009-01-08T08:45:22.093-08:00Supraventricular Tachycardia (SVT)<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjI_1wfqbiKOxEEZJDeKYhyphenhyphenFL2oWUq0C-VpUlwFzhmBCTyl8LIFlKTm6iAyinsPLDZqPJCsAx0vGqBYnmqnu1IHdhzwGRIEeXq_BjtAVGlvaLkwm6_ULxmbmsHz8DhpMa0sfceP4PjxKaf1/s1600-h/electsys.gif"><img id="BLOGGER_PHOTO_ID_5288964861077952850" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 376px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjI_1wfqbiKOxEEZJDeKYhyphenhyphenFL2oWUq0C-VpUlwFzhmBCTyl8LIFlKTm6iAyinsPLDZqPJCsAx0vGqBYnmqnu1IHdhzwGRIEeXq_BjtAVGlvaLkwm6_ULxmbmsHz8DhpMa0sfceP4PjxKaf1/s400/electsys.gif" border="0" /></a><br /><div align="left"><a name="navskip"></a><strong>Supraventricular Tachycardia (SVT)</strong></div><strong></strong><br /><div align="left">Supraventricular (originating above the ventricles) tachycardia (SVT) is a series of fast atrial heartbeats that can cause the heart to contract at rates of 250 times per minute or faster. SVT can be uncomfortable and frightening. The type of treatment depends on whether the electrical impulses reenter the atria via a bypass tract (Wolff-Parkinson-White syndrome), through the atrioventricular (AV) node, or are caused by a single abnormal group of cells.</div><br /><div align="left"><br /></div><a id="Jump_Link_One" name="Jump_Link_One"></a><br /><div align="left"><strong>Wolff-Parkinson-White (WPW) Syndrome</strong></div><br /><div align="left">Wolff-Parkinson-White Syndrome is a common cause of SVT. In WPW there is an abnormal electrical connection between the atria and ventricles. This extra tissue is a short circuit between these chambers. It provides an extra pathway for electrical impulses to be conducted through the tissue that normally blocks electrical impulses between the atria and ventricles. This short circuit is called an accessory pathway, and it allows electrical impulses to travel between the atria and the ventricles without going through the AV node. In WPW, an SVT is usually started when an impulse travels down the AV node to the ventricles and then up through the short circuit tissue to the atria. This impulse can then travel through the atria and down the AV node before the SA node can start the next heartbeat. If the impulse continues to travel in this repeating, circular pattern, it can cause the heart to beat very rapidly.</div><br /><div align="left"><br /><a id="Jump_Link_Two" name="Jump_Link_Two"></a><strong>AV Nodal Reentrant Tachycardia</strong><br />AV Nodal Reentrant Tachycardia (AVNRT) is another common form of SVT. In AVNRT, there is an extra electrical pathway in or near the AV node. If an electrical impulse is conducted in this pathway, it may direct the impulse through both the AV node and the extra pathway in a repeating, circular pattern. The AV node and the extra pathway are located essentially in the center of the heart. This causes the upper and lower chambers to beat rapidly at the same time instead of in the normal sequence (upper chambers beating first, followed by the lower chambers).<br /><a href="http://www.sjm.com/globals/printpageview.aspx?area=html&param1=/generatedtemplates/conditions//overview/supraventriculartachycardia.htm&param2=&param3=#top"></a><br /><a id="Jump_Link_Three" name="Jump_Link_Three"></a><strong>Rapid AV Nodal Conduction<br /></strong>In some SVTs the atria may spontaneously generate multiple rapid impulses. Many of these impulses can travel through the AV node to the ventricles in an erratic manner. As a result, the heart rhythm can become irregular and rapid. If this happens, the heart will not pump blood efficiently.</div><br /><div align="left"><br /><a id="Jump_Link_Four" name="Jump_Link_Four"></a><strong>Atrial Flutter<br /></strong>Atrial flutter is one of the more common SVTs, where the upper chambers of the heart (atria) beat anywhere from 240 to 320 times per minute. This arrhythmia is similar to atrial fibrillation, as it originates entirely within the upper chambers, but it produces a more organized, regular rhythm. It is usually not harmful, but can result in symptoms such as palpitations, shortness of breath, chest tightness, fatigue, and lightheadedness. If left untreated, atrial flutter may eventually lead to conditions associated with other arrhythmias that result in abnormally high heart rates.</div><br /><div align="left"><br />Even though the upper chambers are beating rapidly, the AV node allows only one-half to one-third of the electrical impulses to reach the hearts lower chambers (the ventricles). This prevents the arrhythmia from becoming life-threatening, and keeps the wrist pulse rate at only 100 to 150 beats per minute. This arrhythmia can last for hours or days; therefore, most people with atrial flutter require treatment.</div><br /><div align="left"><br /><strong>Normal Rhythm</strong><br />Every normal heart has a normal rhythm. That rhythm varies from person to person. In most healthy people, the heart at rest beats about 60 to 100 times per minute. A small bunch of heart cells called the sinoatrial node keeps time.</div><br /><div align="left"><br /><a href="http://www.sjm.com/globals/printpageview.aspx?area=html&param1=/generatedtemplates/conditions//overview/supraventriculartachycardia.htm&param2=&param3=#top"></a><span style="font-size:78%;">© 2008 St. Jude Medical, Inc.</span> </div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-8329673722619204352009-01-08T08:34:00.000-08:002009-01-08T08:41:30.640-08:00Appendicitis<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjv2qigTrqBE64Uz-E0LUYYCEvhbcjyUUMeCR6xf4LcXpPjFJTj952akHZvvj4gvCWmGAJI-ZIQ4Aig-ngIPUcyZ9IU3iqw3QyRfLaYxIcWTcADRbmuh2-btdQWAARo7x_RtbslWVW2Am50/s1600-h/Appendicitis.jpg"><span style="color:#000000;"><img id="BLOGGER_PHOTO_ID_5288962715443172546" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 393px; CURSOR: hand; HEIGHT: 310px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEjv2qigTrqBE64Uz-E0LUYYCEvhbcjyUUMeCR6xf4LcXpPjFJTj952akHZvvj4gvCWmGAJI-ZIQ4Aig-ngIPUcyZ9IU3iqw3QyRfLaYxIcWTcADRbmuh2-btdQWAARo7x_RtbslWVW2Am50/s400/Appendicitis.jpg" border="0" /></span></a><span style="color:#000000;"><br /></span><div align="center"><a name="tocb"><strong><span style="color:#000000;">What is the appendix?</span></strong></a><span style="color:#000000;"> </span></div><div><span style="color:#000000;">The </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=2312"><span style="color:#000000;">appendix</span></a><span style="color:#000000;"> is a closed-ended, narrow tube up to several inches in length that attaches to the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=2658"><span style="color:#000000;">cecum</span></a><span style="color:#000000;"> (the first part of the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=2787"><span style="color:#000000;">colon</span></a><span style="color:#000000;">) like a worm. (The anatomical name for the appendix, vermiform appendix, means worm-like appendage.) The inner lining of the appendix produces a small amount of </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4450"><span style="color:#000000;">mucus</span></a><span style="color:#000000;"> that flows through the open center of the appendix and into the cecum. The wall of the appendix contains lymphatic tissue that is part of the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=3907"><span style="color:#000000;">immune system</span></a><span style="color:#000000;"> for making antibodies. Like the rest of the colon, the wall of the appendix also contains a layer of muscle, but the muscle is poorly developed. </span></div><div align="center"><br /><a name="tocc"><strong><span style="color:#000000;">What is appendicitis and what causes appendicitis?</span></strong></a></div><div><span style="color:#000000;"> Appendicitis means </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=3979"><span style="color:#000000;">inflammation</span></a><span style="color:#000000;"> of the appendix. It is thought that appendicitis begins when the opening from the appendix into the cecum becomes blocked. The blockage may be due to a build-up of thick mucus within the appendix or to </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=5564"><span style="color:#000000;">stool </span></a><span style="color:#000000;">that enters the appendix from the cecum. The mucus or stool hardens, becomes rock-like, and blocks the opening. This rock is called a fecalith (literally, a rock of stool). At other times, the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=10347"><span style="color:#000000;">lymphatic tissue</span></a><span style="color:#000000;"> in the appendix may swell and block the appendix. After the blockage occurs, bacteria which normally are found within the appendix begin to invade (infect) the wall of the appendix. The body responds to the invasion by mounting an attack on the bacteria, an attack called inflammation. An alternative theory for the cause of appendicitis is an initial rupture of the appendix followed by spread of bacteria outside the appendix.. The cause of such a rupture is unclear, but it may relate to changes that occur in the lymphatic tissue, for example, inflammation, that line the wall of the appendix.) </span></div><div align="center"><br /><span style="color:#000000;">If the inflammation and </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=12923"><span style="color:#000000;">infection</span></a><span style="color:#000000;"> spread through the wall of the appendix, the appendix can rupture. After rupture, infection can spread throughout the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=2081"><span style="color:#000000;">abdomen</span></a><span style="color:#000000;">; however, it usually is confined to a small area surrounding the appendix (forming a peri-appendiceal abscess).<br />Sometimes, the body is successful in containing ("healing") the appendicitis without surgical treatment if the infection and accompanying inflammation do not spread throughout the abdomen. The inflammation, pain and symptoms may disappear. This is particularly true in elderly patients and when antibiotics are used. The patients then may come to the doctor long after the episode of appendicitis with a lump or a mass in the right lower abdomen that is due to the scarring that occurs during healing. This lump might raise the suspicion of </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=13931"><span style="color:#000000;">cancer</span></a><span style="color:#000000;">.<br /><br /></span><a name="tocd"><strong><span style="color:#000000;">What are the complications of appendicitis?</span></strong></a><span style="color:#000000;"> </span></div><div><span style="color:#000000;">The most frequent complication of appendicitis is perforation. Perforation of the appendix can lead to a periappendiceal abscess (a collection of infected </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=5140"><span style="color:#000000;">pus</span></a><span style="color:#000000;">) or diffuse </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4843"><span style="color:#000000;">peritonitis</span></a><span style="color:#000000;"> (infection of the entire lining of the abdomen and the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4824"><span style="color:#000000;">pelvis</span></a><span style="color:#000000;">). The major reason for appendiceal perforation is delay in diagnosis and treatment. In general, the longer the delay between diagnosis and surgery, the more likely is perforation. The risk of perforation 36 hours after the onset of symptoms is at least 15%. Therefore, once appendicitis is diagnosed, surgery should be done without unnecessary delay. </span></div><div><br /><span style="color:#000000;">A less common complication of appendicitis is blockage of the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4004"><span style="color:#000000;">intestine</span></a><span style="color:#000000;">. Blockage occurs when the inflammation surrounding the appendix causes the intestinal muscle to stop working, and this prevents the intestinal contents from passing. If the intestine above the blockage begins to fill with liquid and gas, the abdomen distends and </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4510"><span style="color:#000000;">nausea</span></a><span style="color:#000000;"> and vomiting may occur. It then may be necessary to drain the contents of the intestine through a tube passed through the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=13183"><span style="color:#000000;">nose</span></a><span style="color:#000000;"> and </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=3326"><span style="color:#000000;">esophagus</span></a><span style="color:#000000;"> and into the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=5560"><span style="color:#000000;">stomach</span></a><span style="color:#000000;"> and intestine. </span></div><div><br /><span style="color:#000000;">A feared complication of appendicitis is </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=5449"><span style="color:#000000;">sepsis</span></a><span style="color:#000000;">, a condition in which infecting bacteria enter the blood and travel to other parts of the body. This is a very serious, even life-threatening complication. Fortunately, it occurs infrequently. </span></div><div align="center"><br /><a name="toce"><strong><span style="color:#000000;">What are the symptoms of appendicitis?</span></strong></a><span style="color:#000000;"> </span></div><div><span style="color:#000000;">The main symptom of appendicitis is </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=1908"><span style="color:#000000;">abdominal pain</span></a><span style="color:#000000;">. The pain is at first diffuse and poorly localized, that is, not confined to one spot. (Poorly localized pain is typical whenever a problem is confined to the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=5512"><span style="color:#000000;">small intestine </span></a><span style="color:#000000;">or colon, including the appendix.) The pain is so difficult to pinpoint that when asked to point to the area of the pain, most people indicate the location of the pain with a circular motion of their hand around the central part of their abdomen. A second, common, early symptom of appendicitis is loss of appetite which may progress to nausea and even vomiting. Nausea and vomiting also may occur later due to intestinal obstruction.<br />As appendiceal inflammation increases, it extends through the appendix to its outer covering and then to the lining of the abdomen, a thin </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4344"><span style="color:#000000;">membrane</span></a><span style="color:#000000;"> called the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4842"><span style="color:#000000;">peritoneum</span></a><span style="color:#000000;">. Once the peritoneum becomes inflamed, the pain changes and then can be localized clearly to one small area. Generally, this area is between the front of the right hip bone and the belly button. The exact point is named after Dr. Charles McBurney--McBurney's point. If the appendix ruptures and infection spreads throughout the abdomen, the pain becomes diffuse again as the entire lining of the abdomen becomes inflamed. </span></div><div align="center"><br /><a name="tocf"><strong><span style="color:#000000;">How is appendicitis diagnosed?</span></strong></a><span style="color:#000000;"> </span></div><div><span style="color:#000000;">The diagnosis of appendicitis begins with a thorough history and physical examination. Patients often have an elevated temperature, and there usually will be moderate to severe tenderness in the right lower abdomen when the doctor pushes there. If inflammation has spread to the peritoneum, there is frequently rebound tenderness. Rebound tenderness is pain that is worse when the doctor quickly releases his hand after gently pressing on the abdomen over the area of tenderness. </span></div><div align="left"><br /><span style="color:#000000;"><em>White Blood Cell Count</em> </span></div><div><span style="color:#000000;">The </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=9983"><span style="color:#000000;">white blood cell count</span></a><span style="color:#000000;"> in the blood usually becomes elevated with infection. In early appendicitis, before infection sets in, it can be normal, but most often there is at least a mild elevation even early. Unfortunately, appendicitis is not the only condition that causes elevated white blood cell counts. Almost any infection or inflammation can cause this count to be abnormally high. Therefore, an elevated white blood cell count alone cannot be used as a sign of appendicitis. </span></div><div><br /><span style="color:#000000;"><em>Urinalysis</em> </span></div><div><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=7542"><span style="color:#000000;">Urinalysis</span></a><span style="color:#000000;"> is a microscopic examination of the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=5915"><span style="color:#000000;">urine</span></a><span style="color:#000000;"> that detects red blood cells, white blood cells and bacteria in the urine. Urinalysis usually is </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=22433"><span style="color:#000000;">abnormal</span></a><span style="color:#000000;"> when there is inflammation or stones in the kidneys or bladder. The urinalysis also may be abnormal with appendicitis because the appendix lies near the ureter and bladder. If the inflammation of appendicitis is great enough, it can spread to the ureter and bladder leading to an abnormal urinalysis. Most patients with appendicitis, however, have a normal urinalysis. Therefore, a normal urinalysis suggests appendicitis more than a urinary tract problem.<br />Abdominal X-Ray An abdominal x-ray may detect the fecalith (the hardened and calcified, pea-sized piece of stool that blocks the appendiceal opening) that may be the cause of appendicitis. This is especially true in children. </span></div><div><br /><span style="color:#000000;"><em>Ultrasound</em> </span></div><div><span style="color:#000000;">An </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=510"><span style="color:#000000;">ultrasound</span></a><span style="color:#000000;"> is a painless procedure that uses sound waves to identify organs within the body. Ultrasound can identify an enlarged appendix or an abscess. Nevertheless, during appendicitis, the appendix can be seen in only 50% of patients. Therefore, not seeing the appendix during an ultrasound does not exclude appendicitis. Ultrasound also is helpful in women because it can exclude the presence of conditions involving the ovaries, fallopian tubes and uterus that can mimic appendicitis. </span></div><div><br /><span style="color:#000000;"><em>Barium Enema</em> </span></div><div><span style="color:#000000;">A </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=405"><span style="color:#000000;">barium enema</span></a><span style="color:#000000;"> is an x-ray test where liquid barium is inserted into the colon from the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=2294"><span style="color:#000000;">anus</span></a><span style="color:#000000;"> to fill the colon. This test can, at times, show an impression on the colon in the area of the appendix where the inflammation from the adjacent inflammation impinges on the colon. Barium enema also can exclude other intestinal problems that mimic appendicitis, for example Crohn's disease.<br />Computerized tomography (CT) Scan In patients who are not </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=10695"><span style="color:#000000;">pregnant</span></a><span style="color:#000000;">, a </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=315"><span style="color:#000000;">CT Scan</span></a><span style="color:#000000;"> of the area of the appendix is useful in diagnosing appendicitis and peri-appendiceal abscesses as well as in excluding other diseases inside the abdomen and pelvis that can mimic appendicitis.<br />Laparoscopy </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=944"><span style="color:#000000;">Laparoscopy</span></a><span style="color:#000000;"> is a surgical procedure in which a small fiberoptic tube with a camera is inserted into the abdomen through a small puncture made on the abdominal wall. Laparoscopy allows a direct view of the appendix as well as other abdominal and </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4822"><span style="color:#000000;">pelvic</span></a><span style="color:#000000;"> organs. If appendicitis is found, the inflamed appendix can be removed with the laparascope. The disadvantage of laparoscopy compared to ultrasound and CT is that it requires a general </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=2247"><span style="color:#000000;">anesthetic.</span></a><span style="color:#000000;"> </span></div><div><br /><span style="color:#000000;">There is no one test that will diagnose appendicitis with certainty. Therefore, the approach to suspected appendicitis may include a period of observation, tests as previously discussed, or surgery. </span></div><div align="center"><br /><a name="tocg"><strong><span style="color:#000000;">Why can it be difficult to diagnose appendicitis?</span></strong></a><span style="color:#000000;"> </span></div><div><span style="color:#000000;">It can be difficult to diagnose appendicitis. The position of the appendix in the abdomen may vary. Most of the time the appendix is in the right lower abdomen, but the appendix, like other parts of the intestine, has a </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4356"><span style="color:#000000;">mesentery.</span></a><span style="color:#000000;"> This mesentery is a sheet-like membrane that attaches the appendix to other structures within the abdomen. If the mesentery is large, it allows the appendix to move around. In addition, the appendix may be longer than normal. The combination of a large mesentery and a long appendix allows the appendix to dip down into the pelvis (among the pelvic organs in women). It also may allow the appendix to move behind the colon (called a retro-colic appendix). In either case, inflammation of the appendix may act more like the inflammation of other organs, for example, a woman's pelvic organs. </span></div><div><br /><span style="color:#000000;">The diagnosis of appendicitis also can be difficult because other inflammatory problems may mimic appendicitis. Therefore, it is common to observe patients with suspected appendicitis for a period of time to see if the problem will resolve on its own or develop characteristics that more strongly suggest appendicitis or, perhaps, another condition. </span></div><div align="center"><br /><a name="toch"><strong><span style="color:#000000;">What other conditions can mimic appendicitis?</span></strong></a><span style="color:#000000;"> </span></div><div><span style="color:#000000;">The surgeon faced with a patient suspected of having appendicitis always must consider and look for other conditions that can mimic appendicitis. Among the conditions that mimic appendicitis are: Meckel's diverticulitis. A Meckel's diverticulum is a small outpouching of the small intestine which usually is located in the right lower abdomen near the appendix. The diverticulum may become inflamed or even perforate (break open or rupture). If inflamed and/or perforated, it usually is removed surgically. </span></div><div><br /><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4823"><span style="color:#000000;">Pelvic inflammatory disease.</span></a><span style="color:#000000;"> The right </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=3375"><span style="color:#000000;">fallopian tube</span></a><span style="color:#000000;"> and </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4705"><span style="color:#000000;">ovary</span></a><span style="color:#000000;"> lie near the appendix. Sexually active women may contract infectious diseases that involve the tube and ovary. Usually, antibiotic therapy is sufficient treatment, and surgical removal of the tube and ovary are not necessary. Inflammatory diseases of the right upper abdomen. Fluids from the right upper abdomen may drain into the lower abdomen where they stimulate inflammation and mimic appendicitis. Such fluids may come from a perforated </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=3131"><span style="color:#000000;">duodenal ulcer,</span></a><span style="color:#000000;"> </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=368"><span style="color:#000000;">gallbladder disease,</span></a><span style="color:#000000;"> or inflammatory diseases of the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4179"><span style="color:#000000;">liver</span></a><span style="color:#000000;">, e.g., a liver abscess. </span></div><div><br /><span style="color:#000000;">Right-sided diverticulitis. Although most diverticuli are located on the left side of the colon, they occasionally occur on the right side. When a right-sided diverticulum ruptures it can provoke inflammation they mimics appendicitis. </span></div><div><br /><span style="color:#000000;">Kidney diseases. The right kidney is close enough to the appendix that inflammatory problems in the kidney-for example, an abscess-can mimic appendicitis.<br /></span><a name="toci"><span style="color:#000000;">How is appendicitis treated?</span></a><span style="color:#000000;"> Once a diagnosis of appendicitis is made, an appendectomy usually is performed. Antibiotics almost always are begun prior to surgery and as soon as appendicitis is suspected. </span></div><div><br /><span style="color:#000000;">There is a small group of patients in whom the inflammation and infection of appendicitis remain mild and localized to a small area. The body is able not only to contain the inflammation and infection but to resolve it as well. These patients usually are not very ill and improve during several days of observation. This type of appendicitis is referred to as "confined appendicitis" and may be treated with antibiotics alone. The appendix may or may not be removed at a later time. </span></div><div><br /><span style="color:#000000;">On occasion, a person may not see their doctor until appendicitis with rupture has been present for many days or even weeks. In this situation, an abscess usually has formed, and the appendiceal perforation may have closed over. If the abscess is small, it initially can be treated with antibiotics; however, the abscess usually requires drainage. A drain (a small plastic or rubber tube) usually is inserted through the skin and into the abscess with the aid of an ultrasound or CT scan that can determine the exact location of the abscess. The drain allows pus to flow from the abscess out of the body. The appendix may be removed several weeks or months after the abscess has resolved. This is called an interval appendectomy and is done to prevent a second attack of appendicitis. </span></div><div align="center"><br /><a name="tocj"><strong><span style="color:#000000;">How is an appendectomy done?</span></strong></a><span style="color:#000000;"> </span></div><div><span style="color:#000000;">During an appendectomy, an incision two to three inches in length is made through the skin and the layers of the abdominal wall over the area of the appendix. The surgeon enters the abdomen and looks for the appendix which usually is in the right lower abdomen. After examining the area around the appendix to be certain that no additional problem is present, the appendix is removed. This is done by freeing the appendix from its mesenteric attachment to the abdomen and colon, cutting the appendix from the colon, and sewing over the hole in the colon. If an abscess is present, the pus can be drained with drains that pass from the abscess and out through the skin. The abdominal incision then is closed. </span></div><div><br /><span style="color:#000000;">Newer techniques for removing the appendix involve the use of the </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=9931"><span style="color:#000000;">laparoscope</span></a><span style="color:#000000;">. The laparoscope is a thin telescope attached to a video camera that allows the surgeon to inspect the inside of the abdomen through a small puncture wound (instead of a larger incision). If appendicitis is found, the appendix can be removed with special instruments that can be passed into the abdomen, just like the laparoscope, through small puncture wounds. The benefits of the laparoscopic technique include less post-operative pain (since much of the post-surgery pain comes from incisions) and a speedier return to normal activities. An additional advantage of laparoscopy is that it allows the surgeon to look inside the abdomen to make a clear diagnosis in cases in which the diagnosis of appendicitis is in doubt. For example, laparoscopy is especially helpful in menstruating women in whom a rupture of an </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=7738"><span style="color:#000000;">ovarian cysts</span></a><span style="color:#000000;"> may mimic appendicitis.<br />If the appendix is not ruptured (perforated) at the time of surgery, the patient generally is sent home from the hospital after surgery in one or two days. Patients whose appendix has perforated are sicker than patients without perforation, and their hospital stay often is prolonged (four to seven days), particularly if peritonitis has occurred. </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=4021"><span style="color:#000000;">Intravenous</span></a><span style="color:#000000;"> antibiotics are given in the hospital to fight infection and assist in resolving any abscess. </span></div><div><br /><span style="color:#000000;">Occasionally, the surgeon may find a normal-appearing appendix and no other cause for the patient's problem. In this situation, the surgeon may remove the appendix. The reasoning in these cases is that it is better to remove a normal-appearing appendix than to miss and not treat appropriately an early or mild case of appendicitis. </span></div><div align="center"><br /><a name="tock"><strong><span style="color:#000000;">What are the complications of appendectomy?</span></strong></a><span style="color:#000000;"> </span></div><div><span style="color:#000000;">The most common complication of appendectomy is infection of the wound, that is, of the surgical incision. Such infections vary in severity from mild, with only redness and perhaps some tenderness over the incision, to moderate, requiring only antibiotics, to severe, requiring antibiotics and surgical treatment. Occasionally, the inflammation and infection of appendicitis are so severe that the surgeon will not close the incision at the end of the surgery because of concern that the wound is already infected. Instead, the surgical closing is postponed for several days to allow the infection to subside with antibiotic therapy and make it less likely for infection to occur within the incision. Wound infections are less common with laparoscopic surgery.<br />Another complication of appendectomy is an abscess, a collection of pus in the area of the appendix. Although abscesses can be drained of their pus surgically, there are also non-surgical techniques, as previously discussed. </span></div><div align="center"><br /><a name="tocl"><strong><span style="color:#000000;">Are there long-term consequences of appendectomy?</span></strong></a><span style="color:#000000;"> </span></div><div align="left"><span style="color:#000000;">It is not clear if the appendix has an important role in the body in older children and adults. There are no major, long-term health problems resulting from removing the appendix although a slight increase in some diseases has been noted, for example, Crohn's disease.<br /></span><a name="new"></a><br /><span style="color:#000000;">Recently it has been hypothesized that some episodes of appendicitis-like symptoms, especially recurrent symptoms, may be due to an increased sensitivity of the intestine and appendix from a prior episode of inflammation. That is, the recurrent symptoms are not due to recurrent episodes of inflammation. Rather, prior inflammation has made the nerves of the intestines and appendix or the central nervous system that innervate them more sensitive to normal stimuli, that is, with stimuli other than inflammation. This will be a difficult, if not impossible, hypothesis to confirm. </span></div><div align="left"><br /><a name="glance"><strong><span style="color:#000000;">Appendectomy At A Glance</span></strong></a><span style="color:#000000;"><strong><br /></strong>The appendix is a small, worm-like appendage attached to the colon.<br />Appendicitis occurs when bacteria invade and infect the wall of the appendix.<br />The most common complications of appendicitis are abscess and peritonitis.<br />The most common manifestations of appendicitis are pain, </span><a href="http://www.medicinenet.com/script/main/art.asp?articlekey=3425"><span style="color:#000000;">fever</span></a><span style="color:#000000;">, and abdominal tenderness.<br />Appendicitis usually is suspected on the basis of a patient's history and physical examination; however, a white blood cell count, urinalysis, abdominal x-ray, barium enema, ultrasonography, CT, and laparoscopy also may be helpful in diagnosis.<br />Due to the varying size and location of the appendix and the proximity of other organs to the appendix, it may be difficult to differentiate appendicitis from other abdominal and pelvic diseases.<br />The treatment for appendicitis usually is antibiotics and appendectomy (surgery to remove the appendix).<br />Complications of appendectomy include wound infection and abscess.</span></div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-10753687498373453602008-12-14T19:43:00.000-08:002009-07-03T21:35:43.642-07:00Tetralogy of Fallot<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi3fSttjPUEmFaqUKvY0v5tNugMCmuNxnsHcD141fbc54gA32eGhkteG06ubRco_gLaVVUIQIsE02cs4zNgE4uazcpqsmkLLHBl6cyhlSyq-CwPtwo8kqOHGVwFBSHQPBKBCSLaeVco9AKm/s1600-h/13020-inter-full.jpg"><span style="color:#000000;"><img id="BLOGGER_PHOTO_ID_5279857879490943282" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 259px; CURSOR: hand; HEIGHT: 400px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi3fSttjPUEmFaqUKvY0v5tNugMCmuNxnsHcD141fbc54gA32eGhkteG06ubRco_gLaVVUIQIsE02cs4zNgE4uazcpqsmkLLHBl6cyhlSyq-CwPtwo8kqOHGVwFBSHQPBKBCSLaeVco9AKm/s400/13020-inter-full.jpg" border="0" /></span></a><span style="color:#000000;"><br /></span><div align="center"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi3fSttjPUEmFaqUKvY0v5tNugMCmuNxnsHcD141fbc54gA32eGhkteG06ubRco_gLaVVUIQIsE02cs4zNgE4uazcpqsmkLLHBl6cyhlSyq-CwPtwo8kqOHGVwFBSHQPBKBCSLaeVco9AKm/s1600-h/13020-inter-full.jpg"><span style="color:#000000;">What It Is </span></a></div><div align="center"><span style="color:#000000;">Tetralogy of Fallot has four key features. A ventricular septal defect (a hole between the ventricles) and many levels of obstruction from the right ventricle to the lungs (pulmonary stenosis) are the most important. Also, the aorta (major artery from the heart to the body) lies directly over the ventricular septal defect, and the right ventricle develops thickened muscle. Because the aorta overrides the ventricular defect and there's pulmonary stenosis, blood from both ventricles (oxygen-rich and oxygen-poor) is pumped into the body. Sometimes the pulmonary valve is completely obstructed (pulmonary atresia). Infants and young children with unrepaired tetralogy of Fallot are often blue (cyanotic). The reason is that some oxygen-poor blood is pumped to the body. Surgical TreatmentTetralogy of Fallot is treated surgically. A temporary operation may be done at first if the baby is small. Complete repair comes later. Sometimes, the first operation is a complete intracardiac repair. Temporary OperationIn small and very blue infants, a shunt operation may be done first to provide adequate blood flow to the lungs. This lets the baby grow big enough to have a full repair. The shunt is built between the aorta and the pulmonary artery. The shunt is removed when a complete intracardiac repair is done later.</span></div><div align="center"><span style="color:#000000;"><br /></span></div><div align="center"><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEh7QmDNrlsAEWuqKBVUw2AFO5hXnQU3Ajpx1lLvhaYXqoGviKlmYtIx6GDL-_pLjh3h0tTzGthkqHoNRCclh3UyN11a1c1UvSC8EeiRSVyWLY61MjVND0qLF648uzieHLaOwkgpcj8loB5F/s1600-h/13032-inter-full.jpg"><span style="color:#000000;"><img id="BLOGGER_PHOTO_ID_5279857878764797970" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 275px; CURSOR: hand; HEIGHT: 277px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEh7QmDNrlsAEWuqKBVUw2AFO5hXnQU3Ajpx1lLvhaYXqoGviKlmYtIx6GDL-_pLjh3h0tTzGthkqHoNRCclh3UyN11a1c1UvSC8EeiRSVyWLY61MjVND0qLF648uzieHLaOwkgpcj8loB5F/s400/13032-inter-full.jpg" border="0" /></span></a><span style="color:#000000;"> Complete Repair</span></div><div align="center"><span style="color:#000000;">Complete repair tends to be done early in life. Once it was more common to do a temporary operation first and a complete repair later in childhood. To do a complete repair, the surgeon closes the ventricular septal defect with a patch and opens the right ventricular outflow tract by removing some thickened muscle below the pulmonary valve, repairing or removing the pulmonary valve and enlarging the peripheral pulmonary arteries that go to both lungs. Sometimes a tube is placed between the right ventricle and the pulmonary artery. This is sometimes called a Rastelli repair.<br /><br /></span><a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi_9m0tYBjXT6CZFs43RPgEr6SqMdDMzBX6MW7oTsZDZioFkfQRzTD7-BAtpX5XMEw1GucN6Yk6RDpoYv8_M6UiBNYGPsyVZopMPyOh1wZe57Q-TuKiT6kqcvmc4WqvBViD5lyBY6n4Mnx8/s1600-h/13034-inter-full.jpg"><span style="color:#000000;"><img id="BLOGGER_PHOTO_ID_5279857874987781122" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 287px; CURSOR: hand; HEIGHT: 255px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEi_9m0tYBjXT6CZFs43RPgEr6SqMdDMzBX6MW7oTsZDZioFkfQRzTD7-BAtpX5XMEw1GucN6Yk6RDpoYv8_M6UiBNYGPsyVZopMPyOh1wZe57Q-TuKiT6kqcvmc4WqvBViD5lyBY6n4Mnx8/s400/13034-inter-full.jpg" border="0" /></span></a><span style="color:#000000;"> </span></div><div align="center"><span style="color:#000000;">(</span><a href="http://www.americanheart.org/"><span style="color:#000000;">www.americanheart.org</span></a><span style="color:#000000;">)</span></div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-54237147209418740582008-12-12T21:38:00.000-08:002008-12-12T21:43:27.961-08:00Cerebellar Pilocytic Astrocytoma<strong>What is a cerebellar pilocytic astrocytoma?<br /></strong><em>(grade I pilocytic astrocytoma of the cerebellum)<br />(grade II fibrillary astrocytoma of the cerebellum)<br /></em><br />A cerebellar low-grade glioma is a tumor arising from a type of cell of the central nervous system known as a glial cell. These tumors originate from a specific type of glial cell known as an astrocyte. Astrocytes make up the supportive network of the brain. These cells are named for their star-like appearance.<br /><br />As you read further below, you will find general information about cerebellar low-grade glioma. If you would like to view summary information about brain tumors first, see the overview on brain tumors.<br /><br />Cerebellar low-grade gliomas are astrocytomas that arise in the location of the brain known as the cerebellum. The cerebellum is the center of the brain that controls balance and coordination. Of patients with cerebellar low-grade gliomas, 80-85 percent have what is called grade I pilocytic astrocytomas, and the remaining 15-20 percent have what is called grade II fibrillary astrocytomas. These cerebellar astrocytomas account for 10-20 percent of all childhood brain tumors. They tend to occur before the age of 10 years, most commonly between the ages of 6 and 9.<br /><br /><strong>What causes cerebellar low-grade gliomas? </strong><br />Children with certain genetic syndromes, including neurofibromatosis type I and tuberous sclerosis, are at higher risk of developing tumors of glial origin, including cerebellar low-grade gliomas. The vast majority of children with cerebellar low-grade gliomas however, develop these tumors spontaneously, meaning there is no identifiable cause.<br /><br /><strong>What are the symptoms of a cerebellar low-grade glioma?</strong><br />Due to the relative slow growth rate of cerebellar low-grade gliomas, children with these tumors tend to present to the doctor with symptoms that have been occurring for many months. Some children, however, have a more sudden onset of symptoms. The following are the most common symptoms of a cerebellar low-grade glioma, however, each child may experience symptoms differently. Common symptoms may include:<br /><br />Oh more that 90 percent of patients present with symptoms of increased pressure within the brain. These symptoms include:<br />*headache (generally upon awakening in the morning)<br />*vomiting<br />*fatigue<br />*Oh the majority of children have evidence of difficulty with balance and coordination<br /><br />The symptoms of a brain tumor may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.<br /><br /><strong>How is a cerebellar low-grade glioma diagnosed?</strong><br />Diagnostic procedures for a cerebellar low-grade glioma may include:<br />*physical examination - the child may demonstrate difficulty walking and coordinating movements of the hands and/or legs<br />*computerized tomography scan (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays. For cerebellar low-grade gliomas, a CT scan of the brain is usually done.<br />*magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. For cerebellar low-grade gliomas, an MRI of the brain is usually done. In rare cases when cerebellar low-grade gliomas spread to the spine, an MRI of the spine may also be ordered.<br />*biopsy - in many cases, a tissue sample from the tumor will be taken through a needle during a simple surgical procedure performed by a surgeon to confirm the diagnosis<br /><br /><strong>What are the treatments for a cerebellar low-grade glioma?</strong><br />Specific treatment for a cerebellar low-grade glioma will be determined by your child's physician based on:<br />*your child's age, overall health, and medical history<br />*type, location, and size of the tumor<br />*extent of the disease<br />*your child's tolerance for specific medications, procedures, or therapies<br />*how your child's doctors expects the disease to progress<br />*your opinion or preference<br /><br />Treatment may include (alone or in combination):<br />*surgery - the initial treatment is surgery, and complete tumor removal is associated with a very high rate of cure. In cases when the tumor recurs after initial surgical removal, or if the tumor re-grows after partial surgical removal, the recommended treatment would be a second attempt at surgical removal/de-bulking of the tumor.<br />*radiation and chemotherapy - in cases of residual/recurrent disease, where maximal surgical removal has been achieved, alternative treatment options include chemotherapy and radiation therapy:<br />*chemotherapy - a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.A number of combinations of chemotherapy drugs are being tested to treat cerebellar low-grade gliomas, including vincristine with carboplatin, and vincristine with CCNU, procarbazine and thioguanine. While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible. Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells.<br /><br />Chemotherapy can be given:<br />*as a pill to swallow<br />*as an injection into the muscle or fat tissue<br />*intravenously (directly to the bloodstream; also called IV)<br />*intrathecally - chemotherapy given directly into the spinal column with a needle<br />*radiation therapy - using high-energy rays (radiation) from a specialized machine to damage or *kill cancer cells and shrink tumors. Radiation therapy to the tumor bed is also being used for recurrent disease.<br /><br />The effectiveness of these methods of treatment is still being studied. There is some evidence to suggest that the use of chemotherapy and/or radiation therapy may increase long-term survival in children with incompletely removal tumors.<br /><br /><strong>How are side effects of treatment managed?</strong><br />Children with cerebellar low-grade gliomas may have side effects related to the tumor itself and its treatment. Symptoms at the time of diagnosis related to increased pressure within the brain, such as headache, vomiting and lethargy, are often relieved by surgical removal of the tumor. Effects on coordination and balance are also often improved with surgical removal of the tumor that is compressing structures that control these functions. Dexamethasone, an oral steroid, is often used, especially in the post-operative period, to assist in controlling these systems as well as any additional tissue swelling that may occur post-operatively.<br /><br />Common side effects related to chemotherapy include nausea, vomiting and decreased blood counts (i.e. anemia). Anti-emetics (anti-nausea) medications are administered with the chemotherapy to control symptoms of nausea and vomiting. Occasionally, children receiving chemotherapy will require transfusion of red blood cells and/or platelets to replace these cells, since chemotherapy temporarily decreases the body's ability to produce red blood cells and platelets. White blood cells are reduced with chemotherapy, however these cells are not transfused. Occasionally, children will receive a medication to assist the body in producing white blood cells. Radiation therapy may cause swelling related to tissue inflammation. This inflammation may lead to symptoms of headache or difficulty with coordination. These symptoms, if significant, may be treated with the oral medication dexamethasone.<br /><br /><strong>What is the expected outcome after treatment for cerebellar low-grade glioma?</strong><br />Grade I cerebellar gliomas are associated with a 10-year survival rate of 70-100 percent after surgical removal alone. Grade II cerebellar gliomas are more likely to recur after surgical removal. Recurrent disease may necessitate the use of chemotherapy and/or radiation therapy. Inability to achieve a complete surgical removal and the presence of recurrent disease decreases prognosis and long-term survival.<br /><br /><strong>What about progressive or recurrent disease?</strong><br />The recommended treatment for progressive or recurrent cerebellar low-grade glioma is reattempt at surgical removal. In cases of progressive/recurrent disease, where maximal surgical removal has been achieved, chemotherapy and/or radiation therapy will be recommended. The Dana-Farber Cancer Institute is one of nine institutes in the nation belonging to the Pediatric Brain Tumor Consortium. The consortium is dedicated to the development of new and innovative treatments for children with progressive/recurrent brain tumors not responsive to standard therapies. Children with progressive/recurrent low-grade glioma of the cerebellum would be eligible for a number of experimental therapies available through the consortium.<br /><br /><span style="color:#000000;">(</span><a href="http://www.childrenshospital.org/"><span style="color:#000000;">http://www.childrenshospital.org/</span></a><span style="color:#000000;">)</span>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-54431893031395150102008-12-12T21:31:00.000-08:002008-12-12T21:33:33.831-08:00Respiratory Acidosis<strong>Definition</strong><br />Respiratory acidosis is a condition in which a build-up of carbon dioxide in the blood produces a shift in the body's pH balance and causes the body's system to become more acidic. This condition is brought about by a problem either involving the lungs and respiratory system or signals from the brain that control breathing.<br /><br /><strong>Description</strong><br />Respiratory acidosis is an acid imbalance in the body caused by a problem related to breathing. In the lungs, oxygen from inhaled air is exchanged for carbon dioxide from the blood. This process takes place between the alveoli (tiny air pockets in the lungs) and the blood vessels that connect to them. When this exchange of oxygen for carbon dioxide is impaired, the excess carbon dioxide forms an acid in the blood. The condition can be acute with a sudden onset, or it can develop gradually as lung function deteriorates.<br /><br /><strong>Causes and symptoms<br /></strong>Respiratory acidosis can be caused by diseases or conditions that affect the lungs themselves, such as emphysema, chronic bronchitis, asthma, or severe pneumonia. Blockage of the airway due to swelling, a foreign object, or vomit can induce respiratory acidosis. Drugs like anesthetics, sedatives, and narcotics can interfere with breathing by depressing the respiratory center in the brain. Head injuries or brain tumors can also interfere with signals sent by the brain to the lungs. Such neuromuscular diseases as Guillain-Barré syndrome or myasthenia gravis can impair the muscles around the lungs making it more difficult to breathe. Conditions that cause chronic metabolic alkalosis can also trigger respiratory acidosis.<br />The most notable symptom will be slowed or difficult breathing. Headache, drowsiness, restlessness, tremor, and confusion may also occur. A rapid heart rate, changes in blood pressure, and swelling of blood vessels in the eyes may be noted upon examination. This condition can trigger the body to respond with symptoms of metabolic alkalosis, which may include cyanosis, a bluish or purplish discoloration of the skin due to inadequate oxygen intake. Severe cases of respiratory acidosis can lead to coma and death.<br /><br /><strong>Diagnosis<br /></strong>Respiratory acidosis may be suspected based on symptoms. A blood sample to test for pH and arterial blood gases can be used to confirm the diagnosis. In this type of acidosis, the pH will be below 7.35. The pressure of carbon dioxide in the blood will be high, usually over 45 mmHg.<br /><br /><strong>Treatment</strong><br />Treatment focuses on correcting the underlying condition that caused the acidosis. In patients with chronic lung diseases, this may include use of a bronchodilator or steroid drugs. Supplemental oxygen supplied through a mask or small tubes inserted into the nostrils may be used in some conditions, however, an oversupply of oxygen in patients with lung disease can make the acidosis worse. Antibiotics may be used to treat infections. If the acidosis is related to an overdose of narcotics, or a drug overdose is suspected, the patient may be given a dose of naloxone, a drug that will block the respiratory-depressing effects of narcotics. Use of mechanical ventilation like a respirator may be necessary. If the respiratory acidosis has triggered the body to compensate by developing metabolic alkalosis, symptoms of that condition may need to be treated as well.<br /><br /><strong>Prognosis<br /></strong>If the underlying condition that caused the respiratory acidosis is treated and corrected, there may be no long term effects. Respiratory acidosis may occur chronically along with the development of lung disease or respiratory failure. In these severe conditions, the patient may require the assistance of a respirator or ventilator. In extreme cases, the patient may experience coma and death.<br /><br /><strong>Prevention</strong><br />Patients with chronic lung diseases and those who receive sedatives and narcotics need to be monitored closely for development of respiratory acidosis.<br /><br />(www.healthatoz.com)LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-61627007046419165182008-12-09T02:41:00.000-08:002008-12-09T02:51:49.350-08:00Childhood Asthma<strong>Definition<br /></strong>Childhood asthma has become more widespread in recent decades. As the most common chronic illness in children, childhood asthma causes more missed school and places more limits on activity than does any other disease in the United States. Childhood asthma and adult asthma have the same underlying cause — inflammation of the airways. This inflammation makes the airways overly sensitive, leading to signs and symptoms that range from minor coughing or wheezing to serious flare-ups that interfere with breathing.<br />Fortunately, childhood asthma is treatable. Although childhood asthma can't be cured, you and your child can keep symptoms under control with a written plan, monitoring, regular doctor visits and making treatment changes as needed.<br /><p><br /><strong>Symptoms</strong><br />Childhood asthma can be very disruptive, causing bothersome daily symptoms that interfere with play, sports, school and sleep. In some children, unmanaged asthma can cause serious or even life-threatening asthma attacks. <br />Common childhood asthma symptoms include:<br />*Coughing<br />*A whistling or wheezing sound when exhaling<br />*Shortness of breath<br />*Chest congestion or tightness<br /></p><p>Other signs and symptoms of asthma in children include:<br />*Trouble sleeping caused by shortness of breath, coughing or wheezing<br />*Bouts of coughing or wheezing that get worse with a respiratory infection such as a cold or the flu<br />*Delayed recovery or bronchitis after a respiratory infection<br />*Fatigue or trouble breathing during active play or exercise — signs of exercise-induced asthma<br /></p><p>Asthma signs and symptoms vary from child to child, and may get worse or better over time. While wheezing is most commonly associated with asthma, not all children with asthma wheeze. Your child may have only one sign or symptom, such as a lingering cough or chest congestion.<br />Sometimes it's difficult to tell whether your child's symptoms are caused by asthma. Wheezing episodes and other asthma-like symptoms may be caused by infectious bronchitis or other respiratory problem. </p><p>When to see a doctor Take your child to see the doctor as soon as possible if you suspect he or she may have asthma. Early treatment will not only help control disruptive asthma flare-ups, it may also improve breathing every day.<br /></p><p>Make an appointment if you notice:<br />*Coughing that's constant, intermittent or associated with physical activity<br />*Wheezing or whistling sounds when your child exhales<br />*Shortness of breath or rapid breathing that may or may not be associated with exercise<br />*Complaints of chest tightness<br />*Repeated episodes of suspected bronchitis or pneumonia<br /></p><p>Pay attention to cues from a child who says, "My chest feels funny" or "I'm always coughing." Asthma can be worse at night, so listen for coughing during sleep or coughing that wakes your child in the night. Crying, laughing, yelling, or strong emotional reactions and stress also may trigger coughing or wheezing. If your child is diagnosed with asthma, creating an asthma action plan can help you monitor symptoms and be ready if an asthma attack does occur.<br />When to seek emergency treatment Even if your child hasn't been diagnosed with asthma, seek medical attention immediately if he or she has any trouble breathing. Although episodes of asthma vary in severity, asthma attacks can start with coughing, which progresses to wheezing and rapid breathing. </p><p>In severe cases, you may see your child's chest and sides pulling inward as he or she struggles to breathe. Your child may have an increased heartbeat, sweating and chest pain. Seek emergency care if your child is:<br />*Breathing so hard that he or she has to stop in midsentence to catch his or her breath<br />*Using abdominal muscles to breathe<br />*Widening the nostrils when breathing in<br />*Trying so hard to breathe that the abdomen is sucked under the ribs when he or she breathes in<br /></p><p><strong>Causes</strong><br />In children with asthma, an overly sensitive immune system makes airways become inflamed and swollen when exposed to triggers such as smoke or allergens. Sometimes, asthma symptoms occur with no apparent triggers. When asthma flares up, airway muscles constrict, the lining of the airways swell, and thick mucus fills the bronchial tubes, leading to asthma symptoms.<br /></p><p>Asthma triggers differ from child to child and include:<br />*Viral infections such as the common cold<br />*Allergens such as dust mites, pet dander, pollen or mold<br />*Tobacco smoke or other environmental pollutants<br />*Exercise<br />*Weather changes or cold air<br />*Conditions linked to asthma include:<br />*A chronic runny or stuffy nose (rhinitis)<br />*Inflamed sinuses (sinusitis)<br />*Heartburn (gastroesophageal reflux disease)<br /></p><p><strong>Risk factors<br /></strong>It isn't clear why some children get asthma and others don't, but it's probably due to a combination of genetic (inherited) and environmental factors. Children with a family history of asthma are at greater risk of developing the disease. Other environmental factors that may increase your child's chances of developing asthma include:<br />*Exposure to tobacco smoke<br />*Previous allergic reactions, including skin reactions, food allergies or allergic rhinitis (hay fever)<br />*Living in a large urban area with increased exposure to air pollution<br />*A family history of asthma, allergic rhinitis, hives or eczema<br />*Low birth weight<br />*Obesity<br />*Complications<br /></p><p>Asthma may cause a number of complications, including:<br />*Severe asthma attacks that require emergency room visits or even hospitalization<br />*Permanent narrowing of the bronchial tubes (airway remodeling)<br />*Side effects from long-term use of some medications used to stabilize severe asthma (oral corticosteroids)<br />*Slightly slowed growth in children caused by long-term use of inhaled corticosteroids<br /></p><p><strong>Preparing For Your Appointment</strong><br />A big part of diagnosing childhood asthma depends on accurately reporting your child's symptoms. Be prepared to talk to the doctor about:<br />Exactly what symptoms your child has<br />Whether symptoms occur at certain times of day<br />Whether symptoms get worse or improve at certain times of the year<br />What, if any, triggers seem to set off asthma flare-ups<br />Whether your child has allergies, and whether he or she has a family history of allergies or asthma<br /></p><p><strong>Tests and Diagnosis<br /></strong>Asthma is a very individual condition. Your child's doctor will consider the nature and frequency of symptoms along with results from tests to rule out other diseases before diagnosing asthma.<br />First, the doctor will ask for a detailed description of your child's symptoms and ask about your family history of asthma or allergic diseases such as eczema, hives or allergic rhinitis (hay fever).<br />In children 6 years of age and older, doctors diagnose asthma with the same tests used to identify the disease in adults. Lung function tests (spirometry) measure how quickly and how much air your child can exhale. Your child may have pulmonary function tests at rest, after exercising and after taking asthma medication. Allergy tests also may be part of the evaluation.<br />In younger children, diagnosis can be difficult because lung function tests aren't accurate before 6 years of age. Some children also simply outgrow asthma-like symptoms over time. Your doctor will rely on detailed information about symptom type and frequency when considering an asthma diagnosis in a young child. Sometimes a diagnosis is not made until later, after months or years of observing symptoms. </p><p><br />If you suspect your child has asthma, it's important to start the testing process early. Early diagnosis and proper treatment can prevent disruptions from daily activities such as sleep, play, sports and school. It may also prevent dangerous or even life-threatening asthma attacks.<br />For children younger than age 3 who have symptoms of asthma, many times doctors will use a "wait-and-see" approach. This is because the long-term effects of asthma medication on infants and young children aren't clear. If an infant or toddler has frequent or severe wheezing episodes, a course of medication may be prescribed to see if the wheezing improves symptoms.<br /></p><p><strong>Treatments and Drugs</strong><br />The goal of asthma treatment is to get the asthma under control.<br />Well-controlled asthma means that your child has:<br />*Minimal or no symptoms<br />*Few or no asthma flare-ups<br />*No limitations on physical activities or exercise<br />*Minimal use of fast-acting "rescue" inhalers<br />*Few or no side effects from medications<br /></p><p>Treating asthma involves both preventing asthma symptoms and treating an asthma attack in progress. Preventive medications reduce the inflammation in your child's airways that can lead to symptoms. Quick-relief (rescue) medications quickly open airways that are swollen and limiting breathing. </p><p>While quick-relief medications work quickly, they can't keep your child's symptoms from coming back. If your child has frequent or severe symptoms, he or she will need to take a long-term control medication as well. Your child's symptoms and triggers are likely to change over time. You and your child will need to carefully monitor asthma symptoms and work with your doctor to adjust medications as needed. </p><p>Long-term control medications These preventive anti-inflammatory medications are generally taken every day on a long-term basis to control persistent asthma. In some cases, these medications are taken seasonally if asthma symptoms get worse at certain times of the year. These medications include:<br />*Inhaled corticosteroids, used to treat persistent asthma. These medications reduce chronic inflammation in the airways and reduce the need for other medications. Examples include fluticasone (Flovent), budesonide (Pulmicort), mometasone (Asmanex), triamcinolone (Azmacort), flunisolide (Aerobid) and beclomethasone (Qvar). Continuous inhaled corticosteroids have been associated with slightly slowed growth in children, but research has shown that the effect is minor. In most cases, the benefits of good asthma control outweigh the risks of possible side effects.<br />*Combination inhalers, which contain inhaled corticosteroids plus a long-acting bronchodilator. Advair combines the corticosteroid fluticasone and the bronchodilator salmeterol. Symbicort contains the corticosteroid budesonide plus the bronchodilator formoterol.<br />Leukotriene modifiers, which include montelukast (Singulair) and zafirlukast (Accolate) and zileuton (Zyflo).<br />*Cromolyn, which may help prevent mild to moderate asthma attacks. Cromolyn needs to be taken two to four times a day and is usually taken along with an inhaled corticosteroid.<br />Theophylline, a daily pill that opens the airways (bronchodilator). It relaxes the muscles around the airways to make breathing easier.<br />*Quick-relief (rescue) medications These medications — called short-acting bronchodilators — provide immediate relief of asthma attack signs and symptoms such as coughing, wheezing, chest tightness or shortness of breath. These inhaled medications are delivered using a small, hand-held device called a metered dose inhaler. Albuterol is the most commonly used short-acting bronchodilator. Others include pirbuterol and levalbuterol. These bronchodilators begin working within minutes and last four to six hours.<br />*Allergy-desensitization shots (immunotherapy) Immunotherapy may help if your child has allergic asthma that can't be controlled by avoiding triggers. With immunotherapy, your child will probably need injections once a week for a few months, then once a month for a period of three to five years. Your child's allergic reactions will gradually diminish, lessening the immune system response that triggers asthma symptoms.<br />*Inhaled medication devices Inhaled short- and long-term control medications are used by inhaling a measured dose of medication.<br /></p><p>Older children and teens may use a small, hand-held device called a pressurized metered dose inhaler or an inhaler that releases a fine powder.<br />Infants and toddlers need to use a face mask attached to a metered dose inhaler or a nebulizer to get the correct amount of medication.<br />Babies need to a use a device called a nebulizer, a machine that turns liquid medication into fine droplets. Your baby wears a face mask and breathes normally while the nebulizer delivers the correct dose of medication.<br />*HFA inhalers: A recent change The chlorofluorocarbon (CFC) propellant in quick-relief asthma inhalers has been replaced with a propellant called hydrofluoroalkane (HFA). Unlike CFC inhalers, HFA inhalers don't harm the environment. The spray from the new inhalers may taste different. Although the spray from an HFA inhaler may not seem as strong, your child is still getting the full dose of medication.<br /></p><p><strong>Lifestyle and Home Remedies<br /></strong>Taking steps to reduce your child's exposure to things that trigger asthma symptoms will help your child stay healthy and lessen the possibility of asthma attacks. Here are some things you can do:<br />*Use your air conditioner. Air conditioning helps reduce the amount of airborne pollen from trees, grasses and weeds that finds its way indoors. Air conditioning also lowers indoor humidity and can reduce your exposure to dust mites. If you don't have air conditioning, try to keep your windows closed during pollen season.<br />*Make your home more asthma friendly. Minimize dust that may aggravate nighttime symptoms by replacing certain items in your bedroom. For example, encase pillows, mattresses and box springs in dust-proof covers. Remove carpeting and install hardwood or linoleum flooring. Use washable curtains and blinds.<br />*Maintain low humidity. If you live in a damp climate, talk to your doctor about using a dehumidifier.<br />*Keep indoor air clean. Have a utility company check your air conditioner and furnace once a year. Change the filters in your furnace and air conditioner according to the manufacturer's instructions. Also consider installing a small-particle filter in your ventilation system.<br />*Reduce pet dander. If your child is allergic to dander, it's best to avoid pets with fur or feathers. *Regular bathing or grooming also may reduce the amount of dander in your surroundings.<br />*Clean regularly. Clean your home at least once a week.<br />*Reduce exposure to cold air. If your child's asthma is worsened by cold, dry air, a face mask can help.<br />*Help your child stay healthy Staying active and treating other conditions linked to asthma will help keep your child's asthma under control. </p><p>Make sure your child:<br />*Gets regular exercise. Asthma symptoms can interfere with active play or sports. But don't let asthma sideline your child. Exercise is proved to reduce asthma symptoms and is a critical part of your child's cardiovascular health. With asthma under control, there usually is no limit to your child's physical activity level.<br />*Maintains a healthy weight. Being overweight can worsen asthma symptoms, and it puts your child at higher risk of other health problems.<br />*Controls heartburn and gastroesophageal reflux disease (GERD). It's possible that the acid reflux that causes heartburn may damage lung airways and worsen asthma symptoms.<br /></p><p>Alternative medicine<br />*While some alternative remedies are used for asthma, in most cases more research is needed to see how well they work and to measure the extent of possible side effects. </p><p>(Information derived from: MayoClinic.com)</p>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-3733884953815230002008-12-06T20:49:00.001-08:002008-12-06T21:09:39.037-08:00Hypoplastic Left Heart Syndrome (HLHS)<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEj7hKTzP1N3exeFKH5qxOVgGTCTSjN1KLKIkRpak0bPpfH49pvrTlVVKkURB9RptlmNQ9L6dAPz63JQJYo5gVsuzaHJJdH6qz4znGAR5LfMwSdxjN_9XhdlHWCtWLb1cWlARfyCk0fr0K_L/s1600-h/Hypoplastic-Left-Heart.jpg"><img id="BLOGGER_PHOTO_ID_5276910780868422706" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 309px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEj7hKTzP1N3exeFKH5qxOVgGTCTSjN1KLKIkRpak0bPpfH49pvrTlVVKkURB9RptlmNQ9L6dAPz63JQJYo5gVsuzaHJJdH6qz4znGAR5LfMwSdxjN_9XhdlHWCtWLb1cWlARfyCk0fr0K_L/s400/Hypoplastic-Left-Heart.jpg" border="0" /></a><br /><div align="left"><strong><em>What is HLHS?</em></strong></div><br /><div align="left">Hypoplastic means ‘not developed’. Left Heart – the structures on the left side of the heart.Syndrome – a number of anomalies together. So HLHS means that the left side of the heart has not developed properly – the left ventricle, which should pump red (oxygenated) blood to the body, is very small, and there is a complete blockage between it and the aorta. The aorta, which should carry red blood to the body, is much too small.</div><br /><div align="left"></div><br /><div align="left"><strong><em>Diagnosis</em></strong></div><br /><div align="left">Your baby may have been diagnosed as having a heart condition before birth. HLHS can be seen on a scan of the foetal heart from about 16 weeks. HLHS can develop if there is a blocked or narrow valve, when the left side of the heart does not grow as it should during the later stages of pregnancy.</div><br /><div align="left"><br />After birth, your baby can survive before the foetal circulation system has closed down. This is because red blood will still be crossing from the left atrium to the right. From there it will be pumped, with blue blood, into the pulmonary artery. While the ductus arteriosus is still open, blood will flow through it into the aorta, and thus round the body.</div><br /><div align="left"><br />Often your baby will only be diagnosed because, as the foetal circulation closes down, less and less oxygen reached organs and he or she became extremely ill.</div><br /><p><br />When a heart problem is detected the tests used can be:</p><br /><ol><br /><li>pulse, blood pressure, temperature, and number of breaths a baby takes a minute </li><br /><li>listening with a stethoscope for changes in the heart sounds </li><br /><li>an oxygen saturation monitor to see how much oxygen is getting into the blood </li><br /><li>a chest x-ray to see the size and position of the heart </li><br /><li>an ECG (electrocardiogram) to check the electrical activity </li><br /><li>an ultrasound scan (echocardiogram) to see how the blood moves through the heart </li><br /><li>checks for chemical balance in blood and urine </li><br /><li>a catheter or Magnetic Resonance Imaging test may be needed. </li></ol><br /><p><strong><em>Treatment</em></strong></p><br /><p>This is a very difficult problem to treat: The treatment for HLHS is palliative. Your child’s heart cannot be corrected – that is, made to work like a normal heart – but in some cases it can be improved with a Norwood operation (palliated). The aim of the treatment is to use the pumping power of the right ventricle to get blood to the body and the lungs. This means that your child will have one working ventricle.<br /></p><br /><p>There is a risk to your child in all the procedures, but how great that risk is depends on the shape of the individual heart, and how well your child is otherwise. The doctors will discuss risks with you in detail before asking you to consent to any of the operations.<br /></p><br /><p>If you have had the condition diagnosed during pregnancy, there is time to think about the treatment options, but time is often very short to take decisions on behalf of a newborn.<br /></p><br /><p><strong>Norwood Procedure:</strong> This is a high risk procedure. It is a series of three surgical operations which would eventually allow the right side of your child’s heart to take over the work of the left. The aim of the three operations is for the right ventricle to pump red blood to the body, while the blue blood is allowed to flow directly to the lungs.<br /></p><br /><p><em>Stage One</em> – in the first few days of life, the wall between the left and right atrium is removed so that red blood coming back from the lungs will pass into the right atrium, and from there to the right ventricle. The pulmonary artery is attached to the aorta. A shunt (passage) is created between the aorta and the pulmonary artery branches to the lungs. Mixed red and blue blood will now be pumped through the pulmonary artery to the aorta, to both lungs and to the body.<br /></p><br /><p><em>Stage Two</em> – between the ages of four to nine months the blood flow to the lungs is increased. The SVC (superior vena cava), which carries blue blood from the top of the body to the right atrium, is joined directly to the pulmonary arteries. The shunt between the aorta and the branch arteries created at stage one is closed.<br /></p><br /><p><em>Stage Three</em> – the IVC (Inferior vena cava), which carries blue blood from the lower part of the body to the right atrium, is joined directly to the pulmonary arteries (Fontan operation).<br /></p><br /><p>Other forms of treatment:<br /></p><br /><p><strong>Transplantation:</strong> This is very rarely available in the UK. It will involve trying to keep your baby’s foetal circulation open, using medicines or a stent in the ductus arteriosus, while waiting for a heart to become available. Transplants into very young babies are often more successful than older children or adults, but a majority of babies die waiting for a heart to become available.Other forms of treatment are being used in some Paediatric Cardiology Units and these are developing all the time.<br /></p>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.comtag:blogger.com,1999:blog-3495323378908877416.post-78708845618892821062008-12-06T18:14:00.000-08:002008-12-06T18:15:16.678-08:00Hypoplastic Right Heart Syndrome<a href="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhb-zsDb8mcOokKlpYXQTFSt-thg6MYyYb1bzZBfYAMGpN2lQmevPAWBAW4fPCc_ZRxXtJ7wZ3K_Kd0Au9FoZjO5CSYCIQ6L8S-7RzDw7Dvxxlc_nu20-3XltibgWaIFth9340QPMzkmF5Y/s1600-h/hyporight.jpg"><img id="BLOGGER_PHOTO_ID_5276865913580531874" style="DISPLAY: block; MARGIN: 0px auto 10px; WIDTH: 400px; CURSOR: hand; HEIGHT: 336px; TEXT-ALIGN: center" alt="" src="https://blogger.googleusercontent.com/img/b/R29vZ2xl/AVvXsEhb-zsDb8mcOokKlpYXQTFSt-thg6MYyYb1bzZBfYAMGpN2lQmevPAWBAW4fPCc_ZRxXtJ7wZ3K_Kd0Au9FoZjO5CSYCIQ6L8S-7RzDw7Dvxxlc_nu20-3XltibgWaIFth9340QPMzkmF5Y/s400/hyporight.jpg" border="0" /></a><br /><div align="center"><br />Hypoplastic right heart syndrome (HRHS) refers to underdevelopment of the right sided structures of the heart. These defects cause inadequate blood flow to the lungs and thus, a blue or cyanotic infant. The major problem is pulmonary valve atresia (absence). This valve normally opens and closes to let blood flow to the pulmonary artery. Secondary problems include a very small (hypoplastic) right ventricle (lower chamber which normally pumps blood to the lungs); a small tricuspid valve (this valve allows blood to flow into the right ventricle) and a small (hypoplastic) pulmonary artery. Also, the blood flow into the coronary arteries may be abnormal causing damage to the heart muscle. </div><div align="center"><br />The infant is born with two connections that help blood flow. These are a foramen ovale (hole between the atria) and patent ductus arteriosus (or PDA, a blood vessel between the aorta and pulmonary artery). As these connections begin to close, the infant becomes critically ill.<br />Because the blue blood cannot pass through the right side of the heart to get to the lungs, it crosses into the left atrium and mixes with red blood returning from the lungs. This mixed blood is pumped out of the aorta. The only way in which blood gets to the lungs is through the PDA. The PDA must be maintained open with medicine (PGE1). Surgery is usually performed shortly after starting PGE1 to create an artificial connection (shunt) between the aorta and the pulmonary artery to deliver blood to the lungs. </div>LindseyBhttp://www.blogger.com/profile/09876077349247957261noreply@blogger.com