Uncommon Kawasaki Disease - Essay Example

Kawasaki Disease Kawasaki disease may actually be known as Kawasaki syndrome because its effect is not limited to one organ but it affects manyorgans of the body which include the skin, walls of the blood vessels and the heart. It is a uncommon disease and its occurrence is seen mostly in children and it is described as an acute febrile vasculitic syndrome. This disease is named after its discoverer that is Tomisaku Kawaski who was a Japanese pediatrician and he was the first one to explain this disease. The disease is characterized by high grade fever associated with conjunctivitis which is the reddening and inflammation of the whites of the eyes without pus. Swelling and reddening of the palms of hand and feet is also observed. Peeling of the skin starts about two weeks after the onset of the disease. Swollen lymph glands and rashes on the body are also observed. Lips become dry and red and they start to crack and bleed and a bumpy red tongue which is also known as strawberry tongue is seen. Gingivitis which is an ulcerative gum disease is also seen in certain cases and there is inflammation of the mucous membranes. The disease may also lead to meningitis which is the inflammation of the covering of the brain and spinal cord and it may also lead to pericarditis which is the inflammation of the lining of the heart. Joint pain and swelling is a sign of the disease and this may be due to the inflammation of the joints which is known as arthritis. The disease may also lead to inflammation of the vessels which may eventually lead to myocardial infarction due to the cardiac aneurysms. Irritability is alsonoted in the patients. In Kawasaki disease generalized vasculitis that is inflammation of all the vessels of the body is seen.the aneurysms are not only limitied to the coronary arteries but they may also affect other extraparenchymal muscular arteries such as celiac, mesenteric, femoral, renal, axillary and brachial arteries.there is edematous dissociation of the smooth muscle cells observed in the media of the affected vessels. Endothelial cell swelling and subendothelial edema are seen but the internal elastic lamina remains intact. An increased entry of neutrophills is found in the first 7 to 9 days and these rapidly change into large mononuclear cells together with the lymphocytes and IgA plasma cells. The internal elastic lamina becomes destroyed and eventually fibroblastic proliferation occurs. After this active inflammation over a period of months to week progressive fibrosis occurs alongwith scar formation. Coronary arteritis may also be seen in which there is intimal proliferation, neoangiogenesis, the intima is thickened and consists of linearly arranged micro vessels where there is normally presence of the smooth muscle cells and fibrous layers. In the lymph nodes thrombotic arteriolitis is seen and severe lymphadenitis with necrosis. In the first week the lymph nodes show abnormal hypesplasia of the endothelium of the postcapillary venule and hyperplasia of reticular cells around the postcapillary venule. There is no specific laboratory test for the diagnosis of Kawasaki disease. another problem faced is that immediately a doctor may not be able to know if the patient is suffering from Kawasaki disease or not. This is because the symptoms become very clear only after a week or so. To make sure that a child is suffering from Kawasaki's disease it has been suggested that if fever along with conjunctivitis, strawberry tongue, peeling of the skin of palms of feet and hands and cracked lips is seen, doctors should start off with the treatment needed for a patient suffering from Kawasaki disease. at the same time certain tests which assist in finding out about the disease may also be performed. These tests include the electrocardiography and the echocardiography. A low cardiac output syndrome and shock may be seen due to poor myocardial function and in the electrocardiography arrhythmia, prolonged PR interval or non specific ST and T wave changes are observed. A complete blood count (CBC) may be carried out which will show leukocytosis with increase in immature and mature granulocytes. Anemia with normal red blood cell indexes may also be seen and there might also be occurrence of thrombocytosis. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels are elevated. Liver function tests are also carried out which may show low albumin levels, elevations in serum transaminases, hyperbilirubinemia and plasma gamma glutamyl transpeptidases are also elevated. Lumbar punctures show aseptic meningitis. Elevation of serum cardiac troponin I is also observed in the disease which relates to the myocardial damage caused by the disease. For the treatment of Kawasaki disease high doses of aspirin that is salicylic acid are administered and also used for treatmet is gamma globulin which is administered intravenously with other fluids and is known as intravenous immunoglobulin (IVIG). The pain in the joints which is due to inflammation is treated with anti-inflammatory drugs such as ibuprofen or naproxen. Corticosteroids are also administered for the treatment of the disease if IVIG therapy fails and they are efficient in problems relating to vasculitis. Pentoxifylline is also used for the cure of the disease. it inhibits TNF- messenger RNA transcription and it has a role in the inflammatory effects of the disease pentoxyfylline prevents the inflammatory effects. Patients who do not respond to aspirin and gamma globulin are given an alternative treatment known as plasma exchange (plasmaphoresis). In this process the plasma of the patient is removed and replaced with protein containing fluids. The benefit of removing the plasma is that all the antibodies and proteins which are causing the inflammatory effects of the disease are removed. ulinastatin is also used for the curing of this ailment though its effectiveness is not proven but still it does have mediating effects. Ulinastatin is a human trypsin inhibitor purified from human urine. Abciximab is also used to treat patients especially suffering from coronary aneurysms as it works on the recovering of the vessels. Kawasaki disease if diagnosed in the initial stages that is within ten days may be cured immediately and there may not be any cardiac associations and death can also be avoided. Only few of the patients who are diagnosed and treated upon in the early stages might have long lasting coronary problems. But in later stages permanent effect upon the coronary vessels is seen and the risk of death is also increases. It is also seen in certain cases that there is a relapse of the disease but retreatment can lead to complete cure. High doses of aspirin can also be harmful because they may lead to Reye's syndrome or it may also lead to excessive bleeding. Bibliography Newburger, J. W., M. Takahashi, M. A. Gerber, M. H. Gewitz, L. Y. Tani, J. C. Burns, S. T. Shulman, A. F. Bolger, P. Ferrieri, and R. S. Baltimore. "Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Statement for Health Professionals From the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association." CIRCULATION. 110 (2004): 2747-2771. Inoue Y, T Kobayashi, and A Morikawa. "Treatment of Kawasaki Disease." The New England Journal of Medicine. 356. 26 (2007): 2746-7. Trager, J D K. "Kawasaki's Disease." The New England Journal of Medicine. 333. 21 (1995): 1391. Read More