Kawasaki hastalığı

Kawasaki hastalığı çocukluk döneminin etiyolojisi belli olmayan akut, febril bir vaskülitidir. Tanı klinik bulgulara dayanılarak konur. Koroner arterlerde anevrizma gelişimi ve tromboz hastalığın ciddi komplikasyonlarıdır. Tanı için gerekli kriterler şunlardır: En az beş gündür yüksek ateş, generalize polimorf eritematöz döküntü, konjunktivalarda enjeksiyon, ağız ve dudaklarda tipik değişklikler, el ve ayaklarda bilateral şişlik ve kızarıklık ve tek taraflı fluktuasyon vermeyen > 1.5 cm. servikal lenfadenomegali. Ateş dışındaki beş kriterden dördünün birlikte olmadığı hallerde inkomplet (atipik) Kawasaki tanısı söz konusu olabilir. Hastalık akut, subakut ve konvalesan dönemlerden oluşur. Akut faz reaktanlarında nonspesifik artış vardır. Etkilenen damar etrafında inflamatuar infiltrasyon (mononükleer hücreler, CD 8+ T lenfositler ve immunglobülin A plazma hücreleri) görülür. Tanı konur konmaz, ekokardiyografi yapılarak koroner arterlerde hasar varlığı araştrılmalıdır. Tedavi için 2 g/kg tek doz intravenöz immunglobülin (İVİG) ve 80-100 mg/kg/gün asetil salisilik asit başlanmalıdır. Onuncu günden sonra İVİG verilmesinin yararı tartışmalıdır. İVİG ile yanıt alınamazsa pulse steroid denenebilir. İmmunsüpresif tedavinin etkinliği ve güvenirliği belirsizdir. Koroner arterlerin etkilenme durumu ve derecesine göre prognoz değişkenlik gösterir. Miyokard infarktüsü Kawasaki hastalığındaki en sık ölüm nedenidir. Koroner hasar, prematür ve ciddi ateroskleroz gelişiminde rol oynayabilir. Kawasaki hastalığında koroner arterlerdeki sekeller, genç erişkinlerde iskemik kalp hastalığının önemli bir nedeni olabilir.

Anahtar Kelimeler:

Vaskülit, Koroner anevrizma, Mukokutanöz lenf düğümü sendromu

Kawasaki disease

Kawasaki disease is an acute, febrile vasculitis of young pediatric age with unknown etiology. The diagnosis is based primarily on clinical features. Aneurysmatic changes and thromboses of coronary arteries are serious complications of the disease. Diagnostic criteria for Kawasaki disease are as follows: Fever for at least five days, generalized polymorphous erythematous rash, conjunctival injection, typical changes in the lips and oral cavity, erythema and swelling of hands and feet and unilateral nonfluctuating cervical lymphadenopathy >1.5 cm in diameter. Incomplete (atypical) Kawasaki disease can be considered in cases which does not have four of five criteria. The clinical course of the disease can be divided into acute, subacute and convalescent phases. Acute phase reactants are increased nonspecifically. Inflammatory infiltration (mononuclear cells, CD 8+ T lymphocytes and immunoglobulin A plasma cells) around the affected vessel can be detected. Any damage to coronary arteries should be sought using echocardiography as soon as the diagnosis is ascertained. Single use of intravenous immunglobulin (IVIG) 2 g/kg over 10-12 hours and acetyl salicylic acid 100 mg/kg/day usually is sufficient. It is controversial to give IVIG after 10 days. Pulse steroid can be used if there is resistance to IVIG therapy. The effectiveness and safety of treatment with immunosuppressive agents remain undetermined. Prognosis depends on the severity of the coronary artery disease. Myocardial infarction is the most frequent cause of mortality in Kawasaki disease. Coronary damage could have a role in the development of premature and serious atherosclerosis. Sequela in coronary arteries are thought to have a significant role in ischemic heart disease in young adults.

Keywords:

Vasculitis, Coronary Aneurysm, Mucocutaneous Lymph Node Syndrome,

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