Kawasaki Hastalığı

Öz

Kawasaki hastalığı (mukokutanöz lenf nodu sendromu ) nedeni bilinmeyen ve genellikle 5 yaşın altındaki çocuklarda görülen bir sistemik vaskülit sendromudur. Çocukluk çağında Henoch-Schonlein vaskülitinden sonra en sık görülen ikinci vaskülit nedenidir. Hastalığın en önemli komplikasyonu koroner arter anevrizmasıdır. Bu nedenle erken tanı ve tedavi hastalığın iyi prognozu açısından çok önemlidir. Kawasaki hastalığının kesin tanı koydurucu kriterleri yoktur. Atipik veya inkomplet seyirli vakaların varlığı nedeniyle 5 günden uzun süren ateşli olgularda Kawasaki hastalığı akla getirilmeli ve hastalar kardiak komplikasyonlar açısından ekokardiografi ile değerlendirilmelidir. Erken tanı ve tedavinin önemini bir kez daha vurgulamak amacıyla hastanemize uzamış ateş nedeniyle refere edilen, koroner arter anevrizması gelişmesi nedeniyle Kawasaki hastalığı tanısı alan 5.5 aylık erkek hastayı sunuyoruz.

Anahtar Kelimeler

• Kawasaki hastalığı, sütçocuğu, koroner arter anevrizması

KAWASAKI DISEASE

Öz

Kawasaki disease (mucocutaneous lymph node syndrome) is a systemic vasculitic syndrome with unknown etiology which occurs predominantly in children younger than 5 years.It is the second common cause of vasculitis following Henoch-Schonlein vasculitis in childhood. The most important complication of the disease is coronary artery aneurysm. For that reason, early diagnosis and therapy is very important for good prognosis. Kawasaki disease has no certain diagnostic criteria. Because of atypical or incomplete cases, Kawasaki disease should be considered in any infant or young child with prolonged and unexplained fever and these patients should be evaluated by echocardiography for cardiac complications. We present a 5.5 month old boy referred to our hospital for prolonged fever, developing coronary artery aneurysm and diagnosed as Kawasaki disease to emphasize the importance of early diagnosis and therapy once more

Anahtar Kelimeler

• Kawasaki disease, infant, coronary artery aneurysm

Kaynakça

1. Belay ED, Maddox RA, Holman RC, Curns AT, Ballah K, Schonberger LB. Kawasaki Syndrome and Risk Factors for Coronary Artery Abnormalities. United-States, 1994-2003. Pediatr Infect Dis J. 2006; 25:245-249.
2. Yavuz T, Nişli K, Öner N, Ömeroğlu R.E, Dindar A, Aydoğan Ü, Ertuğrul T. Kawasaki hastalığı: 34 olgu sunumu. Türk Pediatri Arşivi. 2006; 41: 197-200.
3. Beiser AS, Takahashi M, Baker AL, Sundel RP, Newburger JW. A pre- dictive instrument for coronary artery aneurysms in Kawasaki disease. US Multicenter Kawasaki Disease Study Group. Am J Cardiol. 1998; 81:1116-1120.
4. Sundel RP. Update on the treatment of Kawasaki disease in childhood. Curr Rheumatol Rep. 2002; 4: 474-482.
5. Rauch AM. Kawasaki syndrome:critical review of U.S. epidemiology. Prog Clin Biol Res. 1987; 250: 33-34.
6. Han RK, Silverman ED, Newman A, McCrindle BW. Management and outcome of persistent or recurrent fever after initial intravenous gamma globulin therapy in acute Kawasaki disease. Arch Pediatr Adolesc Med. 2000; 154: 694-699.
7. Hirata S, Nakamura Y, Yanagawa H. Incidence rate of recurrent Kawasaki disease and related risk factors: from the results of nationwide surveys of Kawasaki disease in Japan. Acta Paediatr. 2001; 90: 40-44.
8. Okada Y, Shinohara M, Kobayashi T, Inove Y, Tomomasa T, Kobayashi T, Morikowa A.Gunma Kawasaki Disease Study Groyp. Effect of corticos- teroids in addition to intravenous gamma globulin therapy on serum cyto- kine levels in the acute phase of Kawasaki disease in children. J Pediatr 2003;143:363.

9. Sundel RP, Baker AL, Fulton DR, Newburger JW. Corticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial. J Pediatr. 2003; 142: 611-616.
10. Newburger JW, Sleeper LA, McCrindle BW, Minich LL, Gersony W, Vetter VL, Atz AM, Li JS, Takahaski M, Baker AL, Colan SD; Mitchell PD, Klein GL, Sundel RP;Pediatric Haet Network Investiqators. Randomised Trial of Pulsed Corticosteroid Therapy for Primart Treatment of Kawasaki Disease. N Engl J Med. 2007; 356;7:663-675.
11. Weiss JE, Eberhard BA, Chowdhury D, Gottlieb BS. Infliximab as a novel therapy for refractory Kawasaki disease. J Rheumatol. 2004;31:808-810.