Clinical characteristics of Kawasaki disease according to age at diagnosis

Abstract

[한글]

목적: 치명적인 관상동맥 합병증을 일으킬 수 있는 가와사키병에 있어, 연령별에 따른 임상적 특성 및 혈액학적 차이를 알아 보기 위하여 본 연구를 시행하였다.

대상 및 분석 방법: 2006-2007년까지 연세대학교 의과대학 세브란스 아동병원에서 가와사키병으로 진단받은 전체 환아 198례 중 재발된 13례를 제외한 185례를 대상으로 하여 조사하였으며, 이를 연령별로 세 그룹으로 나누어 (A군: ≤6개월, B군: 6개월~5세, C군: ≥5세) 발열기간, 결막충혈, 입술, 구강의 발적, 경부림프절종창, 피부발진, 손발의 변화, BCG 접종부위의 발적 등의 임상적 특성과 백혈구, 혈소판, CRP, ESR, AST/ALT 등의 검사소견을 의무기록을 통하여 후향적으로 비교분석을 하였다.

결과: 전체 환아 185례 중 34%인 63례가 전형적인 가와사키군, 66%인 122례가 비정형적 가와사키군이었으며 5개월이하가 22명(A군), 6개월-4세가 122명(B군), 5세 이상이 32명인(C군) 분포를 보였다. 임상적 특성상 BCG 접종부위가 A군에서 의미있게 높게 나타났으며 경부림프절부종이 C군에서 높게 관찰되었다. 혈액학적 차이로는 A군에서 의미있게 백혈구, 혈소판의 증가소견이 관찰되었다. 기대했던 것과는 달리, A나 C군에서 관상동맥질환이 높게 관찰되지 않았으며 이는 어린 나이라도 조기치료를 받은 환아는 관상동맥질환에 이행되지 않는 것으로 생각되었다.

결론: 연령에 따른 임상특성, 혈액학적 소견이 다양하지만 각 연령별 특성을 고려하여 조기발견, 조기치료를 하여 가와사키병의 치명적 합병증인 관상동맥 합병증을 예방해야한다.

[영문]

Object: The diagnosis of Kawasaki disease (KD) in children outside the typical age range (6 months?5 years) is often delayed, potentially worsening the prognosis with an increased risk of coronary artery involvement. In an effort to better understand the differences, a comparison of the clinical features of KD in children ≤6 months and ≥5 years with those in the more typical age range at diagnosis (6 months?5 years) was made.

Patients and methods: All patients with a discharge diagnosis of KD at Severance Children's Hospital (2006-2007) were retrospectively reviewed and grouped according to their age at presentation.

Results: A total of 185 children were identified. American Heart Association (AHA) criteria were met in 63 (34%) and not met in 122 (66%) children. N-terminal fragment of B-type natriuretic peptide (NT-proBNP) was markedly elevated in all age groups with statistic insignificance (P = 0.69). BCG site change was significantly more common in group A (< 6mo.) (P = 0.01). More marked leukocytosis, thrombocytosis were noted in group A (P = 0.001 and 0.01), which indicated a more marked inflammatory process. Also hemoglobin decreased more significantly in group A than in the two other groups (P =0.01). Cervical lymphadenopathy was seen more common in group C than in the two other groups with statistic insignificance (P = 0.06). While infants < 6mo are reported with a higher risk of coronary artery lesions (CAL) in some studies, there were no different coronary artery complications in any of the age groups in this study.

Conclusion: The clinical and laboratory phenotype of KD varied with age. In incomplete forms of KD and younger children, the clinical features were fewer, more subtle; suggesting that diagnosis is consequently more difficult and delayed. With an absence of a specific diagnostic test for KD, some characteristic clinical and laboratory findings help with diagnosis. BCG site change and NT-proBNP may be highly suggestive indicators for acute KD. Younger children who are treated timely and appropriately may not have a higher risk of CAL.