儿科精品课件-皮肤黏膜淋巴结综合征Kawasaki--Disease.ppt

Kawasaki Disease Department of Pediatric Wang Xiu-ying 中南大学儿科学教研室 第九章第八节 ■Understand the etiology and pathology of Kawasaki Disease (K D) ■Master the clinical manifestations and diagnostic criterion of KD. ■Master the treatment of KD. Purpose and Requirement Kawasaki disease formerly known as mucocutaneous lymphnode syndrome（MCLS 皮肤粘膜淋巴结综合征）. 80% of patients are younger than 5yr, Approximately 20% of untreated patients develop coronary artery abnormalities including aneurysms （动脉瘤）.kawasaki disease has replaced acute rheumatic fever as the leading cause of acquired heart disease in children in our country. Conception Etiology and Pathogenesis ※infectious factors(感染因素) ※ immune reaction（免疫反应） ※ other factors(其他因素) A severe vasculitis（血管炎） of all blood vessels Arteria(动脉) Vena(静脉) Capillary(毛细血管) PATHOLOGY Fever Rash of various forms（多形红斑） Mucosa vary（粘膜变化） Cervical lymphnode swelling （颈淋巴结肿大） Changes of the peripheral extremities （周围四肢的变化） CLINICAL MANIFESTATION Rash involving the trunk and the extremites Rash of various forms Swelling of the hands Swelling of the feet Periungual desquamation tips of the fingers and toes Strawberry tongue, dry cracked lips erythema Conjunctivitis erythema of the oral and pharyngeal mucosa Usually unilateral with a node size of 1.5cm or greater in diameter Cervical lymph node swelling Mild anemia WBC↑,N↑, Pt normal - ↑ ESR ↑, CRP ↑ Hemoglobin electrophoresis α2 ↑ CD4 Tc↑, CD8 Tc↓ IgA ↑, IgM ↑ , C3 normal- ↑ Laboratory examination Two-dimensional echocardiography is the most useful test to monitor the potential development of coronary abnormalities ※ Fever lasting for at least 5 days Presence of at lease four of the following five signs: ☆ Bilateral bulbar conjunctival injection, generally nonpurulent ☆ changes in the mucosa of the oropharynx , including injected pharynx,injected and/or dry fissured lips, strawberry tongue Diagnosis criter