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
formerly known as m 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 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 Conjunctivitis
Usually unilateral with a node size of 1.5cm or greater in diameter Cervical lymph swelling node swelling Cervical lymph swelling node swelling
Mild anemia WBC↑,N↑, Pt normal - ↑ ESR ↑, CRP ↑ ↑ Hemoglobin electrophoresis α 2 ↑ CD 4 Tc↑, CD 8 Tc↓ IgA ↑, IgM ↑, C3 normal- ↑ Laboratory examination
Two-dimensional echocardiography Two-dimensional echocardiography is the most useful test to monitor is the most useful test to monitor the potential development of 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 criteria
☆ Changes of the peripheral extremities,such as edema and/or erythema of the hands or feet in the acute phase; or periungual desquamation in the subacute phase ☆ Rash, primarily truncal;poiymorphous but nonvesicular ☆ Cervical adenopathy, ≥1.5cm,usually unilateral lymphadenopathy Illness not explained by other known disease process
必备条件：原因不明的发热至少持续 5 天以上 其他：应具备下列 5 项体征中的 4 项 非渗出性球结膜充血 口腔咽部改变 四肢末端变化 多形性皮疹 急性期非化脓性颈淋巴结肿大 如果临床体征少于上述 4 项，但明确有 冠状动脉病变，仍可确诊为川崎病 诊断标准 皮肤黏膜淋巴结综合征皮肤黏膜淋巴结综合征
High risk factor for coronary artery aneurysms High risk factor for coronary artery aneurysms Boy, age ＜ 1yr Haematocrit ＜ 0.35 Plasma protein ＜ 35g/L Earlier period (first week): Pt ＜ 200×10 9 /L Pt ＜ 200×10 9 /LCRP↑↑
Aspirin Aspirin High doses of intravenous High doses of intravenous immune globulin (IVIG) immune globulin (IVIG) Corticosteroids Corticosteroids Treatment Treatment