13例不完全川崎病的临床分析

Clinical Anlysis of Incomplete Kasawaki Disease:13 Cases

目的分析不完全川崎病的临床资料,观察其临床特征,为临床诊断治疗提供指导。方法诊断参照第三届国际KD会议修订的诊断标准和美国心脏协会推荐的不完全川崎病诊断指南,结合临床经验,分析总结13例不完全川崎病患儿各种临床表现及其出现时间和几率。结果小于1岁者发病者5例,1～5岁者4例,大于5岁发病4例,平均年龄3.5岁,年龄最小3个月,年龄最大9岁。发热、皮疹、淋巴结肿大、指(趾)末端脱皮、双球结膜充血、唇红皲裂的发生率分别为100%、38.4%、38.4%、38.4%、46.1%、53.8%。指(趾)末端脱皮、肛周皮肤、阴囊皮肤潮红在起病在病程的第1～2周,病程2周内发生心脏冠状动脉病变达60%。本病主要用口服阿司匹林和大剂量静脉注射丙种球蛋白治疗。结论发热、淋巴结肿大、皮疹、唇和口腔黏膜改变是川崎病的早期主要临床表现。肛周潮红及其脱皮、阴囊脱皮、血小板(PLT)增高、肝酶、C反应蛋白(CRP)和血沉(ESR)升高有早期诊断的价值。不完全川崎病疑似病例应尽早行超声心动图检查,心脏冠脉病变可以为诊断提供有力证据,且尽早正规治疗可明显提高预后。

Objective Retrospectively study clinical datas of 13 children with incomplete Kawasaki disease （KD）, observe and an- lyze their clinical characteristics. Methods The diagenosis of incomplete KD was based on the criteria refined on the third international KD conference and the supplemental laboratory criteria and endocardiographic crteria prepared by Amecican Heart Assocication （AHA）. meanwhile, pediatricians ~xperiences played a important role in the diagnosis of incomplete KD. anlyze their varified and atyp- ical performances as well their onset time and probablities of occurence. Results Average age of onset was three and a half years old, with extremities of three months and 9 years. Occurence rate of fever,polymorphous truncal rash,lymphadenopathy,changes of periph- era; extremities,conjuncfivM congestion,changes of lips and oral cavity was 100% ,38.4% ,38.4% ,38.4% ,46.1% ,53.8% respec- tively, desquamation of skin from the figertips ,blush of perianal and scrotum skins occured in the first to sencond week of the disease course, as hagh as 60% coronal abnomabilities took place in the first two weeks. Orally administration of aspirin and intravenous drip of immLme globin were the main treatments. Conclusion Fever, lymph node elargement, rash, changes of lips and oral cavity are the main- ly ealy manifestition, blush and desquamation around the perineum, elevation of PLT, AST, CRP and ESR has great value for early daignosis, once suspicion of incomplete KD founded, a ultrasonic cardiogram should be implemented immediately, coronary abnomality can be used as a vowerful evidence for KD.