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[奥地利的黏膜皮肤淋巴结综合征。4例,1例死亡。2. 病理结果]

[Mucocutaneous lymph node syndrome in Austria. Four cases with one fatal outcome. 2. Pathological findings].

作者信息

Becker H, Höfler H, Urban C, Grubbauer H M, Beitzke A

出版信息

Klin Padiatr. 1981 Jul;193(4):318-21. doi: 10.1055/s-2008-1034485.

Abstract

First pathoanatomic case report of mucocutaneous lymph-node syndrome (MCLS) from Austria: A 3 1/2 year old boy was admitted to the Pediatric Department with a six day history of fever and bilateral conjunctivitis. He showed a maculous exanthema, red and fissuring lips, reddened tonsils and a coated tongue. Submandibular lymph nodes were swollen (1 cm diameter); ESR 128/138; peripheral leukocytosis 17 x 10(9)/l. On the tenth day of illness membranous desquamation from the fingertips and a strawberry tongue was observed. The ECG showed a myocardial infarction in the apical portion of the left ventricle. The boy died on the 25th day of illness. Pathological findings were found predominantly in the heart: Coronary arteries showed fibrous thickening of intimal layer, focal destruction of elastic membrane and aneurysms in both arteries. The aneurysm in the descending branch of the left coronary artery was 15 mm long and had a maximum diameter of 8 mm. It was completely occluded by a thrombus. Recurring anteroseptal myocardial infarction. Other investigated arteries were not affected. Tonsils as well as (paratracheal and parapancreatic) lymph-nodes showed a few ceroid pigment containing histiocytes.

摘要

奥地利首例皮肤黏膜淋巴结综合征(MCLS)的病理解剖病例报告:一名3岁半男孩因发热和双侧结膜炎6天入住儿科。他出现了斑丘疹、嘴唇红肿皲裂、扁桃体发红及舌苔。颌下淋巴结肿大(直径1厘米);血沉128/138;外周血白细胞增多,为17×10⁹/升。病程第10天,观察到指尖有膜状脱皮及草莓舌。心电图显示左心室心尖部心肌梗死。该男孩于病程第25天死亡。病理发现主要在心脏:冠状动脉内膜层纤维增厚,弹性膜局灶性破坏,两条动脉均有动脉瘤形成。左冠状动脉降支的动脉瘤长15毫米,最大直径8毫米,完全被血栓阻塞。反复发生前间隔心肌梗死。其他检查的动脉未受影响。扁桃体以及(气管旁和胰腺旁)淋巴结可见少数含类蜡样色素的组织细胞。

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