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先天性雀斑样痣中的心血管系统疾病

[Disorders of the cardiovascular system in congenital lentiginosis].

作者信息

Jurko A, Rösslerová V, Srsen S, Galanda V, Lányi A, Kulisek D

机构信息

Detská klinika LF UK v Martine.

出版信息

Cesk Pediatr. 1991 Apr;46(4):241-5.

PMID:1893462
Abstract

The authors investigated during the last 10 years four patients with lentiginosis and affection of the heart. In none of them the symptoms were complete enough to include it under the leopard syndrome. In two patients the authors found changes in the outflow portion of the right ventricle which was in their opinion caused by hypertrophy of the interventricular septum and musculature of the right ventricle. The authors consider the term lentiginocardiomyopathic syndrome suitable for this rare clinical and genetic entity. The authors check patients with lentiginocardiomyopathic syndrome regularly after 6-month intervals. In two children they recorded progression of the heart disease.

摘要

在过去10年里,作者对4例患有雀斑样痣病且伴有心脏病变的患者进行了研究。他们中没有一人的症状足以将其纳入豹斑综合征。在两名患者中,作者发现右心室流出道有变化,他们认为这是由室间隔和右心室肌肉肥厚引起的。作者认为“雀斑样痣心肌病综合征”这一术语适用于这种罕见的临床和遗传实体。作者每隔6个月定期检查患有雀斑样痣心肌病综合征的患者。在两名儿童中,他们记录到了心脏病的进展。

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