1型神经纤维瘤病患儿的心脏性猝死

Sudden cardiac death in young children with neurofibromatosis type 1.

作者信息

Kanter Ronald J, Graham Matthew, Fairbrother David, Smith S Victor

机构信息

Division of Pediatric Cardiology, Department of Pediatrics, Duke University School of Medicine, Durham, NC 27710, USA.

出版信息

J Pediatr. 2006 Nov;149(5):718-20. doi: 10.1016/j.jpeds.2006.07.046.

DOI:10.1016/j.jpeds.2006.07.046

PMID:17095352

Abstract

Patients with neurofibromatosis type 1 (NF1) can manifest a characteristic vasculopathy that in adults is rarely associated with fatal coronary artery occlusion. We describe the clinical and pathological findings from 2 unrelated young children with NF1, a similar vasculopathy affecting their coronary arteries, and sudden cardiac death.

摘要

1型神经纤维瘤病（NF1）患者可表现出一种特征性血管病变，在成人中很少与致命性冠状动脉闭塞相关。我们描述了2名无亲缘关系的患有NF1的幼儿的临床和病理发现，他们患有影响冠状动脉的类似血管病变，并发生了心源性猝死。