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[患有雷克林霍增氏神经纤维瘤病的婴儿的退行性肥厚性心肌病]

[Regressive hypertrophic myocardiopathy in an infant with Recklinghausen's neurofibromatosis].

作者信息

Tillous-Borde I, Challier P, Fontaine J L

出版信息

Arch Fr Pediatr. 1986 Mar;43(3):197-200.

PMID:3092779
Abstract

A case of neonatal hypertrophic cardiomyopathy (HCM) without obstruction is reported. Von Recklinghausen neurofibromatosis in the mother and several relatives and the occurrence of café-au-lait spots in the child at 6 weeks of life led to the association of HCM with this phacomatosis. The spontaneous regression of the myocardial hypertrophy after 6 weeks makes this first report of neonatal HCM in the course of Von Recklinghausen neurofibromatosis peculiar. Several reports in the literature mention the possibility of such a spontaneous regression of HCM or diffuse cardiac tumors, especially in the course of phacomatosis.

摘要

报告了一例无梗阻性新生儿肥厚型心肌病(HCM)病例。母亲和几位亲属患有冯·雷克林豪森神经纤维瘤病,患儿在6周龄时出现咖啡牛奶斑,这导致了HCM与这种斑痣性错构瘤病的关联。6周后心肌肥厚自发消退,这使得在冯·雷克林豪森神经纤维瘤病病程中首次报告新生儿HCM显得尤为特殊。文献中的几篇报告提到了HCM或弥漫性心脏肿瘤自发消退的可能性,尤其是在斑痣性错构瘤病病程中。

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