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结节性硬化症的肺部受累情况。

Pulmonary involvement in tuberous sclerosis.

作者信息

Vicente M P, Pons M, Medina M

机构信息

Pediatric Intensive Care Unit (UCIP), Hospital Sant Joan de Déu, Esplugues de Llobregat, Spain.

出版信息

Pediatr Pulmonol. 2004 Feb;37(2):178-80. doi: 10.1002/ppul.10414.

Abstract

Tuberous sclerosis is a neurocutaneous syndrome involving many tissues. Pulmonary involvement is rare. Pulmonary cases are usually women of child-bearing age who present with spontaneous pneumothorax or progressive dyspnea. In childhood and, even more, in male patients, pulmonary involvement by tuberous sclerosis is exceptional. We report on a 4-year-old male patient diagnosed in the first months of life with tuberous sclerosis with predominantly respiratory symptoms (frequently with acute RDS criteria), in whom lung involvement was pathologically confirmed.

摘要

结节性硬化症是一种累及多个组织的神经皮肤综合征。肺部受累较为罕见。肺部受累的病例通常是育龄期女性,表现为自发性气胸或进行性呼吸困难。在儿童期,尤其是男性患者中,结节性硬化症累及肺部的情况极为罕见。我们报告一例4岁男性患者,在出生后的头几个月被诊断为结节性硬化症,主要表现为呼吸道症状(常符合急性呼吸窘迫综合征标准),其肺部受累经病理证实。

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