Vicente M P, Pons M, Medina M
Pediatric Intensive Care Unit (UCIP), Hospital Sant Joan de Déu, Esplugues de Llobregat, Spain.
Pediatr Pulmonol. 2004 Feb;37(2):178-80. doi: 10.1002/ppul.10414.
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.
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。