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[结节性硬化症合并肺部受累]

[Tuberous sclerosis with pulmonary involvement].

作者信息

Cruz Neto M, Reis P G, Riso M N

机构信息

Serviço de Medicina Interna, Hospital de Arroios, Lisboa.

出版信息

Acta Med Port. 1992 Jan;5(1):35-8.

PMID:1570752
Abstract

We describe the case of a 28 years old caucasian woman with tuberous sclerosis diagnosed at 23 when she underwent nephrectomy for a left renal angiomyolipoma with massive hematuria. She had adenoma but she presented on mental deficit and there was no record of seizures. Familiar history was negative except for the presence of adenoma sebaceum in her father, aged 63, and in her only daughter, aged 10. Five years after nephrectomy she was admitted to our hospital because of exertional dyspnoea and haemoptysis. Pulmonary involvement was confirmed by chest X-rays and CT scans.

摘要

我们描述了一名28岁的白种女性病例,她在23岁时因左侧肾血管平滑肌脂肪瘤伴大量血尿接受肾切除术,被诊断为结节性硬化症。她有腺瘤,但存在智力缺陷,且无癫痫发作记录。家族史呈阴性,不过她63岁的父亲和10岁的独生女有皮脂腺瘤。肾切除术后五年,她因劳力性呼吸困难和咯血入住我院。胸部X光和CT扫描证实了肺部受累。

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