Nakamura Y, Iwata M, Ida M, Oda S, Takeuchi E, Asada K, Nakano H, Horiguchi T, Ooi S, Sato A
Department of Respiratory Medicine, Haibara General Hospital, Shizuoka, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1997 Sep;35(9):985-90.
This is a rare case of Dyskeratosis Congenita (DC) with acute interstitial pneumonia. A 51-year-old man with DC was admitted to our hospital because of cough, sputum and fever. Chest X-ray film showed ground glass opacities in all lung fields for a while steroid's therapy proved effective, but about seven months later the patient's condition became serious. Methylprednisolone, cyclophosphamide and mechanical ventilation therapy were not effective. He died and an autopsy was performed. The lung specimen showed Organizing Diffuse Alveolar Damage, and some parts pointed to bacterial infection. But Pneumocystic carinii pneumonia and Fungal infections were not found. It is therefore necessary to conduct intensive examinations of lung involvement of patients with Dyskeratosis Congenita.
这是一例伴有急性间质性肺炎的先天性角化不良（DC）罕见病例。一名患有DC的51岁男性因咳嗽、咳痰和发热入住我院。胸部X线片显示所有肺野均有磨玻璃样阴影，一段时间的类固醇治疗证明有效，但约七个月后患者病情加重。甲泼尼龙、环磷酰胺和机械通气治疗均无效。患者死亡并进行了尸检。肺标本显示机化性弥漫性肺泡损伤，部分提示细菌感染。但未发现卡氏肺孢子虫肺炎和真菌感染。因此，有必要对先天性角化不良患者的肺部受累情况进行深入检查。
