Jansa P, Stríteský M, Homolka J
II. interní klinika 1. lékarské fakulty UK a VFN, Praha.
Vnitr Lek. 2002 Jan;48(1):34-7.
Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of a large amount of both components of surfactant, proteins and phospholipids in alveoli and terminal bronchioles. This cumulation may lead to impaired gas exchange and result in respiratory insufficiency. The disease may develop in neonatal age or later in adult age. Two congenital forms are known. The disease occurs however also in people with neoplastic disease, during cytostatic treatment, during inhalation of inorganic dusts. A dominant symptom is progressing dyspnoea. Asymptomatic forms exist also. The most effective therapeutic method is overall pulmonary lavage. The authors implemented in 1999 the first overall pulmonary lavage in the Czech Republic in the General Faculty Hospital in Prague in a 60-year-old female patient.
肺泡蛋白沉积症是一种罕见疾病,其特征是肺泡和终末细支气管中大量表面活性剂成分(蛋白质和磷脂)蓄积。这种蓄积可能导致气体交换受损并导致呼吸功能不全。该疾病可在新生儿期或成年后期发病。已知有两种先天性形式。然而,该疾病也发生在患有肿瘤性疾病的人群、进行细胞毒性治疗期间以及吸入无机粉尘期间。主要症状是进行性呼吸困难。也存在无症状形式。最有效的治疗方法是全肺灌洗。作者于1999年在布拉格综合大学医院为一名60岁女性患者实施了捷克共和国首例全肺灌洗。