下周開始，我又要做回一名學生啦～
0812
——Current Medical Diagnosis and Treatment
D42 Pulmonary Disorders 之 Pulmonary Alveolar Proteinosis
Pulmonary Alveolar Proteinosis
肺泡蛋白沉積症
又是一個罕見病。
Pulmonary alveolar proteinosis is a rare disease in which phospholipids accumulate within alveolar spaces. The condition may be primary (idiopathic) or secondary (occurring in immunodeficiency; hematologic malignancies; inhalation of mineral dusts; or following lung infections, including tuberculosis and viral infections).
肺泡蛋白沉積症是磷脂在肺泡內積聚的一種罕見病。肺泡蛋白沉積症可以是原發性的或稱特發；也可是繼發性的，繼發於免疫缺陷、惡性血液腫瘤、礦物粉塵吸入，或繼發於包括肺結核和病毒感染等肺部感染之後。
Progressive dyspnea is the usual presenting symptom, and chest radiograph shows bilateral alveolar infiltrates suggestive of pulmonary edema. The diagnosis is based on demonstration of characteristic findings on bronchoalveolar lavage (milky appearance and PAS-positive lipoproteinaceous material) in association with typical clinical and radiographic features. In secondary disease, an elevated anti-GM-CSF titer in serum or bronchoalveolar lavage fluid is highly sensitive and specific. In some cases, transbronchial or surgical lung biopsy (revealing amorphous intra-alveolar phospholipid) is necessary.
肺泡蛋白沉積症常見的症狀是進行性呼吸困難，胸片常見雙側肺泡浸潤，提示有肺水腫。支氣管肺泡灌洗物檢查，結合典型臨床表現和影像學檢查結果可做出診斷。支氣管肺泡灌洗物檢查的典型表現為乳白色外觀，內含PAS陽性的脂蛋白物質。繼發性肺泡蛋白沉積症時，血清或支氣管肺泡灌洗液中抗粒細胞集落刺激因子滴度升高，這一結果具極高敏感性和特異性。某些病例需經支氣管肺活檢或外科肺活檢，肺泡內可檢出無定形磷脂。
The course of the disease varies. Some patients experience spontaneous remission; others develop progressive respiratory insufficiency. Pulmonary infection with Nocardia or fungi may occur. Therapy for alveolar proteinosis consists of periodic whole-lung lavage. Patients who can not tolerate whole lung lavage or who fail to respond may benefit from inhalational or subcutaneous GM-CSF.
肺泡蛋白沉積症的過程各不相同。部分患者可自行緩解，另一部分可發展為進行性呼吸功能不全。病程中可發生諾卡氏菌或真菌引起的肺部感染。本病通過定期全肺灌洗進行治療。不能耐受全肺灌洗或全肺灌洗無效的患者可吸入或皮下注射粒細胞集落刺激因子。
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以上英文來自Current Medical Diagnosis and Treatment，版權屬原作者。中文是我的筆譯練習，不妥之處，敬請指正。