Munir Zeeshan, Khosa Muhammad Zeeshan, Qazi Muhammad Yaqoob
Department of Paediatric Medicine, The Children's Hospital and ICH, Lahore.
J Coll Physicians Surg Pak. 2015 Jul;25(7):541-2.
Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome in the paediatric age group and characterized by intra-alveolar accumulation of proteinaceous phospholipid-laden material called surfactant. The diagnosis is made by High Resolution Computed Tomography (HRCT) chest which shows characteristic crazy paving appearance and diagnosis confirmed by Bronchoalveolar Lavage (BAL). We report two cases. First was a 9-month old infant who presented with respiratory distress and peripheral cyanosis since birth. He was diagnosed on High Resolution Computed Tomography (HRCT) chest as a case of pulmonary alveolar proteinosis and broncho-alveolar lavage confirmed his diagnosis. Second case was a 10-year old female child who had a history of repeated chest infections for 5 years and now presented with cough and respiratory distress for 45 days. She was also diagnosed on HRCT chest but unfortunately she died before bronchoalveolar lavage.
肺泡蛋白沉积症（PAP）是儿科年龄组中的一种罕见综合征，其特征是肺泡内积聚一种名为表面活性剂的含蛋白质磷脂物质。诊断通过胸部高分辨率计算机断层扫描（HRCT）进行，其显示出特征性的铺路石样外观，诊断通过支气管肺泡灌洗（BAL）得以证实。我们报告两例病例。第一例是一名9个月大的婴儿，自出生以来出现呼吸窘迫和外周发绀。他经胸部高分辨率计算机断层扫描（HRCT）诊断为肺泡蛋白沉积症，支气管肺泡灌洗证实了他的诊断。第二例是一名10岁女童，有5年反复胸部感染病史，现出现咳嗽和呼吸窘迫45天。她也经HRCT胸部诊断，但不幸的是，她在支气管肺泡灌洗前死亡。
