Al Umairi Rashid, Al Lawati Fatma, Al-Riyami Mahmood, Al Kindi Faiza, Kamona Atheel, Al Busaidi Fadhila, Al Lawati Nabil, Al Lawati Adil, Habibulla Zulfikar
Department of Radiology, Royal Hospital, Muscat, Oman.
Department of Pathology, Royal Hospital, Muscat, Oman.
Oman Med J. 2020 Apr 20;35(2):e115. doi: 10.5001/omj.2020.33. eCollection 2020 Mar.
Pulmonary alveolar microlithiasis (PAM) is a rare disease caused by a mutation in the SLC34A2 gene encoding the type IIb sodium phosphate cotransporter in alveolar type II cells. This results in the formation and accumulation of calcium phosphates crystals in the alveoli. Early in the disease, most patients are asymptomatic or might experience mild symptoms. However, in some patients, PAM can progress resulting in pulmonary fibrosis, cor pulmonale, and respiratory failure. We report the case of a 33-year-old Omani male who was referred to our institute with a history of fever and shortness of breath. A chest radiograph revealed bilateral dense consolidation. Chest computed tomography showed bilateral dense interlobular thickening and extensive consolidations with a lower lung predominance. Our findings were highly suggestive of PAM. The diagnosis was confirmed by bronchoalveolar lavage.
肺泡微石症(PAM)是一种罕见疾病,由编码II型肺泡细胞中IIb型磷酸钠共转运蛋白的SLC34A2基因突变引起。这导致肺泡中磷酸钙晶体的形成和积累。在疾病早期,大多数患者无症状或可能出现轻微症状。然而,在一些患者中,PAM会进展,导致肺纤维化、肺心病和呼吸衰竭。我们报告了一例33岁阿曼男性病例,该患者因发热和呼吸急促病史转诊至我院。胸部X线片显示双侧致密实变。胸部计算机断层扫描显示双侧致密的小叶间隔增厚和广泛实变,以下肺为主。我们的发现高度提示为PAM。通过支气管肺泡灌洗确诊。