Deniz Omer
Department of Chest Diseases, Gulhane Military Medical Academy, Ankara, Turkey.
Tuberk Toraks. 2005;53(3):293-8.
Pulmonary alveolar microlithiasis (PAM) is a rare, chronic lung disease characterized by extensive intra alveolar calcium and phosphate deposition throughout both lung parenchyma. Etiology and pathogenesis of PAM is not known. There are some hypothesis for etiology and pathogenesis of PAM but none of them are satisfactory. The incidence of PAM is high in countries such as Turkey, Italy and USA. There is a surprising discordancy between radiological appearance and clinical presentation. Chest X-ray appearance of PAM is almost pathognomonic. Patients with PAM may have all findings of interstitial lung disease in varying degrees as well as micro-nodules on their HRCTs. Patients with PAM usually asymptomatic until the underlying process affects alveolar gas exchange however patients may ultimately develop hypoxemia and cor pulmonale. A definitive therapy for PAM did not exist. Patients with advanced lung disease may benefit from lung transplantation.
肺泡微石症(PAM)是一种罕见的慢性肺部疾病,其特征是在整个肺实质内广泛存在肺泡内钙和磷酸盐沉积。PAM的病因和发病机制尚不清楚。关于PAM的病因和发病机制有一些假说,但都不尽人意。PAM在土耳其、意大利和美国等国家发病率较高。其影像学表现与临床表现之间存在惊人的不一致。PAM的胸部X线表现几乎具有诊断意义。PAM患者在不同程度上可能有间质性肺疾病的所有表现,以及高分辨率CT(HRCT)上的微小结节。PAM患者通常无症状,直到潜在病变影响肺泡气体交换,但患者最终可能会出现低氧血症和肺心病。目前尚无针对PAM的确定性治疗方法。晚期肺部疾病患者可能从肺移植中获益。