肺泡微石症临床病理观察

Pulmonary alveolar microlithiasis:clinical and pathological observations

目的探讨肺泡微石症的临床病理特点、诊断及鉴别诊断。方法对1例肺泡微石症患者的临床、影像学和病理特征进行观察并复习文献。结果患儿反复出现逐渐加重的上呼吸道感染症状,临床以慢性间质性肺炎收治。CT示双肺弥漫性斑点状和网格状高密度影,边缘模糊。肺活检肺组织大体切面有轻微的砂砾感,镜下约30%的肺泡腔内见紫色的钙化小体,呈同心层状钙化。结论肺泡微石症是以双肺肺泡腔内弥漫分布微小结石为特征的常染色体隐性遗传病,具有独特的临床、影像学及病理学特征,预后较差。

Objective To study the clinical and pathological features of pulmonary alveolar microlithiasis and to explore its diagnosis and differential diagnosis. Methods The clinical, image and pathologic features were analysed a case of pulmonary alveolar microlithiasis in childhood and the relevant literatures were reviewed. Results A six year-old girl was admitted to hospital with repeated, progressive appearance of upper respiratory tract infections and chronic interstitial pneumonia. CT showed diffusely distributed mieronodular calcified and high-density shadows with ill-defined edge in both lungs. Open lung biopsy in the girl was performed to determine the diagnosis. The formation of concentrically purple mieroliths were seen clearly within the alveoli under microscope. Diagnosis of pulmonary alveolar mierofithiasis was made on the basis of image and pathological features. Conclusion Pulmonary alveolar microlithiasis is a rare autosomal recessive inherited disease eharacteristicd by the widespread intra-alveolar deposition of laminated calcifications in both lung, which has special features in clinic, image and pathology with unfavorable outcome.