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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 investigate the clinicopathological characteristics, diagnosis and differential diagnosis of alveolar microlithiasis. Methods One case of alveolar microlithiasis was observed and the clinical, imaging and pathological features were reviewed. The results of repeated recurrent children with upper respiratory tract infection symptoms, clinical treatment of chronic interstitial pneumonia. CT showed diffuse pleomorphic lungs and grid-like high-density shadow, fuzzy edge. Pulmonary biopsy of the lung tissue in general have a slight gritty, about 30% of microscopic alveolar cavity see purple calcified bodies, was concentric layered calcification. Conclusions Alveolar microlithiasis is an autosomal recessive disease characterized by diffuse distribution of microlithiasis in the pulmonary alveoli. It has unique clinical, radiological and pathological features with a poor prognosis.