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肺泡微石症。病例报告及鉴别诊断讨论。

Pulmonary alveolar microlithiasis. A case report with a discussion of differential diagnosis.

作者信息

Saputo V, Zocchi M, Mancosu M, Bonaldi U, Croce P

出版信息

Helv Paediatr Acta. 1979;34(3):245-55.

PMID:500384
Abstract

The rare disease of pulmonary alveolar microlithiasis is characterized by innumerable microscopic stones, mainly of tribasic calcium phosphate, within the pulmonary alveoli. In a 13-year-old boy an earlier radiological examination showed diffuse lung opacity. The main differential diagnoses taken into consideration were sarcoidosis, idiopathic hemosiderosis, diffuse interstitial fibrosis and alveolar proteinosis. The correct diagnosis was made after a lung biopsy which showed intra-alveolar microliths. The radiological findings are in agreement with the reports in the literature. The specific X-ray features of pulmonary alveolar microlithiasis are calcified opacities, nodules, hilifugal trabeculations and pleural striae. These radiological signs are sufficiently diagnostic to restrict lung biopsy to particularly difficult cases. The disease is resistant to all therapeutic measures.

摘要

肺泡微石症这种罕见疾病的特征是肺泡内存在无数微小结石,主要成分为磷酸三钙。一名13岁男孩早期的放射学检查显示肺部弥漫性模糊影。考虑的主要鉴别诊断包括结节病、特发性含铁血黄素沉着症、弥漫性间质性纤维化和肺泡蛋白沉积症。在进行肺活检显示肺泡内有微石后做出了正确诊断。放射学检查结果与文献报道一致。肺泡微石症的特异性X线特征为钙化影、结节、离肺门的条索状影和胸膜条纹。这些放射学征象具有足够的诊断价值,可将肺活检限制在特别困难的病例中。该疾病对所有治疗措施均有抵抗性。

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