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Pulmonary Alveolar Microlithiasis - Clinico-Radiological dissociation - A case report with Radiological review

机译:肺泡微结石症-临床放射学解离-病例报告并进行放射学审查

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摘要

Pulmonary alveolar microlithiasis (PAM) is a rare chronic lung disease characterized by deposition of intra alveolar calcium and phosphate in bilateral lung parenchyma with predominance in lower and mid zones. Etiology and pathogenesis is not fully understood. However, mutation in SLC34A2 gene that encodes a sodium phosphate co-transporter in alveolar type-II cells resulting in formation and accumulation of microliths rich in calcium phosphate due to impaired clearance is considered the cause of disease. Patients with PAM are asymptomatic till development of hypoxemia and cor pulmonale. It remains static, while in some it progresses to pulmonary fibrosis, respiratory failure and cor pulmonale. We report a case of 44 year old male patient presenting with progressive shortness of breath on exertion for one year in duration with dry cough, more since last six months. Chest radiograph showed dense micronodular opacities giving classical sandstorm appearance. High resolution computed tomography (HRCT) showed microcalcification, subpleural cystic changes and calcified pleura. Lung biopsy showed calcospherites within alveolar spaces.
机译:肺泡微结石症(PAM)是一种罕见的慢性肺部疾病,其特征是肺泡内钙和磷酸盐沉积在双侧肺实质中,主要分布在中下部。病因和发病机理尚未完全了解。但是,在肺泡II型细胞中编码磷酸钠共转运蛋白的SLC34A2基因突变导致清除率受损导致富含磷酸钙的微石形成和积累,被认为是疾病的原因。 PAM患者在出现低氧血症和肺心病之前无症状。它保持静态,而在某些情况下会发展为肺纤维化,呼吸衰竭和肺心病。我们报告了一例44岁男性患者,由于持续六个月的干咳,持续一年的劳累性呼吸急促。胸部X光片显示密集的微结节混浊,呈现出经典的沙尘暴外观。高分辨率计算机断层扫描(HRCT)显示微钙化,胸膜下囊性改变和钙化胸膜。肺活检显示在肺泡腔内有包囊石。

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