A case of pulmonary alveolar microlithiasis with Cor Pulmonale

[摘要] Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by the formation and deposition of microlithswithin the alveoli and a paucity of symptoms in contrast to the imaging findings. It has familial tendency and is thoughtto be an autosomal recessive disorder with the mutation in the SLC34A2 gene. We describe a case of PAM with CorPulmonale. Ultrasonic cardiogram showed pulmonary hypertension (82 mmHg). Chest radiography revealed diffuse,bilateral sandstorm-like micronodules with greater density in the lower lung fields. HRCT scans demonstrated diffuseground-grass opacities, thickening and calcification of interlobular septa and confluent calcified nodules. A diagnosis ofPAM was suggested and confirmed by transbronchial lung biopsy (TBLB).

[学科分类] 外科医学

[关键词] Pulmonary alveolar microlithiasis;Cor Pulmonale;HRCT