Abstract
Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by an intra-alveolar and interstitial accumulation of tiny, roundish corpuscles called ”microliths”. PAM may lead to hypoxemia, pulmonary hypertension (PH), respiratory failure and cor pulmonale. Lung transplantation is reserved for the extreme patient. We report a 27-year-old male presenting with shortness of breath for 5 years; his hemoglobin and hematocrit were 23.5 mg/dl and 68.6 vol%, respectively. A pulmonary artery catheter revealed severe hypoxemia, PH, increased pulmonary vascular resistance, and a venous admixture. The chest X-ray showed a characteristic sandstorm pattern. High resolution computed tomography revealed diffuse micronodules and interlobar septal thickness. The bronchoalveolar lavage study disclosed characteristic concentric lamellar calcified microliths. The patient responded well to therapeutic phlebotomy and nasal continuous positive airway pressure.
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