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- Atsushi Saito and Francis X McCormack.
- Department of Biochemistry, Sapporo Medical University, School of Medicine, Sapporo 0608543, Japan; Department of Respiratory Medicine and Allergology, Sapporo Medical University, School of Medicine, Sapporo 0608556, Japan.
- Clin. Chest Med. 2016 Sep 1; 37 (3): 441-8.
AbstractPulmonary alveolar microlithiasis (PAM) is a genetic lung disorder that is characterized by the accumulation of calcium phosphate deposits in the alveolar spaces of the lung. Mutations in the type II sodium phosphate cotransporter, NPT2b, have been reported in patients with PAM. PAM progresses gradually, often producing incremental dyspnea on exertion, desaturation in young adulthood, and respiratory insufficiency by late middle age. Treatment remains supportive, including supplemental oxygen therapy. For patients with end-stage disease, lung transplantation is available as a last resort. The recent development of a laboratory animal model has revealed several promising treatment approaches for future trials.Published by Elsevier Inc.
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