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10.1016/j.ccm.2016.04.007

http://scihub22266oqcxt.onion/10.1016/j.ccm.2016.04.007
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C4987712!4987712 !27514591
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suck abstract from ncbi


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pmid27514591
      Clin+Chest+Med 2016 ; 37 (3 ): 441-8
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  • Pulmonary Alveolar Microlithiasis #MMPMID27514591
  • Saito A ; McCormack FX
  • Clin Chest Med 2016[Sep]; 37 (3 ): 441-8 PMID27514591 show ga
  • Pulmonary 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.
  • |*Lung Transplantation [MESH]
  • |*Oxygen Inhalation Therapy [MESH]
  • |Bone Density Conservation Agents/*therapeutic use [MESH]
  • |Calcinosis/complications/diagnostic imaging/genetics/*therapy [MESH]
  • |Calcium Phosphates/metabolism [MESH]
  • |Etidronic Acid/*therapeutic use [MESH]
  • |Genetic Diseases, Inborn/complications/diagnostic imaging/genetics/*therapy [MESH]
  • |Humans [MESH]
  • |Lung Diseases/complications/diagnostic imaging/genetics/*therapy [MESH]
  • |Mutation [MESH]
  • |Radiography, Thoracic [MESH]
  • |Respiratory Insufficiency/etiology/*therapy [MESH]
  • |Sodium-Phosphate Cotransporter Proteins, Type IIb/genetics [MESH]


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