Modeling pulmonary alveolar microlithiasis by epithelial deletion of the Npt2b sodium phosphate cotransporter reveals putative biomarkers and strategies for treatment

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Modeling pulmonary alveolar microlithiasis by epithelial deletion of the Npt2b sodium phosphate cotransporter reveals putative biomarkers and strategies for treatment.

Pulmonary alveolar microlithiasis (PAM) is a rare, autosomal recessive lung disorder associated with progressive accumulation of calcium phosphate microliths. Inactivating mutations in SLC34A2, which encodes the NPT2b sodium-dependent phosphate cotransporter, has been proposed as a cause of PAM. We show that epithelial deletion of Npt2b in mice results in a progressive pulmonary process charact...

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Inorganic phosphate homeostasis in sodium-dependent phosphate cotransporter Npt2b⁺/⁻ mice.

An inorganic phosphate (P(i))-restricted diet is important for patients with chronic kidney disease and patients on hemodialysis. Phosphate binders are essential for preventing hyperphosphatemia and ectopic calcification. The sodium-dependent P(i) (Na/P(i)) transport system is involved in intestinal P(i) absorption and is regulated by several factors. The type II sodium-dependent P(i) transport...

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A Case Report of Pulmonary Alveolar Microlithiasis

Pulmonary alveolar microlithiasis is a rare autosomal recessive disease characterized by the formation of calcium phosphate deposition in the alveoli. Although the disease most often occurs in children, most patients with the disease are diagnosed in adulthood due to the slow progression of the disease inside the lungs. In childhood, it often causes no symptoms, and changes in the lung parenchy...

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Pulmonary Alveolar Microlithiasis: A Rare Case Report

Pulmonary alveolar microlithiasis is an uncommon infiltrative pulmonary disease characterized by deposition of microliths in the alveoli. We present the case of a young adult with complaints of shortness of breath on exertion. Chest radiograph showed innumerable small, dense nodules, diffusely involving both the lungs - predominantly in the lower zones. High-resolution CT scan illustrated wides...

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[Pulmonary alveolar microlithiasis].

The authors present two of their own observations where, based on the clinical course of the disease and the X-ray finding, they diagnosed the condition as pulmonary alveolar microlithiasis. They present a review of the literature as regards new views on the pathogenesis, diagnosis and therapy of the condition. They evaluate the contribution of scintigraphy 99 mTc, bronchial lavage and X-ray me...

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ژورنال

عنوان ژورنال: Science Translational Medicine

سال: 2015

ISSN: 1946-6234,1946-6242

DOI: 10.1126/scitranslmed.aac8577