2020
DOI: 10.1055/s-0040-1702211
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Pulmonary Alveolar Microlithiasis

Abstract: Pulmonary alveolar microlithiasis (PAM) is a rare, autosomal recessive disorder that is caused by mutations in SCL34A2 that encodes for the type IIb sodium-dependent phosphate cotransporter (Npt2b). The loss of Npt2b transporter function from alveolar epithelial cells results in failure to export inorganic phosphate from the alveolar lining fluid, which then accumulates, binds to calcium, and forms hydroxyapatite microliths. Radiographs and computed tomography of the chest demonstrate hyperdense infiltrates th… Show more

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Cited by 9 publications
(5 citation statements)
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“…[ 23 ] Moreover, etidronate interferes with phosphate incorporation into hydroxyapatite, culminating in reduced crystal formation. [ 24 ] The response to etidronate treatment has shown variable results. [ 25 ] Saito et al .…”
Section: Discussionmentioning
confidence: 99%
“…[ 23 ] Moreover, etidronate interferes with phosphate incorporation into hydroxyapatite, culminating in reduced crystal formation. [ 24 ] The response to etidronate treatment has shown variable results. [ 25 ] Saito et al .…”
Section: Discussionmentioning
confidence: 99%
“…PAM is characterized by a defect in SLC34A2, a type IIb sodium-dependent phosphate cotransporter found in alveolar epithelial cells. Dysfunction of this transporter results in the failure to export inorganic phosphate from the alveolar lining fluid, leading to calcium accumulation and hydroxyapatite microlith formation [ 15 , 16 ]). On chest radiograph, PAM may exhibit diffuse or scattered micronodules, hyperdense infiltrates with a predilection for the lung bases, ground-glass attenuation, linear subpleural calcifications or subpleural nodules, small parenchymal nodules, dense consolidations, and a mosaic pattern of attenuation.…”
Section: Discussionmentioning
confidence: 99%
“…The loss of this transporter function results in accumulation of inorganic phosphate in alveolar fluid, which binds to calcium to form calcium phosphate deposits. 81 82 The most common CT findings are ground-glass attenuation and linear and nodular subpleural calcifications ( Fig. 31 ).…”
Section: Pulmonary Alveolar Microlithiasismentioning
confidence: 99%