Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection.
Case Rep Pathol
; 2021: 6674173, 2021.
Article
en En
| MEDLINE
| ID: mdl-33884208
ABSTRACT
BACKGROUND:
Pulmonary alveolar microlithiasis (PAM) is a rare lung disease characterized by the deposition of calcium phosphate microliths or calcospherites, within the alveolar airspace. Typical imaging findings demonstrate a "sandstorm" appearance due to bilateral, interstitial sand-like micronodularities with basal predominance. METHODS ANDRESULTS:
We describe an unusual case of a 48-year-old male with severe, familial PAM ultimately treated with a bilateral lung transplant.CONCLUSIONS:
PAM is a rare lung disease caused by a mutation in the SLC34A2 gene, which encodes for a sodium-phosphate cotransporter in type II alveolar cells, leading to accumulation of intra-alveolar phosphate causing microlith formation. PAM has an indolent course but can progress to chronic hypoxic respiratory failure, ultimately requiring lung transplant, the only known effective treatment.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Idioma:
En
Revista:
Case Rep Pathol
Año:
2021
Tipo del documento:
Article
País de afiliación:
Estados Unidos