Artigo de Periódico
Pulmonary alveolar microlithiasis. State-of-the-art review
Fecha
2013Registro en:
0954-6111
v. 107, n. 1
Autor
Francisco, Flávia Angélica Ferreira
Silva, Jorge Luiz Pereira e
Hochhegger, Bruno
Zanetti, Gláucia Maria Ribeiro
Marchiori, Edson dos Santos
Francisco, Flávia Angélica Ferreira
Silva, Jorge Luiz Pereira e
Hochhegger, Bruno
Zanetti, Gláucia Maria Ribeiro
Marchiori, Edson dos Santos
Institución
Resumen
Pulmonary alveolar microlithiasis (PAM) is a rare genetic lung disease characterized by calcifications
within the alveoli. Mutations in the SLC34A2 gene, which encodes a type IIb sodiumphosphate
cotransporter, are responsible for this disease, leading to intra-alveolar accumulation
of phosphate that favors the formation of microliths. The hallmark of this disorder is
clinical-radiological dissociation, with typical imaging findings that correlate well with specific
pathological findings. The long-term prognosis is poor and no treatment has been discovered to
date. The aim of this review is to describe the main pathological, clinical, and imaging aspects
of PAM, ranging from its genetic basis to treatment.