https://repositorio.ufba.br/handle/ri/13423
Tipo: | Artigo de Periódico |
Título: | Pulmonary alveolar microlithiasis. State-of-the-art review |
Título(s) alternativo(s): | Respiratory Medicine |
Autor(es): | Francisco, Flávia Angélica Ferreira Silva, Jorge Luiz Pereira e Hochhegger, Bruno Zanetti, Gláucia Maria Ribeiro Marchiori, Edson dos Santos |
Autor(es): | Francisco, Flávia Angélica Ferreira Silva, Jorge Luiz Pereira e Hochhegger, Bruno Zanetti, Gláucia Maria Ribeiro Marchiori, Edson dos Santos |
Abstract: | 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. |
Palavras-chave: | Pulmonary alveolar microlithiasis Pulmonary diseases Congenital diseases Computed tomography |
Editora / Evento / Instituição: | Respiratory Medicine |
Tipo de Acesso: | Acesso Aberto |
URI: | http://repositorio.ufba.br/ri/handle/ri/13423 |
Data do documento: | 2013 |
Aparece nas coleções: | Artigo Publicado em Periódico (Faculdade de Medicina) |
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