Respiratory Medicine
| Autor: | Francisco, Flávia Angélica Ferreira, Silva, Jorge Luiz Pereira e, Hochhegger, Bruno, Zanetti, Gláucia Maria Ribeiro, Marchiori, Edson dos Santos |
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| Jazyk: | angličtina |
| Rok vydání: | 2013 |
| Předmět: | |
| Zdroj: | Repositório Institucional da UFBA Universidade Federal da Bahia (UFBA) instacron:UFBA |
| DOI: | 10.1016/j.rmed.2012.10.014 |
| Popis: | Texto completo. Acesso restrito. p. 1–9 Submitted by Santiago Fabio (fabio.ssantiago@hotmail.com) on 2013-07-09T16:37:25Z No. of bitstreams: 1 aaaaaaaaaaaaaaaa.pdf: 796358 bytes, checksum: b0cf02f9274694442e46df1cdc7d3936 (MD5) Approved for entry into archive by Rodrigo Meirelles (rodrigomei@ufba.br) on 2013-10-31T18:02:25Z (GMT) No. of bitstreams: 1 aaaaaaaaaaaaaaaa.pdf: 796358 bytes, checksum: b0cf02f9274694442e46df1cdc7d3936 (MD5) Made available in DSpace on 2013-10-31T18:02:25Z (GMT). No. of bitstreams: 1 aaaaaaaaaaaaaaaa.pdf: 796358 bytes, checksum: b0cf02f9274694442e46df1cdc7d3936 (MD5) Previous issue date: 2013 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. Salvador |
| Databáze: | OpenAIRE |
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