Microlitíase alveolar pulmonar em gêmeos univitelinos: relato de dois casos

Pulmonary alveolar microlithiasis (PAM) is a rare disease characterised by the widespread intra-alveolar accumulation of minute calculi called microliths. It is caused by mutation of the SLC34A2 gene encoding the type IIb sodium phosphate cotransporter in alveolar type II cells. The present study explores the epidemiological, familial, genetic, clinical, diagnostic, radiological and therapeutic aspects with the aim of contributing to a better understanding of this uncommon disease.We searched articles on PAM published up to December 2014 and 544 papers were found, accounting for 1022 cases.PAM is present in all continents and in many nations, in particular in Turkey, China, Japan, India, Italy and the USA. Familiality is frequent. The clinical course is not uniform and the causes of this clinical variability seem to be largely nongenetic. The optimal diagnostic procedure is the association of chest high-resolution computed tomography (HRCT) with bronchoalveolar lavage, but a chest radiograph may suffice in families in which a case has already been diagnosed. Moreover, chest radiography and HRCT allow the classification of the evolutionary phase of the disease and its severity. At present lung transplantation is the only effective therapy. However, better knowledge of the gene responsible offers hope for new therapies. @ERSpublications Familial, genetic, clinical, radiological and therapeutic aspects of pulmonary alveolar microlithiasis epidemiology http://ow.ly/St3Mw

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Pulmonary alveolar microlithiasis: review of the 1022 cases reported worldwide

Castellana

et al. 2015

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Microlitíase alveolar pulmonar: achados na tomografia computadorizada de alta resolução do tórax em 10 pacientes

et al. 2007

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Microlitíase alveolar pulmonar em gêmeos univitelinos: relato de dois casos

Abstract: Os autores apresentam dois casos de microlitíase alveolar pulmonar em gêmeos monozigóticos. Os principais achados obtidos em exames radiográficos e de tomografia computadorizada (técnica de alta resolução) são enfatizados, com base em breve revisão literária.

Cited by 2 publications

References 11 publications

Pulmonary alveolar microlithiasis: review of the 1022 cases reported worldwide

Pulmonary alveolar microlithiasis: review of the 1022 cases reported worldwide

Microlitíase alveolar pulmonar: achados na tomografia computadorizada de alta resolução do tórax em 10 pacientes

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