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[Pulmonary involvement in familial amyloid polyneuropathy type I].

作者信息

Díaz Lobato S, Guerrero E, González P, Crespo M, Esteban R, Villasante C

机构信息

Servicio de Neumología, Hospital La Paz.

出版信息

Rev Clin Esp. 1991 Nov;189(7):335-7.

PMID:1767091
Abstract

Familial amyloid polyneuropathy type 1 or Corino Andrade's disease (FAP 1) is classified amongst the hereditary systemic amyloidosis, being autosomically dominant and preferably affecting the peripheral nervous system. Our country is the fourth world focus with regard to incidence, preceded only by Portugal, Japan, and Sweden. Amyloid involvement is rare, generally following primary forms. There are only three cases described of these hereditary forms, none of which is of the FAP type 1. Our goal is to present a patient diagnosed of FAP type 1 who developed hemoptysis during the course of her disease, observing an amyloid bronchial infiltrate in the samples obtained by bronchoscopy. This is the first case described of pulmonary involvement in this type of amyloidosis according to the literature reviewed.

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