Resumo

PEREZ-BEJARANO, Domingo. Family pulmonary fibrosis in Hermansky-Pudlak syndrome. Mem. Inst. Investig. Cienc. Salud [online]. 2022, vol.20, n.3, pp.142-147. ISSN 1812-9528.  https://doi.org/10.18004/mem.iics/1812-9528/2022.020.03.142.

Family pulmonary fibrosis in the context of Hermansky-Pudlak syndrome is a rare nosological entity whose only definitive therapeutic approach is lung transplantation in the advanced stages. We describe some diagnostic and therapeutic clinical aspects that shape the management of orphan diseases in countries like ours.

Palavras-chave : Family pulmonary fibrosis; Hermansky-Pudlak syndrome; interstitial idiopathic pneumonia; oculocutaneous albinism.

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