Fibrosis pulmonar familiar en el síndrome de Hermansky-Pudlak
DOI:
https://doi.org/10.18004/mem.iics/1812-9528/2022.020.03.142Keywords:
Fibrosis pulmonar familiar, síndrome de Hermansky-Pudlak, neumonía intersticial idiopática, albinismo oculo-cutáneoAbstract
La fibrosis pulmonar familiar en el contexto del síndrome de Hermansky-Pudlak es una entidad nosológica poco frecuente y cuyo único enfoque terapéutico definitivo es el trasplante pulmonar en los estadios avanzados. Describimos algunos aspectos clínicos diagnósticos y terapéuticos que configuran el manejo de las enfermedades huérfanas en países como el nuestro.
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