Pulmonary Hypertension in Patient with Neurofibromatosis Type 1 / 고신대학교의과대학학술지
Kosin Medical Journal
;
: 227-232, 2017.
Artigo
em Inglês
| WPRIM
| ID: wpr-60698
ABSTRACT
Neurofibromatosis type 1 (NF1) is a rare genetic disease. Precapillary pulmonary hypertension (PH) with NF1 is an extremely severe complication. A 65-year-old woman was admitted in our hospital with 3-year history of gradually worsening dyspnea on exertion (New York Heart Association functional class III-IV). Considering her clinical feature and examination findings, she could be diagnosed as PH associated with NF1. She was treated with endothelin receptor antagonist. However her dyspnea was not significantly improved. This is the first Korean case of NF1 patient with PH which confirmed with right heart catheterization.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Cateterismo Cardíaco
/
Neurofibromatose 1
/
Receptores de Endotelina
/
Neurofibromatoses
/
Dispneia
/
Cateteres Cardíacos
/
Coração
/
Concentração de Íons de Hidrogênio
/
Hipertensão Pulmonar
Limite:
Idoso
/
Feminino
/
Humanos
Idioma:
Inglês
Revista:
Kosin Medical Journal
Ano de publicação:
2017
Tipo de documento:
Artigo
Similares
MEDLINE
...
LILACS
LIS