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Jeong-Hoon Song 1 Article
Pulmonary Hypertension in Patient with Neurofibromatosis Type 1
Yeong min Lee, Tae Hyun Yang, HeeSoo Jung, Tae-Seon Oh, Jeong-Hoon Song, Seung-Jung Yu
Kosin Med J. 2017;32(2):227-232.   Published online December 29, 2017
DOI: https://doi.org/10.7180/kmj.2017.32.2.227
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Abstract PDFPubReader   ePub   CrossRef-TDMCrossref - TDM

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.

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KMJ : Kosin Medical Journal