Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1

Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic disorder with an incidence of approximately 1 in 4,000 live births ([1]). Pulmonary arterial hypertension (PAH) is a rare but extremely life-threatening complication associated with NF1. Timely recognition of this unusual and severe ass...

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Autores principales: Rojas, Melanie, Mubarik, Ateeq, Henderson, Elizabeth Ann, Agha, Fatima, Chauhan, Lakshpaul, Iqbal, Arshad Muhammad, Vaziri, Ali, Muddassir, Salman
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2019
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451192/
https://www.ncbi.nlm.nih.gov/pubmed/30997327
http://dx.doi.org/10.1016/j.rmcr.2019.100832
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author Rojas, Melanie
Mubarik, Ateeq
Henderson, Elizabeth Ann
Agha, Fatima
Chauhan, Lakshpaul
Iqbal, Arshad Muhammad
Vaziri, Ali
Muddassir, Salman
author_facet Rojas, Melanie
Mubarik, Ateeq
Henderson, Elizabeth Ann
Agha, Fatima
Chauhan, Lakshpaul
Iqbal, Arshad Muhammad
Vaziri, Ali
Muddassir, Salman
author_sort Rojas, Melanie
collection PubMed
description Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic disorder with an incidence of approximately 1 in 4,000 live births ([1]). Pulmonary arterial hypertension (PAH) is a rare but extremely life-threatening complication associated with NF1. Timely recognition of this unusual and severe association between NF1 and PAH is imperative in prolonging the survival in this specific patient population. We present the clinical outcomes of a 47-year old female previously diagnosed with NF1, who presented with progressively worsening dyspnea.
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spelling pubmed-64511922019-04-17 Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1 Rojas, Melanie Mubarik, Ateeq Henderson, Elizabeth Ann Agha, Fatima Chauhan, Lakshpaul Iqbal, Arshad Muhammad Vaziri, Ali Muddassir, Salman Respir Med Case Rep Article Neurofibromatosis Type 1 (NF1) is an autosomal dominant genetic disorder with an incidence of approximately 1 in 4,000 live births ([1]). Pulmonary arterial hypertension (PAH) is a rare but extremely life-threatening complication associated with NF1. Timely recognition of this unusual and severe association between NF1 and PAH is imperative in prolonging the survival in this specific patient population. We present the clinical outcomes of a 47-year old female previously diagnosed with NF1, who presented with progressively worsening dyspnea. Elsevier 2019-04-01 /pmc/articles/PMC6451192/ /pubmed/30997327 http://dx.doi.org/10.1016/j.rmcr.2019.100832 Text en © 2019 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Article
Rojas, Melanie
Mubarik, Ateeq
Henderson, Elizabeth Ann
Agha, Fatima
Chauhan, Lakshpaul
Iqbal, Arshad Muhammad
Vaziri, Ali
Muddassir, Salman
Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1
title Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1
title_full Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1
title_fullStr Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1
title_full_unstemmed Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1
title_short Pulmonary arterial hypertension: A rare yet fatal complication of Neurofibromatosis Type 1
title_sort pulmonary arterial hypertension: a rare yet fatal complication of neurofibromatosis type 1
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451192/
https://www.ncbi.nlm.nih.gov/pubmed/30997327
http://dx.doi.org/10.1016/j.rmcr.2019.100832
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