Article ; Online: Neurofibromatosis type 1 and pulmonary arterial hypertension: A case report.
2022 Volume 41, Issue 6, Page(s) 511.e1–511.e5
Abstract: Neurofibromatosis type 1 (NF1) is a common autosomal dominant genetic disorder that affects multiple organ systems and has a wide range of clinical manifestations. Pulmonary hypertension (PH) associated with NF1 (PH-NF1) is rarely seen, but confers a ... ...
Abstract | Neurofibromatosis type 1 (NF1) is a common autosomal dominant genetic disorder that affects multiple organ systems and has a wide range of clinical manifestations. Pulmonary hypertension (PH) associated with NF1 (PH-NF1) is rarely seen, but confers a dismal prognosis. In the literature this association has been described in only 31 cases. The authors report the case of a 77-year-old female patient with NF1 complicated by severe precapillary PH despite triple disease-specific oral combination therapy. Because no data are available on the efficacy of specific PH therapy in PH-NF1, these patients should be assessed in expert PH centers and referred for lung transplantation at an early stage. |
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Language | Portuguese |
Publishing date | 2022-05-20 |
Publishing country | Portugal |
Document type | Case Reports |
ZDB-ID | 632718-7 |
ISSN | 2174-2030 ; 0870-2551 ; 0304-4750 |
ISSN (online) | 2174-2030 |
ISSN | 0870-2551 ; 0304-4750 |
DOI | 10.1016/j.repc.2019.05.018 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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