دورية أكاديمية
Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1)
| العنوان: | Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1) |
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| المؤلفون: | Seiya Tanaka, Fuko Kawahara, Taro Miyamoto, Satoshi Tsurusaki, Yoshihito Sanuki, Kiyoshi Ozumi, Takashi Harada, Hiromi Tasaki |
| المصدر: | Case Reports in Cardiology, Vol 2019 (2019) |
| بيانات النشر: | Hindawi Limited, 2019. |
| سنة النشر: | 2019 |
| المجموعة: | LCC:Diseases of the circulatory (Cardiovascular) system |
| مصطلحات موضوعية: | Diseases of the circulatory (Cardiovascular) system, RC666-701 |
| الوصف: | A 56-year-old woman was diagnosed as having chronic obstructive pulmonary disease with heavy smoking. Mild pulmonary hypertension (mean pulmonary arterial pressure: 31 mmHg) was detected at the first visit. She was diagnosed with pulmonary hypertension due to pulmonary disease and medicated only with bronchodilators. Simultaneous, multiple freckling in the trunk of her body and café au lait macules in her back with some cutaneous neurofibromas were also detected. A plastic surgeon removed one of the neurofibromas and pathologically diagnosed it as neurofibromatosis type 1 (NF1). We finally rediagnosed her with pulmonary hypertension with unclear and/or multifactorial factors when she deteriorated 1 year after being treated only with bronchodilators. We then administrated upfront combination therapy with macitentan and tadalafil. Mean pulmonary arterial pressure rapidly improved. Learning Objective. Pulmonary arterial hypertension (PAH) in neurofibromatosis type 1 (NF1) can occur due to lung disease or due to certain involvement of pulmonary arteries, or a combination of both. Increased awareness of PAH in NF1 is very important for patients survival. The current therapeutic strategy is almost identical to that of idiopathic PAH; however, there is no clinical evidence. Insights gained from clinical experiences should help identify promising novel therapeutic approaches in NF1-PAH. |
| نوع الوثيقة: | article |
| وصف الملف: | electronic resource |
| اللغة: | English |
| تدمد: | 2090-6404 2090-6412 |
| Relation: | https://doaj.org/toc/2090-6404; https://doaj.org/toc/2090-6412 |
| DOI: | 10.1155/2019/2987461 |
| URL الوصول: | https://doaj.org/article/0d71100e5883498899a8d0067e1d029b |
| رقم الأكسشن: | edsdoj.0d71100e5883498899a8d0067e1d029b |
| قاعدة البيانات: | Directory of Open Access Journals |
Full Text Finder | تدمد: | 20906404 20906412 |
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| DOI: | 10.1155/2019/2987461 |