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Hémothorax spontané: complication rare de la neurofibromatose type 1
Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen’s disease is an autosomal dominant genetic disorder. It is the most common of phacomatoses. Pulmonary complications have been rarely described in the literature. Vascular complications have been reported in 3.6% of patients. We here re...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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The African Field Epidemiology Network
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5724956/ https://www.ncbi.nlm.nih.gov/pubmed/29255555 http://dx.doi.org/10.11604/pamj.2017.28.85.13820 |
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author | Fdil, Soumia Bouchikhi, Saad Bourkadi, Jamal-Eddine |
author_facet | Fdil, Soumia Bouchikhi, Saad Bourkadi, Jamal-Eddine |
author_sort | Fdil, Soumia |
collection | PubMed |
description | Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen’s disease is an autosomal dominant genetic disorder. It is the most common of phacomatoses. Pulmonary complications have been rarely described in the literature. Vascular complications have been reported in 3.6% of patients. We here report the case of a 38-year old female patient, followed-up for neurofibromatosis type 1, admitted to the Emergency Department with hemorrhagic shock. Clinical examination showed several coffee-with-milk colored spots, many plexiform neurofibromas, left-sided pleural effusion syndrome. Pleural puncture objectified coagulable haemorrhagic fluid. The patient received transfusion and emergency chest drainage. Patient’s assessment was completed by angioscanner which showed no pulmonary embolism or other associated lesions. Spontaneous hemothorax is a rare and severe complication of neurofibromatosis. It is probably due to vascular injury caused by this disease. |
format | Online Article Text |
id | pubmed-5724956 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | The African Field Epidemiology Network |
record_format | MEDLINE/PubMed |
spelling | pubmed-57249562017-12-18 Hémothorax spontané: complication rare de la neurofibromatose type 1 Fdil, Soumia Bouchikhi, Saad Bourkadi, Jamal-Eddine Pan Afr Med J Case Report Neurofibromatosis type 1 (NF1), also known as Von Recklinghausen’s disease is an autosomal dominant genetic disorder. It is the most common of phacomatoses. Pulmonary complications have been rarely described in the literature. Vascular complications have been reported in 3.6% of patients. We here report the case of a 38-year old female patient, followed-up for neurofibromatosis type 1, admitted to the Emergency Department with hemorrhagic shock. Clinical examination showed several coffee-with-milk colored spots, many plexiform neurofibromas, left-sided pleural effusion syndrome. Pleural puncture objectified coagulable haemorrhagic fluid. The patient received transfusion and emergency chest drainage. Patient’s assessment was completed by angioscanner which showed no pulmonary embolism or other associated lesions. Spontaneous hemothorax is a rare and severe complication of neurofibromatosis. It is probably due to vascular injury caused by this disease. The African Field Epidemiology Network 2017-09-27 /pmc/articles/PMC5724956/ /pubmed/29255555 http://dx.doi.org/10.11604/pamj.2017.28.85.13820 Text en © Soumia Fdil et al. http://creativecommons.org/licenses/by/2.0/ The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Fdil, Soumia Bouchikhi, Saad Bourkadi, Jamal-Eddine Hémothorax spontané: complication rare de la neurofibromatose type 1 |
title | Hémothorax spontané: complication rare de la neurofibromatose type 1 |
title_full | Hémothorax spontané: complication rare de la neurofibromatose type 1 |
title_fullStr | Hémothorax spontané: complication rare de la neurofibromatose type 1 |
title_full_unstemmed | Hémothorax spontané: complication rare de la neurofibromatose type 1 |
title_short | Hémothorax spontané: complication rare de la neurofibromatose type 1 |
title_sort | hémothorax spontané: complication rare de la neurofibromatose type 1 |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5724956/ https://www.ncbi.nlm.nih.gov/pubmed/29255555 http://dx.doi.org/10.11604/pamj.2017.28.85.13820 |
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