Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome
Ehlers-Danlos syndrome (EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Critical Care |
| Online Access: | http://dx.doi.org/10.1155/2015/804252 |
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| _version_ | 1832553199102328832 |
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| author | Filipa Pereira Teresa Cardoso Paula Sá |
| author_facet | Filipa Pereira Teresa Cardoso Paula Sá |
| author_sort | Filipa Pereira |
| collection | DOAJ |
| description | Ehlers-Danlos syndrome (EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome. |
| format | Article |
| id | doaj-art-6b7847a22c9c4710b5c6b05caabaa431 |
| institution | Kabale University |
| issn | 2090-6420 2090-6439 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Critical Care |
| spelling | doaj-art-6b7847a22c9c4710b5c6b05caabaa4312025-02-03T05:55:18ZengWileyCase Reports in Critical Care2090-64202090-64392015-01-01201510.1155/2015/804252804252Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos SyndromeFilipa Pereira0Teresa Cardoso1Paula Sá2Serviço de Anestesiologia, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalUnidade de Cuidados Intensivos Polivalente, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalServiço de Anestesiologia, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, PortugalEhlers-Danlos syndrome (EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome.http://dx.doi.org/10.1155/2015/804252 |
| spellingShingle | Filipa Pereira Teresa Cardoso Paula Sá Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome Case Reports in Critical Care |
| title | Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome |
| title_full | Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome |
| title_fullStr | Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome |
| title_full_unstemmed | Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome |
| title_short | Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome |
| title_sort | spontaneous dissection of the renal artery in vascular ehlers danlos syndrome |
| url | http://dx.doi.org/10.1155/2015/804252 |
| work_keys_str_mv | AT filipapereira spontaneousdissectionoftherenalarteryinvascularehlersdanlossyndrome AT teresacardoso spontaneousdissectionoftherenalarteryinvascularehlersdanlossyndrome AT paulasa spontaneousdissectionoftherenalarteryinvascularehlersdanlossyndrome |