Total colectomy in Vascular Ehlers Danlos syndrome a case report and literature review
Sign Up to like & getrecommendations! Published in 2021 at "Annals of Medicine and Surgery"
DOI: 10.1016/j.amsu.2021.102948
Abstract: Introduction Ehlers Danlos syndromes (EDS) are a group of genetic disorders, characterized by skin hyperelasticity, joint hyperlaxity and tissue weakness. Vascular EDS is rare and is differs from other types of EDS by an inconsistent… read more here.
Keywords: perforation; danlos syndrome; case; vascular ehlers ... See more keywords
A multi-institutional experience in vascular Ehlers-Danlos syndrome diagnosis.
Sign Up to like & getrecommendations! Published in 2019 at "Journal of vascular surgery"
DOI: 10.1016/j.jvs.2019.04.487
Abstract: OBJECTIVE Vascular Ehlers-Danlos syndrome (vEDS) is a rare disorder and 1 of 13 types of EDS. The syndrome results in aortic and arterial aneurysms and dissections at a young age. Diagnosis is confirmed with molecular… read more here.
Keywords: diagnosis; vascular ehlers; multi institutional; ehlers danlos ... See more keywords
Natural History and Surgical Management of Colonic Perforations in Vascular Ehlers-Danlos Syndrome: A Retrospective Review.
Sign Up to like & getrecommendations! Published in 2019 at "Diseases of the colon and rectum"
DOI: 10.1097/dcr.0000000000001383
Abstract: BACKGROUND Vascular Ehlers-Danlos syndrome is a rare and severe genetic condition leading to spontaneous, potentially life-threatening arterial and digestive complications. Colonic ruptures are a common feature of the disease, but clear recommendations on their management… read more here.
Keywords: management; danlos syndrome; ehlers danlos; vascular ehlers ... See more keywords
The dysmorphic phenotype in vascular Ehlers Danlos syndrome
Sign Up to like & getrecommendations! Published in 2022 at "Clinical Dysmorphology"
DOI: 10.1097/mcd.0000000000000437
Abstract: The Ehlers Danlos syndromes are identified by their connective tissue features and are not rich in dysmorphic handles. Vascular Ehlers Danlos syndrome (vEDS) however, is characterised by a recognisable phenotypic constellation of internal and external… read more here.
Keywords: dysmorphic phenotype; danlos syndrome; vascular ehlers; phenotype vascular ... See more keywords
Natural history of gastrointestinal manifestations in vascular Ehlers–Danlos syndrome: A 17‐year retrospective review
Sign Up to like & getrecommendations! Published in 2019 at "Journal of Gastroenterology and Hepatology"
DOI: 10.1111/jgh.14522
Abstract: Vascular Ehlers–Danlos syndrome (vEDS) is a rare connective tissue disorder due to heterozygous mutations in the COL3A1 gene with a dominant negative effect. Spontaneous bowel perforation and intra‐abdominal organ rupture are common complications of vEDS.… read more here.
Keywords: gastrointestinal manifestations; danlos syndrome; vascular ehlers; natural history ... See more keywords
Fulminant myocardial bleeding: another clinical course of vascular Ehlers-Danlos Syndrome
Sign Up to like & getrecommendations! Published in 2017 at "BMJ Case Reports"
DOI: 10.1136/bcr-2017-220786
Abstract: Vascular Ehlers-Danlos Syndrome (vEDS) is a dominantly inherited connective tissue disorder characterised by colon rupture and arterial aneurysm, dissection and rupture. A patient was diagnosed with vEDS after a spontaneous colon rupture when he was… read more here.
Keywords: myocardial bleeding; fulminant myocardial; danlos syndrome; vascular ehlers ... See more keywords
Disappearing multiple visceral aneurysms in Vascular Ehlers-Danlos syndrome.
Sign Up to like & getrecommendations! Published in 2023 at "Vascular"
DOI: 10.1177/17085381231162126
Abstract: OBJECTIVE To assess the complex management of arterial anomalies in Vascular Ehlers-Danlos Syndrome (vEDS). METHODS We report the case of a 34-year-old male, diagnosed with vEDS, who presented with acute intraperitoneal hemorrhage caused by the… read more here.
Keywords: disappearing multiple; multiple visceral; visceral aneurysms; danlos syndrome ... See more keywords
Comparative therapeutic strategies for preventing aortic rupture in a mouse model of vascular Ehlers-Danlos syndrome
Sign Up to like & getrecommendations! Published in 2022 at "PLoS Genetics"
DOI: 10.1371/journal.pgen.1010059
Abstract: Vascular Ehlers-Danlos syndrome is a rare inherited disorder caused by genetic variants in type III collagen. Its prognosis is especially hampered by unpredictable arterial ruptures and there is no therapeutic consensus. We created a knock-in… read more here.
Keywords: therapeutic strategies; danlos syndrome; vascular ehlers; ehlers danlos ... See more keywords
Vascular Ehlers-Danlos Syndrome with a Novel Missense Mutation in COL3A1: A Man in His 50s with Aortic Dissection after Interventional Treatment for Hemothorax as the First Manifestation
Sign Up to like & getrecommendations! Published in 2019 at "Internal Medicine"
DOI: 10.2169/internalmedicine.2983-19
Abstract: Type III collagen is the major protein in the walls of blood vessels and hollow organs; it is decreased in patients with vascular Ehlers-Danlos syndrome (EDS). A 52-year-old man was admitted for severe back pain,… read more here.
Keywords: danlos syndrome; aortic dissection; vascular ehlers; man ... See more keywords
Uncommon association between vascular Ehlers–Danlos syndrome and ocular complications
Sign Up to like & getrecommendations! Published in 2023 at "Frontiers in Medicine"
DOI: 10.3389/fmed.2023.1089652
Abstract: Ehlers–Danlos syndromes (EDS) represent a group of rare inherited disorders that affect connective tissues. There are 13 types of disease, most of them affecting joints or skin; symptoms usually include loose joints, joint pain, stretchy… read more here.
Keywords: vascular ehlers; association vascular; ocular complications; uncommon association ... See more keywords