Vascular phenotypes in nonvascular subtypes of the Ehlers-Danlos syndrome: a systematic review.

ABSTRACT: PurposeWithin the spectrum of the Ehlers-Danlos syndromes (EDS), vascular complications are usually associated with the vascular subtype of EDS. Vascular complications are also observed in other EDS subtypes, but the reports are anecdotal and the information is dispersed. To better document the nature of vascular complications among "nonvascular" EDS subtypes, we performed a systematic review.MethodsWe queried three databases for English-language studies from inception until May 2017, documenting both phenotypes and genotypes of patients with nonvascular EDS subtypes. The outcome included the number and nature of vascular complications.ResultsA total of 112 papers were included and data were collected from 467 patients, of whom 77 presented with a vascular phenotype. Severe complications i

SUBMITTER: D'hondt S 

PROVIDER: S-EPMC5993673 | biostudies-literature | 2018 Jun

REPOSITORIES: biostudies-literature