title: [The Elhers-Danlos syndrome: an insidious pathology. Presentation of a familiary group].
abstract: BACKGROUND
  Elhers-Danlos Syndrome (EDS) is an hereditary disease of the connective tissue, which may have clinical appearances varying in its severity from subclinical asymptomatic forms up to dramatic uncontrollable haemorrhage.
  
  
  METHODS
  We have observed three siblings who were carriers of vascular type EDS. They were otherwise healthy at the time of the disease presentation and have become symptomatic since then, developing severe and uncontrollable haemorrhage.
  
  
  RESULTS
  We have reported the clinical presentation of this syndrome, the clinical and laboratory characteristics, the treatment and the outcome.
  
  
  CONCLUSIONS
  Although the severity of this disease, Elhers-Danlos Syndrome becomes symptomatic lately and suddenly in its course, often during emergency surgery for haemorrhagic shock. Several issues are raised such as the possibility for early diagnosis of this disease, the psychological features of a genetic counseling, the need for the patients to have adequate lifestyle and last but not least the risk of genetic transmission of the disease.
authors.affiliation: U.O.C Chirurgia d'Urgenza e del Trauma, Ospedale Maggiore, Bologna, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Giordani
authors.forename: Gregorio
authors.initials: G
authors.lastname: Tugnoli
authors.forename: Salomone
authors.initials: S
authors.lastname: di Saverio
authors.forename: Silvia
authors.initials: S
authors.lastname: Villani
authors.forename: Andrea
authors.initials: A
authors.lastname: Biscardi
authors.forename: Gianluca
authors.initials: G
authors.lastname: Senatore
authors.forename: Eleonora
authors.initials: E
authors.lastname: Giorgini
authors.forename: Franco
authors.initials: F
authors.lastname: Baldoni
mesh_terms: D000328:Adult; D004535:Ehlers-Danlos Syndrome / Q000175:diagnosis* / Q000235:genetics*; D005260:Female; D006801:Humans; D008297:Male