title: Familial occurrence of membranous obstruction of the inferior vena cava: arguments in favor of a congenital etiology.
abstract: Membranous obstruction of the inferior vena cava is a rare disease. The etiology of the membrane is believed to be thrombotic or congenital. In three of 11 siblings from a single family, symptoms of membranous obstruction of the inferior vena cava developed during early adult life. All had signs of more long-standing disease, as judged by the presence of collaterals, cirrhosis and, in one case, hepatocellular carcinoma. On family screening no further cases of membranous obstruction of the inferior vena cava were found. There was also no evidence of inherited defects in the natural coagulation inhibitors (protein C, protein S and antithrombin III) and plasminogen deficiency. This familial occurrence of membranous obstruction of the inferior vena cava supports a congenital etiology, although a thrombotic etiology cannot be totally excluded.
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Groningen, The Netherlands.
authors.forename: S C
authors.initials: SC
authors.lastname: Riemens
authors.forename: E B
authors.initials: EB
authors.lastname: Haagsma
authors.forename: T
authors.initials: T
authors.lastname: Kok
authors.forename: A S
authors.initials: AS
authors.lastname: Gouw
authors.forename: E J
authors.initials: EJ
authors.lastname: van der Jagt
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000792:Angiography; D015901:Angiography, Digital Subtraction; D006502:Budd-Chiari Syndrome / Q000235:genetics; D002648:Child; D005260:Female; D005820:Genetic Testing; D006801:Humans; D008297:Male; D008875:Middle Aged; D010690:Phlebography; D014652:Vascular Diseases / Q000151:congenital* / Q000000981:diagnostic imaging / Q000235:genetics*; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging