title: Successful kidney transplantation from donor with Marfan's syndrome.
abstract: Marfan's syndrome is caused by mutations in the extracellular matrix protein fibrillin-1 with aortic aneurysm and dissection being its most life-threatening manifestations. Kidney transplantation from donors with Marfan's syndrome has never been reported in the literature, possibly because of reticences due to the underlying connective tissue disease. Here, we report two patients with end-stage renal disease, transplanted with the kidneys from a donor with Marfan's syndrome who died of aortic dissection and cerebral hemorrhage. After delayed graft function in both recipients, renal function normalized with no renovascular complications and negative proteinuria for 6 years in one patient and 2 years in the other patient, who died from an ischemic cerebrovascular insult. Kidneys from organ donors with Marfan's syndrome might be suitable for transplantation.
authors.affiliation: Clinic and Policlinic of Nephrology and Hypertension, University Hospital Inselspital, Berne, Switzerland.
authors.forename: S
authors.initials: S
authors.lastname: Farese
authors.forename: B
authors.initials: B
authors.lastname: Vogt
authors.forename: F J
authors.initials: FJ
authors.lastname: Frey
authors.forename: U
authors.initials: U
authors.lastname: Huynh-Do
mesh_terms: D000328:Adult; D002102:Cadaver; D005500:Follow-Up Studies; D006801:Humans; D016030:Kidney Transplantation / Q000009:adverse effects; D008297:Male; D008382:Marfan Syndrome / Q000601:surgery*; D008875:Middle Aged; D014019:Tissue Donors