title: Changes in renal function in patients with familial amyloid polyneuropathy treated with orthotopic liver transplantation.
abstract: BACKGROUND
  Familial amyloid polyneuropathy (FAP) is an autosomal dominant disease caused by a point mutation in the gene encoding transthyretin, which is secreted by the liver. Orthotopic liver transplantation (OLT) has been proposed to prevent disease progression. Little is known about long-term changes in renal function and lesions after OLT.
  
  
  METHODS
  The renal function of 33 patients with FAP was evaluated (proteinuria, serum creatinine, creatinine clearance) before OLT and over a period of at least 5 years afterwards. A pre-transplantation renal biopsy was performed in 14 patients and a follow-up biopsy in eight patients.
  
  
  RESULTS
  Before transplantation, mean serum creatinine concentration was 86 micromol/l (47-126 micromol/l) and creatinine clearance was 71.9+/-31.6 ml/min/1.73 m(2). Proteinuria was detected in 54% of patients (0.3-4 g/day). Pre-transplant renal biopsies (n = 14) revealed glomerular, tubular and vascular amyloid deposits in 90, 58 and 66% of patients, respectively. Eleven patients (33%) died after OLT. Death occurred most frequently in patients having weight losses >7 kg (P<0.05). After transplantation, 25 patients (76%) suffered acute renal failure but only one required dialysis. One month after transplantation, the mean serum creatinine concentration was 134.1+/-73 micromol/l and remained constant during follow-up. Eight patients underwent a second renal biopsy 2 years after transplantation. No significant changes in deposits or renal toxicity due to calcineurin inhibitors were detected.
  
  
  CONCLUSIONS
  Although liver transplantation in FAP does not affect existing renal amyloid deposits, it prevents the progression of renal disease.
authors.affiliation: Service de Néphrologie, Hémodialyse et Transplantation, Villejuif, France.
authors.forename: Renaud
authors.initials: R
authors.lastname: Snanoudj
authors.forename: Antoine
authors.initials: A
authors.lastname: Durrbach
authors.forename: Eric
authors.initials: E
authors.lastname: Gauthier
authors.forename: David
authors.initials: D
authors.lastname: Adams
authors.forename: Didier
authors.initials: D
authors.lastname: Samuel
authors.forename: Sophie
authors.initials: S
authors.lastname: Ferlicot
authors.forename: Pierre
authors.initials: P
authors.lastname: Bedossa
authors.forename: Alain
authors.initials: A
authors.lastname: Prigent
authors.forename: Henri
authors.initials: H
authors.lastname: Bismuth
authors.forename: Bernard
authors.initials: B
authors.lastname: Charpentier
mesh_terms: D000328:Adult; D028227:Amyloid Neuropathies, Familial / Q000473:pathology / Q000503:physiopathology* / Q000601:surgery*; D001706:Biopsy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007668:Kidney / Q000473:pathology / Q000503:physiopathology*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged