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.

CONCLUSION:

Although liver transplantation in FAP does not affect existing renal amyloid deposits, it prevents the progression of renal disease.

• Authors: Renaud Snanoudj, Antoine Durrbach, Eric Gauthier, David Adams, Didier Samuel, Sophie Ferlicot, Pierre Bedossa, Alain Prigent, Henri Bismuth, Bernard Charpentier
• Journal: Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association (Nephrol Dial Transplant) Vol. 19 Issue 7 Pg. 1779-85 (Jul 2004) ISSN: 0931-0509 [Print] England
• PMID: 15150345 (Publication Type: Journal Article)

Topics

• Adult
• Amyloid Neuropathies, Familial (pathology, physiopathology, surgery)
• Biopsy
• Female
• Follow-Up Studies
• Humans
• Kidney (pathology, physiopathology)
• Liver Transplantation
• Male
• Middle Aged