NDT Advance Access originally published online on May 18, 2004
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Nephrol Dial Transplant (2004) 19: 1779-1785
Nephrol Dial Transplant Vol. 19 No. 7 © ERA-EDTA 2004; all rights reserved
Original Article
Changes in renal function in patients with familial amyloid polyneuropathy treated with orthotopic liver transplantation
1 Service de Néphrologie, Hémodialyse et Transplantation, 2 Service de Neurologie, 3 Service Anatomopathologie and 4 Service de Biophysique, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France, 5 INSERM U542, Villejuif and 6 Service d’Hépatologie, Hôpital Paul Brousse, Villejuif, France
Correspondence and offprint requests to: A. Durrbach, MD, PhD, Nephrology Unit, Le Kremlin Bicetre Hospital, 78 rue du General Leclerc, 94270 Le Kremlin Bicetre, France. Email: antoine.durrbach{at}bct.ap-hop-paris.fr
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 µmol/l (47–126 µmol/l) and creatinine clearance was 71.9±31.6 ml/min/1.73 m2. 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 µmol/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.
Keywords: amylosis; familial amyloid polyneuropathy; liver transplantation; renal failure; survival
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