Begin typing your search above and press return to search.
Volume: 4 Issue: 2 December 2006 - Supplement - 1

FULL TEXT

RENAL TRANSPLANTATION IN ALPORT SYNDROME

Alport syndrome is a hereditary nephritis with progressive glomerular disease, hearing loss and ocular abnormalities with prevalence of 1 in 50,000 live births and 2% in new cases of end stage renal disease. Three to 4 percent of these patients develop de novo anti-GBM antibody disease after transplant (Tx). Affected patients are at risk (up to 75 percent) for crescentic glomerulonephritis and graft loss especially during one year after Tx. We planned a study to evaluate the survival rates in our transplanted Alport patients. From 1986 to January 2005, a total of 1760 renal Txs were performed at Hasheminejad Hospital. There were 10 cases of Alport syndrome (9 males and one female) in this group. Diagnostic criteria were based on clinical findings of neurologic deafness, positive family history and renal involvement in 9 cases and renal biopsy in one case. Data analyzed by Spss program and Kaplan-Meier test for survival analysis. The mean age of patients was 25.9± 8.3 years. The source of kidney was from living unrelated in 11 Txs and living related in one Tx. Two patients received second Txs. One and 8 years graft survival were 83.3% and 71.4% respectively. Two of three graft losses occurred in three months after Tx. Patient survival was 91.7% in one and 10 years after Tx. In our last center report the 8 years graft survival was 77 % and 10 years patient survival was 75.5%. Our data showed in spite of better patient survival in Alport syndrome patients, our graft survival is a lower than our general Tx population with higher risk of graft loss in first year after Tx. We conclude that anti GBM level should be checked regularly in this group after Tx and in the cases with Anti GBM disease and graft loss retransplantation carried out under specific conditions.



Volume : 4
Issue : 2
Pages : 97


PDF VIEW [1214] KB.