Recurrence of Fabryʼs Disease in a Renal Allograft Eleven Years After Successful Renal Transplantation

Recurrence of Fabry's disease in a renal allograft 14 years after transplantation

“…Light-microscopy showed no abnormalities, but electron-microscopy revealed occasional small myelin figures in the vascular endothelium compatible with early accumulation of ceramid-trihexosid. Mosnier et al [5] also described a case in which autopsy was performed II years after successful transplantation. Sphingolipid inclusions were found in endothelial cells of capillaries by electronmicroscopy.…”

“…We found only four other cases in the literature [4][5][6][7]. In 1981 we reported a case from our centre in which, 14 years after successful transplantation, no signs of de novo Fabry's disease in the transplanted kidney could be found [8].…”

The Evaluation of the Renal Transplant Candidates: Clinical Practice Guidelines

“…It was initially hoped that kidney transplantation would provide sufficient enzyme to prevent disease progression. However, this proved not to be the case (410), and it is clear that the disease can recur in the transplanted kidney (411)(412)(413)(414). In addition, the disease appears to progress in other organs as well (413,415).…”

“…However, this proved not to be the case (410), and it is clear that the disease can recur in the transplanted kidney (411)(412)(413)(414). In addition, the disease appears to progress in other organs as well (413,415). However, histologic recurrences have mostly been documented several years after transplantation (411)(412)(413)(414), and long-term graft survival is common (416)(417)(418).…”

“…In addition, the disease appears to progress in other organs as well (413,415). However, histologic recurrences have mostly been documented several years after transplantation (411)(412)(413)(414), and long-term graft survival is common (416)(417)(418). Most recurrences have been associated with few renal manifestations, e.g.…”