Renal Transplantation for Childhood Cystinosis

Four cystinotic children with renal failure received a renal allograft from a parent. The follow-up durations for the four patients were eight, 14, 16 and 32 months; final creatinine clearances were 115, 66, 130 and 41 ml per minute per 1.73 m² respectively. No decrease in cystine crystals was visible on corneal and bone-marrow examinations. Although the allografts accumulated intracellular cystine, the distribution of cystine was different from that in the natural disease. Cystine crystals were plentiful in interstitial cells of both the patients' original and their transplanted kidneys, but crystals were found in glomerular and tubular epithelial cells only in the original kidneys. Moreover, changes in renal function correlated with complications of transplantation and clinical rejection episodes rather than with the amount of accumulated cystine. So far the Fanconi syndrome does not appear to be developing, and all patients have returned to full activity.