Renal Failure in Sisters with Cystinuria and the Management of Amino Acids in Chronic Hemodialysis

Two sisters received long-term hemodialysis to manage chronic renal failure resulting from treatment of their cystinuria. Both had progressive renal failure caused by D-penicillamine treatment after surgery for urolithiasis. These are the first reported cases involving sisters. The 34-year-old sister had received dialysis since April 1975 and her 26-year-old sister had been on dialysis since October 1977. There has been little variation in the treatment of chronic renal failure whether caused by cystinuria or by other diseases. However, we treated these sisters with cystinuria, along with other patients, by differential diet management. The balance of plasma amino acids improved remarkably after 3.5 years of treatment and both sisters are now undergoing excellent dialysis. The diet restricts methionine and crystine to 2.5 g/day.