ABSTRACT
Cystinuria is an autosomal recessive defect in reabsorptive transport of cystine and the dibasic amino acids ornithine, arginine, and lysine from the luminal fluid of the renal proximal tubule and small intestine. The clinical manifestation of cystinuria is cystine urolithiasis, which often recurs throughout a patient's lifetime. Surgical intervention is often necessary to treat the calculi.
