Cystine accumulation disorder is a genetic defect of the renal tubules. The reabsorption of dibasic amino acids (lysine, arginine, ornithine) is also affected, but does not cause symptoms because they share a transport channel with cystine, in addition to another transport system. They are also more soluble in urine than cystine and thus do not cause crystallization and stone formation. Their absorption (including cystine) in the small intestine is also reduced. Renal aminoaciduria is a group of inherited membrane transport deficiencies that result in the excretion of large amounts of amino acids from the urine due to impaired reabsorption of amino acids by the proximal renal tubules. Diagnostic criteria: 1. Family history of hereditary disease. 2, Signs and symptoms of kidney stones, such as colic, hematuria, urinary tract obstruction and/or urinary tract infection. 3, Recurrent cystine stones in the urinary tract with hexagonal flat crystals visible on urine sediment microscopy. 4, KUB X-ray plain film shows multiple, thinly shadowed, variable size stones in the urinary tract bilaterally. 5, Positive urine nitrohydrocyanate test can confirm the diagnosis. The most common symptom of cystinuria is renal colic, which usually occurs between the ages of 10 and 30. It can cause urinary sensation and renal failure due to urinary tract obstruction. Radiopaque cystine stones occur in the renal pelvis or bladder. Antler-shaped stones are common, with cystine forming yellowish-brown, hexagonal crystals in the urine. Excess cystine in the urine can be detected by the nitroprusside cyanide test. Chromatography and electrophoresis can further clarify the diagnosis.