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Cystine is an amino acid generated by the union of two cysteine molecules, and is the chief sulfur-containing compound in protein. Cystinosis is a condition in which the body accumulates the amino acid cystine within cells forming crystals that can build up and damage the cells. These crystals negatively affect many systems in the body, especially the kidneys and eyes. Cystinosis is the most common cause of Fanconi syndrome which occurs when the function of the cells in renal tubules are impaired, leading to abnormal amounts of carbohydrates and amino acids in the urine, excessive urination, and low blood levels of potassium and phosphates. This test is used to diagnose cystinosis, since it is characterized by excessive amounts of cystine in urine.
Do not add acid or other preservatives to the sample.