Cystinuria

These high levels of cystine lead to the formation of cystine stones in the kidneys, ureter and bladder. These cystine stones generate various complications, such as hypertension, intense abdominal pain, recurrent urinary tract infections, renal function impairment in up to 70% of patients and chronic renal insufficiency. These complications can ultimately lead to renal failure.

Of the available alkalizing therapies for the management of cystinuria, citrate is the intervention of choice as citrate excretion in urine potentially prevents calcium salt crystallization in alkali conditions (over a pH of 7.5). Potassium salts do not lead to the adverse consequences observed with sodium salts, which had been historically used as an alkalizing therapy. Moreover, it is recommended that potassium deficiency be avoided in cystinuric patients since this condition may decrease citrate excretion and cause renal acidosis, a buildup of acids in the blood.