children s health issues
Cystinuria
Treatment of Cystinuria
Increasing fluid intake Medications to make the urine more alkaline Medications to dissolve cystine Decreasing salt and animal protein in the diet Treatment of cystinuria consists of preventing cystine stones from forming by keeping the concentration of cystine in the urine low. To keep the cystine concentration low, a person must drink enough fluids to produce at least 6 to 8½ pints (3 to 4 liters) of urine each day. During the night, however, when the person is not drinking, less urine is produced and stone formation is more likely. This risk is reduced by drinking fluids before going to bed. Another treatment approach involves takingpotassium citrateorpotassium bicarbonateand sometimesacetazolamideto make the urine more alkaline (that is, less acidic) because cystine dissolves more easily in alkaline urine than in acidic urine. Efforts to increase intake of water and make the urine more alkaline can lead to abdominal bloating, making the treatment difficult for some people to tolerate. Consuming less salt and animal protein may help reduce the concentration of cystine in the urine. If stones continue to form despite these measures, medications such aspenicillamine,tiopronin, orcaptoprilmay be tried. These drugs react with cystine to keep it dissolved. is effective in keeping the concentration of cystine in the urine low but it can be toxic, so doctors give Vitamin B6 Deficiency ( Tiopronincan be used instead ofpenicillamineto treat some children because it has a lower frequency of side effects. Captoprilis slightly less effective thanpenicillaminebut is less toxic. Although the medications are usually effective, there is a fairly high risk that stones will continue to form. 