Xanthinuria Treatment & Management

Updated: Dec 30, 2020
  • Author: Sahar Fathallah-Shaykh, MD; Chief Editor: Craig B Langman, MD  more...
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Treatment

Medical Care

Alkalinization of the urine has little effect on the solubility of xanthine.

No specific therapies are available for classic xanthinuria; however, some general measures are recommended as follows:

  • High fluid intake: A reasonable goal for fluid intake is 1.5-2 times maintenance spaced over 24 hours. Drinking water at night is important to prevent the usual development of a concentrated morning urine.

  • Dehydration prevention: Educate the patient about the importance of preventing dehydration.

  • Low-purine diet: Sources of food with lower purine content include cheese and eggs, grains, fruits, nuts, and most vegetables. The foods that contain higher amounts of purines are beef, pork, poultry, seafood, liver, kidney, and heart as well as peas, beans, spinach, and lentils.

  • Xanthine dehydrogenase and aldehyde oxidase metabolize certain medications, and the enzyme-deficient or inhibited state can lead to toxic accumulation of the parent drug. Xanthine dehydrogenase is involved in degradation of azathioprine or 6-mercaptopurine, and aldehyde oxidase metabolizes allopurinol, cyclophosphamide, methotrexate, and quinine.

Consultation with a nephrologist is helpful.

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Surgical Care

Xanthine stones are sensitive to extracorporeal shockwave lithotripsy. Consultation with a urologist is warranted because other techniques are available for stone removal.

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Diet and Activity

Diet

Recommend a low-purine diet (see Medical Care).

Activity

Advise older patients with xanthine myopathy to avoid vigorous physical activity (eg, long-distance running).

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