Dent's disease medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

As of today, there is no agreed-upon treatment of Dent’s disease and no therapy has been formally accepted. Most treatment measures are mostly supportive in nature and they include:

• Thiazide diuretics which have been used with success in reducing the calcium output in urine, but they are also known to cause hypokalemia.
  • In rats with diabetes insipidus thiazide diuretics inhibit the NaCl co-transporter in the renal distal convoluted tubule leading indirectly to less water and solutes being delivered to the distal tubule.^[1]
• Amiloride which also increases distal tubular calcium reabsorption and has been used as a therapy for idiopathic hypercalciuria.
  • A combination of 25 mg of chlorthalidone plus 5 mg of amiloride daily led to a substantial reduction in urine calcium in Dent’s patients, however urine pH was “significantly higher in patients with Dent’s disease than in those with idiopathic hypercalciuria (P < 0.03), and supersaturation for uric acid was consequently lower (P < 0.03).”^[2]
• For patients with osteomalacia, Vitamin D or derivatives have been employed, apparently with success.
• Some lab tests on mice with CLC-5 related tubular damage showed that a high citrate diet preserved renal function and delayed progress of renal disease.^[3]

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