Dent's Disease - Treatment

Treatment

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; they include:

  • Thiazide diuretics (i.e. hydrochlorothiazide) 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 cotransporter in the renal distal convoluted tubule, leading indirectly to less water and solutes being delivered to the distal tubule. The impairment of Na transport in the distal convoluted tubule induces natriuresis and water loss, while increasing the reabsorption of calcium in this segment in a manner unrelated to sodium transport.
  • Amiloride 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, but 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)."
  • 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 a high-citrate diet preserved renal function and delayed progress of renal disease.

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