Condition Drug Dose
Complete central DI dDAVP 10-20 (g intranasally q 12-24 h
Partial central DI Vasopressin tannate 2-5 U IM q 24-48 h
Aqueous vasopressin 5-10 U SC q 4-6 h
Chlorpropamide 250-500 mg/d
Clofibrate 500 mg tid-qid
Carbamazepine 400-600 mg/d
Treatment of central diabetes insipidus (DI). Central DI may be treated with hormone replacement or drugs. In acute settings when renal water losses are extensive, aqueous vasopressin (pitressin) is useful. It has a short duration of action that allows for careful monitoring and avoiding complications like water intoxication. This drug should be used with caution in patients with underlying coronary artery disease and peripheral vascular disease, as it can cause vascular spasm and prolonged vasoconstriction. For the patient with established central DI, desmopressin acetate (dDAVP) is the agent of choice. It has a long half-life and does not have significant vasoconstrictive effects like those of aqueous vasopressin. It can be conveniently administered intranasally every 12 to 24 hours. It is usually tolerated well. It is safe to use in pregnancy and resists degradation by circulating vasopressinase. In patients with partial DI, agents that potentiate release of antidiuretic hormone can be used. These include chlorpropamide, clofibrate, and carbamazepine. They work effectively only if combined with hormone therapy, decreased solute intake, or diuretic administration .
Congenital nephrogenic diabetes insipidus, X-linked-recessive form. This is a rare disease of male patients who do not concentrate their urine after administration of antidiuretic hormone. The pedigrees of affected families have been linked to a group of Ulster Scots who emigrated to Halifax, Nova Scotia in 1761 aboard the ship called "Hopewell." According to the Hopewell hypothesis, most North American patients with this disease are descendants of a common ancestor with a single gene defect. Recent studies, however, disproved this hypothesis . The gene defect has now been traced to 87 different mutations in the gene for the vaso-pressin receptor (AVP-R2) in 106 presumably unrelated families . (From Bichet, et al. ; with permission.)
Congenital nephrogenic diabetes insipidus (NDI), autosomal-recessive form. In the autosomal recessive form of NDI, mutations have been found in the gene for the antiiuretic hormone (ADH)-sensitive water channel, AQP-2. This form of NDI is exceedingly rare as compared with the X-linked form of NDI . Thus far, a total of 15 AQP-2 mutations have been described in total of 13 families . The acquired form of NDI occurs in various kidney diseases and in association with various drugs, such as lithium and amphotericin B. (From Canfield et al. ; with permission.)
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