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- Pablo Saborio, MD*
- Gary A. Tipton, MD†
- James C.M. Chan, MD‡
- *Visiting Scholar to Medical College of Virginia (MCV) Campus of Virginia Commonwealth University, Richmond, VA from the University of Costa Rica, San Jose, Costa Rica.
- †Associate Professor of Pediatrics, Division of General Pediatrics, Medical College of Virginia, Richmond, VA.
- ‡Editorial Board
OBJECTIVES
After completing this article, readers should be able to:
Describe the simple test that will establish the diagnosis of diabetes insipidus.
Explain how to differentiate central diabetes insipidus from nephrogenic diabetes insipidus and compulsive water drinking.
Delineate the inheritance pattern of central diabetes insipidus and nephrogenic diabetes insipidus.
Describe the treatments of choice for central diabetes insipidus and nephrogenic diabetes insipidus.
Definition and Epidemiology
Polydipsia and polyuria with dilute urine, hypernatremia, and dehydration are the hallmarks of diabetes insipidus in infants and children. Patients who have diabetes insipidus are unable to conserve water and can become severely dehydrated when deprived of water. The polyuria exceeds 5 mL/kg per hour of dilute urine, with a documented specific gravity of less than 1.010. The hypernatremia is evidenced by a serum sodium concentration in excess of 145 mmol/L (145 mEq/L).
Three conditions give rise to polydipsia and polyuria. The most common condition is central or neurogenic diabetes insipidus related to a deficiency of vasopressin. Less common is nephrogenic diabetes insipidus, including the X-linked recessive, autosomal recessive, and autosomal dominant types due to renal tubular resistance to vasopressin. Finally, these conditions can occur in the compulsive water drinker who demonstrates physiologic inhibition of vasopressin secretion.
The incidence of diabetes insipidus in the general population is 3 in 100,000, with a slightly higher incidence among males (60%). X-linked nephrogenic diabetes insipidus is very rare, with arginine vasopressin receptor2 (AVPR2) gene mutations among males estimated to be 4 in 1,000,000. The incidence of compulsive water drinking is unknown, but there appears to be a female predisposition (80%). Although the compulsive water drinker commonly presents in the third decade of life, cases have been described in patients from 8 to 18 years of age. Compulsive water drinking is encountered in 10% to 40% of patients who have schizophrenia. …
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