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Successful treatment with hydrochlorothiazide and amiloride in an infant with congenital nephrogenic diabetes insipidus
Authors:Timothy M. Uyeki  Floyd L. Barry  Stephen M. Rosenthal  Robert S. Mathias
Affiliation:(1) Departments of Pediatrics, University of California San Francisco Medical Center, 533 Parnassus Avenue, Box 0748, 94 143-0748 San Francisco, California, USA;(2) Departments of Endocrinology, University of California San Francisco Medical Center, 533 Parnassus Avenue, Box 0748, 94 143-0748 San Francisco, California, USA;(3) Departments of Children's Renal Center, University of California San Francisco Medical Center, 533 Parnassus Avenue, Box 0748, 94 143-0748 San Francisco, California, USA
Abstract:We report a 9-month-old male latino infant with congenital nephrogenic diabetes insipidus (NDI) who presented with hypernatremic dehydration aggravated by severe gastroenteritis. Initially, the infant was managed with intravenous fluids followed by standard 20 cal/ounce formula and pharmacological therapy, resulting in normalization of his serum sodium level. While hydrochlorothiazide therapy alone or in combination with prostaglandin inhibitors or amiloride has been successful in children and adolescents, this is the first report of the successful use of hydrochlorothiazide and amiloride in an infant with congenital NDI.
Keywords:Nephrogenic diabetes insipidus  Vasopressin V2 receptor gene  X-linked recessive inheritance  Hypernatremia  Amiloride
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