Medical term:

pseudohypoaldosteronism



pseudohypoaldosteronism

 [soo″do-hi″po-al-dos´ter-ōn-izm]
a hereditary disorder of infancy, characterized by severe salt loss by the kidneys despite elevated secretion and urinary excretion of aldosterone; it is thought to be due to unresponsiveness of the distal renal tubule to aldosterone.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

pseudohypoaldosteronism

A heterogeneous group of salt wasting syndromes due to distal renal tubular insensitivity to mineralocorticoids–eg, aldosterone or due to a defect in the mineralocortioid receptor in the colonic mucosa, salivary gland, sweat glands, resulting in salt loss but normal adrenocortical and renal function, and a hyperactive renin-angiotensin system. Cf Hypoaldosteronism Types Type I Albright's hereditary osteodystrophy More common, and is almost invariably X-linked MIM 300100; AD MIM 103580 cases–designated type IA have been reported; there is inadequate cAMP response to PTH; Type II Due to inadequate end-organ response to ↑cAMP levels; affected children are short and stocky with a round facies, brachydactyly, tetany, foci of bony demineralization, osteitis fibrosa.
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.


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