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pseudohypoaldosteronism |
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pseudohypoaldosteronism /pseu·do·hy·po·al·dos·ter·on·ism/ (-hi″po-al-dos´ter-ōn-izm)
1. a hereditary disorder of infancy characterized by severe salt and water depletion and other signs of aldosterone deficiency, although aldosterone secretion is normal or increased; causes include aldosterone receptor defects and renal dysfunction. 2. the endocrine abnormality associated with sodium-losing nephropathy, usually due to chronic pyelonephritis, seen primarily in adults.
pseudohypoaldosteronism [-hī′pōaldos′tərōn′izəm] 1 a hereditary disorder of infancy characterized by severe salt and water depletion and other signs of aldosterone deficiency, even though normal or elevated amounts of aldosterone are secreted. Causes include aldosterone receptor defects and renal dysfunction. Some affected infants outgrow the need for dietary salt supplements in early childhood. 2 the endocrine abnormality associated with sodium-losing nephropathy, usually resulting from chronic pyelonephritis, seen primarily in adults. See also Gordon's syndrome. 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.
pseudohypoaldosteronism an unresponsiveness of the distal renal tubule to aldosterone, resulting in severe loss of salt in the urine, despite elevated secretion and urinary excretion of aldosterone. An inherited disorder in humans.
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. Want to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit the webmaster's page for free fun content. |
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