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.

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

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.

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.
References in periodicals archive ?
Pathogenesis and causes of hyperkalemia Pathogenesis of Causes of hyperkalemia hyperkalemia False Hyperkalemia Hemolysis Tight turnstile Thrombocytosis Leukocytosis Impaired elimination of ARF, CRF potassium Pseudohypoaldosteronism, hypoaldosteronism, and congenital adrenal hyperplasia Medications (ACEI, ARB, NSAI) Mannitol Drugs interfering with renal potassium excretion (amiloride, spironolactone, and triamteren) Increased shift of Trauma potassium from intra Burn injury to extracellular space Rhabdomyolysis, tumorlyis, hemolysis, and after massive transfusion Acidosis Hyperkalemic periodic paralysis Hyperglisemia Succinylcholine Excessive intake ARF: Acute renal failure, CRF: Chronic renal failure, ACEI: Anjiotensin-converting-enzyme inhibitor, ARB: Anjiotensin
The combination of positive GBS in the urine and abnormal renal ultrasound supported a renal cause of the patient's hypoaldosteronism or pseudohypoaldosteronism as the diagnosis.
Furthermore, patients with the hereditary disease Pseudohypoaldosteronism Type 1 have a reduced ENaC activity in the airways and thus an increased ASL volume [99].
9%) who had pseudohypoaldosteronism becasue of inability to provide a high level of oral salt intake and in one patient who had panhypopituitarism and motor-mental retardation (2.
Novel mutation in the epithelial sodium channel causing type I pseudohypoaldosteronism in a patient misdiagnosed with cystic fibrosis.
Pseudohypoaldosteronism type I (PHA-I) is a rare disorder with only a few cases reported worldwide.
A small sampling of topics: thyroid hormone transporters and resistance, clinical and molecular aspects of androgen insensitivity, pseudohypoaldosteronism, current issues on molecular diagnosis of GH signaling defects, and human congenital perilipin deficiency and insulin resistance.
Ved was diagnosed with a rare condition called pseudohypoaldosteronism when he was nine months old and had to take regular supplements.
Ved, described by his parents as a happy and lively lad, had been diagnosed with a rare condition called pseudohypoaldosteronism when he was nine months old.
Growth hormone activates renin-aldosterone system in children with idiopathic short stature and in a pseudohypoaldosteronism patient with a mutation in epithelial sodium channel alpha subunit.
Disturbances of Na/K balance: pseudohypoaldosteronism revisited.
Pseudohypoaldosteronism with increased sweat and saliva electrolyte values and frequent lower respiratory tract infections mimicking cystic fibrosis.

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