Glucocorticoid-Remediable Aldosteronism

07/12/1969 - "I. Type relieved by glucocorticoid (glucocorticoid-remediable aldosteronism)."
08/01/2007 - "Glucocorticoid-remediable aldosteronism responds well to treatment with low-dose glucocorticoids. "
07/01/1994 - "Glucocorticoid-remediable aldosteronism (GRA) promotes a rare but unusual form of hypertension that is unresponsive to ordinary medications but very responsive to glucocorticoid medications. "
01/01/2016 - "Glucocorticoid remediable aldosteronism (GRA) is rare familial form of primary aldosteronism characterized by a normalization of hypertension with the administration of glucocorticoids. "
06/01/1991 - "Glucocorticoid-remediable aldosteronism is an inherited form of mineralocorticoid excess associated with moderate overproduction of aldosterone, in which biochemical and clinical remission is dramatically induced by small amounts of glucocorticoids. "

12/01/2003 - "g., for glucocorticoid-remediable aldosteronism, Liddle's syndrome, mineralocorticoid excess. "
06/01/1991 - "Syndrome of apparent mineralocorticoid excess and glucocorticoid-remediable aldosteronism."
06/01/2003 - "Success in the search for genes that cause or contribute to hypertension susceptibility has been limited to a few rare Mendelian forms of hypertension (glucocorticoid remediable aldosteronism, apparent mineralocorticoid excess, and Liddle's syndrome). "
12/01/2002 - "Hereditary RAAS syndromes result from intrinsic renal abnormalities (apparent mineralocorticoid excess and Liddle's syndromes) or from mineralocorticoid excess states (congenital adrenal hyperplasia and glucocorticoid-remediable aldosteronism). "
09/01/2001 - "Thus, this model is similar to inherited forms of human hypertension caused by abnormal regulation of transport by mineralocorticoids, such as apparent mineralocorticoid excess and glucocorticoid-remediable aldosteronism. "

05/01/1995 - "3. A novel observation on subjects inheriting glucocorticoid-suppressible hyperaldosteronism from their mothers showed that they had significantly higher plasma aldosterone concentrations and mean arterial blood pressures than those inheriting glucocorticoid-suppressible hyperaldosteronism from their fathers. "
05/01/1995 - "However, there was a significant correlation between basal plasma aldosterone and left ventricular mass index in the subjects with glucocorticoid-suppressible hyperaldosteronism (r = 0.66, P < 0.03). "
10/22/1981 - "Anomalous postural aldosterone response in glucocorticoid-suppressible hyperaldosteronism."
12/01/1996 - "Aldosterone-producing adenomas do not contain glucocorticoid-remediable aldosteronism chimeric gene duplications."
05/01/1995 - "Despite marked elevations in plasma aldosterone concentrations from birth in subjects with glucocorticoid-suppressible hyperaldosteronism, left ventricular hypertrophy did not occur. "

02/01/2007 - "Patients with a positive dexamethasone test underwent genetic testing for glucocorticoid-remediable aldosteronism (GRA). "
10/01/2001 - "We suggest that the dexamethasone suppression test could lead to an incorrect diagnosis of glucocorticoid-remediable aldosteronism."
09/01/2001 - "In conclusion, plasma 18-OHF determination by this ELISA method is reliable for detecting glucocorticoid-remediable aldosteronism, and it does so better than the dexamethasone suppression test."
11/01/1997 - "Evaluation of the dexamethasone suppression test for the diagnosis of glucocorticoid-remediable aldosteronism."
09/01/2001 - "In primary aldosteronism, we evaluated usefulness of plasma 18-OHF determination and the dexamethasone suppression test in the diagnosis of glucocorticoid-remediable aldosteronism using the genetic test as the gold standard. "

02/01/2004 - "Non-glucocorticoid-remediable aldosteronism in an infant with low-renin hypertension."
03/01/2012 - "The study included 62 patients with low-renin essential hypertension (EH), 81 patients with PA (20 APA, 61 BAH), 24 patients with glucocorticoid-remediable aldosteronism, 16 patients with adrenal incidentaloma, and 30 normotensives. "
12/01/1995 - "Glucocorticoid-remediable aldosteronism resembles primary aldosteronism, whereas Liddle's syndrome resembles low-renin essential hypertension. "
05/01/1995 - "The subjects with glucocorticoid-suppressible hyperaldosteronism had a higher mean blood pressure and plasma aldosterone concentration, and lower plasma renin concentration, than the control subjects. "
05/01/2013 - "Primary aldosteronism may either be sporadic or familial, the latter variant occurring in at least three forms: type I (or glucocorticoid-remediable aldosteronism), type II and type III. The diagnosis is based on the aldosterone/renin ratio as a screening test, subsequent confirmatory tests, and on CT/MR imaging studies. "

08/01/2008 - "Genetic studies excluded mutation at the aldosterone synthase locus, further distinguishing this disorder from glucocorticoid-remediable aldosteronism. "
12/01/1995 - "Glucocorticoid-remediable aldosteronism is caused by the presence of a chimeric gene, which incorporates the regulatory region of the 11-beta-hydroxylase gene and the structural portion of the aldosterone synthase gene. "
01/10/2022 - "GRAde: a long-read sequencing approach to efficiently identifying the CYP11B1/CYP11B2 chimeric form in patients with glucocorticoid-remediable aldosteronism."
01/01/2021 - "Preclinical diagnosis and identification of the chimeric CYP11B1/CYP11B2 gene in two pediatric cases of a Japanese family with glucocorticoid-remediable aldosteronism."
09/01/2015 - "A chimeric CYP11B1/CYP11B2 gene causes glucocorticoid-remediable aldosteronism (GRA), while the rare CYP11B2/CYP11B1 chimeric gene leads to 11β-hydroxylase deficiency (11-OHD). "

07/01/1999 - "Of note, all of these hypertensive syndromes (Liddle's syndrome, glucocorticoid-remediable aldosteronism, and the apparent mineralocorticoid excess syndrome) share an underlying dysregulation of the activity of the epithelial sodium channel in the cortical collecting tubule. "
01/01/2019 - "We report our long-term single-practice experience in an unusual group of five patients with chronic hyperaldosteronism (HA, including three with glucocorticoid-remediable aldosteronism, GRA) treated with low-dose amiloride (a specific epithelial sodium channel [ENaC] blocker) 5-10 (mean 7) mg daily for 14-28 (mean 20) years. "
01/01/2019 - "We report our long-term single-practice experience in 5 patients with chronic hyperaldosteronism (HA, including 3 with glucocorticoid remediable aldosteronism, GRA) treated with low-dose amiloride (a specific epithelial sodium channel [ENaC] blocker) 5-10 (mean 7) mg daily for 14-28 (mean 20) years. "
06/01/2000 - "A major focus has been the epithelial sodium channel (ENaC), which can be activated by mutations (eg, Liddle's syndrome), changes in the response to mineralocorticoids (apparent mineralocorticoid excess syndrome), or production of mineralocorticoids (glucocorticoid-remediable aldosteronism). "
03/01/1997 - "Two forms of hypertension transmitted on an autosomal recessive basis have been identified: one is glucocorticoid-suppressible hyperaldosteronism (GSH) and the other is Liddle's syndrome (amiloride-suppressible hyperactivity of the epithelial sodium channel). "

06/01/1996 - "Altered 11 beta-hydroxylase activity in glucocorticoid-suppressible hyperaldosteronism."
11/01/1996 - "11 beta-Hydroxylase activity in glucocorticoid suppressible hyperaldosteronism: lessons for essential hypertension?"
01/10/2022 - "GRAde: a long-read sequencing approach to efficiently identifying the CYP11B1/CYP11B2 chimeric form in patients with glucocorticoid-remediable aldosteronism."
01/01/2021 - "Preclinical diagnosis and identification of the chimeric CYP11B1/CYP11B2 gene in two pediatric cases of a Japanese family with glucocorticoid-remediable aldosteronism."
09/01/2015 - "A chimeric CYP11B1/CYP11B2 gene causes glucocorticoid-remediable aldosteronism (GRA), while the rare CYP11B2/CYP11B1 chimeric gene leads to 11β-hydroxylase deficiency (11-OHD). "

01/01/2002 - "For example, genes responsible for Liddle syndrome, glucocorticoid remediable aldosteronism, apparent mineralocorticoid excess have been characterized and have demonstrated the importance of sodium and water homeostasis in blood pressure control."
01/01/2000 - "Recently, rare mendelian forms of hypertension such as glucocorticoid-remediable aldosteronism (GRA), apparent mineralocorticoid excess (AME) and Liddle Syndrome caused by single gene mutations have been identified in which the mechanism is an increased sodium retention. "
09/01/1995 - "The three conditions are characterized by inappropriate control of aldosterone secretion (glucocorticoid remediable aldosteronism), sodium retention (Liddle's syndrome) or aldosterone action (apparent mineralocorticoid excess), and underline a potential role of an aldosterone: salt imbalance in mineralocorticoid hypertension. "
10/01/2003 - "To date, the molecular mechanisms of five such syndromes have been largely clarified, including glucocorticoid-remediable aldosteronism, Liddle's syndrome, apparent mineralocorticoid excess, an activating mutation of the mineralocorticoid receptor, and pseudohypoaldosteronism type 2. Each of these conditions features salt sensitivity with increased sodium and volume reabsorption by the kidney and low plasma renin activity. "
04/15/2003 - "Four types of monogenic hypertension belong to the group of mineralocorticoid hypertension, which are characterized by high renal water and sodium retention and resulting suppression of plasma renin activity (PRA), high urinary potassium secretion and consecutive low plasma potassium:1. increased production of the hormone aldosterone: glucocorticoid-remediable aldosteronism (GRH), 2. prereceptor disorder with loss of selectivity of the mineralocorticoid receptor: apparent mineralocorticoid excess (AME), 3. receptor disorder with constitutive activation of the mineralocorticoid receptor: "Geller syndrome", 4. postreceptor disorder with enhanced function of the epithelial sodium channel: Liddle's syndrome. "

06/01/2017 - "A/C = cortisol-corrected aldosterone concentration; ACE = angiotensin-converting enzyme; APA = aldosterone-producing adenoma; APCC = aldosterone-producing cell cluster; ARB = angiotensin receptor blocker; ARR = aldosterone-to-renin ratio; AVS = adrenal venous sampling; CT = computed tomography; ENaC = epithelial sodium channel; GRA = glucocorticoid remediable aldosteronism; IHA = idiopathic hyperaldosteronism; LI = lateralization ratio; MR = mineralocorticoid receptor; MRI = magnetic resonance imaging; PA = primary aldosteronism; PRA = plasma renin activity; SRA = surgical remediable aldosteronism."
11/01/1993 - "Deoxyribonucleic acid (DNA) markers of lipid abnormalities or hypertension have included LDL-receptor defects, lipoprotein lipase deficiency, high Lp(a), familial defective apo B, decreased quantitative levels of apo B, apo E phenotype, angiotensinogen, and 'glucocorticoid remediable aldosteronism (GRA) hypertension.' Also tested in Utah studies, but not found to be DNA markers for hypertension, were the genetic loci for the structural genes for renin and angiotensin-converting enzyme, and the sodium antiport system. "

04/01/2011 - "Delayed arterial switch operation for d-transposition of the great arteries and glucocorticoid remediable aldosteronism."