I. Dexamethasone-suppressible hyperaldosteronism: a large new kindred. The clinical picture can also occur with certain forms of congenital adrenal hyperplasia (in which other mineralocorticoids are elevated) and the dominantly inherited dexamethasone-suppressible hyperaldosteronism. Careers. Dexamethasone-suppressible hyperaldosteronism. Dexamethasone-suppressible hyperaldosteronism. Dexamethasone-suppressible hyperaldosteronism (DSH) is a rare familial variety of primary aldosteronism in which the biochemical features of mineralocorticoid … dexamethasone-suppressible hyperaldosteronism, Arteaga E, Kater CE, Biglieri EG (1986) Aldosterone production during prolonged ACTH administration. Small adenomas are increasingly recognized as a cause of primary hypertension even when serum potassium levels are normal. Salti IS, Stiefel M, Ruse JL, Laidlaw JC. The responses of renin, aldosterone, and blood pressure to ACTH suppression with dexamethasone in a 61-yr-old man with glucocorticoid-suppressible hyperaldosteronism were modified by coexistent atheromatous renal artery stenosis (RAS). J Endocrinol Invest. DSH should be considered in all children who present with low renin hypertension. Glucocorticoid remediable aldosteronism also describable as aldosterone synthase hyperactivity, is an autosomal dominant disorder in which the increase in aldosterone secretion produced by ACTH is no longer transient.. 8600 Rockville Pike Description What are the signs and symptoms of primary hyperaldosteronism? In: Mantero F, Biglieri EG, Funder JW, Scoggins BA (eds) The adrenal gland and hypertension. FOIA Genetic studies show no HLA linkage and point to an autosomal dominant mode of inheritance, suggesting that the prevalence of this disease has been underestimated in the past. Endocrinology 115:462–466, Gomez-Sanchez CE, Gomez-Sanchez EP, Smith JS, Ferriss MW, Foecking MW (1985) Receptor binding and biologic activity of 18-oxocortisol. N Engl J Med. Regulation of adrenocortical function and body electrolytes was studied in two affected brothers. L'identification moléculaire de trois d'entre elles s'est faite entre 1992 et 1995. J Clin Endocrinol Metab 43:115–131, Sonino N, Levine LS, New MI (1981) Mineralocorticoid and metabolic response to metyrapone in normotensive children and children with dexamethasone-suppressible and primary aldosteronism. O'Mahony S, Burns A, Murnaghan DJ. Several other members of the family were investigated. Fallo F, Sonino N, Boscaro M, Armanini D, Mantero F, Dörr HG, Knorr D, Kuhnle U. Klin Wochenschr. Am Heart J 95:375–388, Ganguly A (1985) Is glucocorticoid-suppressible hyperaldosteronism mediated by ACTH? Primary hyperaldosteronism and idiopathic hyperaldosteronism, although their presenting features of raised aldosterone secretion and hypertension are similar, are probably not variations of the same disease. Secretion rates of cortisol and aldosterone precursors in various forms of congenital adrenal hyperplasia. https://doi.org/10.1007/BF01712834, Over 10 million scientific documents at your fingertips, Not logged in Genotyping and HLA typing were also performed. DSH should be considered in all children who present with low renin hypertension. Biochemically DSH and APA can be differentiated from IAH since in both aldosterone does not respond to upright posture, to angiotensin II infusion, and to angiotensin-converting enzyme (ACE) captopril. Clin Sci Mol Med 51:329s-332s, Mantero F, Fallo F, Opocher G, Armanini D, Boscaro M, Scaroni C (1981) Effect of angiotensin II and converting enzyme inhibitor (captopril) on blood pressure, plasma renin activity and aldosterone in primary aldosteronism. The main pathogenetic hypotheses point to a pituitary and/or an adrenal abnormality, but the intrinsic nature of the disease remains to be elucidated. Dexamethasone, spironolactone and eplerenone have been used in treatment. ACHIEVEMENT. This case of dexamethasone suppressible hyperaldosteronism (DSH) illustrates that the degree of hypertension in this syndrome may produce severe renal microvascular lesions. Rauh W, Levine LS, Gottesdiener K, New MI. Hypertension, hypokalemia, suppressed renin, and high aldosterone values characterize DSH in the basal state, similar to the other forms of primary aldosteronism, i.e., aldosterone-producing adenoma (APA) or bilateral idiopathic adrenal hyperplasia (IAH). In some cases hypertension is unresponsive to dexamethasone and needs alternative treatment. J Clin Endocrinol Metab 53:331–337, Gomez-Sanchez CE, Montgomery M, Ganguly A, Holland OB, Gomez-Sanchez EP, Grim CE, Weinberger MH (1984) Elevated urinary excretion of 18-oxocortisol in glucocorticoid-suppressible aldosteronism. Reinvestigation of an 18-yr-old male patient with dexamethasone-suppressible hyperaldosteronism after 6 yr of little or no treatment demonstrates that elevated We use cookies to enhance your experience on our website.By continuing to use our website, you are agreeing to our use of cookies. The evidence in favour of this conclusion has been fully discussed previously [5, 12]. COVID-19 is an emerging, rapidly evolving situation. 10, Rome, pp 89–104, Mizuno K, Haruyama K, Gotoh M, Matsui J, Fukuchi S (1984) Evidence for the role of kinins in the acute antihypertensive effect of captopril in low-renin hypertension. The response of the adrenal glomerulosa to renin stimulation was determined in 10 patients with dexamethasone-suppressible hyperaldosteronism. Under normal conditions, aldosterone production is regulated by the renin-angiotensin system and potassium balance. Clin Sci 61:289s-293s, Miura K, Ioshinaga K, Goto K, Katsushima K, Maebashi M, Demura H, Iino M, Demura R, Torikai T (1968) A case of glucocorticoid-responsive hyperaldosteronism. With DEX J Hypertens 1:197–205, Biglieri EG, Schambelan M, Slaton PE, Stockigt JR (1970) The intercurrent hypertension in primary aldosteronism. On the basis of further examinations, dexamethasone was administered and returned blood pressure to normal, and also normalized serum potassium, plasma aldosterone, and renin activity. Clin Pharmacol Ther 30:246–250, Ganguly A, Melada GA, Luetscher JA, Dowdy AJ (1973) Control of plasma aldosterone in primary aldosteronism: distinction between adenoma and hyperplasia. 1987 Jan;1(1):99-108. doi: 10.1007/BF00866890. J Clin Endocrinol Metab 28:1807–1815, Miura K, Yamakita N, Yasuda K, Murase H, Takeda N, Takeuchi K, Yasujima H, Murakami O, Abe K, Yoshinaga K, Sasano N (1986) Clinical features of dexamethasone-suppressible hyperaldosteronism in Japan with study in our case followed for 18 years. (Abstract). We report a patient who presented in 1974 at age 15 yr with blood pressure of 240/120 mm Hg, … Hypertension 8:669–676, DiMartino J, Stoner E, New MI (1985) Isolation of an immunoreactive substance in patients with dexamethasone-suppressible hyperaldosteronism (DSH). Type relieved by glucocorticoid (glucocorticoid-remediable aldosteronism). In: New MI, Borrelli P (eds) Dexamethasone-suppressible hyperaldosteronism, Serono symposia review no. The final diagnosis of DSH rests upon the prompt reversal of the features of mineralocorticoid excess by glucocorticoid therapy. Clin Exp Hypertens A4:1771–1777, Ulick S, Land M, Chu M (1983) 18-Oxocortisol, a naturally occurring mineralocorticoid agonist. Regulation of adrenocortical function and body electrolytes was studied in two affected brothers. Would you like email updates of new search results? In all conditions, the feedback system fails and aldosterone secretion continues despite a low blood renin level. Rarely, the condition is hereditary - so called glucocorticoid or dexamethasone-suppressible hyperaldosteronism.Rarer still, large adrenal carcinomas may secrete aldosterone. - 201.144.13.194. Raven Press, New York, pp 405–420, Connell JMC, Kenyon CJ, Corrie JET, Fraser R, Watt R, Lever AF (1986) Dexamethasone-suppressible hyperaldosteronism: adrenal transition cell hyperplasia? The clinical phenotype varies from severe, early onset hypertension to much milder blood pressure elevation; hypokalaemia is usually mild. Clinical, biochemical and molecular data on five kindreds with dexamethasone-suppressible hyperaldosteronism are reviewed. 1970 Mar;30(3):361-71. doi: 10.1210/jcem-30-3-361. Klin Wochenschr 56 [Suppl 1]:161–168, Rocco S, Fallo F, Luzzi T, Opocher G, Mantero F (1985) Diagnostic relevance of acute converting enzyme blockade in differentiating idiopathic hyperaldosteronism (IHA) from aldosterone-producing adenoma (APA). J Clin Endocrinol Metab 57:388–392, Ganguly A, Weinberger MH, Guthrie GP, Fineberg NS (1984) Adrenal steroid response to ACTH in glucocorticoid-suppressible hyperaldosteronism. Bethesda, MD 20894, Copyright Dexamethasone-suppressible hyperaldosteronism. 10, Rome, pp 133–137, Lan NC, Matulich DT, Stockigt JR, Biglieri EG, New MI, Baxter J (1981) Role of steroids in various states of mineralocorticoid excess hypertension: analysis by mineralocorticoid receptor assay. In GRA, the hypersecretion of aldosterone and the accompanying hypertension are remedied when ACTH secretion is suppressed by administering glucocorticoids. Dexamethasone-suppressible hyperaldosteronism: Pathophysiology, clinical aspects, and new insights into the pathogenesis. Circ Res 27 [Suppl 1]:I195-I202, Bravo E, Tarazi R, Dustan H, Fouad FM, Textor SC, Gifford RW, Vidt DG (1983) The changing clinical spectrum of primary aldosteronism. Acta Endocrinol 99:251–255, Mantero F, Gion M, Armanini D, Opocher G (1976) Aldosterone regulation in primary aldosteronism: differences between adenoma and hyperplasia. Clin Endocrinol 22:777–785, Fallo F, Boscaro M, Sonino N, Mantero F (1986) Abnormal adrenal responsiveness in dexamethasone-suppressible hyperaldosteronism: further evidence. Rarely, the condition is hereditary – so called glucocorticoid or dexamethasone-suppressible hyperaldosteronism. MRI Magnetic Resonance Imaging; WHO World Health Organization; CNS Central Nervous System; SNF Skilled Nursing Facility; AA Alcoholics Anonymous; SMI Serious Mental Illness; SA Semen Analysis; BFN Big Fat Negative; IVF In Vitro Fertilization; BPD Borderline Personality Disorder; APA American Psychiatric Association; LMP Last … Accessibility [ Links ] This cause of mineralocorticoid excess is primary hyperaldosteronism reflecting excess production of aldosterone by adrenal zona glomerulosa. Klin Wochenschr 65, 437–444 (1987). Raven Press, New York, pp 395–404, Ganguly A, Weinberger MH (1981) Triamterene-thiazide combination: alternative therapy for primary aldosteronism. Dexamethasone-suppressible hyperaldosteronism (DSH) is a rare familial variety of primary aldosteronism in which the biochemical features of mineralocorticoid excess are corrected by treatment with glucocorticoids. NUCLÉAIRE MÉDECINE. Endocrinology 113:2320–2322, Ulick S, Chu M, Land M (1983) Biosynthesis of 18-oxocortisol by aldosterone-producing adrenal tissue. Please enable it to take advantage of the complete set of features! In contrast, morphologically DSH is similar to IAH, since neither macroscopic nor histologic examinations of the adrenals give evidence of any unilateral abnormality. Unable to load your collection due to an error, Unable to load your delegates due to an error. Hypertension 8:317–322, Keller-Wood ME, Dallman MF (1984) Corticosteroid inhibition of ACTH secretion. In most pat… We report a large new kindred with this syndrome, the second such family described in the British Isles and the first in Ireland. In: New MI, Borrelli P (eds) Dexamethasone-suppressible hyperaldosteronism, Serono symposia review no. The clinical picture can also occur with certain forms of congenital adrenal hyperplasia (in which other mineralocorticoids are elevated) and the dominantly inherited dexamethasone-suppressible hyperaldosteronism. J Clin Endocrinol Metab 43:1283–1293, New MI, Oberfield SE, Levine LS, Dupont B, Pollack M, Gill JR, Bartter FC (1980) Autosomal dominant transmission and absence of HLA linkage in dexamethasone-suppressible hyperaldosteronism. In: New MI, Borrelli P (eds) Dexamethasone-suppressible hyperaldosteronism, Serono symposia review no. Dexamethasone-suppressible hyperaldosteronism is a rare familial syndrome in which hypokalemia, suppression of plasma renin concentration, and elevated aldosterone secretion are corrected by treatment with glucocorticoids. In addition, long-term treatment with dexamethasone was associated with recurrence of hypertension and was accompanied by loss of aldosterone suppression with glucocorticoid administration in one report.34 It is unclear whether this reflects emergence of autonomy of the GRA adrenal gland, or possible normalization of adrenal function due to diminished expression of the … ACE, APA, IAH: 8 : 1987: Effects of naloxone on the pituitary-adrenal axis in patients with dexamethasone-suppressible hyperaldosteronism. Glucocorticoid-remediable aldosteronism (GRA): diagnosis, variability of phenotype and regulation of potassium homeostasis. Acta Endocrinol 98:87–94, Speiser PW, Martin KO, Kao-Lo G, New MI (1985) Excess mineralocorticoid receptor activity in patients with dexamethasone-suppressible hyperaldosteronism is under adrenocorticotropin control. PMID: 4351777 [PubMed - indexed for MEDLINE] MeSH Terms. In summary, we did not find other genetic alterations or high levels of 18-hydroxycortisol that could explain a positive dexamethasone suppression test in idiopathic hyperaldosteronism. PRA: 9 : 1985 J Clin Endocrinol Metab 61:297–302, Stockigt JR, Scoggins BA (1986) Evolution of dexamethasone-suppressible to idiopathic hyperaldosteronism. One hundred and seventeen consecutive patients (61 males and 56 females, aged 28–67 yr), including 2 pairs of siblings, referred since 1994 to our centers were studied. Most people will not have symptoms. Mineralocorticoids, salt balance and blood pressure after prolonged ACTH administration in juvenile hypertension. J Clin Endocrinol Metab 52:195–198, Woodland E, Tunny TJ, Hamlet SM, Gordon RD (1985) Hypertension corrected and aldosterone responsiveness to renin-angiotensin restored by long-term dexamethasone in glucocorticoid-supressible hyperaldosteronism. Genetic studies show no HLA linkage and point to an autosomal dominant mode of inheritance, suggesting that the prevalence of this disease has been underestimated in the past. All were hypertensive, and the slight majority (64 of 117 = 54.7%) had spontaneous hypokalemia of varying degrees. Pediatr Nephrol. Endocr Rev 5:1–24, Kuhnle U, Armanini D, Fallo F, Wehling M, Dörr H, Sonino N, Mantero F (1986) Plasma mineralocorticoid activity, mineralocorticoid receptors and intracellular electrolytes in patients with dexamethasone-suppressible hyperaldosteronism (DSH). 1969 Jul 12;101(1):1-10. Klinische Wochenschrift It is generally held that idiopathic hyperaldosteronism and glucocorticoid-suppressible hyperaldosteronism (GSH) are distinct entities, distinguishable by thorough investigation. Klin Wochenschr. J Clin Endocrinol Metab 62:934–940, Laidlaw JC (1986) Dexamethasone-suppressible hyperaldosteronism: patients JS and LS twenty years later. Dexamethasone-suppressible hyperaldosteronism: pathophysiology, clinical aspects, and new insights into the pathogenesis. The genetic basis of the syndrome reflects the presence of a chimaeric gene derived from an unequal crossover between … Rarer still, large adrenal carcinomas may secrete aldosterone. Clin Endocrinol 26:163–168, Ferris JB, Beevers DG, Brown JJ (1978) Clinical, biochemical and pathological features of low-renin (primary) hyperaldosteronism. Clin Exp Pharmacol Physiol 12:245–248, Istituto di Semeiotica Medica, Università di Padova, Italia, F. Fallo, N. Sonino, M. Boscaro, D. Armanini & F. Mantero, Kinderklinik der Universität München, Germany, You can also search for this author in Aldosterone is a hormone manufactured by the adrenal glands which helps the body retain water and sodium and excrete potassium. It is caused by a fusion of the CYP11B1 and CYP11B2 genes and is inherited in an autosomal dominant manner. It is a cause of primary hyperaldosteronism. Dexamethasone-suppressible hyperaldosteronism: pathophysiology, clinical aspects, and new insights into the pathogenesis. Pediatrics 65:597–604, Grim CE, Ganguly A, Weinberger MH (1986) A rapid method to differentiate glucocorticoid-suppressible hyperaldosteronism from other causes of primary aldosteronism with an anomalous postural response of plasma aldosterone. L'hyperaldostéronisme suppressible par la dexaméthasone associe une hypertension précoce, souvent apparue a […] Lire la suite. Can Med Assoc J. Can Med Ass J 95:1109–1119, Ulick S, Chu M (1982) Hypersecretion of a new corticosteroid, 18-hydroxycortisol in two types of adrenocortical hypertension. In: New MI, Borrelli P (eds) Dexamethasone-suppressible hyperaldosteronism, Serono symposia review no. This case of dexamethasone suppressible hyperaldosteronism (DSH) illustrates that the degree of hypertension in this syndrome may produce severe renal microvascular lesions. New MI, Siegal EJ, Peterson RE. This site needs JavaScript to work properly. A case of glucocorticoid-responsive hyperaldosteronism. © 2021 Springer Nature Switzerland AG. J Clin Endocrinol Metab 37:93–100, New MI, Peterson RE, Saenger P, Levine LS (1976) Evidence for an unidentified ACTH-induced steroid causing hypertension. Aldosterone is a hormone manufactured by the adrenal glands which helps the body retain water and sodium and excrete potassium. J Endocrinol Invest 8 [Suppl 1]:177, Ruse JL, Price C, Stiefel M, Laidlaw JC (1972) The influence of glucocorticoids and heparin on aldosterone production. In: Giovannelli G, New MI, Gorini S (eds) Hypertension in children and adolescents. In: New MI, Borrelli P (eds) Dexamethasone-suppressible hyperaldosteronism, Serono symposia review no. Lancet I:550–551, Oberfield SE, Levine LS, Stoner E, Chow D, Rauh W, Greig F, Lee MS, Lightner E, Witte M, New MI (1981) Adrenal glomerulosa function in patients with dexamethasone-suppressible hyperaldosteronism. The patients were treated continuously with 2 mg/day dexamethasone (DEX) and were studied on a regular sodium diet (87 meq/m2. We report a patient who presented in 1974 at age 15 yr with blood pressure of 240/120 mm Hg, serum K of 3.1 mM, low renin, and high normal aldosterone excretion, with findings diagnostic of GSH. Endocrinology 116:6–10, Gomez-Sanchez CE, Gill JR, Ganguly A, Gordon RD (1986) Glucocorticoid-suppressible aldosteronism: a disorder of the transitional zone? (Abstract). 1995 Jul-Aug;18(7):512-7. doi: 10.1007/BF03349762. This report describes investigations in a new kindred with dexamethasone-suppressible hyperaldosteronism affecting three successive generations. It is caused by a fusion of the CYP11B1 and CYP11B2 genes and is inherited in an autosomal dominant manner. 10, Rome, pp 79–87, Beretta-Piccoli C, Davies DL, Brown JJ, Ferris B, Fraser R, Lever AF, Morton JJ, Robertson JS, Semple PF, Watt RR (1983) Relation of blood pressure with body and plasma electrolytes in Conn's syndrome. The family has a dramatic history of premature … 17-Hydroxycorticosteroids/urine; 17-Ketosteroids/urine; Adolescent; Adrenocorticotropic Hormone; Aldosterone/secretion; Aldosterone/urine; Corticosterone/secretion; Desoxycorticosterone/secretion; Dexamethasone… Raven Press, New York, pp 165–175, Lee SM, Lightner E, Witte M, Oberfield SE, Levine LS, New MI (1982) Dexamethasone-suppressible hyperaldosteronism in a child with nephrosclerosis. Glucocorticoid-remediable aldosteronism is one of three types of familial hyperaldosteronism. Of 60 subjects given 4-day dexamethasone treatment, 23 were classified as APA and 37 as IHA. 11. 1971 Sep 23;285(13):735-9. doi: 10.1056/NEJM197109232851306. Rarer still, large adrenal carcinomas may secrete aldosterone. Non-tumorous "primary" aldosteronism. Miura K, Yoshinaga K, Goto K, Katsushima I, Maebashi M, Demura H, Iino M, Demura R, Torikai T. J Clin Endocrinol Metab. In all conditions, the feedback system fails and aldosterone secretion continues despite a low blood renin level. [ Links ] 12. The presumptive diagnosis was first made in a 7-yr-old boy and led to the identification of the disorder in his mother and grandmother. Acta Endocrinol 111 [Suppl 274]:179–180, Kuhnle U, Land M, Ulick S (1986) Evidence for the secretion of an antimineralocorticoid in congenital adrenal hyperplasia. Dexamethasone-suppressible hyperaldosteronism (DSH) is a rare familial variety of primary aldosteronism in which the biochemical features of mineralocorticoid … 10, Rome, pp 41–48, Grim CE, Weinberger MH (1980) Familial dexamethasone-suppressible normokalemic hyperaldosteronism. Immunoreactive plasma levels of the proopiolipomelanocortin- derived peptides, ACTH, β-endorphin-lipotropin, and γ 3 MSH, were measured in patients with primary hyperaldosteronism, idiopathic hyperaldosteronism with bilateral adrenal hyperplasia, and dexamethasone-suppressible hyperaldosteronism. 1978;56 Suppl 1:161-7. doi: 10.1007/BF01477468. Dr. Maria New identified a new form of hypertension, “apparent mineralcorticoid excess,” which has resulted in a new area of research to determine how gluticoscoritcoids and mineralcorticoids interact with receptors. J Hum Hypertens 1989;3:255-8. The apparent responsiveness of aldosterone to angiotensin-II … A profile of dexamethasone-suppressible hyperaldosteronism (DSH), a variant of primary aldosteronism, is drawn by reviewing its pathophysiological and clinical aspects. Steroids 1995;60: 48-51. 10, Rome, pp 105–117, Fallo F, Boscaro M, Sonino N, Mantero F (1987) Effects of naloxone on the pituitary-adrenal axis in patients with dexamethasone-suppressible hyperaldosteronism. [Renin-angiotensin-aldosterone function tests]. Jpn Heart J 25:379–386, Mulrow PJ (1981) Glucocorticoid-suppressible hyperaldosteronism: a clue to the missing hormone? At the time of the study, subjects were all hospitalized, had been consuming a daily diet containing 120–150 mmol sodium and 60 mmol potassium for at least 2 weeks, and had been off all medications for at least 1 month. In: New MI, Borrelli P (eds) Dexamethasone-suppressible hyperaldosteronism, Serono symposia review no. A profile of dexamethasone-suppressible hyperaldosteronism (DSH), a variant of primary aldosteronism, is drawn by reviewing its pathophysiological and clinical aspects. Ann Int Med 90:386–395, Wisgerhof M, Brown RD, Mogen MJ, Carpenter PC, Edis AJ (1981) The plasma aldosterone response to angiotensin II infusion in aldosterone-producing adenoma and in idiopathic hyperaldosteronism. We suggest that the dexamethasone suppression test could lead to an incorrect diagnosis of … Dexamethasone-Suppressible normokalemic hyperaldosteronism 1968 Dec ; 28 ( 12 ):1807-15. doi: 10.1210/jcem-30-3-361 of congenital adrenal hyperplasia EG 1986... Subjects given 4-day dexamethasone treatment, 23 were classified as APA and 37 as IHA body... Scientific documents at your fingertips, Not logged in - 201.144.13.194 Hall CE, Weinberger (... In - 201.144.13.194 naloxone on the pituitary-adrenal axis in patients with dexamethasone-suppressible:. Hyperaldosteronism was studied in two affected brothers: pathophysiology, clinical aspects, and New insights into the pathogenesis,! Corticosteroid inhibition of ACTH secretion collection due to an error, unable to load your delegates due to error... And 37 as IHA fusion of the disease remains to be elucidated by glucocorticoid therapy secretion continues a... Take advantage of the complete set of features who present with low hypertension! Are normal in juvenile hypertension, 12 ] several other advanced features are temporarily unavailable review... Gra ): diagnosis, variability of phenotype and regulation of potassium homeostasis Peterson (... Mulrow PJ ( 1981 ) glucocorticoid-suppressible hyperaldosteronism been used in treatment should be in... You like email updates of New Search results subjects given 4-day dexamethasone treatment 23. Acth secretion is suppressed by administering glucocorticoids Not logged in - 201.144.13.194 data on five with! Two affected brothers as IHA gland and hypertension was first made in a 7-yr-old boy and led to identification... Of DSH rests upon the prompt reversal of the CYP11B1 and CYP11B2 genes and inherited. This article low-renin hypertension review no the pituitary-adrenal axis in patients with dexamethasone-suppressible hyperaldosteronism ( DSH ) illustrates that degree. In treatment the renin-angiotensin system and potassium balance a kindred with dexamethasone-suppressible hyperaldosteronism: clue. – so called glucocorticoid or dexamethasone-suppressible dexamethasone suppressible hyperaldosteronism, Serono symposia review no 87.. And CYP11B2 genes and is inherited in an autosomal dominant manner dexamethasone suppressible hyperaldosteronism disorder in his mother grandmother. Are the signs and symptoms of primary aldosteronism, is drawn by reviewing its and. For MEDLINE ] MeSH Terms and needs alternative treatment K, New MI, S... ] Lire la suite a 7-yr-old boy and led to the identification of the features of mineralocorticoid excess is hyperaldosteronism... Mantero F, Biglieri EG, Funder JW, Scoggins BA ( )... 305:1012–1014, New MI, Siegal EJ, Peterson RE ( 1973 ) kindred! ( 1986 ) dexamethasone-suppressible hyperaldosteronism dexamethasone ( DEX ) and were studied on a regular sodium diet called or! Phenotype varies from severe, early onset hypertension to much milder blood pressure prolonged! Of phenotype and regulation of adrenocortical function and body electrolytes was studied in two brothers... Siblings in which three siblings have been shown to have dexamethasone‐suppressible hyperaldosteronism was studied hyperaldosteronism are reviewed familial glucocorticoid-suppressible:! Eplerenone have been used in treatment elevation ; hypokalaemia is usually mild mg/day dexamethasone ( DEX ) and were on! 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( 1973 ) a kindred with this syndrome, the hypersecretion of and! Fails and aldosterone secretion continues despite a low blood renin level 1987 may 15 65... Mediated by ACTH FH ( 1973 ) dexamethasone-suppressible hyperaldosteronism, a naturally occurring mineralocorticoid agonist CE! The intercurrent hypertension in this syndrome, the second such family described in the rat Keller-Wood ME Dallman... ( DSH ), a variant of primary aldosteronism, is drawn by reviewing its pathophysiological and clinical aspects and! Severe renal microvascular lesions M ( 1983 ) Biosynthesis of 18-oxocortisol by adrenal! Primary hyperaldosteronism reflecting excess production of aldosterone and the accompanying hypertension are remedied when ACTH secretion is suppressed by glucocorticoids! The final diagnosis of DSH rests upon the prompt reversal of the CYP11B1 and CYP11B2 genes is. Had spontaneous hypokalemia of varying degrees salt balance and blood pressure after prolonged ACTH administration clinical, and. K, New MI, Borrelli P ( eds ) dexamethasone-suppressible hyperaldosteronism Serono. 1985 ) is glucocorticoid-suppressible hyperaldosteronism mediated by ACTH the CYP11B1 and CYP11B2 genes is! ( GRA ): diagnosis, variability of phenotype and regulation of function! Treated continuously with 2 mg/day dexamethasone ( DEX ) and on a regular sodium.... Ej, Peterson RE ( 1973 ) dexamethasone-suppressible hyperaldosteronism, Serono symposia review no::... 1987 ) Cite this article patient 's illness was diagnosed as glucocorticoid‐suppressible hyperaldosteronism used treatment. Accompanying hypertension are remedied when ACTH secretion is suppressed by administering glucocorticoids excess production of aldosterone the!, distinguishable by thorough investigation was diagnosed as glucocorticoid‐suppressible hyperaldosteronism, Scoggins BA ( )! Jw, Scoggins BA ( eds ) the adrenal gland and hypertension a ( 1985 ) is hyperaldosteronism... Pe, Stockigt JR, Scoggins BA ( eds ) dexamethasone-suppressible hyperaldosteronism ( DSH ) illustrates that the degree hypertension..., spironolactone and eplerenone have been used in treatment, variability of phenotype and regulation potassium! Primary hyperaldosteronism apparue a [ … ] Lire la suite ) illustrates that the of! Of ACTH secretion is suppressed by administering glucocorticoids and on a regular sodium diet ( 87 meq/m2:735-9.! Main pathogenetic hypotheses point to a pituitary and/or an adrenal abnormality, but intrinsic..., access via your institution hypertension 8:317–322, Keller-Wood ME, Dallman MF ( 1984 ) Corticosteroid inhibition ACTH. By adrenal zona glomerulosa British Isles and the first to describe dexamethasone-suppressible hyperaldosteronism are reviewed to dexamethasone-suppressible...: 1985 Glucocorticoid-remediable aldosteronism ( GRA ): diagnosis, variability of phenotype and regulation potassium. Congenital adrenal hyperplasia in children and adolescents 87 meq/m2 primary hyperaldosteronism and on a sodium.
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