Apparent mineralocorticoid excess syndromes

Shimojo, M.; Stewart, Paul M.

doi:10.1007/bf03349763

Cited by 23Hormonal Regulation and HypertensionAdrenal Hormones and DisordersSexual Differentiation and Disorders

Apparent mineralocorticoid excess syndromes

Shimojo, M., Stewart, Paul M.·1995·Journal of Endocrinological Investigation

DOI 10.1007/bf03349763

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Abstract

Apparent mineralocorticoid excess (AME) is a syndrome attributable to congenital deficiency of the enzyme 11 beta-dehydrogenase (11 beta-OHSD) which converts active glucocorticoid cortisol to inactive cortisone. When 11 beta-OHSD activity is impaired, cortisol acts as a potent mineralocorticoid and causes hypertension and hypokalemia with a suppression of the renin-angiotensin-aldosterone system. The increased ratio of urinary cortisol/cortisone metabolites and a prolonged half-life of cortisol are useful for the diagnosis. Dexamethasone and/or potassium sparing diuretics have been used for medication of AME. Licorice ingestion induces a mineralocorticoid excess state, and it seems that this is the result of acquired inhibition of 11 beta-DH by glycyrrhetinic acid. The existence of a second 11 beta-OHSD isoform has been suggested strongly for a long time, and recently, a human 11 beta-OHSD 2 cDNA has been isolated. It appears that 11 beta-OHSD 2 conveys specificity upon the renal MR, and a defect in its activity seems likely to account for the phenotype of AME.

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HSD11B2 converts cortisol to cortisone to protect the MR from activation by cortisol, allowing aldosterone to bind.
“HSD11B2 converts cortisol to cortisone to protect the MR from activation by cortisol, thus allowing the dedicated MR agonist aldosterone to bind [[228], [229], [230], [231]].”
From Human steroid biosynthesis, metabolism and excretion are differentially reflected by serum and urine steroid metabolomes: a comprehensive review (2019) · cites this paper as [229]
HSD11B2 deficiency caused by inborn mutations or excess consumption of HSD11B2 inhibitors leads to unwanted excess mineralocorticoid receptor activation by cortisol, resulting in apparent mineralocorticoid excess.
“Therefore, HSD11B2 deficiency caused by inborn mutations or excess consumption of HSD11B2 inhibitors (carbenoxolone, licorice) (102, 103) leads to unwanted excess MR activation by cortisol, resulting in apparent mineralocorticoid excess (AME) (104).”
From Steroid Metabolome Analysis in Disorders of Adrenal Steroid Biosynthesis and Metabolism (2019) · cites this paper as [104]

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APPARENT MINERALOCORTICOID EXCESS AND DEFICIENT 11β‐OXIDATION OF CORTISOL IN A YOUNG FEMALE
Harinck, H. I. J., van Brummelen, P., van Seters, A. P., & Moolenaar, A. J. (1984) · Clinical Endocrinology · doi:10.1111/j.1365-2265.1984.tb01388.x · Cited by 36 · see citing works
Extravascular CBG-like sites in rat kidney and mineralocorticoid receptor specificity
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Localization of Renal 1 β-Dehydrogenase byin SituHybridization: Autocrine not Paracrine Protector of the Mineralocorticoid Receptor
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The “Mineralocorticoid-Like” Actions Conferred on Corticosterone by Carbenoxolone Are Inhibited by the Mineralocorticoid Receptor (Type I) Antagonist RU28318*
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From behandling van ulcus ventriculi to ulcus duodeni met succus liquiritiae.
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Spironolactone-reversible rickets associated with 11β-hydroxysteroid dehydrogenase deficiency syndrome
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Endogenous inhibitors of 11 beta-hydroxysteroid dehydrogenase in hypertension
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Corticosteroids, kidneys, sweet roots and dirty drugs
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The prismatic case of apparent mineralocorticoid excess.
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Investigation of the mechanism of hypertension in apparent mineralocorticoid excess
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Evidence for Cortisol as the mineralocorticoid in the syndrome of apparent mineralocorticoid excess
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THE ACTION OF MONO-AMMONIUM GLYCYRRHIZINATE ON ADRENALECTOMIZED SUBJECTS AND ITS SYNERGISM WITH HYDROCORTISONE*
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Endocrine involvement in licorice hypertension
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Type I corticosteroid receptor-like immunoreactivity in the rat salivary glands and distal colon: modulation by corticosteroids
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11 beta-hydroxysteroid dehydrogenase and corticosteroid hormone receptors in the rat colon
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Steroid characteristics of mineralocorticoid adrenocortical hypertension
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Enzyme activities and maturation in unstimulated and exogenous gonadotropin-stimulated human oocytes
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Pseudoaldosteronism (Liddle's Syndrome): Evidence for Increased Cell Membrane Permeability to Na+
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Liddle syndrome
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Flagged first, then by citations

2 of the 106 references couldn’t be matched to a known paper.

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11β-Hydroxysteroid Dehydrogenase
Stewart, P. M., & Krozowski, Z. S. (1997) · Vitamins and hormones · doi:10.1016/s0083-6729(08)60646-9 · Cited by 716 · see citing works
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Steroid Metabolome Analysis in Disorders of Adrenal Steroid Biosynthesis and Metabolism
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Liquorice and its health implications
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Cortisol and cortisone analysis in serum and plasma by atmospheric pressure photoionization tandem mass spectrometry
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Molecular Basis for Hypertension in the “Type II Variant” of Apparent Mineralocorticoid Excess
Li, A., Tedde, R., Krozowski, Z., Pala, A., Li, K. X. Z., Shackleton, C. H. L., Mantero, F., Palermo, M., & Stewart, P. M. (1998) · The American Journal of Human Genetics · doi:10.1086/301955 · Cited by 105 · see citing works
Apparent mineralocorticoid excess syndrome: an overview
Palermo, M., Quinkler, M., & Stewart, P. M. (2004) · Arquivos Brasileiros de Endocrinologia & Metabologia · doi:10.1590/s0004-27302004000500015 · Cited by 92 · see citing works
Endometrial Stromal Decidualization Responds Reversibly to Hormone Stimulation and Withdrawal
Yu, J., Berga, S. L., Johnston-MacAnanny, E. B., Sidell, N., Bagchi, I. C., Bagchi, M. K., & Taylor, R. N. (2016) · Endocrinology · doi:10.1210/en.2015-1942 · Cited by 61 · see citing works
Congenital deficiency of 11β-hydroxysteroid dehydrogenase (apparent mineralocorticoid excess syndrome): Diagnostic value of urinary free cortisol and cortisone
Palermo, M., Delitala, G., Mantero, F., Stewart, P. M., & Shackleton, C. H. L. (2001) · Journal of Endocrinological Investigation · doi:10.1007/bf03343803 · Cited by 51 · see citing works
Effect of the neuropeptide substance P on the rat bone marrow-derived osteogenic cells in vitro
Adamus, M., & browski, Z. J. D. (2001) · Journal of Cellular Biochemistry · doi:10.1002/1097-4644(20010601)81:3 · Cited by 54 · see citing works
Quantitative analysis of human serum corticosterone by high-performance liquid chromatography coupled to electrospray ionization mass spectrometry
Ghulam, A., Kouach, M., Racadot, A., Boersma, A., Vantyghem, M., & Briand, G. (1999) · Journal of Chromatography B Biomedical Sciences and Applications · doi:10.1016/s0378-4347(99)00048-1 · Cited by 26 · see citing works
Recent advances in the assessment of the ratios of cortisol to cortisone and of some of their metabolites in urine by LC‐MS‐MS
Saba, A., Raffaelli, A., Cupisti, A., Petri, A., Marcocci, C., & Salvadori, P. A. (2008) · Journal of Mass Spectrometry · doi:10.1002/jms.1530 · Cited by 24 · see citing works
Does kidney transplantation normalise cortisol metabolism in apparent mineralocorticoid excess syndrome?
Palermo, M., Delitala, G., Sorba, G., Cossu, M. L., Satta, R. P., Tedde, R., Pala, A., & Shackleton, C. H. L. (2000) · Journal of Endocrinological Investigation · doi:10.1007/bf03343755 · Cited by 24 · see citing works
Grapefruit juice inhibits 11β‐hydroxysteroid dehydrogenase in vivo, in man
Palermo, M., Armanini, D., & Delitala, G. (2003) · Clinical Endocrinology · doi:10.1046/j.1365-2265.2003.01806.x · Cited by 18 · see citing works
Adrenal minerlocorticoids pathway and its clinical applications
Ghulam, A., Vantyghem, M., Wémeau, J.-L., & Boersma, A. (2003) · Clinica Chimica Acta · doi:10.1016/s0009-8981(03)00045-7 · Cited by 14 · see citing works
The adrenal cortex
Bertholf, R. L., Jialal, I., & Winter, W. E. (2012) · Tietz Textbook of Clinical Chemistry and Molecular Diagnostics · doi:10.1016/b978-1-4160-6164-9.00054-8 · Cited by 5 · see citing works
Edema, Enigma: 11 B-Hydroxysteroid Dehydrogenase Type 2 Inhibition by Sweetener “Stevia”
Esmail, S., & Kabadi, U. M. (2012) · Open Journal of Endocrine and Metabolic Diseases · doi:10.4236/ojemd.2012.23007 · Cited by 4 · see citing works
Evaluation of three herbal compounds used for the management of lower urinary tract disease in healthy cats: a pilot study
Daniels, M., Bartges, J. W., Raditic, D., Marsden, S., COX, S. K., & Callens, A. J. (2017) · Journal of Feline Medicine and Surgery · doi:10.1177/1098612x17748241 · Cited by 4 · see citing works
Hypertension and Severe Hypokalemia Associated With Oral Ingestion of Topical Hydrocortisone Cream
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Aldosterone and Vascular Damage
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revisão Apparent Mineralocorticoid Excess Syndrome: An Overview
Palermo, M., Quinkler, M. O., & Stewart, P. M. (2004)

Apparent mineralocorticoid excess: Type I and type II
Mantero, F., Palermo, M., Petrelli, M., Tedde, R., Stewart, P. M., & Shackleton, C. H. L. (1996) · Steroids · doi:10.1016/0039-128x(96)00012-8 · Cited by 69 · see citing works
Congenital and acquired syndromes of apparent mineralocorticoid excess
Edwards, C. R. W., Walker, B. R., Benediktsson, R., & Seckl, J. R. (1993) · The Journal of Steroid Biochemistry and Molecular Biology · doi:10.1016/0960-0760(93)90114-c · Cited by 49 · see citing works
Apparent Mineralocorticoid Excess
Benediktsson, R., & Edwards, C. R. W. (2006) · Springer eBooks · doi:10.1007/3-540-29623-9_6194 · Cited by 8 · see citing works
11β-Hydroxysteroid Dehydrogenase and Its Role in the Syndrome of Apparent Mineralocorticoid Excess
White, P. C., Mune, T., Rogerson, F. M., Kayes, K. M., & Agarwal, A. K. (1997) · Pediatric Research · doi:10.1203/00006450-199701000-00004 · Cited by 46 · see citing works
The advances in research of apparent mineralocorticoid excess
Xiang-hu, M. (2009) · International Journal of Internal Medicine

Authors: Shimojo, M., & Stewart, P. M.
Affiliations: Queen Elizabeth Hospital Birmingham (GB)
ORCID: Stewart, Paul M.
Venue: Journal of Endocrinological Investigation
Publisher: Springer Science and Business Media LLC
Published: 1995-07
Volume: 18
Issue: 7
Pages: 518-532
ISSN: 0391-4097 (ISSN-L)
1720-8386
DOI: 10.1007/bf03349763
OpenAlex: W2152483462
Cited by: 23
References: 106
Citations by year: 2022: 1 · 2019: 2 · 2017: 1 · 2016: 1 · 2012: 2
FWCI: 0.23
Type: journal article
Language: en
Open access: closed
Licence: www.springernature.com/gp/researchers/text-and-data-mining (tdm)
www.springernature.com/gp/researchers/text-and-data-mining (vor)
Locations: Journal of Endocrinological Investigation (publishedVersion)
PubMed (publishedVersion)
Topics: Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal Regulation and Hypertension
Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
Keywords: apparent mineralocorticoid excess, cortisol, cortisone, hypertension, hypokalemia, dexamethasone, potassium-sparing diuretics, licorice, glycyrrhetinic acid, mineralocorticoid receptor
MeSH terms: Animals, Humans, Hydrocortisone, Hydroxysteroid Dehydrogenases, Hypertension, Hypokalemia, Mineralocorticoids, Syndrome, 11-beta-Hydroxysteroid Dehydrogenases

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Apparent mineralocorticoid excess syndromes

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