Hyperaldosteronism: Difference between revisions
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{{CMG}}; {{AE}} {{MJ}} | {{CMG}}; {{AE}} {{MJ}} {{HK}} | ||
'''This page contains general information about | '''This page contains general information about hyperaldosteronism. For more information on specific types, please visit the pages on [[Primary hyperaldosteronism|primary hyperaldosteronism]]. | ||
{{SK}} Aldosteronism | {{SK}} Aldosteronism | ||
== Overview == | == Overview == | ||
Hyperaldosteronism is a clinical scenario of [[mineralocorticoid excess]] with resistant [[hypertension]], [[hypokalemia]], and [[metabolic alkalosis]] due to increased [[hydrogen]] ion excretion. Aldosteronism may be classified into three types, [[primary hyperaldosteronism]] (Conn's syndrome), secondary hyperaldosteronism, and pseudohyperaldosteronism. [[Primary hyperaldosteronism]] can caused by [[aldosterone]]-secreting [[adenoma]], bilateral [[hyperplasia]] of the adrenal glands, and ectopic secretion of aldosterone from ovaries and kidneys. [[Primary hyperaldosteronism]] features overproduction of [[aldosterone]] despite suppressed [[plasma renin activity]] (PRA). The resulting Na+ retention produces [[hypertension]], and increased K+ excretion may cause hypokalemia. Secondary hyperaldosteronism is caused by high [[renin]] and subsequently [[aldosterone]] level, such as [[renovascular]] causes and reninoma. Pseudohyperaldosteronism is the clinical presentation of hyperaldosteronism such as resistant [[hypertension]], [[hypokalemia]], and [[metabolic alkalosis]] due to factors other than [[renin]] and [[aldosterone]]. The treatment should be prescribed for the blockade of [[aldosterone]] effects, or based on underlying disease. | |||
== Classification == | == Classification == | ||
Aldosteronism and [[mineralocorticoid excess]] may be classified into | Aldosteronism and [[mineralocorticoid excess]] may be classified into three types, [[primary hyperaldosteronism]] (conn's syndrome), secondary hyperaldosteronism, and pseudohyperaldosteronism. The different types of mineralocorticoid excess are described below. | ||
== Primary hyperaldosteronism (conn's syndrome) == | |||
Primary hyperaldosteronism causes categorizes as below table: <ref name="pmid24800505">{{cite journal |vauthors=Wada N, Jin S, Hui SP, Yanagisawa K, Kurosawa T, Chiba H |title=[Differential diagnosis of primary aldosteronism by measurement of hybrid steroids using mass spectrometry] |language=Japanese |journal=Rinsho Byori |volume=62 |issue=3 |pages=276–82 |year=2014 |pmid=24800505 |doi= |url=}}</ref><ref name="pmid22487411">{{cite journal |vauthors=Nielsen ML, Pareek M, Andersen I |title=[Liquorice-induced hypertension and hypokalaemia] |language=Danish |journal=Ugeskr. Laeg. |volume=174 |issue=15 |pages=1024–5 |year=2012 |pmid=22487411 |doi= |url=}}</ref><ref name="pmid21962616">{{cite journal |vauthors=Chow KM, Ma RC, Szeto CC, Li PK |title=Polycystic kidney disease presenting with hypertension and hypokalemia |journal=Am. J. Kidney Dis. |volume=59 |issue=2 |pages=270–2 |year=2012 |pmid=21962616 |doi=10.1053/j.ajkd.2011.08.020 |url=}}</ref><ref name="pmid22154539">{{cite journal |vauthors=Sarafidis PA, Georgianos PI, Germanidis G, Giavroglou C, Nikolaidis P, Lasaridis AN, Madias NE |title=Hypertension and symptomatic hypokalemia in a patient with simultaneous unilateral stenoses of intrarenal arteries and mesangioproliferative glomerulonephritis |journal=Am. J. Kidney Dis. |volume=59 |issue=3 |pages=434–8 |year=2012 |pmid=22154539 |doi=10.1053/j.ajkd.2011.11.001 |url=}}</ref><ref name="pmid17275580">{{cite journal |vauthors=Khosla N, Hogan D |title=Mineralocorticoid hypertension and hypokalemia |journal=Semin. Nephrol. |volume=26 |issue=6 |pages=434–40 |year=2006 |pmid=17275580 |doi=10.1016/j.semnephrol.2006.10.004 |url=}}</ref><ref name="pmid23953804">{{cite journal |vauthors=Weiner ID |title=Endocrine and hypertensive disorders of potassium regulation: primary aldosteronism |journal=Semin. Nephrol. |volume=33 |issue=3 |pages=265–76 |year=2013 |pmid=23953804 |pmc=3748390 |doi=10.1016/j.semnephrol.2013.04.007 |url=}}</ref><ref name="pmid25715092">{{cite journal |vauthors=Martell-Claros N, Abad-Cardiel M, Alvarez-Alvarez B, García-Donaire JA, Pérez CF |title=Primary aldosteronism and its various clinical scenarios |journal=J. Hypertens. |volume=33 |issue=6 |pages=1226–32 |year=2015 |pmid=25715092 |doi=10.1097/HJH.0000000000000546 |url=}}</ref><ref name="pmid10818057">{{cite journal |vauthors=Franse LV, Pahor M, Di Bari M, Somes GW, Cushman WC, Applegate WB |title=Hypokalemia associated with diuretic use and cardiovascular events in the Systolic Hypertension in the Elderly Program |journal=Hypertension |volume=35 |issue=5 |pages=1025–30 |year=2000 |pmid=10818057 |doi= |url=}}</ref><ref name="pmid21525970">{{cite journal |vauthors=Rossi E, Farnetti E, Nicoli D, Sazzini M, Perazzoli F, Regolisti G, Grasselli C, Santi R, Negro A, Mazzeo V, Mantero F, Luiselli D, Casali B |title=A clinical phenotype mimicking essential hypertension in a newly discovered family with Liddle's syndrome |journal=Am. J. Hypertens. |volume=24 |issue=8 |pages=930–5 |year=2011 |pmid=21525970 |doi=10.1038/ajh.2011.76 |url=}}</ref><ref name="pmid25968592">{{cite journal |vauthors=Ruecker B, Lang-Muritano M, Spanaus K, Welzel M, l'Allemand D, Phan-Hug F, Katschnig C, Konrad D, Holterhus PM, Schoenle EJ |title=The Aldosterone/Renin Ratio as a Diagnostic Tool for the Diagnosis of Primary Hypoaldosteronism in Newborns and Infants |journal=Horm Res Paediatr |volume=84 |issue=1 |pages=43–8 |year=2015 |pmid=25968592 |doi=10.1159/000381852 |url=}}</ref><ref name="pmid25908467">{{cite journal |vauthors=Ardhanari S, Kannuswamy R, Chaudhary K, Lockette W, Whaley-Connell A |title=Mineralocorticoid and apparent mineralocorticoid syndromes of secondary hypertension |journal=Adv Chronic Kidney Dis |volume=22 |issue=3 |pages=185–95 |year=2015 |pmid=25908467 |doi=10.1053/j.ackd.2015.03.002 |url=}}</ref><ref name="pmid19174076">{{cite journal |vauthors=Iglesias P, Tajada P, Martínez I, Díez JJ |title=[Salt-wasting congenital adrenal hyperplasia associated to hyperreninemic hyperaldosteronism] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=132 |issue=2 |pages=80–1 |year=2009 |pmid=19174076 |doi=10.1016/j.medcli.2008.09.002 |url=}}</ref><ref name="pmid3413779">{{cite journal |vauthors=Kikuta Y, Sanjo K, Nakajima K, Ashizawa I, Ojima M |title=Primary aldosteronism in childhood due to primary adrenal hyperplasia |journal=Tohoku J. Exp. Med. |volume=155 |issue=1 |pages=57–70 |year=1988 |pmid=3413779 |doi= |url=}}</ref><ref name="pmid21494136">{{cite journal |vauthors=Hassan-Smith Z, Stewart PM |title=Inherited forms of mineralocorticoid hypertension |journal=Curr Opin Endocrinol Diabetes Obes |volume=18 |issue=3 |pages=177–85 |year=2011 |pmid=21494136 |doi=10.1097/MED.0b013e3283469444 |url=}}</ref><ref name="pmid4299011">{{cite journal |vauthors=Bartter FC, Henkin RI, Bryan GT |title=Aldosterone hypersecretion in "non-salt-losing" congenital adrenal hyperplasia |journal=J. Clin. Invest. |volume=47 |issue=8 |pages=1742–52 |year=1968 |pmid=4299011 |pmc=297334 |doi=10.1172/JCI105864 |url=}}</ref> | |||
{| class="wikitable" | {| class="wikitable" | ||
! rowspan="9" |Primary hyperaldosteronism | ! rowspan="9" |[[Primary hyperaldosteronism]] | ||
!Category | !Category | ||
!Diseases | !Diseases | ||
|- | |- | ||
| rowspan="2" |[[Adrenal gland|Adrenal]] causes | | rowspan="2" |[[Adrenal gland|Adrenal]] causes | ||
| | |[[Aldosterone]]-secreting [[Adrenal gland|adrenal]] [[adenoma]] | ||
|- | |- | ||
| | |Idiopathic hyperaldosteronism | ||
* | * Bilateral [[hyperplasia]] of the [[adrenal gland]] | ||
|- | |- | ||
|Extra-adrenal causes | |Extra-adrenal causes | ||
| | |[[Ectopic]] secretion of [[aldosterone]] | ||
* From the [[ovaries]] and [[kidneys]] | |||
|- | |- | ||
| rowspan="3" |Familial hyperaldosteronism | | rowspan="3" |Familial hyperaldosteronism | ||
| | |Familial hyperaldosteronism type I | ||
* Glucocorticoid-remediable aldosteronism [GRA] | |||
|- | |- | ||
| | |Familial hyperaldosteronism II | ||
* Familial | * Familial occurrence of [[Aldosterone|aldosterone-]]<nowiki/>secreting [[Adrenal gland|adrenal]] [[adenoma]] or bilateral idiopathic hyperplasia or both | ||
|- | |- | ||
| | |Familial hyperaldosteronism type III | ||
* Associated with the germline mutation in the [[KCNJ5]] potassium channel) | |||
|- | |- | ||
| rowspan="2" |Other | | rowspan="2" |Other | ||
| | |Pure [[aldosterone]]-producing [[Adrenal carcinoma|adrenocortical carcinomas]] | ||
|- | |- | ||
| | |Unilateral [[adrenal hyperplasia]] | ||
|} | |||
| | |||
== Secondary hyperaldosteronism == | |||
| | Secondary hyperaldosteronism causes are categorized as below, and each specific disease is described in the related micro-chapter. <ref name="pmid12943516">{{cite journal |vauthors=Stewart PM |title=Tissue-specific Cushing's syndrome, 11beta-hydroxysteroid dehydrogenases and the redefinition of corticosteroid hormone action |journal=Eur. J. Endocrinol. |volume=149 |issue=3 |pages=163–8 |year=2003 |pmid=12943516 |doi= |url=}}</ref><ref name="pmid12788832">{{cite journal |vauthors=Quinkler M, Stewart PM |title=Hypertension and the cortisol-cortisone shuttle |journal=J. Clin. Endocrinol. Metab. |volume=88 |issue=6 |pages=2384–92 |year=2003 |pmid=12788832 |doi=10.1210/jc.2003-030138 |url=}}</ref><ref name="pmid1569172">{{cite journal |vauthors=Ulick S, Wang JZ, Blumenfeld JD, Pickering TG |title=Cortisol inactivation overload: a mechanism of mineralocorticoid hypertension in the ectopic adrenocorticotropin syndrome |journal=J. Clin. Endocrinol. Metab. |volume=74 |issue=5 |pages=963–7 |year=1992 |pmid=1569172 |doi=10.1210/jcem.74.5.1569172 |url=}}</ref><ref name="pmid8504732">{{cite journal |vauthors=Whorwood CB, Sheppard MC, Stewart PM |title=Licorice inhibits 11 beta-hydroxysteroid dehydrogenase messenger ribonucleic acid levels and potentiates glucocorticoid hormone action |journal=Endocrinology |volume=132 |issue=6 |pages=2287–92 |year=1993 |pmid=8504732 |doi=10.1210/endo.132.6.8504732 |url=}}</ref><ref name="pmid13879332">{{cite journal |vauthors=CHRISTY NP, LARAGH JH |title=Pathogenesis of hypokalemic alkalosis in Cushing's syndrome |journal=N. Engl. J. Med. |volume=265 |issue= |pages=1083–8 |year=1961 |pmid=13879332 |doi=10.1056/NEJM196111302652203 |url=}}</ref><ref name="pmid26934393">{{cite journal |vauthors=Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, Stowasser M, Young WF |title=The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline |journal=J. Clin. Endocrinol. Metab. |volume=101 |issue=5 |pages=1889–916 |year=2016 |pmid=26934393 |doi=10.1210/jc.2015-4061 |url=}}</ref> | ||
|- | {| class="wikitable" | ||
! | |||
| | !Category | ||
| | !Diseases | ||
| | |||
| | |||
|- | |- | ||
| | | rowspan="9" |Secondary hyperaldosteronism | ||
| | |[[Genetics|Genetic]] mutation | ||
| | |[[Bartter syndrome|Bartter]] and [[Gitelman syndrome|Gitelman syndromes]] (hyperplasia of the [[juxtaglomerular apparatus]], the source of [[renin]] in the [[kidney]]) | ||
| | |||
|- | |- | ||
| rowspan="2" |Endocrine causes | | rowspan="2" |Endocrine causes | ||
|Cushing syndrome | |[[Cushing's syndrome|Cushing syndrome]] | ||
* The main pathogenetic mechanism is linked to the excess | |||
of [[cortisol]] which saturates 11-HSD2 activity, | |||
* This allows [[cortisol]] to bind [[mineralocorticoid receptor]] | |||
|- | |- | ||
|Ectopic ACTH production | |Ectopic [[ACTH]] production (Secondary to [[carcinomas]] such as [[lung cancer]]) | ||
|- | |- | ||
| rowspan="4" |Renovascular | | rowspan="4" |[[Renovascular disease|Renovascular]] | ||
|Kidney transplant | |[[Kidney]] transplant | ||
|- | |- | ||
|Renin-secreting juxtaglomerular cell tumors | |[[Renin]]-secreting [[Juxtaglomerular cells|juxtaglomerular cell tumors]] | ||
|- | |- | ||
|Scleroderma renal crisis | |[[Scleroderma|Scleroderma renal crisis]] | ||
|- | |- | ||
|Malignant hypertension | |[[Malignant hypertension]] | ||
|- | |- | ||
|Tumors | |[[Tumors]] | ||
|Reninoma | |[[Reninoma]] | ||
|- | |- | ||
|Intravascular hypovolemia | |Intravascular [[hypovolemia]] | ||
| | | | ||
* [[Heart failure]] | |||
* Hepatic [[cirrhosis]] | |||
* [[Nephrotic syndrome]] | |||
|} | |} | ||
Pseudohyperaldosteronism causes: | == Pseudohyperaldosteronism causes (low renin) == | ||
Pseudohyperaldosteronism causes are classified as below: <ref name="pmid24800505">{{cite journal |vauthors=Wada N, Jin S, Hui SP, Yanagisawa K, Kurosawa T, Chiba H |title=[Differential diagnosis of primary aldosteronism by measurement of hybrid steroids using mass spectrometry] |language=Japanese |journal=Rinsho Byori |volume=62 |issue=3 |pages=276–82 |year=2014 |pmid=24800505 |doi= |url=}}</ref><ref name="pmid22487411">{{cite journal |vauthors=Nielsen ML, Pareek M, Andersen I |title=[Liquorice-induced hypertension and hypokalaemia] |language=Danish |journal=Ugeskr. Laeg. |volume=174 |issue=15 |pages=1024–5 |year=2012 |pmid=22487411 |doi= |url=}}</ref><ref name="pmid21962616">{{cite journal |vauthors=Chow KM, Ma RC, Szeto CC, Li PK |title=Polycystic kidney disease presenting with hypertension and hypokalemia |journal=Am. J. Kidney Dis. |volume=59 |issue=2 |pages=270–2 |year=2012 |pmid=21962616 |doi=10.1053/j.ajkd.2011.08.020 |url=}}</ref><ref name="pmid22154539">{{cite journal |vauthors=Sarafidis PA, Georgianos PI, Germanidis G, Giavroglou C, Nikolaidis P, Lasaridis AN, Madias NE |title=Hypertension and symptomatic hypokalemia in a patient with simultaneous unilateral stenoses of intrarenal arteries and mesangioproliferative glomerulonephritis |journal=Am. J. Kidney Dis. |volume=59 |issue=3 |pages=434–8 |year=2012 |pmid=22154539 |doi=10.1053/j.ajkd.2011.11.001 |url=}}</ref><ref name="pmid17275580">{{cite journal |vauthors=Khosla N, Hogan D |title=Mineralocorticoid hypertension and hypokalemia |journal=Semin. Nephrol. |volume=26 |issue=6 |pages=434–40 |year=2006 |pmid=17275580 |doi=10.1016/j.semnephrol.2006.10.004 |url=}}</ref><ref name="pmid23953804">{{cite journal |vauthors=Weiner ID |title=Endocrine and hypertensive disorders of potassium regulation: primary aldosteronism |journal=Semin. Nephrol. |volume=33 |issue=3 |pages=265–76 |year=2013 |pmid=23953804 |pmc=3748390 |doi=10.1016/j.semnephrol.2013.04.007 |url=}}</ref><ref name="pmid25715092">{{cite journal |vauthors=Martell-Claros N, Abad-Cardiel M, Alvarez-Alvarez B, García-Donaire JA, Pérez CF |title=Primary aldosteronism and its various clinical scenarios |journal=J. Hypertens. |volume=33 |issue=6 |pages=1226–32 |year=2015 |pmid=25715092 |doi=10.1097/HJH.0000000000000546 |url=}}</ref><ref name="pmid10818057">{{cite journal |vauthors=Franse LV, Pahor M, Di Bari M, Somes GW, Cushman WC, Applegate WB |title=Hypokalemia associated with diuretic use and cardiovascular events in the Systolic Hypertension in the Elderly Program |journal=Hypertension |volume=35 |issue=5 |pages=1025–30 |year=2000 |pmid=10818057 |doi= |url=}}</ref><ref name="pmid21525970">{{cite journal |vauthors=Rossi E, Farnetti E, Nicoli D, Sazzini M, Perazzoli F, Regolisti G, Grasselli C, Santi R, Negro A, Mazzeo V, Mantero F, Luiselli D, Casali B |title=A clinical phenotype mimicking essential hypertension in a newly discovered family with Liddle's syndrome |journal=Am. J. Hypertens. |volume=24 |issue=8 |pages=930–5 |year=2011 |pmid=21525970 |doi=10.1038/ajh.2011.76 |url=}}</ref><ref name="pmid25968592">{{cite journal |vauthors=Ruecker B, Lang-Muritano M, Spanaus K, Welzel M, l'Allemand D, Phan-Hug F, Katschnig C, Konrad D, Holterhus PM, Schoenle EJ |title=The Aldosterone/Renin Ratio as a Diagnostic Tool for the Diagnosis of Primary Hypoaldosteronism in Newborns and Infants |journal=Horm Res Paediatr |volume=84 |issue=1 |pages=43–8 |year=2015 |pmid=25968592 |doi=10.1159/000381852 |url=}}</ref><ref name="pmid25908467">{{cite journal |vauthors=Ardhanari S, Kannuswamy R, Chaudhary K, Lockette W, Whaley-Connell A |title=Mineralocorticoid and apparent mineralocorticoid syndromes of secondary hypertension |journal=Adv Chronic Kidney Dis |volume=22 |issue=3 |pages=185–95 |year=2015 |pmid=25908467 |doi=10.1053/j.ackd.2015.03.002 |url=}}</ref><ref name="pmid19174076">{{cite journal |vauthors=Iglesias P, Tajada P, Martínez I, Díez JJ |title=[Salt-wasting congenital adrenal hyperplasia associated to hyperreninemic hyperaldosteronism] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=132 |issue=2 |pages=80–1 |year=2009 |pmid=19174076 |doi=10.1016/j.medcli.2008.09.002 |url=}}</ref><ref name="pmid3413779">{{cite journal |vauthors=Kikuta Y, Sanjo K, Nakajima K, Ashizawa I, Ojima M |title=Primary aldosteronism in childhood due to primary adrenal hyperplasia |journal=Tohoku J. Exp. Med. |volume=155 |issue=1 |pages=57–70 |year=1988 |pmid=3413779 |doi= |url=}}</ref><ref name="pmid21494136">{{cite journal |vauthors=Hassan-Smith Z, Stewart PM |title=Inherited forms of mineralocorticoid hypertension |journal=Curr Opin Endocrinol Diabetes Obes |volume=18 |issue=3 |pages=177–85 |year=2011 |pmid=21494136 |doi=10.1097/MED.0b013e3283469444 |url=}}</ref><ref name="pmid4299011">{{cite journal |vauthors=Bartter FC, Henkin RI, Bryan GT |title=Aldosterone hypersecretion in "non-salt-losing" congenital adrenal hyperplasia |journal=J. Clin. Invest. |volume=47 |issue=8 |pages=1742–52 |year=1968 |pmid=4299011 |pmc=297334 |doi=10.1172/JCI105864 |url=}}</ref> | |||
{| class="wikitable" | {| class="wikitable" | ||
! rowspan="2" |Pseudohyperaldosteronism causes | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Pseudohyperaldosteronism causes | ||
! rowspan="2" |Disease | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease | ||
! rowspan="2" |Etiology | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Etiology | ||
! rowspan="2" |Clinical features | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Clinical features | ||
! colspan="4" |Labratory | ! colspan="4" align="center" style="background:#4479BA; color: #FFFFFF;" + |Labratory | ||
! | ! rowspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Treatment | ||
|- | |- | ||
!Elevated mineralocorticoid | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Elevated mineralocorticoid | ||
!Renin | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Renin | ||
!Aldosterone | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Aldosterone | ||
!Other | ! align="center" style="background:#4479BA; color: #FFFFFF;" + |Other | ||
|- | |- | ||
| rowspan="9" |Endogenous causes | | rowspan="9" |Endogenous causes | ||
|17 alpha-hydroxylase deficiency | |[[17 alpha-hydroxylase deficiency]] | ||
|Mutations in the [[CYP17A1]] gene | |Mutations in the [[CYP17A1]] gene | ||
| | | | ||
Line 157: | Line 126: | ||
* Minimal [[body hair]] | * Minimal [[body hair]] | ||
| rowspan="2" |Deoxycorticosterone (DOC) | | rowspan="2" |[[Deoxycorticosterone]] ([[Deoxycorticosterone|DOC]]) | ||
| rowspan="2" |↓ | | rowspan="2" |↓ | ||
| rowspan="2" |↓ | | rowspan="2" |↓ | ||
|Cortisol ↓ | |[[Cortisol]] ↓ | ||
| rowspan="2" | | | rowspan="2" |[[Corticosteroids]] | ||
|- | |- | ||
|11β-hydroxylase deficiency | |[[11β-hydroxylase deficiency]] | ||
|Mutations in the [[CYP11B1]] gene | |Mutations in the [[CYP11B1]] gene | ||
| | | | ||
Line 170: | Line 139: | ||
* [[Hypertension]] and [[hypokalemia]] | * [[Hypertension]] and [[hypokalemia]] | ||
* [[Virilization]] | * [[Virilization]] | ||
|Cortisol ↓ | |[[Cortisol]] ↓ | ||
|- | |- | ||
|Apparent mineralocorticoid excess syndrome (AME) | |Apparent mineralocorticoid excess syndrome (AME) | ||
|Genetic or acquired defect of 11-HSD | |Genetic or acquired defect of 11-HSD gene | ||
* [[Cortisone]] decreases and [[cortisol]] accumulates and binds to [[aldosterone]] receptors | |||
| | | | ||
* Severe juvenile [[hypertension]] | |||
* [[Hypercalciuria]], [[nephrocalcinosis]], [[polyuria]] (due to [[hypokalemia]]-induced [[nephrogenic diabetes insipidus]]) | |||
* [[Renal failure]] | |||
|[[Cortisol]] has [[mineralocorticoid]] effects | |||
|↓ | |↓ | ||
|↓ | |↓ | ||
|Urinary free cortisone ↓↓ | |Urinary free [[cortisone]] ↓↓ | ||
| | |[[Dexamethasone]] and/or [[mineralocorticoid]] blockers | ||
|- | |- | ||
|Liddle’s syndrome | |[[Liddle's syndrome|Liddle’s syndrome]] (Pseudohyperaldosteronism type 1) | ||
|Mutation of the epithelial sodium channels (ENaC) gene in the distal renal tubules | |Mutation of the epithelial [[sodium]] channels ([[ENaC]]) [[gene]] in the distal [[renal tubules]] | ||
| | | | ||
* [[Hypertension]] | |||
* [[Hypokalemia]] | |||
|No extra [[mineralocorticoid]] presents, and mutations in [[Sodium|Na]] channels mimic [[aldosterone]] mechanism | |||
|↓ | |↓ | ||
|↓ | |↓ | ||
| | |[[Cortisol]] ↓ | ||
| | |[[Amiloride]] or [[triamterene]] | ||
|- | |- | ||
|Cushing’s syndrome | |[[Cushing’s syndrome]] | ||
| | | | ||
* The main pathogenetic mechanism is linked to the excess | |||
of [[cortisol]] which saturates 11-HSD2 activity, | |||
* This allows [[cortisol]] to bind [[mineralocorticoid receptor]] | |||
|Rapid [[Obesity|weight gain]], particularly of the [[trunk]] and [[face]] with [[limbs]] sparing ([[central obesity]]) | |||
* Proximal [[muscle weakness]] | |||
* A [[round face]] often referred to as a "[[moon face]]" | |||
* Excess [[sweating]] | |||
* [[Headache]] | |||
|[[Cortisol]] has [[mineralocorticoid]] effects | |||
|↓ | |↓ | ||
| | | | ||
* ↓ if excess cortisol saturates 11-HSD2 enzyme activity | * ↓ if excess [[cortisol]] saturates 11-HSD2 enzyme activity | ||
* ↑ in direct activation of renin angiotensin system activation by glucocorticoids | * ↑ in direct activation of [[renin]] [[angiotensin]] system activation by [[glucocorticoids]] | ||
|Urinary free cortisol markedly ↑↑ | |Urinary free [[cortisol]] markedly ↑↑ | ||
| | | | ||
* [[Pasireotide]], [[Cabergoline]], [[Ketoconazole]], and [[Metyrapone]] | |||
* Adrenalectomy | |||
|- | |- | ||
|Insensitivity to glucocorticoids (Chrousos syndrome) | |Insensitivity to [[glucocorticoids]] (Chrousos syndrome) | ||
| | |Mutations in [[glucocorticoid receptor]] (GR) gene | ||
| | | | ||
|Deoxycorticosterone (DOC) | * [[Hypertension]] | ||
* Adrenal [[hyperandrogenism]] | |||
|[[Deoxycorticosterone]] ([[Deoxycorticosterone|DOC]]) | |||
|↓ | |↓ | ||
|↓ | |↓ | ||
| | |[[Cortisol]] | ||
| | |[[Dexamethasone]] | ||
|- | |- | ||
| | |[[Cortisol]]-secreting adrenocortical [[carcinoma]] | ||
| | |Multifactorial | ||
| | | | ||
Rapid [[Obesity|weight gain]], particularly of the [[trunk]] and [[face]] with [[limbs]] sparing ([[central obesity]]) | |||
* Proximal [[muscle weakness]] | |||
* A [[round face]] often referred to as a "[[moon face]]" | |||
* Excess [[sweating]] | |||
* [[Headache]] | |||
|[[Cortisol]] has [[mineralocorticoid]] effects | |||
|↓ | |||
| | | | ||
* ↓ if excess [[cortisol]] saturates 11-HSD2 enzyme activity | |||
* ↑ in direct activation of [[renin]] [[angiotensin]] system activation by [[glucocorticoids]] | |||
|Urinary free [[cortisol]] markedly ↑↑ | |||
|[[Surgery]] | |||
|- | |- | ||
|Geller’s syndrome | |Geller’s syndrome | ||
| | |[[Mutation]] of [[mineralocorticoid]] (MR) receptor that alters its specificity and allows [[progesterone]] to bind MR | ||
| | |Severe [[hypertension]] particularly during [[pregnancy]] | ||
| | |[[Progesterone]] has [[mineralocorticoid]] effects | ||
|↓ | |↓ | ||
|↓ | |↓ | ||
| | | - | ||
| | |[[mineralocorticoid]] blockers | ||
|- | |- | ||
|Gordon’s syndrome | |Gordon’s syndrome (Pseudohypoaldosteronism type 2) | ||
| | |Mutations of at least four genes have been identified, including WNK1 and WNK4 | ||
| | | | ||
* [[Hypertension]] | |||
* [[Hyperkalemia]] | |||
* Normal renal function | |||
|No excess mineralocorticoid; an increased activity of the thiazide-sensitive Na–Cl co-transporter in the distal tubule | |||
|↓ | |↓ | ||
| | |Normal | ||
| | |Hyperkalemia | ||
|thiazide diuretics and/or dietary sodium restriction | |thiazide diuretics and/or dietary sodium restriction | ||
|- | |- | ||
| rowspan=" | | rowspan="4" |Exogenous causes | ||
|Corticosteroids with mineralocorticoid activity | |Corticosteroids with mineralocorticoid activity | ||
|Fludrocortisone or fluoroprednisolone can mimic the action of aldosterone, | |||
| | | | ||
* [[Hypertension]] | |||
* [[Hypokalemia]] | |||
|Medications such as fludrocortisone | |||
|↓ | |||
|↓ | |||
| - | |||
|Change the treatment | |||
| | |||
| | |||
| | |||
|- | |||
| | |||
|- | |- | ||
|Licorice ingestion | |Licorice ingestion | ||
|[[Glycyrrhetinic acid]] that binds [[mineralocorticoid]] receptor and blocks 11-HSD2 at the level of classical target tissues of [[aldosterone]] | |||
| | | | ||
* [[Hypertension]] | |||
| | |||
* [[Hypokalemia]] | |||
|<nowiki>-</nowiki> | |||
|↓ | |↓ | ||
|↓ | |↓ | ||
|Urinary free cortisol Moderate ↑ | |Urinary free cortisol Moderate ↑ | ||
| | |Discontinue licorice | ||
|- | |- | ||
| | |Grapefruit | ||
|High assumption of naringenin, a component of grapefruit, can also block 11-HSD | |||
| | |||
| | | | ||
* [[Hypertension]] | |||
| - | |||
|↓ | |||
|↓ | |||
| - | |||
|Discontinue grapefruit | |||
|- | |- | ||
| | |[[Estrogens]] | ||
|[[Estrogens]] can retain [[sodium]] and water by different mechanisms, causing: | |||
* Increased blood pressure values and suppressing the [[renin]] [[aldosterone]] system, on the other side inducing secondary hyperaldosteronism due to the stimulation of the synthesis of [[angiotensinogen]] | |||
| | |||
| | | | ||
* [[Hypertension]] | |||
* [[Headache]] | |||
* [[Edema]] | |||
* [[Weight gain]] | |||
|<nowiki>-</nowiki> | |||
|↓ | |||
|↓ | |||
| - | |||
|Discontinue [[estrogens]] | |||
|} | |} | ||
=== Other less common causes of pseudohyperaldosteronism are: === | |||
* Sclerosis of [[juxtaglomerular apparatus]] ([[diabetic]] [[microangiopathy]] and/or of the [[elderly]]) | |||
* Low-[[renin]] essential [[hypertension]] | |||
* [[Adrenergic agonist|B-Adrenergic agonists]] | |||
* Partial/total [[nephrectomy]] or removal of [[renal]] tissue | |||
* [[Autonomic dysfunction]] | |||
== Differentiating Diagnosis == | == Differentiating Diagnosis == | ||
Hyperaldosteronism should be differentiated from other diseases causing '''[[hypertension]]''' and '''[[hypokalemia]]''' for example: | Hyperaldosteronism should be differentiated from other diseases causing '''[[hypertension]]''' and '''[[hypokalemia]]''' for example:<ref name="pmid24800505">{{cite journal |vauthors=Wada N, Jin S, Hui SP, Yanagisawa K, Kurosawa T, Chiba H |title=[Differential diagnosis of primary aldosteronism by measurement of hybrid steroids using mass spectrometry] |language=Japanese |journal=Rinsho Byori |volume=62 |issue=3 |pages=276–82 |year=2014 |pmid=24800505 |doi= |url=}}</ref><ref name="pmid22487411">{{cite journal |vauthors=Nielsen ML, Pareek M, Andersen I |title=[Liquorice-induced hypertension and hypokalaemia] |language=Danish |journal=Ugeskr. Laeg. |volume=174 |issue=15 |pages=1024–5 |year=2012 |pmid=22487411 |doi= |url=}}</ref><ref name="pmid21962616">{{cite journal |vauthors=Chow KM, Ma RC, Szeto CC, Li PK |title=Polycystic kidney disease presenting with hypertension and hypokalemia |journal=Am. J. Kidney Dis. |volume=59 |issue=2 |pages=270–2 |year=2012 |pmid=21962616 |doi=10.1053/j.ajkd.2011.08.020 |url=}}</ref><ref name="pmid22154539">{{cite journal |vauthors=Sarafidis PA, Georgianos PI, Germanidis G, Giavroglou C, Nikolaidis P, Lasaridis AN, Madias NE |title=Hypertension and symptomatic hypokalemia in a patient with simultaneous unilateral stenoses of intrarenal arteries and mesangioproliferative glomerulonephritis |journal=Am. J. Kidney Dis. |volume=59 |issue=3 |pages=434–8 |year=2012 |pmid=22154539 |doi=10.1053/j.ajkd.2011.11.001 |url=}}</ref><ref name="pmid17275580">{{cite journal |vauthors=Khosla N, Hogan D |title=Mineralocorticoid hypertension and hypokalemia |journal=Semin. Nephrol. |volume=26 |issue=6 |pages=434–40 |year=2006 |pmid=17275580 |doi=10.1016/j.semnephrol.2006.10.004 |url=}}</ref><ref name="pmid23953804">{{cite journal |vauthors=Weiner ID |title=Endocrine and hypertensive disorders of potassium regulation: primary aldosteronism |journal=Semin. Nephrol. |volume=33 |issue=3 |pages=265–76 |year=2013 |pmid=23953804 |pmc=3748390 |doi=10.1016/j.semnephrol.2013.04.007 |url=}}</ref><ref name="pmid25715092">{{cite journal |vauthors=Martell-Claros N, Abad-Cardiel M, Alvarez-Alvarez B, García-Donaire JA, Pérez CF |title=Primary aldosteronism and its various clinical scenarios |journal=J. Hypertens. |volume=33 |issue=6 |pages=1226–32 |year=2015 |pmid=25715092 |doi=10.1097/HJH.0000000000000546 |url=}}</ref><ref name="pmid10818057">{{cite journal |vauthors=Franse LV, Pahor M, Di Bari M, Somes GW, Cushman WC, Applegate WB |title=Hypokalemia associated with diuretic use and cardiovascular events in the Systolic Hypertension in the Elderly Program |journal=Hypertension |volume=35 |issue=5 |pages=1025–30 |year=2000 |pmid=10818057 |doi= |url=}}</ref><ref name="pmid21525970">{{cite journal |vauthors=Rossi E, Farnetti E, Nicoli D, Sazzini M, Perazzoli F, Regolisti G, Grasselli C, Santi R, Negro A, Mazzeo V, Mantero F, Luiselli D, Casali B |title=A clinical phenotype mimicking essential hypertension in a newly discovered family with Liddle's syndrome |journal=Am. J. Hypertens. |volume=24 |issue=8 |pages=930–5 |year=2011 |pmid=21525970 |doi=10.1038/ajh.2011.76 |url=}}</ref><ref name="pmid25968592">{{cite journal |vauthors=Ruecker B, Lang-Muritano M, Spanaus K, Welzel M, l'Allemand D, Phan-Hug F, Katschnig C, Konrad D, Holterhus PM, Schoenle EJ |title=The Aldosterone/Renin Ratio as a Diagnostic Tool for the Diagnosis of Primary Hypoaldosteronism in Newborns and Infants |journal=Horm Res Paediatr |volume=84 |issue=1 |pages=43–8 |year=2015 |pmid=25968592 |doi=10.1159/000381852 |url=}}</ref><ref name="pmid25908467">{{cite journal |vauthors=Ardhanari S, Kannuswamy R, Chaudhary K, Lockette W, Whaley-Connell A |title=Mineralocorticoid and apparent mineralocorticoid syndromes of secondary hypertension |journal=Adv Chronic Kidney Dis |volume=22 |issue=3 |pages=185–95 |year=2015 |pmid=25908467 |doi=10.1053/j.ackd.2015.03.002 |url=}}</ref><ref name="pmid19174076">{{cite journal |vauthors=Iglesias P, Tajada P, Martínez I, Díez JJ |title=[Salt-wasting congenital adrenal hyperplasia associated to hyperreninemic hyperaldosteronism] |language=Spanish; Castilian |journal=Med Clin (Barc) |volume=132 |issue=2 |pages=80–1 |year=2009 |pmid=19174076 |doi=10.1016/j.medcli.2008.09.002 |url=}}</ref><ref name="pmid3413779">{{cite journal |vauthors=Kikuta Y, Sanjo K, Nakajima K, Ashizawa I, Ojima M |title=Primary aldosteronism in childhood due to primary adrenal hyperplasia |journal=Tohoku J. Exp. Med. |volume=155 |issue=1 |pages=57–70 |year=1988 |pmid=3413779 |doi= |url=}}</ref><ref name="pmid21494136">{{cite journal |vauthors=Hassan-Smith Z, Stewart PM |title=Inherited forms of mineralocorticoid hypertension |journal=Curr Opin Endocrinol Diabetes Obes |volume=18 |issue=3 |pages=177–85 |year=2011 |pmid=21494136 |doi=10.1097/MED.0b013e3283469444 |url=}}</ref><ref name="pmid4299011">{{cite journal |vauthors=Bartter FC, Henkin RI, Bryan GT |title=Aldosterone hypersecretion in "non-salt-losing" congenital adrenal hyperplasia |journal=J. Clin. Invest. |volume=47 |issue=8 |pages=1742–52 |year=1968 |pmid=4299011 |pmc=297334 |doi=10.1172/JCI105864 |url=}}</ref> | ||
*[[Renal artery stenosis]] | *[[Renal artery stenosis]] | ||
*[[Cushing's syndrome]] | *[[Cushing's syndrome]] | ||
*[[Congenital adrenal hyperplasia]] (CAH) | *[[Congenital adrenal hyperplasia]] ([[Congenital adrenal hyperplasia|CAH]]) | ||
**[[17 alpha-hydroxylase deficiency|17 alpha hydroxylase deficiency]] | **[[17 alpha-hydroxylase deficiency|17 alpha hydroxylase deficiency]] | ||
**[[11β-hydroxylase deficiency|11 beta hydroxylase deficiency]] | **[[11β-hydroxylase deficiency|11 beta hydroxylase deficiency]] | ||
Line 399: | Line 333: | ||
== History and symptoms == | == History and symptoms == | ||
===History === | |||
Hyperaldosteronism may be suspected in the following scenarios: | |||
* Patients with a history of spontaneous or unprovoked [[hypokalemia]] along with [[hypertension]]. | |||
* Patients who develop severe and/or persistent [[hypokalemia]] while on low to moderate doses of [[Diuretics|potassium-wasting diuretics]]. | |||
* Patients with a history of treatment-refractory/-resistant [[hypertension]] ([[HTN]]). | |||
Patients with profound [[hypokalemia]] report [[fatigue]], [[muscle weakness]], [[cramping]], [[headaches]], and [[palpitations]]. They can also have [[polydipsia]] and [[polyuria]] from [[hypokalemia]]-induced [[nephrogenic diabetes insipidus]]. Long-standing [[Hypertension|HTN]] may lead to [[cardiac]], [[retinal]], renal, and [[Neurological disease|neurologic]] problems, with all the associated [[Symptoms and Signs|symptoms and signs]]. Patients with primary hyperaldosteronism may have subclinical [[systolic dysfunction]], more [[bradycardia]], [[Blood pressure|higher blood pressure]] and [[vascular resistance]] values than those with the secondary hyperaldosteronism. [[Blood plasma|Plasma]] [[renin]] activity has been found to be lower in primary than in secondary hyperaldosteronism. | |||
===Common Symptoms=== | |||
Common symptoms of Hyperaldosteronism include:<ref name="pmid4714286">{{cite journal |vauthors=Rubidge CJ, O'Dowd PB, Powell SJ |title=Difetarsone in the treatment of Trichuris trichiura infections |journal=S. Afr. Med. J. |volume=47 |issue=23 |pages=991–2 |year=1973 |pmid=4714286 |doi= |url=}}</ref><ref name="pmid16932426">{{cite journal |vauthors=Mattsson C, Young WF |title=Primary aldosteronism: diagnostic and treatment strategies |journal=Nat Clin Pract Nephrol |volume=2 |issue=4 |pages=198–208; quiz, 1 p following 230 |year=2006 |pmid=16932426 |doi=10.1038/ncpneph0151 |url=}}</ref><ref name="pmid3216243">{{cite journal |vauthors=Di Tullio M, Alli C, Avanzini F, Bettelli G, Colombo F, Devoto MA, Marchioli R, Mariotti G, Radice M, Taioli E |title=Prevalence of symptoms generally attributed to hypertension or its treatment: study on blood pressure in elderly outpatients (SPAA) |journal=J Hypertens Suppl |volume=6 |issue=1 |pages=S87–90 |year=1988 |pmid=3216243 |doi= |url=}}</ref><ref name="pmid21278718">{{cite journal |vauthors=Unwin RJ, Luft FC, Shirley DG |title=Pathophysiology and management of hypokalemia: a clinical perspective |journal=Nat Rev Nephrol |volume=7 |issue=2 |pages=75–84 |year=2011 |pmid=21278718 |doi=10.1038/nrneph.2010.175 |url=}}</ref><ref name="pmid546663">{{cite journal |vauthors=Bautista J, Gil-Neciga E, Gil-Peralta A |title=Hypokalemic periodic paralysis in primary hyperaldosteronism. Subclinical myopathy with atrophy of the type 2A muscle fibers |journal=Eur. Neurol. |volume=18 |issue=6 |pages=415–20 |year=1979 |pmid=546663 |doi= |url=}}</ref><ref name="pmid12908077">{{cite journal |vauthors=Bortolotto LA, Cesena FH, Jatene FB, Silva HB |title=Malignant hypertension and hypertensive encephalopathy in primary aldosteronism caused by adrenal adenoma |journal=Arq. Bras. Cardiol. |volume=81 |issue=1 |pages=97–100, 93–6 |year=2003 |pmid=12908077 |doi= |url=}}</ref> | |||
==== Hypertension related symptoms ==== | |||
* [[Headache|Headaches]] | |||
* [[Facial flushing]] | |||
* [[Weakness]] | |||
* [[Visual impairment]] | |||
* Impaired consciousness | |||
* [[Seizure|Seizures]] ([[hypertensive encephalopathy]]) | |||
==== Hypokalemia related symptoms ==== | |||
* [[Constipation]] | |||
* [[Polyuria]] and [[polydipsia]] (because of impaired renal concentrating ability) | |||
* [[Weakness]] | |||
=== Less Common Symptoms === | |||
Less common symptoms of hyperaldosteronism include:<ref name="pmid5596496">{{cite journal |vauthors=Moeller J, Muniz B |title=[Hypokalemic ileus and aldosteronism] |language=German |journal=Med Klin |volume=62 |issue=52 |pages=2019–24 |year=1967 |pmid=5596496 |doi= |url=}}</ref><ref name="pmid15024897">{{cite journal |vauthors=Failor RA, Capell PT |title=Hyperaldosteronism and pheochromocytoma: new tricks and tests |journal=Prim. Care |volume=30 |issue=4 |pages=801–20, viii |year=2003 |pmid=15024897 |doi= |url=}}</ref> | |||
* [[Paralysis]] | |||
* [[Palpitations]] | |||
* [[Ileus]] | |||
==References== | |||
{{reflist|2}} | |||
{{Endocrine pathology}} | {{Endocrine pathology}} | ||
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Resident Survival Guide |
Hyperaldosteronism Main page |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2] Syed Hassan A. Kazmi BSc, MD [3]
This page contains general information about hyperaldosteronism. For more information on specific types, please visit the pages on primary hyperaldosteronism.
Synonyms and keywords: Aldosteronism
Overview
Hyperaldosteronism is a clinical scenario of mineralocorticoid excess with resistant hypertension, hypokalemia, and metabolic alkalosis due to increased hydrogen ion excretion. Aldosteronism may be classified into three types, primary hyperaldosteronism (Conn's syndrome), secondary hyperaldosteronism, and pseudohyperaldosteronism. Primary hyperaldosteronism can caused by aldosterone-secreting adenoma, bilateral hyperplasia of the adrenal glands, and ectopic secretion of aldosterone from ovaries and kidneys. Primary hyperaldosteronism features overproduction of aldosterone despite suppressed plasma renin activity (PRA). The resulting Na+ retention produces hypertension, and increased K+ excretion may cause hypokalemia. Secondary hyperaldosteronism is caused by high renin and subsequently aldosterone level, such as renovascular causes and reninoma. Pseudohyperaldosteronism is the clinical presentation of hyperaldosteronism such as resistant hypertension, hypokalemia, and metabolic alkalosis due to factors other than renin and aldosterone. The treatment should be prescribed for the blockade of aldosterone effects, or based on underlying disease.
Classification
Aldosteronism and mineralocorticoid excess may be classified into three types, primary hyperaldosteronism (conn's syndrome), secondary hyperaldosteronism, and pseudohyperaldosteronism. The different types of mineralocorticoid excess are described below.
Primary hyperaldosteronism (conn's syndrome)
Primary hyperaldosteronism causes categorizes as below table: [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]
Primary hyperaldosteronism | Category | Diseases |
---|---|---|
Adrenal causes | Aldosterone-secreting adrenal adenoma | |
Idiopathic hyperaldosteronism
| ||
Extra-adrenal causes | Ectopic secretion of aldosterone | |
Familial hyperaldosteronism | Familial hyperaldosteronism type I
| |
Familial hyperaldosteronism II
| ||
Familial hyperaldosteronism type III
| ||
Other | Pure aldosterone-producing adrenocortical carcinomas | |
Unilateral adrenal hyperplasia |
Secondary hyperaldosteronism
Secondary hyperaldosteronism causes are categorized as below, and each specific disease is described in the related micro-chapter. [16][17][18][19][20][21]
Category | Diseases | |
---|---|---|
Secondary hyperaldosteronism | Genetic mutation | Bartter and Gitelman syndromes (hyperplasia of the juxtaglomerular apparatus, the source of renin in the kidney) |
Endocrine causes | Cushing syndrome
of cortisol which saturates 11-HSD2 activity,
| |
Ectopic ACTH production (Secondary to carcinomas such as lung cancer) | ||
Renovascular | Kidney transplant | |
Renin-secreting juxtaglomerular cell tumors | ||
Scleroderma renal crisis | ||
Malignant hypertension | ||
Tumors | Reninoma | |
Intravascular hypovolemia |
|
Pseudohyperaldosteronism causes (low renin)
Pseudohyperaldosteronism causes are classified as below: [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]
Pseudohyperaldosteronism causes | Disease | Etiology | Clinical features | Labratory | Treatment | |||
---|---|---|---|---|---|---|---|---|
Elevated mineralocorticoid | Renin | Aldosterone | Other | |||||
Endogenous causes | 17 alpha-hydroxylase deficiency | Mutations in the CYP17A1 gene |
|
Deoxycorticosterone (DOC) | ↓ | ↓ | Cortisol ↓ | Corticosteroids |
11β-hydroxylase deficiency | Mutations in the CYP11B1 gene |
|
Cortisol ↓ | |||||
Apparent mineralocorticoid excess syndrome (AME) | Genetic or acquired defect of 11-HSD gene
|
|
Cortisol has mineralocorticoid effects | ↓ | ↓ | Urinary free cortisone ↓↓ | Dexamethasone and/or mineralocorticoid blockers | |
Liddle’s syndrome (Pseudohyperaldosteronism type 1) | Mutation of the epithelial sodium channels (ENaC) gene in the distal renal tubules | No extra mineralocorticoid presents, and mutations in Na channels mimic aldosterone mechanism | ↓ | ↓ | Cortisol ↓ | Amiloride or triamterene | ||
Cushing’s syndrome |
of cortisol which saturates 11-HSD2 activity,
|
Rapid weight gain, particularly of the trunk and face with limbs sparing (central obesity)
|
Cortisol has mineralocorticoid effects | ↓ |
|
Urinary free cortisol markedly ↑↑ |
| |
Insensitivity to glucocorticoids (Chrousos syndrome) | Mutations in glucocorticoid receptor (GR) gene |
|
Deoxycorticosterone (DOC) | ↓ | ↓ | Cortisol | Dexamethasone | |
Cortisol-secreting adrenocortical carcinoma | Multifactorial |
Rapid weight gain, particularly of the trunk and face with limbs sparing (central obesity)
|
Cortisol has mineralocorticoid effects | ↓ |
|
Urinary free cortisol markedly ↑↑ | Surgery | |
Geller’s syndrome | Mutation of mineralocorticoid (MR) receptor that alters its specificity and allows progesterone to bind MR | Severe hypertension particularly during pregnancy | Progesterone has mineralocorticoid effects | ↓ | ↓ | - | mineralocorticoid blockers | |
Gordon’s syndrome (Pseudohypoaldosteronism type 2) | Mutations of at least four genes have been identified, including WNK1 and WNK4 |
|
No excess mineralocorticoid; an increased activity of the thiazide-sensitive Na–Cl co-transporter in the distal tubule | ↓ | Normal | Hyperkalemia | thiazide diuretics and/or dietary sodium restriction | |
Exogenous causes | Corticosteroids with mineralocorticoid activity | Fludrocortisone or fluoroprednisolone can mimic the action of aldosterone, | Medications such as fludrocortisone | ↓ | ↓ | - | Change the treatment | |
Licorice ingestion | Glycyrrhetinic acid that binds mineralocorticoid receptor and blocks 11-HSD2 at the level of classical target tissues of aldosterone | - | ↓ | ↓ | Urinary free cortisol Moderate ↑ | Discontinue licorice | ||
Grapefruit | High assumption of naringenin, a component of grapefruit, can also block 11-HSD | - | ↓ | ↓ | - | Discontinue grapefruit | ||
Estrogens | Estrogens can retain sodium and water by different mechanisms, causing:
|
- | ↓ | ↓ | - | Discontinue estrogens |
Other less common causes of pseudohyperaldosteronism are:
- Sclerosis of juxtaglomerular apparatus (diabetic microangiopathy and/or of the elderly)
- Low-renin essential hypertension
- Partial/total nephrectomy or removal of renal tissue
Differentiating Diagnosis
Hyperaldosteronism should be differentiated from other diseases causing hypertension and hypokalemia for example:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]
- Renal artery stenosis
- Cushing's syndrome
- Congenital adrenal hyperplasia (CAH)
- Liddle's syndrome
- Diuretic use
- Licorice ingestion
- Renin-secreting tumors
Hypertension and Hypokalemia | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Plasma renin activity | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Normal or High (Plasma Renin/Aldosterone ratio <10 | Suppressed (Plasma Renin/Aldosterone ratio >20 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
*Renin-secreting tumors *Diuretic use *Renovascular hypertension *Coarctation of aorta *Malignant phase hypertension | Urinary aldosterone | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Elevated | Normal | Low | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Conn's syndrome (Primary aldosteronism) | Profound K+ depletion | • 17 alpha hydroxylase deficiency • 11 beta hydroxylase deficiency • Liddle's syndrome • Licorice ingestion • Deoxycortisone producing tumor | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Add Mineralocrticoid antagonist for 8 weeks | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
BP response | No BP response | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency) • Licorice ingestion •Glucocorticoid resistance | Liddle's syndrome) | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
History and symptoms
History
Hyperaldosteronism may be suspected in the following scenarios:
- Patients with a history of spontaneous or unprovoked hypokalemia along with hypertension.
- Patients who develop severe and/or persistent hypokalemia while on low to moderate doses of potassium-wasting diuretics.
- Patients with a history of treatment-refractory/-resistant hypertension (HTN).
Patients with profound hypokalemia report fatigue, muscle weakness, cramping, headaches, and palpitations. They can also have polydipsia and polyuria from hypokalemia-induced nephrogenic diabetes insipidus. Long-standing HTN may lead to cardiac, retinal, renal, and neurologic problems, with all the associated symptoms and signs. Patients with primary hyperaldosteronism may have subclinical systolic dysfunction, more bradycardia, higher blood pressure and vascular resistance values than those with the secondary hyperaldosteronism. Plasma renin activity has been found to be lower in primary than in secondary hyperaldosteronism.
Common Symptoms
Common symptoms of Hyperaldosteronism include:[22][23][24][25][26][27]
- Headaches
- Facial flushing
- Weakness
- Visual impairment
- Impaired consciousness
- Seizures (hypertensive encephalopathy)
- Constipation
- Polyuria and polydipsia (because of impaired renal concentrating ability)
- Weakness
Less Common Symptoms
Less common symptoms of hyperaldosteronism include:[28][29]
References
- ↑ 1.0 1.1 1.2 Wada N, Jin S, Hui SP, Yanagisawa K, Kurosawa T, Chiba H (2014). "[Differential diagnosis of primary aldosteronism by measurement of hybrid steroids using mass spectrometry]". Rinsho Byori (in Japanese). 62 (3): 276–82. PMID 24800505.
- ↑ 2.0 2.1 2.2 Nielsen ML, Pareek M, Andersen I (2012). "[Liquorice-induced hypertension and hypokalaemia]". Ugeskr. Laeg. (in Danish). 174 (15): 1024–5. PMID 22487411.
- ↑ 3.0 3.1 3.2 Chow KM, Ma RC, Szeto CC, Li PK (2012). "Polycystic kidney disease presenting with hypertension and hypokalemia". Am. J. Kidney Dis. 59 (2): 270–2. doi:10.1053/j.ajkd.2011.08.020. PMID 21962616.
- ↑ 4.0 4.1 4.2 Sarafidis PA, Georgianos PI, Germanidis G, Giavroglou C, Nikolaidis P, Lasaridis AN, Madias NE (2012). "Hypertension and symptomatic hypokalemia in a patient with simultaneous unilateral stenoses of intrarenal arteries and mesangioproliferative glomerulonephritis". Am. J. Kidney Dis. 59 (3): 434–8. doi:10.1053/j.ajkd.2011.11.001. PMID 22154539.
- ↑ 5.0 5.1 5.2 Khosla N, Hogan D (2006). "Mineralocorticoid hypertension and hypokalemia". Semin. Nephrol. 26 (6): 434–40. doi:10.1016/j.semnephrol.2006.10.004. PMID 17275580.
- ↑ 6.0 6.1 6.2 Weiner ID (2013). "Endocrine and hypertensive disorders of potassium regulation: primary aldosteronism". Semin. Nephrol. 33 (3): 265–76. doi:10.1016/j.semnephrol.2013.04.007. PMC 3748390. PMID 23953804.
- ↑ 7.0 7.1 7.2 Martell-Claros N, Abad-Cardiel M, Alvarez-Alvarez B, García-Donaire JA, Pérez CF (2015). "Primary aldosteronism and its various clinical scenarios". J. Hypertens. 33 (6): 1226–32. doi:10.1097/HJH.0000000000000546. PMID 25715092.
- ↑ 8.0 8.1 8.2 Franse LV, Pahor M, Di Bari M, Somes GW, Cushman WC, Applegate WB (2000). "Hypokalemia associated with diuretic use and cardiovascular events in the Systolic Hypertension in the Elderly Program". Hypertension. 35 (5): 1025–30. PMID 10818057.
- ↑ 9.0 9.1 9.2 Rossi E, Farnetti E, Nicoli D, Sazzini M, Perazzoli F, Regolisti G, Grasselli C, Santi R, Negro A, Mazzeo V, Mantero F, Luiselli D, Casali B (2011). "A clinical phenotype mimicking essential hypertension in a newly discovered family with Liddle's syndrome". Am. J. Hypertens. 24 (8): 930–5. doi:10.1038/ajh.2011.76. PMID 21525970.
- ↑ 10.0 10.1 10.2 Ruecker B, Lang-Muritano M, Spanaus K, Welzel M, l'Allemand D, Phan-Hug F, Katschnig C, Konrad D, Holterhus PM, Schoenle EJ (2015). "The Aldosterone/Renin Ratio as a Diagnostic Tool for the Diagnosis of Primary Hypoaldosteronism in Newborns and Infants". Horm Res Paediatr. 84 (1): 43–8. doi:10.1159/000381852. PMID 25968592.
- ↑ 11.0 11.1 11.2 Ardhanari S, Kannuswamy R, Chaudhary K, Lockette W, Whaley-Connell A (2015). "Mineralocorticoid and apparent mineralocorticoid syndromes of secondary hypertension". Adv Chronic Kidney Dis. 22 (3): 185–95. doi:10.1053/j.ackd.2015.03.002. PMID 25908467.
- ↑ 12.0 12.1 12.2 Iglesias P, Tajada P, Martínez I, Díez JJ (2009). "[Salt-wasting congenital adrenal hyperplasia associated to hyperreninemic hyperaldosteronism]". Med Clin (Barc) (in Spanish; Castilian). 132 (2): 80–1. doi:10.1016/j.medcli.2008.09.002. PMID 19174076.
- ↑ 13.0 13.1 13.2 Kikuta Y, Sanjo K, Nakajima K, Ashizawa I, Ojima M (1988). "Primary aldosteronism in childhood due to primary adrenal hyperplasia". Tohoku J. Exp. Med. 155 (1): 57–70. PMID 3413779.
- ↑ 14.0 14.1 14.2 Hassan-Smith Z, Stewart PM (2011). "Inherited forms of mineralocorticoid hypertension". Curr Opin Endocrinol Diabetes Obes. 18 (3): 177–85. doi:10.1097/MED.0b013e3283469444. PMID 21494136.
- ↑ 15.0 15.1 15.2 Bartter FC, Henkin RI, Bryan GT (1968). "Aldosterone hypersecretion in "non-salt-losing" congenital adrenal hyperplasia". J. Clin. Invest. 47 (8): 1742–52. doi:10.1172/JCI105864. PMC 297334. PMID 4299011.
- ↑ Stewart PM (2003). "Tissue-specific Cushing's syndrome, 11beta-hydroxysteroid dehydrogenases and the redefinition of corticosteroid hormone action". Eur. J. Endocrinol. 149 (3): 163–8. PMID 12943516.
- ↑ Quinkler M, Stewart PM (2003). "Hypertension and the cortisol-cortisone shuttle". J. Clin. Endocrinol. Metab. 88 (6): 2384–92. doi:10.1210/jc.2003-030138. PMID 12788832.
- ↑ Ulick S, Wang JZ, Blumenfeld JD, Pickering TG (1992). "Cortisol inactivation overload: a mechanism of mineralocorticoid hypertension in the ectopic adrenocorticotropin syndrome". J. Clin. Endocrinol. Metab. 74 (5): 963–7. doi:10.1210/jcem.74.5.1569172. PMID 1569172.
- ↑ Whorwood CB, Sheppard MC, Stewart PM (1993). "Licorice inhibits 11 beta-hydroxysteroid dehydrogenase messenger ribonucleic acid levels and potentiates glucocorticoid hormone action". Endocrinology. 132 (6): 2287–92. doi:10.1210/endo.132.6.8504732. PMID 8504732.
- ↑ CHRISTY NP, LARAGH JH (1961). "Pathogenesis of hypokalemic alkalosis in Cushing's syndrome". N. Engl. J. Med. 265: 1083–8. doi:10.1056/NEJM196111302652203. PMID 13879332.
- ↑ Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, Stowasser M, Young WF (2016). "The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline". J. Clin. Endocrinol. Metab. 101 (5): 1889–916. doi:10.1210/jc.2015-4061. PMID 26934393.
- ↑ Rubidge CJ, O'Dowd PB, Powell SJ (1973). "Difetarsone in the treatment of Trichuris trichiura infections". S. Afr. Med. J. 47 (23): 991–2. PMID 4714286.
- ↑ Mattsson C, Young WF (2006). "Primary aldosteronism: diagnostic and treatment strategies". Nat Clin Pract Nephrol. 2 (4): 198–208, quiz, 1 p following 230. doi:10.1038/ncpneph0151. PMID 16932426.
- ↑ Di Tullio M, Alli C, Avanzini F, Bettelli G, Colombo F, Devoto MA, Marchioli R, Mariotti G, Radice M, Taioli E (1988). "Prevalence of symptoms generally attributed to hypertension or its treatment: study on blood pressure in elderly outpatients (SPAA)". J Hypertens Suppl. 6 (1): S87–90. PMID 3216243.
- ↑ Unwin RJ, Luft FC, Shirley DG (2011). "Pathophysiology and management of hypokalemia: a clinical perspective". Nat Rev Nephrol. 7 (2): 75–84. doi:10.1038/nrneph.2010.175. PMID 21278718.
- ↑ Bautista J, Gil-Neciga E, Gil-Peralta A (1979). "Hypokalemic periodic paralysis in primary hyperaldosteronism. Subclinical myopathy with atrophy of the type 2A muscle fibers". Eur. Neurol. 18 (6): 415–20. PMID 546663.
- ↑ Bortolotto LA, Cesena FH, Jatene FB, Silva HB (2003). "Malignant hypertension and hypertensive encephalopathy in primary aldosteronism caused by adrenal adenoma". Arq. Bras. Cardiol. 81 (1): 97–100, 93–6. PMID 12908077.
- ↑ Moeller J, Muniz B (1967). "[Hypokalemic ileus and aldosteronism]". Med Klin (in German). 62 (52): 2019–24. PMID 5596496.
- ↑ Failor RA, Capell PT (2003). "Hyperaldosteronism and pheochromocytoma: new tricks and tests". Prim. Care. 30 (4): 801–20, viii. PMID 15024897.