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Primary aldosteronism

MedGen UID:
278002
Concept ID:
C1384514
Disease or Syndrome
Synonyms: Conn's syndrome; Primary hyperaldosteronism
SNOMED CT: Idiopathic hyperaldosteronism (258117004); Primary hyperaldosteronism (190507007); Conn's syndrome (190507007); Conn syndrome (190507007); Primary aldosteronism (190507007); Idiopathic aldosteronism (258117004)
 
HPO: HP:0011736
Monarch Initiative: MONDO:0001422

Definition

A form of hyperaldosteronism caused by a defect within the adrenal gland. [from HPO]

Conditions with this feature

Aldosterone-producing adenoma with seizures and neurological abnormalities
MedGen UID:
815939
Concept ID:
C3809609
Disease or Syndrome
A rare, genetic, neurologic disease characterized by primary hyperaldosteronism presenting with early-onset, severe hypertension, hypokalemia and neurological manifestations (including seizures, severe hypotonia, spasticity, cerebral palsy and profound developmental delay/intellectual disability).

Professional guidelines

PubMed

Reincke M, Bancos I, Mulatero P, Scholl UI, Stowasser M, Williams TA
Lancet Diabetes Endocrinol 2021 Dec;9(12):876-892. doi: 10.1016/S2213-8587(21)00210-2. PMID: 34798068
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article
Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, Stowasser M, Young WF Jr
J Clin Endocrinol Metab 2016 May;101(5):1889-916. Epub 2016 Mar 2 doi: 10.1210/jc.2015-4061. PMID: 26934393

Recent clinical studies

Etiology

Turcu AF, Yang J, Vaidya A
Nat Rev Endocrinol 2022 Nov;18(11):665-682. Epub 2022 Aug 31 doi: 10.1038/s41574-022-00730-2. PMID: 36045149
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article
Vaidya A, Carey RM
J Clin Endocrinol Metab 2020 Dec 1;105(12):3771-83. doi: 10.1210/clinem/dgaa606. PMID: 32865201Free PMC Article
Tziomalos K
Curr Hypertens Rev 2020;16(1):11. doi: 10.2174/1573402115666190416161116. PMID: 31038070
Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, Stowasser M, Young WF Jr
J Clin Endocrinol Metab 2016 May;101(5):1889-916. Epub 2016 Mar 2 doi: 10.1210/jc.2015-4061. PMID: 26934393

Diagnosis

Turcu AF, Yang J, Vaidya A
Nat Rev Endocrinol 2022 Nov;18(11):665-682. Epub 2022 Aug 31 doi: 10.1038/s41574-022-00730-2. PMID: 36045149
Reincke M, Bancos I, Mulatero P, Scholl UI, Stowasser M, Williams TA
Lancet Diabetes Endocrinol 2021 Dec;9(12):876-892. doi: 10.1016/S2213-8587(21)00210-2. PMID: 34798068
Zennaro MC, Boulkroun S, Fernandes-Rosa FL
Nat Rev Endocrinol 2020 Oct;16(10):578-589. Epub 2020 Jul 28 doi: 10.1038/s41574-020-0382-4. PMID: 32724183
Tziomalos K
Curr Hypertens Rev 2020;16(1):11. doi: 10.2174/1573402115666190416161116. PMID: 31038070
Hundemer GL, Vaidya A
Endocrinol Metab Clin North Am 2019 Dec;48(4):681-700. doi: 10.1016/j.ecl.2019.08.002. PMID: 31655770Free PMC Article

Therapy

Forestiero V, Sconfienza E, Mulatero P, Monticone S
Rev Endocr Metab Disord 2023 Feb;24(1):39-48. Epub 2022 May 10 doi: 10.1007/s11154-022-09729-6. PMID: 35536535Free PMC Article
Buffolo F, Tetti M, Mulatero P, Monticone S
Hypertension 2022 Sep;79(9):1899-1911. Epub 2022 Jun 29 doi: 10.1161/HYPERTENSIONAHA.122.17964. PMID: 35766038
Sarathy H, Salman LA, Lee C, Cohen JB
Med Clin North Am 2022 Mar;106(2):269-283. Epub 2022 Feb 2 doi: 10.1016/j.mcna.2021.11.004. PMID: 35227430Free PMC Article
Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, Stowasser M, Young WF Jr
J Clin Endocrinol Metab 2016 May;101(5):1889-916. Epub 2016 Mar 2 doi: 10.1210/jc.2015-4061. PMID: 26934393
Te Riet L, van Esch JH, Roks AJ, van den Meiracker AH, Danser AH
Circ Res 2015 Mar 13;116(6):960-75. doi: 10.1161/CIRCRESAHA.116.303587. PMID: 25767283

Prognosis

Forestiero V, Sconfienza E, Mulatero P, Monticone S
Rev Endocr Metab Disord 2023 Feb;24(1):39-48. Epub 2022 May 10 doi: 10.1007/s11154-022-09729-6. PMID: 35536535Free PMC Article
Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article
Vaidya A, Carey RM
J Clin Endocrinol Metab 2020 Dec 1;105(12):3771-83. doi: 10.1210/clinem/dgaa606. PMID: 32865201Free PMC Article
Tziomalos K
Curr Hypertens Rev 2020;16(1):11. doi: 10.2174/1573402115666190416161116. PMID: 31038070

Clinical prediction guides

Mete O, Erickson LA, Juhlin CC, de Krijger RR, Sasano H, Volante M, Papotti MG
Endocr Pathol 2022 Mar;33(1):155-196. Epub 2022 Mar 14 doi: 10.1007/s12022-022-09710-8. PMID: 35288842Free PMC Article
Rossi GP, Bisogni V, Rossitto G, Maiolino G, Cesari M, Zhu R, Seccia TM
High Blood Press Cardiovasc Prev 2020 Dec;27(6):547-560. Epub 2020 Nov 7 doi: 10.1007/s40292-020-00415-9. PMID: 33159664Free PMC Article
Petramala L, Concistrè A, Olmati F, Saracino V, Chimenti C, Frustaci A, Russo MA, Letizia C
Int J Mol Sci 2020 Jul 17;21(14) doi: 10.3390/ijms21145047. PMID: 32709015Free PMC Article
Brown JM, Siddiqui M, Calhoun DA, Carey RM, Hopkins PN, Williams GH, Vaidya A
Ann Intern Med 2020 Jul 7;173(1):10-20. Epub 2020 May 26 doi: 10.7326/M20-0065. PMID: 32449886Free PMC Article
Monticone S, D'Ascenzo F, Moretti C, Williams TA, Veglio F, Gaita F, Mulatero P
Lancet Diabetes Endocrinol 2018 Jan;6(1):41-50. Epub 2017 Nov 9 doi: 10.1016/S2213-8587(17)30319-4. PMID: 29129575

Recent systematic reviews

Wang A, Wang Y, Liu H, Hu X, Li J, Xu H, Nie Z, Zhang L, Lyu Z
Front Endocrinol (Lausanne) 2022;13:1027841. Epub 2022 Oct 31 doi: 10.3389/fendo.2022.1027841. PMID: 36387892Free PMC Article
Sanga V, Rossitto G, Seccia TM, Rossi GP
Hypertension 2022 Sep;79(9):1912-1921. Epub 2022 Jun 10 doi: 10.1161/HYPERTENSIONAHA.121.18858. PMID: 35686552
Leung AA, Symonds CJ, Hundemer GL, Ronksley PE, Lorenzetti DL, Pasieka JL, Harvey A, Kline GA
Hypertension 2022 Aug;79(8):1835-1844. Epub 2022 Jun 2 doi: 10.1161/HYPERTENSIONAHA.122.19377. PMID: 35652330Free PMC Article
Shi S, Lu C, Tian H, Ren Y, Chen T
Front Endocrinol (Lausanne) 2020;11:574151. Epub 2020 Sep 25 doi: 10.3389/fendo.2020.574151. PMID: 33101208Free PMC Article
Monticone S, D'Ascenzo F, Moretti C, Williams TA, Veglio F, Gaita F, Mulatero P
Lancet Diabetes Endocrinol 2018 Jan;6(1):41-50. Epub 2017 Nov 9 doi: 10.1016/S2213-8587(17)30319-4. PMID: 29129575

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