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Primary hyperaldosteronism

MedGen UID:
278002
•Concept ID:
C1384514
•
Disease or Syndrome
Synonyms: Conn's syndrome; Primary aldosteronism
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).
See: Condition Record
Aldosterone-producing adenoma with seizures and neurological abnormalities

Professional guidelines

PubMed

Diagnosis and management of primary hyperaldosteronism in patients with hypertension: a practical approach endorsed by the British and Irish Hypertension Society.
Faconti L, Kulkarni S, Delles C, Kapil V, Lewis P, Glover M, MacDonald TM, Wilkinson IB
J Hum Hypertens 2024 Jan;38(1):8-18. Epub 2023 Nov 14 doi: 10.1038/s41371-023-00875-1. PMID: 37964158Free PMC Article
Primary Hyperaldosteronism: Approach to Diagnosis and Management.
El-Asmar N, Rajpal A, Arafah BM
Med Clin North Am 2021 Nov;105(6):1065-1080. Epub 2021 Sep 9 doi: 10.1016/j.mcna.2021.06.007. PMID: 34688415
Diagnosis of hypokalemia: a problem-solving approach to clinical cases.
Assadi F
Iran J Kidney Dis 2008 Jul;2(3):115-22. PMID: 19377223

Recent clinical studies

Etiology

Evaluating phase II results of Baxdrostat, an aldosterone synthase inhibitor for hypertension.
Awosika A, Cho Y, Bose U, Omole AE, Adabanya U
Expert Opin Investig Drugs 2023 Jul-Dec;32(11):985-995. Epub 2023 Nov 24 doi: 10.1080/13543784.2023.2276755. PMID: 37883217
The Effect of Aldosterone on Cardiorenal and Metabolic Systems.
Otsuka H, Abe M, Kobayashi H
Int J Mol Sci 2023 Mar 11;24(6) doi: 10.3390/ijms24065370. PMID: 36982445Free PMC Article
Diagnosis of primary hyperaldosteronism.
Araujo-Castro M, Parra-Ramírez P
Med Clin (Barc) 2022 May 13;158(9):424-430. Epub 2021 Dec 16 doi: 10.1016/j.medcli.2021.10.012. PMID: 34924198
Primary Hyperaldosteronism: Approach to Diagnosis and Management.
El-Asmar N, Rajpal A, Arafah BM
Med Clin North Am 2021 Nov;105(6):1065-1080. Epub 2021 Sep 9 doi: 10.1016/j.mcna.2021.06.007. PMID: 34688415
Primary Aldosteronism: Cardiovascular Outcomes Pre- and Post-treatment.
Hundemer GL
Curr Cardiol Rep 2019 Jul 27;21(9):93. doi: 10.1007/s11886-019-1185-x. PMID: 31352525

Diagnosis

Diagnostic and therapeutic approach to hypernatremia.
Ilardi A
Diagnosis (Berl) 2022 Nov 1;9(4):403-410. Epub 2022 Aug 2 doi: 10.1515/dx-2022-0034. PMID: 35918296
Diagnosis of primary hyperaldosteronism.
Araujo-Castro M, Parra-Ramírez P
Med Clin (Barc) 2022 May 13;158(9):424-430. Epub 2021 Dec 16 doi: 10.1016/j.medcli.2021.10.012. PMID: 34924198
Primary Hyperaldosteronism: Approach to Diagnosis and Management.
El-Asmar N, Rajpal A, Arafah BM
Med Clin North Am 2021 Nov;105(6):1065-1080. Epub 2021 Sep 9 doi: 10.1016/j.mcna.2021.06.007. PMID: 34688415
Low-Renin Hypertension.
Athimulam S, Lazik N, Bancos I
Endocrinol Metab Clin North Am 2019 Dec;48(4):701-715. Epub 2019 Sep 25 doi: 10.1016/j.ecl.2019.08.003. PMID: 31655771
Glucocorticoid-remediable aldosteronism.
Halperin F, Dluhy RG
Endocrinol Metab Clin North Am 2011 Jun;40(2):333-41, viii. doi: 10.1016/j.ecl.2011.01.012. PMID: 21565670

Therapy

Evaluating phase II results of Baxdrostat, an aldosterone synthase inhibitor for hypertension.
Awosika A, Cho Y, Bose U, Omole AE, Adabanya U
Expert Opin Investig Drugs 2023 Jul-Dec;32(11):985-995. Epub 2023 Nov 24 doi: 10.1080/13543784.2023.2276755. PMID: 37883217
Primary Hyperaldosteronism: Approach to Diagnosis and Management.
El-Asmar N, Rajpal A, Arafah BM
Med Clin North Am 2021 Nov;105(6):1065-1080. Epub 2021 Sep 9 doi: 10.1016/j.mcna.2021.06.007. PMID: 34688415
Low-Renin Hypertension.
Athimulam S, Lazik N, Bancos I
Endocrinol Metab Clin North Am 2019 Dec;48(4):701-715. Epub 2019 Sep 25 doi: 10.1016/j.ecl.2019.08.003. PMID: 31655771
Glucocorticoid-remediable aldosteronism.
Halperin F, Dluhy RG
Endocrinol Metab Clin North Am 2011 Jun;40(2):333-41, viii. doi: 10.1016/j.ecl.2011.01.012. PMID: 21565670
Primary hyperaldosteronism.
Scoggins BA, Coghlan JP
Pharmacol Ther 1980;9(3):367-94. doi: 10.1016/0163-7258(80)90024-8. PMID: 6997893

Prognosis

Diagnostic and therapeutic approach to hypernatremia.
Ilardi A
Diagnosis (Berl) 2022 Nov 1;9(4):403-410. Epub 2022 Aug 2 doi: 10.1515/dx-2022-0034. PMID: 35918296
Hypertension resolution after adrenalectomy for primary hyperaldosteronism: Which is the best predictive model?
Thiesmeyer JW, Ullmann TM, Greenberg J, Williams NT, Limberg J, Stefanova D, Beninato T, Finnerty BM, Vignaud T, Leclerc J, Fahey TJ 3rd, Mirallie E, Brunaud L, Zarnegar R
Surgery 2021 Jan;169(1):133-137. Epub 2020 Jun 2 doi: 10.1016/j.surg.2020.04.017. PMID: 32507297
Topical Diagnosis and Determination of the Primary Hyperaldosteronism Variant.
Shidlovskyi VO, Shidlovskyi OV, Tovkai OA, Sheremet MI, Maksymyuk VV, Tarabanchuk VV, Ivanovych SM, Heryak MS, Andreychyn MS, Hanberher II, Piddubna AA
J Med Life 2019 Oct-Dec;12(4):322-328. doi: 10.25122/jml-2019-0072. PMID: 32025248Free PMC Article
A Woman with Normotensive Primary Hyperaldosteronism.
Moradi S, Shafiepour M, Amirbaigloo A
Acta Med Iran 2016 Feb;54(2):156-8. PMID: 26997605
Adrenal vein sampling.
Kahn SL, Angle JF
Tech Vasc Interv Radiol 2010 Jun;13(2):110-25. doi: 10.1053/j.tvir.2010.02.006. PMID: 20540920

Clinical prediction guides

Diagnosis of primary hyperaldosteronism.
Araujo-Castro M, Parra-Ramírez P
Med Clin (Barc) 2022 May 13;158(9):424-430. Epub 2021 Dec 16 doi: 10.1016/j.medcli.2021.10.012. PMID: 34924198
Hypertension resolution after adrenalectomy for primary hyperaldosteronism: Which is the best predictive model?
Thiesmeyer JW, Ullmann TM, Greenberg J, Williams NT, Limberg J, Stefanova D, Beninato T, Finnerty BM, Vignaud T, Leclerc J, Fahey TJ 3rd, Mirallie E, Brunaud L, Zarnegar R
Surgery 2021 Jan;169(1):133-137. Epub 2020 Jun 2 doi: 10.1016/j.surg.2020.04.017. PMID: 32507297
Aldosterone and the risk of hypertension.
Xanthakis V, Vasan RS
Curr Hypertens Rep 2013 Apr;15(2):102-7. doi: 10.1007/s11906-013-0330-y. PMID: 23378101Free PMC Article
Adrenal vein sampling.
Kahn SL, Angle JF
Tech Vasc Interv Radiol 2010 Jun;13(2):110-25. doi: 10.1053/j.tvir.2010.02.006. PMID: 20540920
21-Deoxytetrahydroaldosterone excretion in primary hyperaldosteronism.
Abdelhamid S, Lewicka S, Vecsei P, Winter J
Cardiology 1985;72 Suppl 1:102-6. doi: 10.1159/000173954. PMID: 4053120

Recent systematic reviews

Does Intraprocedural CT Improve the Success Rate of Adrenal Venous Sampling? A Systematic Review and Meta-Analysis of Data from 809 Patients.
Hafezi-Nejad N, Gullotti DM, Bailey CR, Lessne ML, Holly BP
Cardiovasc Intervent Radiol 2022 Jan;45(1):29-40. Epub 2021 Sep 13 doi: 10.1007/s00270-021-02954-7. PMID: 34518912
A systematic review of pathophysiology and management of familial hyperaldosteronism type 1 in pregnancy.
Sanga V, Seccia TM, Rossi GP
Endocrine 2021 Oct;74(1):5-10. Epub 2021 May 27 doi: 10.1007/s12020-021-02763-5. PMID: 34043182Free PMC Article

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