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Glucocortocoid-insensitive primary hyperaldosteronism

MedGen UID:
892826
•Concept ID:
C4023208
•
Disease or Syndrome
Synonym: Familial primary hyperaldosteronism type 2
 
HPO: HP:0011740

Definition

A form of primary hyperaldosteronism in which the overproduction of aldosterone cannot be suppressed by the administration of dexamethasone or similar glucocorticoids. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGlucocortocoid-insensitive primary hyperaldosteronism

Professional guidelines

PubMed

Diagnosis and treatment of primary aldosteronism.
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
Pathogenesis and treatment of primary aldosteronism.
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
The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline.
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

Primary aldosteronism - a multidimensional syndrome.
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
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
Evaluation and Management of Primary Hyperaldosteronism.
Lee FT, Elaraj D
Surg Clin North Am 2019 Aug;99(4):731-745. doi: 10.1016/j.suc.2019.04.010. PMID: 31255203
The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline.
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

Primary aldosteronism - a multidimensional syndrome.
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
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
Diagnosis and treatment of primary aldosteronism.
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
Pathogenesis and treatment of primary aldosteronism.
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
Evaluation and Management of Primary Hyperaldosteronism.
Lee FT, Elaraj D
Surg Clin North Am 2019 Aug;99(4):731-745. doi: 10.1016/j.suc.2019.04.010. PMID: 31255203

Therapy

Primary aldosteronism in pregnancy.
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
Aldosterone as a Mediator of Cardiovascular Damage.
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
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: Practical Approach to Diagnosis and Management.
Byrd JB, Turcu AF, Auchus RJ
Circulation 2018 Aug 21;138(8):823-835. doi: 10.1161/CIRCULATIONAHA.118.033597. PMID: 30359120Free PMC Article
The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline.
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

Prognosis

Primary Aldosteronism: A Pragmatic Approach to Diagnosis and Management.
Dogra P, Bancos I, Young WF Jr
Mayo Clin Proc 2023 Aug;98(8):1207-1215. doi: 10.1016/j.mayocp.2023.04.023. PMID: 37536806
Primary aldosteronism in pregnancy.
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
Evolution of the Primary Aldosteronism Syndrome: Updating the Approach.
Vaidya A, Carey RM
J Clin Endocrinol Metab 2020 Dec 1;105(12):3771-83. doi: 10.1210/clinem/dgaa606. PMID: 32865201Free PMC Article
Primary Aldosteronism Diagnosis and Management: A Clinical Approach.
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
Cardiometabolic outcomes and mortality in medically treated primary aldosteronism: a retrospective cohort study.
Hundemer GL, Curhan GC, Yozamp N, Wang M, Vaidya A
Lancet Diabetes Endocrinol 2018 Jan;6(1):51-59. Epub 2017 Nov 9 doi: 10.1016/S2213-8587(17)30367-4. PMID: 29129576Free PMC Article

Clinical prediction guides

Primary hyperaldosteronism is associated with increased mortality and morbidity in patients with hypertension and diabetes.
Pillai K, Fares A, Dargham S, Al Suwaidi J, Jayyousi A, Abi Khalil C
Front Endocrinol (Lausanne) 2023;14:1147225. Epub 2023 May 26 doi: 10.3389/fendo.2023.1147225. PMID: 37305032Free 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
Hyperaldosteronism: Screening and Diagnostic Tests.
Sabbadin C, Fallo F
High Blood Press Cardiovasc Prev 2016 Jun;23(2):69-72. Epub 2016 Mar 14 doi: 10.1007/s40292-016-0136-5. PMID: 26971505
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

Recent systematic reviews

Bone and mineral metabolism in patients with primary aldosteronism: A systematic review and meta-analysis.
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
Management and Outcomes of Primary Aldosteronism in Pregnancy: A Systematic Review.
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
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
Primary Aldosteronism and Bone Metabolism: A Systematic Review and Meta-Analysis.
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
Cardiovascular events and target organ damage in primary aldosteronism compared with essential hypertension: a systematic review and meta-analysis.
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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