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Primary unilateral adrenal hyperplasia

MedGen UID:
905007
•Concept ID:
C4274967
•
Disease or Syndrome
Synonyms: primary unilateral adrenal hyperplasia; PUAH
SNOMED CT: Primary unilateral adrenal hyperplasia (715868005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016504
Orphanet: ORPHA231580

Definition

A surgically correctable form of primary hyperaldosteronism characterized by renin suppression, unilateral aldosterone hypersecretion, and moderate to severe hypertension secondary to hyperplasia of the adrenal gland. May be associated with hypokalemia, which, when present, may be symptomatic with muscular weakness, cramps, paresthesia or palpitations with or without atrial fibrillation.The etiology is not known. Unilateral adrenalectomy abolishes aldosterone hypersecretion and hypokalemia in most patients. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary unilateral adrenal hyperplasia

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
Diagnosis and management of primary aldosteronism.
Vilela LAP, Almeida MQ
Arch Endocrinol Metab 2017 May-Jun;61(3):305-312. doi: 10.1590/2359-3997000000274. PMID: 28699986Free 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

Recent clinical studies

Etiology

Strategies for subtyping primary aldosteronism.
Yang YH, Chang YL, Lee BC, Lu CC, Wang WT, Hu YH, Liu HW, Lin YH, Chang CC, Wu WC, Tseng FY, Lin YH, Wu VC, Hwu CM
J Formos Med Assoc 2024 Mar;123 Suppl 2:S114-S124. Epub 2023 May 17 doi: 10.1016/j.jfma.2023.05.004. PMID: 37202237
Primary bilateral macronodular adrenal hyperplasia: A series of 32 cases and literature review.
Araujo-Castro M, Reincke M
Endocrinol Diabetes Nutr (Engl Ed) 2023 Apr;70(4):229-239. doi: 10.1016/j.endien.2023.04.005. PMID: 37116968
Unilateral adrenalectomy in bilateral adrenal hyperplasia with primary aldosteronism.
Lu YC, Liu KL, Wu VC, Wang SM, Lin YH, Chueh SJ, Wu KD, Su YR, Huang KH; TAIPAI Study Group
J Formos Med Assoc 2023 May;122(5):393-399. Epub 2023 Feb 20 doi: 10.1016/j.jfma.2022.12.015. PMID: 36813699
Prevalence, diagnosis and outcomes of treatment for primary aldosteronism.
Yang Y, Reincke M, Williams TA
Best Pract Res Clin Endocrinol Metab 2020 Mar;34(2):101365. Epub 2019 Dec 5 doi: 10.1016/j.beem.2019.101365. PMID: 31837980
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

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
Diagnosis and management of primary bilateral macronodular adrenal hyperplasia.
Vassiliadi DA, Tsagarakis S
Endocr Relat Cancer 2019 Oct 1;26(10):R567-R581. doi: 10.1530/ERC-19-0240. PMID: 32053747
Diagnosis and management of primary aldosteronism.
Vilela LAP, Almeida MQ
Arch Endocrinol Metab 2017 May-Jun;61(3):305-312. doi: 10.1590/2359-3997000000274. PMID: 28699986Free 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
Primary hyperaldosteronism.
Noth RH, Biglieri EG
Med Clin North Am 1988 Sep;72(5):1117-31. doi: 10.1016/s0025-7125(16)30732-5. PMID: 3045453

Therapy

Primary bilateral macronodular adrenal hyperplasia: A series of 32 cases and literature review.
Araujo-Castro M, Reincke M
Endocrinol Diabetes Nutr (Engl Ed) 2023 Apr;70(4):229-239. doi: 10.1016/j.endien.2023.04.005. PMID: 37116968
Unilateral adrenalectomy in bilateral adrenal hyperplasia with primary aldosteronism.
Lu YC, Liu KL, Wu VC, Wang SM, Lin YH, Chueh SJ, Wu KD, Su YR, Huang KH; TAIPAI Study Group
J Formos Med Assoc 2023 May;122(5):393-399. Epub 2023 Feb 20 doi: 10.1016/j.jfma.2022.12.015. PMID: 36813699
Diagnosis and management of primary bilateral macronodular adrenal hyperplasia.
Vassiliadi DA, Tsagarakis S
Endocr Relat Cancer 2019 Oct 1;26(10):R567-R581. doi: 10.1530/ERC-19-0240. PMID: 32053747
Diagnosis and management of primary aldosteronism.
Vilela LAP, Almeida MQ
Arch Endocrinol Metab 2017 May-Jun;61(3):305-312. doi: 10.1590/2359-3997000000274. PMID: 28699986Free 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

Prevalence, diagnosis and outcomes of treatment for primary aldosteronism.
Yang Y, Reincke M, Williams TA
Best Pract Res Clin Endocrinol Metab 2020 Mar;34(2):101365. Epub 2019 Dec 5 doi: 10.1016/j.beem.2019.101365. PMID: 31837980
Genetics of tumors of the adrenal cortex.
Bonnet-Serrano F, Bertherat J
Endocr Relat Cancer 2018 Mar;25(3):R131-R152. Epub 2017 Dec 12 doi: 10.1530/ERC-17-0361. PMID: 29233839
Subtype Diagnosis of Primary Aldosteronism: Is Adrenal Vein Sampling Always Necessary?
Buffolo F, Monticone S, Williams TA, Rossato D, Burrello J, Tetti M, Veglio F, Mulatero P
Int J Mol Sci 2017 Apr 17;18(4) doi: 10.3390/ijms18040848. PMID: 28420172Free PMC Article
Cushing's disease.
Bertagna X, Guignat L, Groussin L, Bertherat J
Best Pract Res Clin Endocrinol Metab 2009 Oct;23(5):607-23. doi: 10.1016/j.beem.2009.06.001. PMID: 19945026
Correctable subsets of primary aldosteronism. Primary adrenal hyperplasia and renin responsive adenoma.
Irony I, Kater CE, Biglieri EG, Shackleton CH
Am J Hypertens 1990 Jul;3(7):576-82. doi: 10.1093/ajh/3.7.576. PMID: 2194512

Clinical prediction guides

Unilateral adrenalectomy in bilateral adrenal hyperplasia with primary aldosteronism.
Lu YC, Liu KL, Wu VC, Wang SM, Lin YH, Chueh SJ, Wu KD, Su YR, Huang KH; TAIPAI Study Group
J Formos Med Assoc 2023 May;122(5):393-399. Epub 2023 Feb 20 doi: 10.1016/j.jfma.2022.12.015. PMID: 36813699
Therapeutic Outcomes with Surgical and Medical Management of Primary Aldosteronism.
Wachtel H, Fraker DL
Curr Cardiol Rep 2021 Jun 3;23(7):89. doi: 10.1007/s11886-021-01516-0. PMID: 34081226Free PMC Article
Genetics of tumors of the adrenal cortex.
Bonnet-Serrano F, Bertherat J
Endocr Relat Cancer 2018 Mar;25(3):R131-R152. Epub 2017 Dec 12 doi: 10.1530/ERC-17-0361. PMID: 29233839
Subtype Diagnosis of Primary Aldosteronism: Is Adrenal Vein Sampling Always Necessary?
Buffolo F, Monticone S, Williams TA, Rossato D, Burrello J, Tetti M, Veglio F, Mulatero P
Int J Mol Sci 2017 Apr 17;18(4) doi: 10.3390/ijms18040848. PMID: 28420172Free PMC Article
Outcomes of adrenalectomy in patients with unilateral primary aldosteronism: a review.
Steichen O, Zinzindohoué F, Plouin PF, Amar L
Horm Metab Res 2012 Mar;44(3):221-7. Epub 2012 Jan 13 doi: 10.1055/s-0031-1299681. PMID: 22395801

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