ReviewEndocrine2021
Update on primary bilateral macronodular adrenal hyperplasia (PBMAH).
Review in Endocrine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
26 citing papers in PubMed, 55 citations in OpenAlex.
- Hypercortisolism: Causes, Consequences and Clinical Significance - A Review of Pathophysiology.Diabetes, obesity & metabolism · 2026Review
- European Reference Networks - a flagship activity of the EU in the field of rare and complex diseases: from 2017 to 2025.Orphanet journal of rare diseases · 2026Review
- Genetic background and management outcomes in primary bilateral macronodular adrenal hyperplasia: Implications for diagnosis and treatment-A retrospective cohort study.The Journal of international medical research · 2026Article
- Primary bilateral macronodular adrenal hyperplasia presenting as resistant hypertension with bilateral adrenal masses: a case report.Annals of medicine and surgery (2012) · 2026Article
- The Proteome of Bilateral Macronodular Adrenocortical Disease (BMAD) Shows Different Profiles Correlating with the Genetic Causes and Reveals Specific Dysregulation of RNA Polymerase II and Cholesterol Biosynthesis Enzymes.Endocrine pathology · 2026Article
- Bilateral macronodular adrenocortical disease with isolated primary aldosteronism and normocortisolemia: a case report.Frontiers in medicine · 2026Article
- First case report of theAME case reports · 2026Article
- Primary Bilateral Macronodular Adrenal Hyperplasia Associated WithJCEM case reports · 2026Article
- Bilateral Adrenalectomy for Primary Bilateral Macronodular Adrenocortical Hyperplasia With a NovelJournal of clinical medicine research · 2025Article
- Adrenal causes of endocrine hypertension in childhood or adolescence.Journal of endocrinological investigation · 2025Review
- Review
- Sexual dimorphism in benign adrenocortical tumours.European journal of endocrinology · 2025Review
- The mutational landscape of ARMC5 in Primary Bilateral Macronodular Adrenal Hyperplasia: an update.Orphanet journal of rare diseases · 2025Review
- Primary bilateral macronodular adrenal hyperplasia: A rare case report of Cushing syndrome and review of literature.Medicine · 2024Review
- Somatic Molecular Heterogeneity in Bilateral Macronodular Adrenocortical Disease (BMAD) Differs Among the Pathological Subgroups.Endocrine pathology · 2024Article
- Corticotropin-releasing hormone test predicts the outcome of unilateral adrenalectomy in primary bilateral macronodular adrenal hyperplasia.Journal of endocrinological investigation · 2024Article
- Case report: A 51-year-old diabetic patient with primary bilateral macronodular adrenal hyperplasia and primary hyperparathyroidism.Frontiers in endocrinology · 2024Article
- Challenges and controversies in adrenal surgery: A practical approach.Current problems in surgery · 2023Article
- ARMC5-negative primary bilateral macronodular adrenal hyperplasia.BMJ case reports · 2023Article
- Impact of Morphology in the Genotype and Phenotype Correlation of Bilateral Macronodular Adrenocortical Disease (BMAD): A Series of Clinicopathologically Well-Characterized 35 Cases.Endocrine pathology · 2023Article
Corrections and comments
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Authors and funding
5 authors at 4 institutions in 4 countries.
Funding
Abstract
Primary bilateral macronodular adrenal hyperplasia (PBMAH), characterized by bilateral benign adrenal macronodules (>1 cm) potentially responsible for variable levels of cortisol excess, is a rare and heterogeneous disease. However, its frequency increases due to incidentally diagnosed cases on abdominal imaging carried out for reasons other than suspected adrenal disease. Mostly isolated, it can also be associated with dominantly inherited genetic conditions in rare cases. Considering the bilateral nature of adrenal involvement and the description of familial cases, the search of a genetic predisposition has led to the identification of germline heterozygous inactivating mutations of the putative tumor suppressor gene ARMC5, causing around 25% of the apparent sporadic cases. Rigorous biochemical and imaging assessment are key elements in the management of this challenging disease in terms of diagnosis. Treatment is reserved for symptomatic patients with overt or subclinical Cushing syndrome, and was historically based on bilateral adrenalectomy, which nowadays tends to be replaced by unilateral adrenalectomy or lifelong treatment with cortisol synthesis inhibitors.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.