ArticleThe Journal of clinical endocrinology and metabolism2010
Cushing syndrome in the McCune-Albright syndrome.
Article in The Journal of clinical endocrinology and metabolism, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 56 papers, 1 of them a synthesis that pooled it.
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.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
56 citing papers in PubMed, 1 synthesis or guideline pooled it, 139 citations in OpenAlex.
- Treatment of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline.The Journal of clinical endocrinology and metabolism · 2015Guideline
- Genetic background of infantile hypophosphatemia: a narrative review.Translational pediatrics · 2026Review
- cAMP/PKA signaling in endocrine hypertension: genetic mechanisms and pathophysiological insights.Frontiers in endocrinology · 2026Review
- Adrenal causes of endocrine hypertension in childhood or adolescence.Journal of endocrinological investigation · 2025Review
- Fibrous dysplasia/McCune-Albright syndrome: state-of-the-art advances, pathogenesis, and basic/translational research.Orphanet journal of rare diseases · 2025Review
- Cushing syndrome in paediatric population: who and how to screen.Journal of endocrinological investigation · 2025Review
- Successful Use of Metyrapone Suppositories in an Infant with Neonatal Cushing and McCune Albright Syndrome: A Case Report.Hormone research in paediatrics · 2025Article
- Clinical spectrum and uncommon features of McCune-Albright syndrome in children: a cohort study from a National Referral Center.Frontiers in endocrinology · 2025Article
- Posterior Reversible Encephalopathy Syndrome in the Context of McCune-Albright Syndrome: A Case Report.Cureus · 2024Article
- The longest reported survival of a child with McCune-Albright syndrome and a severe early presenting phenotype consisting of neonatal cushing syndrome, cardiac and liver diseases.Journal of surgical case reports · 2023Article
- Neonatal cholestasis as the onset symptom of McCune-Albright syndrome: case reports and a literature review.Frontiers in pediatrics · 2023Article
- Case Report: Severe McCune-Albright syndrome presenting with neonatal Cushing syndrome: navigating through clinical obstacles.Frontiers in endocrinology · 2023Article
- Brain and eye involvement in McCune-Albright Syndrome: clinical and translational insights.Frontiers in endocrinology · 2023Review
- Gene Mutations in Cushing's Syndrome.Molecular syndromology · 2023Review
- Treatment of Primary Pigmented Nodular Adrenocortical Disease.Hormone and metabolic research = Hormon- und Stoffwechselforschung = Hormones et metabolisme · 2022Review
- Primary bilateral macronodular adrenal hyperplasia: definitely a genetic disease.Nature reviews. Endocrinology · 2022Review
- Cushing syndrome as a failed cardiac screen in a patient with McCune-Albright syndrome: a case report.Journal of medical case reports · 2022Article
- Article
- An Overview of the Heterogeneous Causes of Cushing Syndrome Resulting From Primary Macronodular Adrenal Hyperplasia (PMAH).Journal of the Endocrine Society · 2022Review
- Genotype-Phenotype Correlation in Fibrous Dysplasia/McCune-Albright Syndrome.The Journal of clinical endocrinology and metabolism · 2021Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
contextCushing syndrome (CS) is a rare but potentially fatal feature of McCune-Albright syndrome (MAS). Optimal management, prognostic features, and long-term follow-up of this disorder have not been described.
settingThe study was conducted at an academic tertiary care center. PATIENTS: A total of 112 patients participating in a natural history study at the National Institutes of Health (NIH) were evaluated, and 21 published cases were reviewed.
interventionsSubjects received observation, medical management, or bilateral adrenalectomy.
main outcome measuresWe measured prevalence, prognostic factors, and natural history.
resultsThe prevalence of CS among NIH patients was 7.1%. The median age at diagnosis was 3 months. Clinical features included "Cushingoid facies" (66.7%), failure to thrive (60.0%), low birth weight (50.0%), liver disease (36.7%), and heart disease (26.7%). Six patients (20.0%) died, four after adrenalectomy. Death was more likely in patients with comorbid heart disease (odds ratio, 13.3; P < 0.05). Of 23 survivors, 13 underwent adrenalectomy, and 10 exhibited spontaneous resolution. Two patients with spontaneous resolution who were tested later in life (3 and 15 yr after resolution) continued to have low-level, autonomous adrenal function with biochemical adrenal insufficiency. Compared to MAS patients without CS, patients with CS were more likely to have a cognitive/developmental disorder (44.4 vs. 4.8%; P < 0.001; odds ratio, 8.8).
conclusionsComorbid heart and liver disease were poor prognostic markers and may indicate the need for prompt adrenalectomy. The high incidence of cognitive disorders indicates a need for close developmental follow-up and parental counseling. Patients with spontaneous resolution of CS may develop adrenal insufficiency, and they require long-term monitoring.
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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.