ArticleThe Journal of clinical endocrinology and metabolism2020
Central Adrenal Insufficiency Is Rare in Adults With Prader-Willi Syndrome.
Article in The Journal of clinical endocrinology and metabolism, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 21 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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
21 citing papers in PubMed, 43 citations in OpenAlex.
- Long-term administration of hydrocortisone: down-regulating the level of cytokine and resulting in injuring testicular tissue of juvenile mice.BMC pharmacology & toxicology · 2025Article
- Assessment of hypothalamic-pituitary-adrenal axis impairment and effects of hydrocortisone treatment in adults with Prader-Willi syndrome.Frontiers in endocrinology · 2025Article
- Prader-Willi syndrome: guidance for children and transition into adulthood.Endocrine connections · 2024Article
- Endocrine features of Prader-Willi syndrome: a narrative review focusing on genotype-phenotype correlation.Frontiers in endocrinology · 2024Review
- Should we routinely assess hypothalamic-pituitary-adrenal axis in pediatric patients with Prader-Willi syndrome?Frontiers in endocrinology · 2024Article
- Premature adrenarche in Prader-Willi syndrome is associated with accelerated pre-pubertal growth and advanced bone age.Journal of pediatric endocrinology & metabolism : JPEM · 2023Article
- The Italian registry for patients with Prader-Willi syndrome.Orphanet journal of rare diseases · 2023Article
- The transition from pediatric to adult care in individuals with Prader-Willi syndrome.Endocrine connections · 2023Review
- Acute stress response of the HPA-axis in children with Prader-Willi syndrome: new insights and consequences for clinical practice.Frontiers in endocrinology · 2023Article
- Bone Health in Adults With Prader-Willi Syndrome: Clinical Recommendations Based on a Multicenter Cohort Study.The Journal of clinical endocrinology and metabolism · 2022Article
- Health Problems in Adults with Prader-Willi Syndrome of Different Genetic Subtypes: Cohort Study, Meta-Analysis and Review of the Literature.Journal of clinical medicine · 2022Article
- Endocrine and Metabolic Illnesses in Young Adults with Prader-Willi Syndrome.Journal of personalized medicine · 2022Article
- Approach to the Patient With Prader-Willi Syndrome.The Journal of clinical endocrinology and metabolism · 2022Article
- Impact of Deprivation on Obesity in Children with PWS.Journal of clinical medicine · 2022Article
- Evaluation of plasma ACTH in the metyrapone test is insufficient for the diagnosis of secondary adrenal insufficiency.Frontiers in endocrinology · 2022Article
- Family Matters: Trauma and Quality of Life in Family Members of Individuals With Prader-Willi Syndrome.Frontiers in psychiatry · 2022Article
- Adrenal insufficiency in patients with Prader-Willi syndrome.Frontiers in endocrinology · 2022Review
- What Every Internist-Endocrinologist Should Know about Rare Genetic Syndromes in Order to Prevent Needless Diagnostics, Missed Diagnoses and Medical Complications: Five Years of 'Internal Medicine for Rare Genetic Syndromes'.Journal of clinical medicine · 2021Article
- Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients.Journal of clinical medicine · 2021Article
- Hyponatremia in Children and Adults with Prader-Willi Syndrome: A Survey Involving Seven Countries.Journal of clinical medicine · 2021Article
Corrections and comments
- Erratum issued
Authors and funding
13 authors at 8 institutions in 7 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
contextPrader-Willi syndrome (PWS) is associated with several hypothalamic-pituitary hormone deficiencies. There is no agreement on the prevalence of central adrenal insufficiency (CAI) in adults with PWS. In some countries, it is general practice to prescribe stress-dose hydrocortisone during physical or psychological stress in patients with PWS. Side effects of frequent hydrocortisone use are weight gain, osteoporosis, diabetes mellitus, and hypertension-already major problems in adults with PWS. However, undertreatment of CAI can cause significant morbidity-or even mortality.
objectiveTo prevent both over- and undertreatment with hydrocortisone, we assessed the prevalence of CAI in a large international cohort of adults with PWS. As the synacthen test shows variable results in PWS, we only use the metyrapone test (MTP) and insulin tolerance test (ITT).
designMetyrapone test or ITT in adults with PWS (N = 82) and review of medical files for symptoms of hypocortisolism related to surgery (N = 645).
settingOutpatient clinic. PATIENTS OR OTHER
participantsEighty-two adults with genetically confirmed PWS.
main outcome measureFor MTP, 11-deoxycortisol > 230 nmol/L was considered sufficient. For ITT, cortisol > 500 nmol/L (Dutch, French, and Swedish patients) or > 450 nmol/L (British patients) was considered sufficient.
resultsCentral adrenal insufficiency was excluded in 81 of 82 patients. Among the 645 patients whose medical files were reviewed, 200 had undergone surgery without perioperative hydrocortisone treatment. None of them had displayed any features of hypocortisolism.
conclusionsCentral adrenal insufficiency is rare (1.2%) in adults with PWS. Based on these results, we recommend against routinely prescribing hydrocortisone stress-doses in adults with PWS.
Indexed as
Identifiers
What OpenQuestion holds
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.