Evidence map›Paper›PMID 33515368›Full record

ReviewReviews in endocrine & metabolic disorders2021

Paediatric Cushing's disease: Epidemiology, pathogenesis, clinical management and outcome.

Rosario Ferrigno, Valeria Hasenmajer, Silvana Caiulo, Marianna Minnetti, Paola Mazzotta, Helen L Storr, Andrea M Isidori, Ashley B Grossman, Maria Cristina De Martino, Martin O Savage

Open access · hybridAbstract readReview
In one paragraph

Review in Reviews in endocrine & metabolic disorders, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
26citing papers in PubMed, 2 pooled it
6.0field-weighted citation impact, top 3% of its field
1 · What the graph read from it

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.

2 · The registry

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.

3 · Its place in the literature

Who cites it

26 citing papers in PubMed, 2 syntheses or guidelines pooled it, 50 citations in OpenAlex.

  1. Pooled it
  2. Guideline
  3. Article
  4. Cushing's disease across the lifespan: a case-based clinical review.Archives of endocrinology and metabolism · 2026
    Review
  5. Atypical Presentation and Course of ACTH-Independent Cushing’s Syndrome in Two FamiliesJournal of clinical research in pediatric endocrinology · 2026
    Article
  6. Review
  7. Article
  8. Article
  9. Article
  10. Article
  11. Adrenal causes of endocrine hypertension in childhood or adolescence.Journal of endocrinological investigation · 2025
    Review
  12. Cushing syndrome in paediatric population: who and how to screen.Journal of endocrinological investigation · 2025
    Review
  13. Article
  14. Article
  15. Article
  16. Article
  17. Review
  18. Observational
  19. Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors at 5 institutions in 2 countries.

Rosario Ferrigno *Dipartimento di Medicina Clinica e Chirurgia, Federico II University, Naples, Italy.ORCID 0000-0002-8172-2405
Valeria Hasenmajer *Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy.ORCID 0000-0003-4301-6662
Silvana Caiulo *Primary care Paediatrician, Local Health Unit of Brindisi, Brindisi, Italy.
Marianna Minnetti *Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy.ORCID 0000-0002-9091-2809
Paola Mazzotta *Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy.
Helen L StorrCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine & Dentistry, London, UK.
Andrea M IsidoriDepartment of Experimental Medicine, Sapienza University of Rome, Rome, Italy.ORCID 0000-0002-9037-5417
Ashley B GrossmanCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine & Dentistry, London, UK.
Maria Cristina De MartinoDipartimento di Medicina Clinica e Chirurgia, Federico II University, Naples, Italy.ORCID 0000-0003-3354-009X
Martin O SavageCentre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine & Dentistry, London, UK. m.o.savage@qmul.ac.uk.ORCID 0000-0001-7902-3376
Sapienza University of Rome · ITFederico II University Hospital · ITWilliam Harvey Research Institute · GBOspedale A. Perrino · ITThe Royal Free Hospital · GB

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Cushing's disease (CD) is rare in paediatric practice but requires prompt investigation, diagnosis and therapy to prevent long-term complications. Key presenting features are a change in facial appearance, weight gain, growth failure, virilization, disturbed puberty and psychological disturbance. Close consultation with an adult endocrinology department is recommended regarding diagnosis and therapy. The incidence of CD, a form of ACTH-dependent Cushing's syndrome (CS), is equal to approximately 5% of that seen in adults. The majority of ACTH-secreting adenomas are monoclonal and sporadic, although recent studies of pituitary tumours have shown links to several deubiquitination gene defects. Diagnosis requires confirmation of hypercortisolism followed by demonstration of ACTH-dependence. Identification of the corticotroph adenoma by pituitary MRI and/or bilateral inferior petrosal sampling for ACTH may contribute to localisation before pituitary surgery. Transsphenoidal surgery (TSS) with selective microadenomectomy is first-line therapy, followed by external pituitary irradiation if surgery is not curative. Medical therapy to suppress adrenal steroid synthesis is effective in the short-term and bilateral adrenalectomy should be considered in cases unfit for TSS or radiotherapy or when urgent remission is needed after unsuccessful surgery. TSS induces remission of hypercortisolism and improvement of symptoms in 70-100% of cases, particularly when performed by a surgeon with experience in children. Post-TSS complications include pituitary hormone deficiencies, sub-optimal catch-up growth, and persisting excess of BMI. Recurrence of hypercortisolism following remission is recognised but infrequent, being less common than in adult CD patients. With experienced specialist medical and surgical care, the overall prognosis is good. Early referral to an experienced endocrine centre is advised.

Indexed as

Cushing SyndromePituitary ACTH HypersecretionPituitary NeoplasmsAdultChildHumansPituitary GlandTreatment OutcomeCortisolCushing’sHypercortisolismPaediatricPituitary

Identifiers

PMID33515368
PMCPMC8724222
OpenAlexW3123024892

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

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.