Evidence map›Paper›PMID 30343978›Full record

ArticleJournal of pediatric surgery2019

Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children.

N Scott Adzick, Diva D De Leon, Lisa J States, Katherine Lord, Tricia R Bhatti, Susan A Becker, Charles A Stanley

Registry-linked trialAbstract read
In one paragraph

Article in Journal of pediatric surgery, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01916148 (Expanded Access Use of 18F-L-Fluoro-DOPA PET/CT Scan Localization of Focal Pancreatic Lesions in Subjects With Hyperinsulinemic Hypoglycemia), which is not on this map. Cited by 59 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
59citing papers in PubMed, 1 pooled it
7.8field-weighted citation impact, top 2% 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.

NCT01916148 availablenot on this map

Expanded Access Use of 18F-L-Fluoro-DOPA PET/CT Scan Localization of Focal Pancreatic Lesions in Subjects With Hyperinsulinemic Hypoglycemia

Typeexpanded_accessSponsorChildren's Hospital of PhiladelphiaConditionsCongenital Hyperinsulinism (CHI), Beckwith-Wiedemann Syndrome, InsulinomaArms18F-DOPA
3 · Its place in the literature

Who cites it

59 citing papers in PubMed, 1 synthesis or guideline pooled it, 117 citations in OpenAlex.

  1. Guideline
  2. Review
  3. Congenital Hyperinsulinism With Paternally InheritedJournal of the ASEAN Federation of Endocrine Societies · 2026
    Article
  4. Article
  5. Determinants of hyperinsulinism severity in children with Beckwith-Wiedemann syndrome.The Journal of clinical endocrinology and metabolism · 2026
    Article
  6. Article
  7. Article
  8. Article
  9. Article
  10. Review
  11. Article
  12. [EJNMMI research · 2025
    Article
  13. Review
  14. Review
  15. Article
  16. Article
  17. Article
  18. Article
  19. Approach to the Neonate With Hypoglycemia.The Journal of clinical endocrinology and metabolism · 2024
    Review
  20. Review
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

7 authors at 2 institutions in 1 country.

N Scott AdzickDepartment of Surgery and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. Electronic address: adzick@email.chop.edu.
Diva D De LeonDepartment of Pediatrics and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
Lisa J StatesDepartment of Radiology and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
Katherine LordDepartment of Pediatrics and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
Tricia R BhattiDepartment of Pathology and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
Susan A BeckerDepartment of Pediatrics and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
Charles A StanleyDepartment of Pediatrics and the Congenital Hyperinsulinism Center, Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
University of Pennsylvania · USChildren's Hospital of Philadelphia · US

Funding

Neurological Phenotyping in Hyperinsulinism – Administrative Supplement to Islet dysregulation in Infants with Congenital HyperinsulinismR01DK056268 · NIDDK · CHILDREN'S HOSP OF PHILADELPHIA · PI DE LEON, DIVA D., GANGULY, ARUPA · 1999 to 2024
$9.1M
Islet Dysregulation in Infants with Congenital HyperinsulinismR37DK056268 · NIDDK · CHILDREN'S HOSP OF PHILADELPHIA · PI STANLEY, CHARLES ALFRED · 2009 to 2017
$6.0M
Insulin Secretion in Hyperinsulinism Human IsletsR01DK098517 · NIDDK · CHILDREN'S HOSP OF PHILADELPHIA · PI DE LEON, DIVA D. · 2013 to 2023
$4.5M
NIDDK NIH HHS R01 DK056268NIDDK NIH HHS R01 DK098517NIDDK NIH HHS R37 DK056268
6 · The paper itself

Abstract

backgroundCongenital Hyperinsulinism (HI) causes severe hypoglycemia in neonates and children. We reviewed our experience with pancreatectomy for the various types of HI.

methodsFrom 1998 to 2018, 500 patients with HI underwent pancreatectomy: 246 for focal HI, 202 for diffuse HI, 37 for atypical HI (16 for Localized Islet Nuclear Enlargement [LINE], 21 for Beckwith-Wiedemann Syndrome), and 15 for insulinoma. Focal HI neonates were treated with partial pancreatectomy. Patients with diffuse HI who failed medical management underwent near-total (98%) pancreatectomy. Atypical HI patients had pancreatectomies tailored to the PET scan and biopsy findings.

resultsThe vast majority of pancreatectomies for focal HI were < 50%, and many were 2%-10%. 97% of focal HI patients are cured. For diffuse disease patients, 31% were euglycemic, 20% were hyperglycemic, and 49% required treatment for hypoglycemia; the incidence of diabetes increased with long-term follow-up. All 15 insulinoma patients were cured.

conclusionsOur approach to patients with focal HI can distinguish focal from diffuse HI, localize focal lesions, and permit partial pancreatectomy with cure in almost all focal patients. Surgery does not cure diffuse disease but can help prevent severe hypoglycemia and brain damage. Surgery can be curative for insulinoma and for some cases of atypical HI. LEVEL OF EVIDENCE: Level IV.

Indexed as

AdolescentAdultChildChild, PreschoolCongenital HyperinsulinismFemaleFollow-Up StudiesHumansInfantInfant, NewbornInsulinomaLaparoscopyMalePancreasPancreatectomyPancreatic NeoplasmsBeckwith–Wiedemann syndromeCongenital hyperinsulinismInsulinomaLocalized islet nuclear enlargementMultiple endocrine neoplasia-type 1 (MEN1)Pancreatectomy

Identifiers

PMID30343978
PMCPMC6339589
OpenAlexW2894614745

What OpenQuestion holds

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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.