Evidence map›Paper›PMID 41006196›Full record

ArticleThe Journal of clinical endocrinology and metabolism2026

Children With Pancreatic Hypoplasia Experience Poor Weight Gain and Labile Diabetes but Low Incidence of DKA.

Anna M Denson, Karen E Rodriguez, Lisa R Letourneau-Freiberg, Balamurugan Kandasamy, Rochelle N Naylor, Hussein D Abdullatif, Robert W Benjamin, Erin Cobry, Michael S Freemark, Inas H Thomas and 1 more

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In one paragraph

Article in The Journal of clinical endocrinology and metabolism, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
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

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

11 authors.

Anna M DensonDepartment of Medicine, University of Tennessee Health Science Center, Memphis, TN 38163, USA.
Karen E RodriguezDepartment of Medicine, University of Chicago, Chicago, IL 60637, USA.ORCID 0009-0000-5701-1913
Lisa R Letourneau-FreibergDepartment of Medicine, University of Chicago, Chicago, IL 60637, USA.ORCID 0000-0001-9465-4870
Balamurugan KandasamyDepartment of Medicine, University of Chicago, Chicago, IL 60637, USA.
Rochelle N NaylorDepartment of Medicine, University of Chicago, Chicago, IL 60637, USA.
Hussein D AbdullatifDepartment of Pediatrics, University of Alabama at Birmingham, Birmingham, AL 35233, USA.
Robert W BenjaminDivision of Pediatric Endocrinology and the Duke Molecular Physiology Institute, Durham, NC 27710, USA.
Erin CobryDepartment of Pediatrics, University of Colorado, Barbara Davis Center for Diabetes, Aurora, CO 80045, USA.
Michael S FreemarkDivision of Pediatric Endocrinology and the Duke Molecular Physiology Institute, Durham, NC 27710, USA.
Inas H ThomasDepartment of Pediatrics, University of Michigan Health, Ann Arbor, MI 48109, USA.
Siri Atma W GreeleyDepartment of Medicine, University of Chicago, Chicago, IL 60637, USA.ORCID 0000-0002-0741-3567

Funding

Monogenic Diabetes: Next Generation Diagnosis, Treatment and ComplicationsR01DK104942 · NIDDK · UNIVERSITY OF CHICAGO · PI GREELEY, SIRI ATMA W., PHILIPSON, LOUIS H. · 2016 to 2024
$4.7M
NIDDK NIH HHS R01 DK104942NIDDK NIH HHS R01DK104942
6 · The paper itself

Abstract

contextPathogenic variants in GATA6, GATA4, and PDX1 cause pancreatic hypoplasia or agenesis and early onset diabetes mellitus. There is a lack of information about long-term outcomes and clinical management of these complicated patients, including how best to approach their exocrine pancreatic insufficiency (EPI), weight gain, and glycemic management.

participantsWe investigated clinical features and treatment of patients with variants in GATA6, GATA4, and PDX1 identified through the US Monogenic Diabetes Registry. Data were self-reported or extracted from medical records.

resultsEleven children were studied. Pancreatic hypoplasia/agenesis, EPI, gallbladder agenesis, and congenital heart defects were common in this cohort. Novel features were present, such as recurrent infections and epilepsy. All participants were born small for gestational age and many had difficulties with weight gain. Insulin treatment was discontinued and later reinstated for 3 infants. Glycemic control in nearly all patients was suboptimal with the mean hemoglobin A1c being 8.7, but only 1 episode of diabetic ketoacidosis was reported. Fasting and postprandial hypoglycemia were common. Eight of 11 children required pancreatic enzymes. Two children required enteral feedings to maintain nutritional balance.

conclusionChildren with GATA6, GATA4, and PDX1-related neonatal diabetes have labile, insulin-dependent diabetes mellitus and varying degrees of EPI caused by pancreatic hypoplasia/agenesis. Intrauterine growth restriction and postnatal difficulties with weight gain are common but rates of diabetic ketoacidosis are low, which may in part be due to a lack of glucagon, an important driver of ketosis.

Indexed as

Diabetes Mellitus, Type 1Diabetic KetoacidosisExocrine Pancreatic InsufficiencyPancreasPancreatic DiseasesWeight GainChildChild, PreschoolFemaleGATA4 Transcription FactorHomeodomain ProteinsHumansIncidenceInfantInfant, NewbornMaleGATA4 Transcription FactorHomeodomain Proteinspancreatic and duodenal homeobox 1 proteinTrans-Activatorsmonogenic diabetesneonatal diabetespancreas disorderspancreatic agenesispancreatic hypoplasiapediatric endocrinology

Identifiers

PMID41006196
PMCPMC13017217

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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.