Children With Pancreatic Hypoplasia Experience Poor Weight Gain and Labile Diabetes but Low Incidence of DKA
An erratum to this article was published (J Clin Endocrinol Metab, 17 Mar 2026 ↗)
Authors' conclusion
Children 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.
Study context
- Population
- Children with pathogenic variants in GATA6, GATA4 and PDX1 (pancreatic hypoplasia/agenesis, early-onset DM), US Monogenic Diabetes Registry
- Participants
- 11 children
- Outcome
- Clinical features and treatment
Methodological detailsregistry-based descriptive study, US Monogenic Diabetes Registry, self-reported or medical record data, Data self-reported or extracted from medical records
- Registry-based descriptive study
- US Monogenic Diabetes Registry
- Self-reported or medical record data
- Data self-reported or extracted from medical records
Main finding
As reported in the abstract
- Born small for gestational age
- all participants
- Insulin discontinued and later reinstated
- 3 infants
- Mean HbA1c
- 8.7
- DKA episodes reported
- 1
- Required pancreatic enzymes
- 8 of 11
- Required enteral feedings
- 2
Observational data: associations, not effects.
Original publication
Children With Pancreatic Hypoplasia Experience Poor Weight Gain and Labile Diabetes but Low Incidence of DKA. J Clin Endocrinol Metab. Published 27 September 2025. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →