Pediatric endocrinology

Divergent metabolic phenotypes in two genetic syndromes of low insulin secretion

Diabetes Res Clin PractPublished Cross-sectional study

Authors' conclusion

A distinct metabolic phenotype depending on GH counter-regulatory status, associates with diabetes development and excess glucose-induced IAPP secretion.

Study context

Population
Adults with life-long diminished insulin secretion (DIS): Laron syndrome (LS) and Guevara-Rosenbloom syndrome (GRS), and controls
Exposure
LS vs GRS vs controls
Outcome
Metabolic phenotype (body composition, adipokines, IGF axis, 5-hour OGTT)

Main finding

As reported in the abstract

  • Both syndromic cohorts displayed DIS during oral glucose tolerance test (OGTT)
  • LS: higher total and high molecular weight (HMW) adiponectin, lower IGF-I, IGF-II and IGF-Binding Protein-3 than other groups
  • LS: normal glycemic responses during OGTT with the lowest IAPP secretion
  • GRS: higher protein glycation, deficient glucose control during OGTT, increased IAPP secretion

Observational data: associations, not effects.

Original publication

Divergent metabolic phenotypes in two genetic syndromes of low insulin secretion. Diabetes Res Clin Pract. Published 19 December 2022. doi:10.1016/j.diabres.2022.110228

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