Surgical Management of Pediatric Congenital Hyperinsulinism in a Chinese Cohort

27 pediatric patients with diffuse congenital hyperinsulinism are described, with 12 undergoing surgical intervention. The study compares outcomes between subtotal and near-total pancreatectomy over a median follow-up of 2.9 years. It was authored by Xiaoxiang Li and includes genetic and neurodevelopmental assessment data.

Use Cases

• Compare metabolic outcomes like postoperative diabetes rates between subtotal pancreatectomy and near-total pancreatectomy groups.
• Analyze the association between ABCC8/KCNJ11 mutation status and the decision for surgical intervention.
• Assess long-term neurodevelopmental outcomes using standardized tools across surgical and non-surgical patient groups.
• Model surgical intervention selection using clinical features via exploratory Firth penalized logistic regression.
• Examine hypoglycemia control patterns and trade-offs relative to the extent of pancreatic resection.

Strengths

• Contains detailed clinical and genetic data for 27 pediatric patients with a rare condition.
• Provides a median postoperative follow-up period of 2.9 years for longitudinal outcome assessment.
• Includes genetic testing results, with 8 out of 8 tested surgical patients harboring ABCC8/KCNJ11 mutations.

Limitations

• Small sample size of 27 patients limits statistical power and generalizability of findings.
• Single-center study from China may introduce geographic and institutional bias.
• Neurodevelopmental outcomes were assessed at variable ages, which may complicate direct comparisons.

Provenance

Source

figshare, authored by Xiaoxiang Li.

Collection Method

Clinical study summarizing patient characteristics, surgical procedures, and outcomes from a single center.

Time Range

Median postoperative follow-up was 2.9 years (range 2.0–8.8 years).

Freshness

Last updated March 23, 2026.

Geography

Study focuses on Chinese pediatric patients.