ArticleThe Journal of clinical endocrinology and metabolism2021
Long-Term Outcome and Treatment in Persistent and Transient Congenital Hyperinsulinism: A Finnish Population-Based Study.
Article in The Journal of clinical endocrinology and metabolism, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it, 27 citations in OpenAlex.
- International Guidelines for the Diagnosis and Management of Hyperinsulinism.Hormone research in paediatrics · 2024Guideline
- Congenital Hyperinsulinism With Paternally InheritedJournal of the ASEAN Federation of Endocrine Societies · 2026Article
- Knowledge, attitudes, and practices in pediatric hypoglycemia in Italy (KAPPHy study).Journal of endocrinological investigation · 2026Article
- Real-world insights into neurodevelopmental outcomes amongst people with congenital hyperinsulinism.Orphanet journal of rare diseases · 2026Article
- Comprehensive genetic rescreening improves diagnostic yield in congenital hyperinsulinism.Journal of the Endocrine Society · 2026Article
- Umbilical Cord Blood Glucose Concentrations and Transitional Neonatal Hypoglycemia.JAMA network open · 2026Article
- Reassessing Pancreatectomy in Diffuse Congenital Hyperinsulinism: A Tale of 2 Brothers With HomozygousJCEM case reports · 2025Article
- Severe congenital hyperinsulinism with progressive neurological deterioration due to novel HADH-GHSR digenic mutations: the first case report.Pediatric endocrinology, diabetes, and metabolism · 2025Article
- The Birth Prevalence of Congenital Hyperinsulinism: A Narrative Review of the Epidemiology of a Rare Disease.Hormone research in paediatrics · 2025Review
- Characterization of congenital hyperinsulinism in Argentina: Clinical features, genetic findings, and treatment outcomes.PloS one · 2025Article
- Neonatal dysglycemia: a review of dysglycemia in relation to brain health and neurodevelopmental outcomes.Pediatric research · 2024Review
- Congenital hyperinsulinism in the Ukraine: a 10-year national study.Frontiers in endocrinology · 2024Article
- Non-surgical Treatment May be Appropriate for Most Chinese Children With Monogenic Congenital Hyperinsulinism Based on a Retrospective Study of 121 Patients.Pediatric diabetes · 2024Article
- Hyperinsulinemic Hypoglycemia Diagnosed in Childhood Can Be Monogenic.The Journal of clinical endocrinology and metabolism · 2023Article
- New approaches to screening and management of neonatal hypoglycemia based on improved understanding of the molecular mechanism of hypoglycemia.Frontiers in pediatrics · 2023Review
- Syndromic forms of congenital hyperinsulinism.Frontiers in endocrinology · 2023Review
- Standardised practices in the networked management of congenital hyperinsulinism: a UK national collaborative consensus.Frontiers in endocrinology · 2023Review
- Congenital Hyperinsulinaemic Hypoglycaemia-A Review and Case Presentation.Journal of clinical medicine · 2022Review
- Increased referrals for congenital hyperinsulinism genetic testing in children with trisomy 21 reflects the high burden of non-genetic risk factors in this group.Pediatric diabetes · 2022Article
- Congenital Hyperinsulinism: Current Laboratory-Based Approaches to the Genetic Diagnosis of a Heterogeneous Disease.Frontiers in endocrinology · 2022Review
Corrections and comments
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Authors and funding
4 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
contextThe management of congenital hyperinsulinism (CHI) has improved.
objectiveTo examine the treatment and long-term outcome of Finnish patients with persistent and transient CHI (P-CHI and T-CHI).
designA population-based retrospective study of CHI patients treated from 1972 to 2015. PATIENTS: 106 patients with P-CHI and 132 patients with T-CHI (in total, 42 diagnosed before and 196 after year 2000) with median follow-up durations of 12.5 and 6.2 years, respectively.
main outcome measuresRecovery, diabetes, pancreatic exocrine dysfunction, neurodevelopment.
resultsThe overall incidence of CHI (n = 238) was 1:11 300 live births (1972-2015). From 2000 to 2015, the incidence of P-CHI (n = 69) was 1:13 500 and of T-CHI (n = 127) 1:7400 live births. In the 21st century P-CHI group, hyperinsulinemic medication was initiated and normoglycemia achieved faster relative to earlier. Of the 74 medically treated P-CHI patients, 68% had discontinued medication. Thirteen (12%) P-CHI patients had partial pancreatic resection and 19 (18%) underwent near-total pancreatectomy. Of these, 0% and 84% developed diabetes and 23% and 58% had clinical pancreatic exocrine dysfunction, respectively. Mild neurological difficulties (21% vs 16%, respectively) and intellectual disability (9% vs 5%, respectively) were as common in the P-CHI and T-CHI groups. However, the 21st century P-CHI patients had significantly more frequent normal neurodevelopment and significantly more infrequent diabetes and pancreatic exocrine dysfunction compared with those diagnosed earlier.
conclusionsOur results demonstrated improved treatment and long-term outcome in the 21st century P-CHI patients relative to earlier.
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