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Challenges in management of transient hyperinsulinism - a retrospective analysis of 36 severely affected children

Henrike Hoermann1, Marcia Roeper1, Roschan Salimi Dafsari1

  • 1Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children's Hospital, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany.

Journal of Pediatric Endocrinology & Metabolism : JPEM
|April 16, 2021
PubMed

Insights

Transient hyperinsulinism (THI) is a neonatal hypoglycemia disorder. Perinatal stress is a risk factor, and improved postnatal monitoring and standardized diazoxide treatment protocols are needed for better management of THI.

Area of Science:

  • Neonatology
  • Pediatric Endocrinology
  • Metabolic Disorders

Background:

  • Transient hyperinsulinism (THI) is a neonatal hypoglycemia disorder with an unclear pathomechanism, though perinatal stress is a known predisposing factor.
  • Early diagnosis and effective management are crucial for infants with THI.

Purpose of the Study:

  • To characterize the clinical phenotype and treatment of children with THI.
  • To identify potential improvements in the management of THI.

Main Methods:

  • Retrospective analysis of 36 children diagnosed with THI between 2007 and 2019.
  • Review of clinical data, diagnostic methods, and treatment strategies.

Main Results:

  • All patients exhibited risk factors for neonatal hypoglycemia or indicators of perinatal stress.
  • Most infants were diagnosed on day of life 1; later diagnoses were associated with lower blood glucose levels.
  • Diazoxide was effective, but treatment withdrawal and fasting tests lacked standardization, leading to recurrent hypoglycemia in some cases.

Conclusions:

  • Perinatal stress may increase the risk of THI, highlighting the need for enhanced postnatal hypoglycemia monitoring.
  • Diazoxide is effective for THI, but standardized criteria for treatment initiation and discontinuation are required.
  • Establishing consensus diagnostic criteria for THI is essential for improved study comparability and clinical management.
Abstract

Keywords:
congenital hyperinsulinismdiazoxidehypoglycemiatransient hyperinsulinism

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