Related Experiment Video

Updated: May 15, 2026

Hyperinsulinemic-euglycemic Clamps in Conscious, Unrestrained Mice
11:10

Hyperinsulinemic-euglycemic Clamps in Conscious, Unrestrained Mice

Published on: November 16, 2011

Cognitive and developmental outcome of conservatively treated children with congenital hyperinsulinism

Yael Levy-Shraga1, Orit Pinhas-Hamiel, Efrat Kraus-Houminer

  • 1Safra Children’s Hospital, Tel Hashomer, Israel. yael.levy.shraga@gmail.com

Journal of Pediatric Endocrinology & Metabolism : JPEM
|January 19, 2013
PubMed

Insights

Most children with congenital hyperinsulinism (CHI) treated medically achieved normal cognitive development. However, over half showed below-normal adaptive skills, suggesting ongoing challenges despite improved management of this infant hypoglycemia condition.

Area of Science:

  • Pediatric Endocrinology
  • Neurodevelopmental Pediatrics
  • Metabolic Disorders

Background:

  • Congenital hyperinsulinism (CHI) is a leading cause of persistent infant hypoglycemia, posing significant risks for neurological damage.
  • Effective management is crucial to maintain normoglycemia and prevent adverse neurodevelopmental outcomes.
  • Treatment strategies for CHI often involve complex medical therapies and surgical interventions.

Purpose of the Study:

  • To evaluate the cognitive, developmental, and adaptive skill outcomes in children with CHI managed solely through medical treatment.
  • To assess the long-term impact of medical management on neurodevelopmental trajectories in pediatric patients with CHI.

Main Methods:

  • A cohort of 14 children under 10 years with CHI, receiving only medical treatment, was studied.
  • Participants underwent comprehensive physical and neurological examinations.
  • Standardized assessments included the Bayley Scale of Infant and Toddler Development (3rd Ed.) or Kaufman Assessment Battery for Children, Vineland Adaptive Behavior Scales, and Achenbach Child Behavior Checklist (CBCL).

Main Results:

  • 86% of children demonstrated normal cognitive and developmental scores on standardized assessments.
  • Two children (14%) exhibited cognitive achievements below the expected range.
  • Parental reports indicated below-normal adaptive skills in 57% of the patients assessed via the Vineland questionnaire.

Conclusions:

  • Contrary to prior research, this study observed normal cognitive achievements in the majority of medically treated children with CHI.
  • The findings suggest that earlier diagnosis and enhanced medical management over the last decade may contribute to better cognitive outcomes.
  • Despite cognitive normality, a significant proportion of children present with challenges in adaptive functioning, highlighting the need for continued support and monitoring.
Abstract

More Related Videos

Full-Endoscopic Surgery for Hypothalamic Hamartoma Resection02:22

Full-Endoscopic Surgery for Hypothalamic Hamartoma Resection

Published on: April 12, 2024

Studying the Hypothalamic Insulin Signal to Peripheral Glucose Intolerance with a Continuous Drug Infusion System into the Mouse Brain08:32

Studying the Hypothalamic Insulin Signal to Peripheral Glucose Intolerance with a Continuous Drug Infusion System into the Mouse Brain

Published on: January 4, 2018

Related Concept Videos

Diabetes Mellitus: Overview and Type I Subtype01:22

Diabetes Mellitus: Overview and Type I Subtype

Diabetes mellitus is a chronic metabolic disorder characterized by high blood glucose levels due to inadequate insulin production, insulin resistance, or both. The condition affects millions worldwide and can significantly impact their health and quality of life.
Type 1 diabetes is an autoimmune disease in which the immune system mistakenly attacks and destroys the insulin-producing beta cells in the pancreas. As a result, the body is unable to produce sufficient insulin, and individuals with...
Insulin: Dosing Regimen and Adverse Effects01:16

Insulin: Dosing Regimen and Adverse Effects

Insulin-replacement therapy usually includes both long-acting insulin (basal) and short-acting insulin (to cater to postprandial needs). In a diverse group of type 1 diabetes patients, the average daily insulin dose is typically 0.5-0.7 units/kg body weight. However, obese patients and pubertal adolescents may need more due to insulin resistance.
The basal dose constitutes about 40%-50% of the total daily dose, with the rest as premeal insulin. The mealtime insulin dose should mirror...
Diabetes Mellitus: Type 2 and Gestational01:22

Diabetes Mellitus: Type 2 and Gestational

Type 2 diabetes, characterized by insulin resistance, arises when the insulin receptors on cells lose responsiveness to insulin, diminishing the cell's capacity to take up glucose, resulting in elevated blood glucose levels. To receive a diagnosis of Type 2 diabetes, a series of blood glucose tests are necessary to assess whether the blood glucose falls within normal parameters. If the result is out of the normal range, a patient may be diagnosed as prediabetic or diabetic, depending on the...
Type I Diabetes III: Clinical Manifestations01:19

Type I Diabetes III: Clinical Manifestations

Type 1 diabetes mellitus typically presents with rapid-onset symptoms due to the body’s inability to utilize glucose in the absence of insulin. Since insulin is required for glucose uptake into cells, its deficiency leads to hyperglycemia and cellular energy deprivation, resulting in characteristic clinical features.Polyuria and PolydipsiaOne of the earliest, most prominent symptoms is polyuria (excessive urination). When blood glucose concentrations rise above the renal threshold, the kidneys...
Pathophysiology of Diabetes01:20

Pathophysiology of Diabetes

Diabetes mellitus is a chronic metabolic disorder characterized by hyperglycemia. The four categories of diabetes are type 1 diabetes, type 2 diabetes, other specific types of diabetes, and gestational diabetes.
Type 1 diabetes is characterized by autoimmune-mediated destruction of pancreatic β cells, with environmental factors potentially triggering this process in genetically susceptible individuals. Despite many not having a family history, certain genes increase susceptibility, suggesting a...
Type II Diabetes II: Pathophysiology01:24

Type II Diabetes II: Pathophysiology

PathophysiologyType 2 diabetes mellitus (T2DM ) is a chronic metabolic disorder characterized by insulin resistance and progressive pancreatic β-cell dysfunction, leading to impaired glucose homeostasis. It results from interactions among genetic predisposition, environmental factors, and metabolic stressors, such as overnutrition and a sedentary lifestyle.Insulin Resistance and Glucose DysregulationEarly T2DM involves insulin resistance in skeletal muscle, adipose tissue, and the liver.