Hyperinsulinemic hypoglycemia

MONDO:0005803

An inherited autosomal recessive syndrome characterized by the disorganized formation of new islets in the pancreas and congenital hyperinsulinism. It is due to focal hyperplasia of pancreatic islet cells budding off from the ductal structures and forming new islets of langerhans. Mutations in the islet cells involve the potassium channel gene kcnj11 or the atp-binding cassette transporter gene abcc8, both on chromosome 11.

Also known as: hyperinsulinemia hypoglycemia, hyperinsulinemic hypoglycemia (disease), nesidioblastosis

14 clinical trials for this condition and its sub-types, 2 tagged with Hyperinsulinemic hypoglycemia itself.

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Where it sits in the disease tree

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Part of

↑ Endocrine system disorder (12512) ↑ Inborn carbohydrate metabolic disorder (370)

Sub-types of Hyperinsulinemic hypoglycemia

Familial hyperinsulinism 9 trials · 12 incl. sub-types
3 sub-types
- Congenital isolated hyperinsulinism 0 trials · 2 incl. sub-types Sub-types →
- Adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia 1 trial
- Hyperinsulinism due to INSR deficiency 0 trials
Hyperinsulinemic hypoglycemia, familial, 1 1 trial
2 sub-types
- Autosomal recessive hyperinsulinism due to SUR1 deficiency 0 trials
- Diazoxide-resistant focal hyperinsulinism due to SUR1 deficiency 0 trials
Hyperinsulinemic hypoglycemia with polycystic kidney disease 0 trials
Hyperinsulinemic hypoglycemia, familial, 8 0 trials
Insulin autoimmune syndrome 0 trials

Most studied deeper sub-types

Exercise-induced hyperinsulinism (1) Hyperinsulinemic hypoglycemia, familial, 2 (1) Hyperinsulinism-hyperammonemia syndrome (1) Autosomal dominant hyperinsulinism due to Kir6.2 deficiency (0) Autosomal dominant hyperinsulinism due to SUR1 deficiency (0) Autosomal recessive hyperinsulinism due to Kir6.2 deficiency (0) Diazoxide-resistant diffuse hyperinsulinism (0) Diazoxide-resistant focal hyperinsulinism (0) Diazoxide-resistant focal hyperinsulinism due to Kir6.2 deficiency (0) Diazoxide-resistant hyperinsulinism (0) Diazoxide-sensitive diffuse hyperinsulinism (0) Hyperinsulinemic hypoglycemia, familial, 3 (0) Hyperinsulinemic hypoglycemia, familial, 4 (0) Hyperinsulinism due to HNF1A deficiency (0) Hyperinsulinism due to HNF4A deficiency (0) Hyperinsulinism due to UCP2 deficiency (0)

Including sub-types (14) Tagged with Hyperinsulinemic hypoglycemia (2)

Trials to join now! 7 Not yet recruiting 1 Not yet finished but already full! 3 Completed 3

A drop of cord blood at birth could flag a rare condition that harms infant brains

Diagnosis Not yet recruiting

This trial tests whether measuring glucose in umbilical cord blood, already collected at birth, can screen newborns for congenital hyperinsulinism, a condition that causes dangerously low blood sugar and can lead to brain damage if missed. Researchers will check the test's accura…

Sponsor: Odense University Hospital • Aim: Diagnosis

Last updated Sep 02, 2026 00:00 UTC

Hyperinsulinemic hypoglycemia

Where it sits in the disease tree

Most studied deeper sub-types

A drop of cord blood at birth could flag a rare condition that harms infant brains