Related Experiment Video

Updated: Dec 12, 2025

Isolation of Human Islets from Partially Pancreatectomized Patients
11:10

Isolation of Human Islets from Partially Pancreatectomized Patients

Published on: July 30, 2011

16.7K

Persistent Hyperinsulinemic Hypoglycemia with Pancreatic Teratoma in Infancy: A Case Report

Ayse Pinar Cemeroglu1, Faik Sarialioglu2, Fatma Burcu Belen-Apak2

  • 1Deparment of Pediatric Endocrinology, Faculty of Medicine, Baskent University, Ankara, Turkey.

The American Journal of Case Reports
|August 13, 2020
PubMed

Insights

A rare pancreatic teratoma caused severe hypoglycemia in an infant. Surgical removal cured the condition, highlighting a unique link between teratomas and hyperinsulinemic hypoglycemia in pediatrics.

Area of Science:

  • Pediatric Surgery
  • Endocrinology
  • Developmental Biology

Background:

  • Intraabdominal pancreatic teratomas are exceptionally rare in children.
  • Severe hyperinsulinemic hypoglycemia is a critical condition in infants.

Observation:

  • A 6-month-old infant presented with lethargy and abnormal eye movements, diagnosed with hyperinsulinemic hypoglycemia.
  • Abdominal imaging revealed a large intraabdominal mass, initially suspected to be fetus in fetu, but histopathology confirmed a mature teratoma with immature pancreatic tissue.
  • The mass was surgically excised, and the infant's hypoglycemia resolved post-operatively.

Findings:

  • This is the first reported case of severe hyperinsulinemic hypoglycemia in an infant caused by an intraabdominal teratoma.
  • Genetic analysis revealed heterozygous variations in HNF1ß and IRS1 genes, suggesting a role in the teratoma's embryogenesis and associated hypoglycemia.
  • No mutations in the ABCC8 gene were found, ruling out common causes of congenital hyperinsulinism.

Implications:

  • This case expands the differential diagnosis for infantile hyperinsulinemic hypoglycemia.
  • It underscores the importance of thorough investigation for ectopic teratomas in unexplained hypoglycemia cases.
  • The findings suggest a potential genetic predisposition involving HNF1ß and IRS1 in the development of pancreatic teratomas and hypoglycemia.

More Related Videos

Robotic Enucleation of an Intra-Pancreatic Insulinoma in the Pancreatic Head05:42

Robotic Enucleation of an Intra-Pancreatic Insulinoma in the Pancreatic Head

Published on: January 3, 2020

8.6K
Isolation, Culture, and Imaging of Human Fetal Pancreatic Cell Clusters08:09

Isolation, Culture, and Imaging of Human Fetal Pancreatic Cell Clusters

Published on: May 18, 2014

14.6K

Related Concept Videos

Hypoglycemia and Glucagon01:15

Hypoglycemia and Glucagon

Without prolonged fasting, healthy individuals maintain blood glucose levels above 3.5 mM due to a well-adapted neuroendocrine counterregulatory system that effectively prevents acute hypoglycemia, a potentially life-threatening condition. The primary clinical scenarios for hypoglycemia encompass diabetes treatment, inappropriate production of endogenous insulin or insulin-like substances by tumors, and the use of glucose-lowering agents in non-diabetic individuals. Notably, hypoglycemia in the...
674
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...
4.7K
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,...
2.9K
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...
4.1K
Glucose Homeostasis: Pancreatic Islets and Insulin Secretion01:27

Glucose Homeostasis: Pancreatic Islets and Insulin Secretion

The pancreatic islets comprising only 1%-2% of the volume are highly vascularized and innervated mini-organs. They contain five endocrine cell types, including β cells that secrete insulin, which is synthesized as a single polypeptide chain, preproinsulin, processed to proinsulin, and finally to insulin and C-peptide. This process is complex and regulated, involving the Golgi complex, the endoplasmic reticulum, and the secretory granules of the β cell.
Insulin and C-peptide are...
1.9K
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...
542