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Hyperinsulinism
Acta Paediatrica Academiae Scientiarum Hungaricae
|January 1, 1980
Summary
A rare form of persistent hyperinsulinemic hypoglycemia in a child was diagnosed late due to temporary diazoxide treatment. Surgical removal of part of the pancreas successfully normalized blood sugar levels.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Surgical Gastroenterology
Background:
- Persistent hyperinsulinemic hypoglycemia (PHH) is a rare cause of severe neonatal hypoglycemia.
- Early diagnosis and treatment are crucial to prevent neurological complications.
Observation:
- A 2-year-old boy presented with severe, intractable hypoglycemia since birth.
- Diazoxide treatment provided only transient success, delaying diagnosis until 1.5 years of age.
- Diagnostic criteria included specific measurements of plasma insulin, blood glucose, and urinary ketones during a controlled fast.
Findings:
- The patient exhibited a normal glycemic response to glucagon.
- A significantly high rate of glucose infusion was required to maintain normal blood glucose levels.
- Subtotal pancreatectomy resulted in normalization of blood glucose levels.
Implications:
- Subtotal pancreatectomy is an effective treatment for intractable hyperinsulinemic hypoglycemia.
- The surgical intervention restored normoglycemia without compromising exocrine pancreatic function.
- This case highlights the importance of timely diagnosis and appropriate management strategies for congenital hyperinsulinism.