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[Persistent hyperinsulinemic hypoglycemia. Two case reports]
Norberto Sotelo-Cruz1, Andrés Cordero-Olivares, Carlos Ramírez-Rodríguez
1Servicio de Medicina Interna, Hospital Infantil del Estado de Sonora. nsotelo@hmo.megared.net.mx
Cirugia Y Cirujanos
|November 20, 2004
Summary
Persistent hyperinsulinism (PHH) in infants causes seizures and hypoglycemia. A glucose/insulin ratio > 0.3 suggests surgery, like pancreatectomy, may be needed if medical treatments fail.
Area of Science:
- Pediatric Endocrinology
- Surgical Gastroenterology
Background:
- Persistent hyperinsulinism (PHH) is a critical cause of recurrent hypoglycemia in infants.
- Diagnosis can be challenging, often requiring specialized metabolic testing.
Observation:
- Two infants presented with seizures and persistent hypoglycemia.
- Initial diagnostic workup revealed a high glucose/insulin ratio (> 0.3) but no pancreatic abnormalities.
- Medical management with hydrocortisone, diazoxide, and octreotide proved ineffective.
Findings:
- Nesidioblastosis, characterized by islet cell changes, was confirmed histologically post-surgery.
- Sub-total pancreatectomy was the definitive treatment for both cases.
- Histologic findings confirmed nesidioblastosis in the Langerhans islets.
Implications:
- A glucose/insulin ratio > 0.3 in infants with PHH is a strong indicator for surgical intervention.
- Timely surgical treatment, such as pancreatectomy, should be considered to avoid delayed management.
- This highlights the importance of surgical consideration in refractory PHH cases.