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Persistent hyperinsulinaemic hypoglycaemia of infancy: case report
1Department of Paediatrics, Pamukkale University, School of Medicine, Denizli, Turkey.
East African Medical Journal
|March 15, 2003
Summary
Persistent hyperinsulinaemic hypoglycaemia of infancy is a rare condition. This case study details a successful subtotal pancreatectomy and octreotide treatment for a neonate with this challenging diagnosis.
Area of Science:
- Pediatric Endocrinology
- Neonatal Medicine
- Metabolic Disorders
Background:
- Persistent hyperinsulinaemic hypoglycaemia of infancy (PHI) is the most common cause of persistent neonatal hypoglycemia.
- PHI presents significant long-term management challenges due to recurrent hypoglycemia.
Observation:
- A male neonate presented with recurrent convulsions and lethargy at three months old.
- Initial investigations revealed severe hypoglycemia (24 mg/dl) with inappropriately high insulin levels, ketonuria absence, and high glucose infusion requirements.
- Despite maximal medical management including prednisone, diazoxide, and octreotide, normoglycemia was not achieved.
Findings:
- A diagnosis of persistent hyperinsulinaemic hypoglycaemia of infancy was confirmed.
- A subtotal (80%) pancreatectomy was performed, followed by continued intermittent hypoglycemic episodes.
- Post-operative management with low-dose octreotide effectively controlled residual hypoglycemia. Histology confirmed diffuse adenomatous hyperplasia (nesidoblastosis).
Implications:
- Subtotal pancreatectomy combined with targeted medical therapy can be effective in managing severe PHI.
- Early diagnosis and intervention are crucial for improving long-term outcomes in infants with PHI.
- This case highlights the utility of octreotide in managing refractory hyperinsulinemic hypoglycemia post-surgery.