J Korean Pediatr Soc.  2000 Feb;43(2):253-259.

Medical Therapy in Children with Persistent Hyperinsulinemic Hypoglycemia of Infancy(PHHI)

• Kim HJ
• Kim MJ
• Shin HS
• Kim JS
• Uhm MR
• Ko SH
• Lee SH
• Jin DK

Abstract

PURPOSE: Persistent hyperinsulinemic hypoglycemia of infancy(PHHI), which is characterised by inappropriate insulin secretion in spite of hypoglycemia, needs urgent treatment to prevent cerebral hypoglycemic damage. Although pancreatectomy is the treatment of choice for PHHI, there are several complications which follow treatment. We suggest that aggressive medical therapy, when effective, is preferable to partial pancreatectomy.
METHODS
We evaluated 8 patients with PHHI admitted to the Department of Pediatrics, Samsung Medical Center from November 1996 to January 1999. Children with hypoglycemia in the range of 3-50mg/dl were included. Octreotide was administered at dosage of 100-150 microgram/day. When the patients did not respond to octreotide, diazoxide and nifedipine were given in addition.
RESULTS
In four of eight patients, octreotide was discontinued after 15 to 165 days. One patient was given diazoxide instead. The remaining 3 patients are still being treated with octreotide.
CONCLUSION
We believe that maximum effort should be made to attain euglycemia with medication, and pancreatectomy should be reserved for patients in whom these measures fail to restore normoglycemia.

Keyword

Persistent hyperinsulinemic hypoglycemia of infancy(PHHI); Octreotide; Pancreatectomy

MeSH Terms

Child*
Congenital Hyperinsulinism*
Diazoxide
Humans
Hypoglycemia
Insulin
Nifedipine
Octreotide
Pancreatectomy
Pediatrics
Diazoxide
Insulin
Nifedipine
Octreotide