All issues > Volume 43(2); 2000

Original Article

J Korean Pediatr Soc. 2000;43(2):253-259. Published online February 15, 2000.

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

Hyun-Jeong HJ Kim¹, Mi-Jeong MJ Kim², Hwa-Sook HS Shin¹, Jung-Sim JS Kim¹, Mie-Ryung MR Uhm¹, Si-Hwan SH Ko¹, Suk-Hyang SH Lee², Dong-Kyu DK Jin¹

¹Department of Pediatrics, Samsung Jeil Hospital
²Graduate School of Clinical Pharmacy Sookmyung Women`s University

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 100-150μg/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.

Keywords :Persistent hyperinsulinemic hypoglycemia of infancy(PHHI), Octreotide, Pancreatectomy