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

Journal of the Korean Pediatric Society 2000;43(2):253-259.
Published online February 15, 2000.
Medical Therapy in Children with Persistent Hyperinsulinemic Hypoglycemia of Infancy(PHHI)
Hyun-Jeong Kim1, Mi-Jeong Kim2, Hwa-Sook Shin1, Jung-Sim Kim1, Mie-Ryung Uhm1, Si-Hwan Ko1, Suk-Hyang Lee2, Dong-Kyu Jin1
1Department of Pediatrics, Samsung Jeil Hospital
2Graduate School of Clinical Pharmacy Sookmyung Women`s University
유아의 지속적 고인슐린혈성 저혈당증에 대한 내과적 치료의 효능평가
김현정1, 김미정2, 신화숙1, 김정심1, 엄미령1, 고시환1, 이숙향2, 진동규1
1성균관대학교 삼성제일병원 소아과
2숙명여자대학교 약학과
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.
Key Words: Persistent hyperinsulinemic hypoglycemia of infancy(PHHI), Octreotide, Pancreatectomy


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