J Korean Soc Pediatr Endocrinol.  2000 Dec;5(2):171-181.

Clinical Outcome after Pancreatectomy in Patients with Persistent Hyperinsulinemic Hypoglycemia of Infancy

Abstract

PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of infancy(PHHI).
METHODS
Medical records of 10 patients(9 males and 1 female, mean age:40.4+/-1.5 months) who were diagnosed as PHHI and underwent pancreatectomy from 1988 to 2000 were reviewed. Clincal and biochemical data were recorded. Subjects were classified arbitrarily into early-onset or late-onset group according to age of onset. Pathologic appearance of pancreas was divided into 2 forms:diffuse or focal. The former had a focal pancreatic adenomatous hyperplasia and the latter was characterized by increased number of betacells with similar distribution seen in normal neonates.
RESULTS
One patient had focal, and nine had diffuse lesions. After near-total pancreatectomy, 4 patients(40.0%) showed complete response, 4(40.0%) had persistent hypoglycemia, and 2(20.0%) developed diabetes mellitus. As neurological sequelae, 6 patients(60.0%) had persistent seizures, and 6(60.0%) had delayed motor and speech development. No clinical or biochemical factors related to postoperative outcome were found.
CONCLUSION
This data indicate that early diagnosis of patients who present with hypoglycemic symptoms in infancy, especially early in life, and development of more effective therapy are warranted, because there is no clinical or biochemical factor predicting final outcome after near-total pancreatectomy and only 40% of patients with PHHI remained euglycemic after surgery with possible severe neurological sequelae.

Keyword

Persistent hyperinsulinemic hypoglycemia of infancy; Pancreatectomy

MeSH Terms

Age of Onset
Congenital Hyperinsulinism*
Diabetes Mellitus
Early Diagnosis
Female
Humans
Hyperplasia
Hypoglycemia
Infant, Newborn
Male
Medical Records
Pancreas
Pancreatectomy*
Seizures
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