title: Persistent hyperinsulinemic hypoglycemia of infancy: long-term outcome following subtotal pancreatectomy.
abstract: BACKGROUND
  Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of persistent hypoglycemia in infants. The current standard treatment is subtotal pancreatectomy (Px). However, the long-term outcome following surgery needs further attention.
  
  
  METHODS
  We analyzed 10 children (7 M, 3 F) with PHHI who underwent partial (65-80%) and subtotal (81-95%) Px. Follow-up ranged from 2 to 9.4 yr (mean = 4.2 yr). We divided them into 2 groups based upon the age at onset of hypoglycemia: early (< 1 mo) and late (> or = 1 mo).
  
  
  RESULTS
  The seven patients in the early-onset group underwent 85-95% Px between ages of 18 d and 3 mo. Three of them initially treated by 85-90% Px had persistent hypoglycemia postoperatively. Two out of three required a 2nd operation with 95% Px for controlling hypoglycemia, though both still had persistent hypoglycemia and required medication to control blood glucose. The remaining four had 95% Px and had maintained euglycemia postoperatively. One patient developed diabetes 6 yr after surgery. Six of seven patients had delayed development and subnormal IQ. Three patients of the late-onset group (3 mo, 6 mo and 4 yr) underwent partial Px (80%, 65% and 65%, respectively) and maintained euglycemia postoperatively. Despite 65% Px, one developed diabetes 3 yr after surgery.
  
  
  CONCLUSIONS
  These results suggest that children with early-onset hypoglycemia have more severe hyperinsulinism than those with late-onset hypoglycemia. The former require 95% Px for maintaining euglycemia, but long-term complications with diabetes may be common. In contrast, the latter require lower percentage Px which may reduce the incidence of diabetes in the future.
authors.affiliation: Department of Pediatrics, Ramathibodi Hospital, Faculty of Medicine, Mahidol University, Bangkok, Thailand.
authors.forename: P
authors.initials: P
authors.lastname: Mahachoklertwattana
authors.forename: C
authors.initials: C
authors.lastname: Suprasongsin
authors.forename: S
authors.initials: S
authors.lastname: Teeraratkul
authors.forename: C
authors.initials: C
authors.lastname: Preeyasombat
mesh_terms: D000367:Age Factors; D001786:Blood Glucose / Q000378:metabolism; D003920:Diabetes Mellitus / Q000209:etiology; D005260:Female; D005947:Glucose / Q000008:administration & dosage; D005951:Glucose Tolerance Test; D006130:Growth Disorders / Q000209:etiology; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications* / Q000601:surgery*; D007003:Hypoglycemia / Q000150:complications* / Q000188:drug therapy / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D007328:Insulin / Q000097:blood; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D010180:Pancreatectomy; D012086:Reoperation; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D001786:Blood Glucose; D007328:Insulin; D005947:Glucose