title: Persistent hyperinsulinemic hypoglycemia of infancy: experience at Siriraj Hospital.
abstract: BACKGROUND
  Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of recurrent or persistent hypoglycemia in early childhood. Conventionally, pancreatectomy (Px) has often been recommended to control hypoglycemia. However, PHHI can be managed successfully by intensive medical treatment to avoid pancreatectomy.
  
  
  METHODS
  Data from 10 infants (8M, 2F) with PHHI were retrospectively analyzed.
  
  
  RESULTS
  Eight patients (80%) developed symptoms within 72 hours after birth (early-onset). Six patients (60%) underwent 85 per cent-95 per cent Px due to failure of medical treatment. Two patients who underwent less than 95 per cent Px required second Px (97% and 99%). One patient developed permanent diabetes mellitus and malabsorption. Hypoglycemia could be successfully managed by medication alone in four patients (40%). Of these, three patients had early-onset neonatal hypoglycemia. Medication could be discontinued in three patients (75%). Three of ten patients (30%) had delayed development. Pancreatectomies and/or the diagnosis of PHHI were made late for these patients. One of these three children also developed epilepsy.
  
  
  CONCLUSIONS
  Patients with PHHI frequently require pancreatectomy which commonly results in long-term complications especially diabetes mellitus and malabsorption. Our data suggest that PHHI can be managed successfully with an intensive medical regimen even in patients with early-onset hypoglycemia. Although medical management is very laborious for the family and physician, it should be applied until euglycemia is accomplished. Moreover, the early diagnosis of PHHI and the successful hypoglycemic control are very necessary to prevent permanent neurologic sequelae.
authors.affiliation: Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Pairunyar
authors.initials: P
authors.lastname: Sawathiparnich
authors.forename: Supawadee
authors.initials: S
authors.lastname: Likitmaskul
authors.forename: Kitti
authors.initials: K
authors.lastname: Angsusingha
authors.forename: Saroj
authors.initials: S
authors.lastname: Nimkarn
authors.forename: Katharee
authors.initials: K
authors.lastname: Chaichanwatanakul
authors.forename: Mongkol
authors.initials: M
authors.lastname: Laohapansang
authors.forename: Chanika
authors.initials: C
authors.lastname: Tuchinda
mesh_terms: D001786:Blood Glucose / Q000032:analysis; D003981:Diazoxide / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D005934:Glucagon / Q000008:administration & dosage; D005947:Glucose / Q000008:administration & dosage; D006785:Hospitals, University; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage; D006946:Hyperinsulinism / Q000150:complications* / Q000175:diagnosis / Q000628:therapy*; D007003:Hypoglycemia / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy*; D015994:Incidence; D007231:Infant, Newborn; D007262:Infusions, Intravenous; D007328:Insulin / Q000097:blood*; D008297:Male; D010180:Pancreatectomy / Q000379:methods; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D013785:Thailand / Q000453:epidemiology; D016896:Treatment Outcome
chemical_list: D001786:Blood Glucose; D007328:Insulin; D005934:Glucagon; D005947:Glucose; D003981:Diazoxide; D006854:Hydrocortisone