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Persistent hyperinsulinemic hypoglycemia of infancy: experience at Siriraj Hospital
Pairunyar Sawathiparnich1, Supawadee Likitmaskul, Kitti Angsusingha
1Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Insights
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) often leads to pancreatectomy, causing complications. Intensive medical management can successfully treat PHHI, avoiding surgery and preventing long-term issues like diabetes and developmental delays.
Area of Science:
- Pediatric Endocrinology
- Neonatology
- Surgical Gastroenterology
Background:
- Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a leading cause of recurrent childhood hypoglycemia.
- Pancreatectomy (Px) is a conventional treatment, but often leads to complications.
- Intensive medical management offers an alternative to surgery.
Purpose of the Study:
- To evaluate the efficacy of intensive medical management for PHHI.
- To assess the outcomes and complications associated with pancreatectomy versus medical treatment.
- To determine the necessity of early diagnosis and intervention in PHHI.
Main Methods:
- Retrospective analysis of data from 10 infants diagnosed with PHHI.
- Review of treatment strategies, including medical management and pancreatectomy.
- Assessment of clinical outcomes, complications, and developmental status.
Main Results:
- 40% of patients were successfully managed with medication alone, including those with early-onset hypoglycemia.
- 60% of patients required pancreatectomy (85%-95%), with some needing repeat surgeries.
- Long-term complications such as diabetes mellitus, malabsorption, and delayed development occurred in patients undergoing pancreatectomy or experiencing delayed diagnosis.
Conclusions:
- Intensive medical management can successfully treat PHHI, even in early-onset cases, potentially avoiding pancreatectomy and its complications.
- Early diagnosis and effective glycemic control are crucial for preventing neurological sequelae.
- While medically intensive, this approach should be prioritized to achieve euglycemia and improve long-term outcomes.
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.
Method:
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.