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Persistent hyperinsulinemic hypoglycemia of infancy: long-term outcome following subtotal pancreatectomy
P Mahachoklertwattana1, C Suprasongsin, S Teeraratkul
1Department of Pediatrics, Ramathibodi Hospital, Faculty of Medicine, Mahidol University, Bangkok, Thailand.
Insights
Infants with early-onset persistent hyperinsulinemic hypoglycemia (PHHI) require extensive pancreatectomy (Px) and often face long-term complications. Late-onset PHHI can be managed with less extensive Px, potentially reducing future diabetes risk.
Area of Science:
- Pediatric Endocrinology
- Surgical Gastroenterology
- Neonatal Care
Background:
- Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is a common cause of infant hypoglycemia.
- Subtotal pancreatectomy (Px) is the standard surgical treatment.
- Long-term outcomes after Px for PHHI require further investigation.
Purpose of the Study:
- To evaluate the long-term outcomes of pancreatectomy (Px) in infants with persistent hyperinsulinemic hypoglycemia (PHHI).
- To compare outcomes between early-onset and late-onset PHHI.
- To assess the impact of Px extent on euglycemia and future complications.
Main Methods:
- Analysis of 10 children with PHHI who underwent partial (65-80%) or subtotal (81-95%) Px.
- Follow-up duration ranged from 2 to 9.4 years.
- Patients were categorized into early (<1 month) and late (≥1 month) onset groups based on age at hypoglycemia diagnosis.
Main Results:
- Early-onset PHHI patients required 85-95% Px, with some needing repeat surgery for persistent hypoglycemia.
- Six of seven early-onset patients experienced delayed development or subnormal IQ; one developed diabetes.
- Late-onset PHHI patients underwent less extensive Px (65-80%) and maintained euglycemia, though one developed diabetes.
Conclusions:
- Early-onset PHHI indicates more severe disease, necessitating extensive Px with a higher risk of diabetes.
- Less extensive Px in late-onset PHHI may reduce the incidence of future diabetes.
- Age at onset is a critical factor in predicting PHHI severity and surgical outcomes.
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.