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High Risk of Diabetes and Neurobehavioral Deficits in Individuals With Surgically Treated Hyperinsulinism
Katherine Lord1, Jerilynn Radcliffe1, Paul R Gallagher1
1Division of Endocrinology and Diabetes (K.L., C.A.S., D.D.D.L.), The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104; Department of Pediatrics (K.L., J.R., C.A.S., D.D.D.L.), The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104; Biostatistics Core (P.R.G.), The Clinical and Translational Research Center, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104; and Department of Surgery (N.S.A.), The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104.
Insights
Children undergoing pancreatectomy for congenital hyperinsulinism (HI) face high risks of diabetes and neurobehavioral deficits. Early developmental assessments are crucial for managing these long-term complications in HI patients.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Surgical Outcomes
Background:
- Congenital hyperinsulinism (HI) often necessitates pancreatectomy in severe cases to manage hypoglycemia.
- Pancreatectomy increases diabetes risk, while persistent hypoglycemia can lead to neurocognitive dysfunction.
- The long-term prevalence of these complications following surgical treatment for HI is not well-defined.
Purpose of the Study:
- To determine the prevalence of diabetes and neurobehavioral deficits in individuals who underwent pancreatectomy for congenital hyperinsulinism (HI).
Main Methods:
- A cross-sectional study design was employed, analyzing data from 121 subjects who had pancreatectomy for HI between 1960 and 2008.
- Diabetes outcomes were assessed via patient interviews and medical records.
- Neurobehavioral outcomes were evaluated using the Adaptive Behavior Assessment System, 2nd edition (ABAS-II) and the Child Behavior Checklist (CBCL).
Main Results:
- 36% of subjects developed diabetes, with a higher proportion of pancreatectomy in those with diabetes (95%) compared to those without (65%).
- Neurobehavioral abnormalities were reported in 48% of subjects, including abnormal scores on the ABAS-II (28%) and CBCL (16%).
- Diabetes onset varied, with some diagnosed immediately post-surgery and others later in childhood or adolescence.
Conclusions:
- Near-total pancreatectomy for congenital hyperinsulinism (HI) places children at significant risk for developing diabetes.
- Neurobehavioral deficits are common sequelae in surgically treated HI patients.
- Routine developmental and behavioral assessments are essential for comprehensive care of children with HI.
Context:
Children with the most common and severe type of congenital hyperinsulinism (HI) frequently require pancreatectomy to control the hypoglycemia. Pancreatectomy increases the risk for diabetes, whereas recurrent hypoglycemia places children at risk of neurocognitive dysfunction. The prevalence of these complications is not well defined.
Objective:
The objective was to determine the prevalence of diabetes and neurobehavioral deficits in surgically treated HI.
Design:
This was designed as a cross-sectional study of individuals who underwent pancreatectomy for HI between 1960 and 2008.
Outcomes:
Diabetes outcomes were assessed through patient interview and medical record review. Neurobehavioral outcomes were assessed through the Adaptive Behavior Assessment System, 2nd edition (ABAS-II), and the Child Behavior Checklist (CBCL).
Results:
A total of 121 subjects were enrolled in the study at a median age of 8.9 years (range, 3.5-50.7 y). Thirty-six percent (44 of 121) of subjects had diabetes. Nine subjects developed diabetes immediately after pancreatectomy. Of the remaining 35 subjects who developed diabetes, the median age at diabetes diagnosis was 7.7 years (range, 8 mo to 43 y). In subjects with diabetes, the median hemoglobin A1c was 7.4% (range, 6.5-12.6%), and 38 (86%) subjects required insulin. Subjects with diabetes had a greater percentage of pancreatectomy than subjects without diabetes (95% [range, 65-98] vs 65% [1-98]). Neurobehavioral abnormalities were reported in 58 (48%) subjects. Nineteen (28%) subjects had abnormal ABAS-II scores, and 10 (16%) subjects had abnormal CBCL scores.
Conclusions:
Children, who undergo near-total pancreatectomy are at high risk of developing diabetes. Neurobehavioral deficits are common, and developmental assessment is essential for children with HI.
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