High Risk of Diabetes and Neurobehavioral Deficits in Individuals With Surgically Treated Hyperinsulinism.
BACKGROUND
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.


METHODS
This was designed as a cross-sectional study of individuals who underwent pancreatectomy for HI between 1960 and 2008.


RESULTS
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.
Division 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.
Katherine
K
Lord
Division 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.
Jerilynn
J
Radcliffe
Division 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.
Paul R
PR
Gallagher
Division 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.
N Scott
NS
Adzick
Division 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.
Charles A
CA
Stanley
Division 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.
Diva D
DD
De León
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology* / Q000523:psychology*; D044903:Congenital Hyperinsulinism / Q000150:complications* / Q000523:psychology / Q000601:surgery*; D003430:Cross-Sectional Studies; D003920:Diabetes Mellitus / Q000209:etiology* / Q000503:physiopathology / Q000523:psychology; D005260:Female; D006442:Glycated Hemoglobin / Q000032:analysis; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use; D007328:Insulin / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009483:Neuropsychological Tests; D010180:Pancreatectomy; D011183:Postoperative Complications / Q000503:physiopathology* / Q000523:psychology*; D011247:Pregnancy; D012307:Risk Factors; D055815:Young Adult
D006442:Glycated Hemoglobin; D007004:Hypoglycemic Agents; D007328:Insulin