4.7 Article

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

期刊

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
卷 100, 期 11, 页码 4133-4139

出版社

ENDOCRINE SOC
DOI: 10.1210/jc.2015-2539

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资金

  1. National Center for Research Resources [UL1RR024134]
  2. National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases [R01 DK098517, R37 DK056268, T32-DK63688-09]

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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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