If your child had a near-total pancreatectomy to control congenital hyperinsulinism (HI), it is almost certain that he or she will develop diabetes, new research from CHOP’s Congenital Hyperinsulinism Center shows. Neurobehavioral deficits are also common in children, whether they have focal or diffuse disease, pointing out the importance of early identification of hypoglycemia and the need for developmental assessments in a child’s first few years.
In the largest study to date of children who undergo pancreatectomy for treatment of HI, CHOP researchers, led by endocrinologists Katherine Lord, MD, and Diva D. De León-Crutchlow, MD, MSCE, studied the outcomes of 121 patients who had undergone a pancreatectomy. They found that 36 percent later developed diabetes.
The greater the percentage of the pancreas that was removed, the higher the incidence of diabetes. Ninety-three percent of those with diabetes underwent a 95 percent or greater pancreatectomy. Children with diffuse disease also were far more likely to later develop diabetes, compared to those with focal disease. “This is why it is so important that we identify focal patients,” Lord says. “Using genetic tests and the 18F-DOPA PET scan offered at CHOP, our surgeon can remove as little of the pancreas as needed and, most likely, spare the child from getting diabetes down the road.”
Of the 44 patients who developed diabetes, nine children developed it immediately post-pancreatectomy. Of the remaining 35 children who developed diabetes, the median age at diabetes diagnosis was 7.7 years.
Nearly half of the children had neurobehavioral abnormalities, probably due to repeated periods of low blood sugar during times of critical brain development. Psychiatric/behavioral problems and speech delay were the most common problems reported. “Even with improved treatments, the rate of neurobehavioral deficits remains high,” Lord says. “That suggests the damage is done in the first few days of life, before the child is diagnosed and treatment is started.”
Lord K, Radcliffe J, Gallagher PR, Adzick NS, Stanley CA, De León DD. High risk of diabetes and neurobehavioral deficits in individuals with surgically treated hyperinsulinism. J Clin Endocrinol Metab. 2015;100(11):4133-4139.