Risks for diabetes, neurobehavioral abnormalities rise in children after surgery for hyperinsulinism

SAN DIEGO — Children who receive surgical treatment for hyperinsulinism, particularly the diffuse form, are at an elevated risk for developing diabetes and neurobehavioral problems, according to research presented at The Endocrine Society annual meeting.

Screening for diabetes on an annual basis is necessary in children who undergo a near-total pancreatectomy, and developmental assessment is essential in all children with hyperinsulinism, according to researchers.

“We found a significant proportion of children developed diabetes and neurobehavioral problems, but many are not receiving the appropriate screening for these complications,” Katherine Lord, MD, of the Children's Hospital of Philadelphia, Pennsylvania, told Endocrine Today.

Katherine Lord

Lord and colleagues conducted a cross-sectional study of individuals with hyperinsulinism who had a pancreatectomy between 1960 and 2008 and received care at their hospital to examine the prevalence of diabetes and neurobehavioral deficits; 121 patients with a median age of 8.9 years (range, 3.5-50.7 years) were included.

The researchers collected clinical data through patient interviews and medical record reviews. The Adaptive Behavior Assessment System-2nd Edition and the Child Behavior Checklist were used to evaluate neurobehavioral outcomes.

The median age at pancreatectomy was 1.7 months (range 0.2-144) and pancreatic histology showed diffuse hyperinsulinism in 59 patients (49%), focal hyperinsulinism in 54 (45%) and other histology in 8 (7%).

Patients with diffuse hyperinsulinism had a greater extent pancreatectomy compared with focal (95%, range 75-99% vs. 50%, range 1-98%; P < .0005).

Diabetes occurred in 44 patients (36%); 8 developed diabetes immediately following pancreatectomy, and the median age of diabetes diagnosis in the remaining 36 was 7.7 years (range, 8 months-43 years). The median HbA1c at the time of the study was 7.4% (range, 6.5%-12.6%) and 38 patients (86%) with diabetes required insulin.

Diva D. De Leon

Patients who developed diabetes diffuse hyperinsulinism more often than focal (84% vs. 14%, P < .0005) and a greater extent pancreatectomy compared with patients who did not develop the condition (95%, range 65-98 vs. 65%, range 1-98).

Neurobehavioral dysfunction was reported in 58 patients (48%) including 16 (13%) with seizures, 22 (18%) with speech delay, 19 (16%) with a learning disability, 13 (11%) with a physical disability and 25 (21%) with behavioral issues.

Among 71 patients assessed through the Adaptive Behavior Assessment System, 19 (28%) had abnormal scores, or more than 1 standard deviation below the mean; among 62 assessed through the Child Behavior Checklist, 10 (16%) had abnormal scores, or more than 1 standard deviation above the mean.

“This study speaks to the need for new medical treatments for children with diffuse disease so they can avoid near-total pancreatectomies as well the importance of early diagnosis and appropriate treatment for [hyperinsulinism] so neurologic injury is less likely,” Diva D. De Leon, MD, MSCE, told Endocrine Today. “New guidelines on the screening and management of hypoglycemia will soon be published by the Pediatric Endocrine Society.” – by Allegra Tiver

Reference:

Lord K. Abtract OR41-6. Presented at: The Endocrine Society Annual Meeting; March 5-8, 2015, San Diego.

Disclosure: Lord reports no relevant financial disclosures.