AUTHOR=Avatapalle Bindu H., Banerjee Indraneel (Indi) , Shah Sajni , Pryce Megan , Nicholson Jacqueline , Rigby Lindsey , Caine Louise , Didi Mohammed , Skae Mars , Ehtisham Sarah , Patel Leena , Padidela Raja , Cosgrove Karen E., Dunne Mark , Clayton Peter 

TITLE=Abnormal Neurodevelopmental Outcomes are Common in Children with Transient Congenital Hyperinsulinism

JOURNAL=Frontiers in Endocrinology

VOLUME=4

YEAR=2013

URL=https://www.frontiersin.org/journals/endocrinology/articles/10.3389/fendo.2013.00060

DOI=10.3389/fendo.2013.00060

ISSN=1664-2392

ABSTRACT=<p><bold>Introduction:</bold> Neuroglycopenia is recognized to be associated with abnormal neurodevelopmental outcomes in 26–44% of children with persistent congenital hyperinsulinism (P-CHI). The prevalence of abnormal neurodevelopment in transient CHI (T-CHI) is not known. We have aimed to investigate abnormal neurodevelopment and associated factors in T-CHI and P-CHI.</p><p><bold>Materials and Methods:</bold> A cohort of children with CHI (<italic>n</italic> = 67, age 2.5–5 years) was assessed at follow-up review and noted to have normal or abnormal (mild or severe) neurodevelopmental outcomes for the domains of speech and language, motor, and vision. Children were classified as P-CHI (<italic>n</italic> = 33), if they had undergone surgery or remained on medical therapy, or T-CHI (<italic>n</italic> = 34), if medical treatment for hypoglycemia was stopped.</p><p><bold>Results:</bold> Overall, abnormal neurodevelopment was present in 26 (39%) children with CHI, of whom 18 (69%) were severe. Importantly, the incidence of abnormal neurodevelopment in T-CHI was similar to that in P-CHI (30 vs. 47% respectively, <italic>p</italic> = 0.16). The prevalence of severe abnormal neurodevelopment in speech, motor, and vision domains was similar in both T-CHI and P-CHI children. For this cohort, we found that the severity of disease [based upon maximal diazoxide dose (odds ratio 95% confidence intervals) 1.3 (1.1; 1.5), <italic>p</italic> = 0.03], and early presentation of CHI &lt;7 days following birth [5.9 (1.3; 27.8), <italic>p</italic> = 0.02] were significantly associated with abnormal neurodevelopment. There was no significant association with gender, genotype, or the histopathological basis of CHI.</p><p><bold>Conclusion:</bold> Abnormal neurodevelopment was evident in one third of children with both T-CHI and P-CHI, early presentation and severe CHI being risk factors. Early recognition and rapid correction of hypoglycemia are advocated to avoid abnormal neurodevelopment in children with CHI.</p>