Abnormal Neurodevelopmental Outcomes are Common in Children with Transient Congenital Hyperinsulinism

Abnormal Neurodevelopmental Outcomes are Common in Children with Transient Congenital Hyperinsulinism

INTRODUCTION 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. MATERIALS AND METHODS A cohort of chil...

Feeding Problems Are Persistent in Children with Severe Congenital Hyperinsulinism

BACKGROUND Congenital hyperinsulinism (CHI) is a rare but severe disorder of hypoglycemia in children, often complicated by brain injury. In CHI, the long-term prevention of hypoglycemia is dependent on reliable enteral intake of glucose. However, feeding problems (FPs) often impede oral glucose delivery, thereby complicating the management of hypoglycemia. FPs have not been systematically char...

Vineland adaptive behavior scales to identify neurodevelopmental problems in children with Congenital Hyperinsulinism (CHI)

BACKGROUND Congenital Hyperinsulinism (CHI) is a disease of severe hypoglycaemia caused by excess insulin secretion and associated with adverse neurodevelopment in a third of children. The Vineland Adaptive Behavior Scales Second Edition (VABS-II) is a parent report measure of adaptive functioning that could be used as a developmental screening tool in patients with CHI. We have investigated th...

Neurodevelopmental outcomes in children with hemifacial microsomia.

OBJECTIVE To determine whether preadolescent children with hemifacial microsomia (HFM) have higher risk of neurodevelopmental delays than unaffected control individuals. DESIGN Case-control follow-up study of neurodevelopment in children with and without HFM. SETTING Case individuals were originally recruited from 26 craniofacial centers across the United States and Canada, and controls wer...

Defining Genotype-Phenotype Correlations in Children with Congenital Hyperinsulinism