TY - JOUR
T1 - Abnormal neurodevelopmental outcomes are common in children with transient congenital hyperinsulinism
AU - Avatapalle, Hima Bindu
AU - Banerjee, Indraneel
AU - Shah, Sajni
AU - Pryce, Megan
AU - Nicholson, Jacqueline
AU - Rigby, Lindsey
AU - Caine, Louise
AU - Didi, Mohammed
AU - Skae, Mars
AU - Ehtisham, Sarah
AU - Patel, Leena
AU - Padidela, Raja
AU - Cosgrove, Karen E.
AU - Dunne, Mark J.
AU - Clayton, Peter E.
PY - 2013
Y1 - 2013
N2 - 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 children with CHI (n = 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 (n = 33), if they had undergone surgery or remained on medical therapy, or T-CHI (n = 34), if medical treatment for hypoglycemia was stopped. Results: 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, p = 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), p = 0.03], and early presentation of CHI
AB - 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 children with CHI (n = 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 (n = 33), if they had undergone surgery or remained on medical therapy, or T-CHI (n = 34), if medical treatment for hypoglycemia was stopped. Results: 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, p = 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), p = 0.03], and early presentation of CHI
KW - Congenital hyperinsulinism
KW - Developmental delay
KW - Hypoglycemia
KW - Neurodevelopment
KW - Neurological outcome
KW - Seizures
KW - Transient congenital hyperinsulinism
U2 - 10.3389/fendo.2013.00060
DO - 10.3389/fendo.2013.00060
M3 - Article
C2 - 23730298
VL - 4
JO - Frontiers in Endocrinology
JF - Frontiers in Endocrinology
IS - MAY
M1 - Article 60
ER -