Objectives:To characterize the clinical features and insulin regulation in in fants with hypoglycemia due to prolonged neonatal hyperinsulinism.Study design:Data were collected on 26 infants with hypoglycemia due to n...Objectives:To characterize the clinical features and insulin regulation in in fants with hypoglycemia due to prolonged neonatal hyperinsulinism.Study design:Data were collected on 26 infants with hypoglycemia due to neonatal hyperinsuli nism that later resolved.Acute insulin response(AIR)tests to calcium,leucine,glucose,and tolbutamide were performed in 11 neonates.Results were compared to children with genetic hyperinsulinism due to mutations of the adenosine triph osphate-dependent potassium(KATP)channel and glutamate dehydrogenase(GDH).R esults:Among the 26 neonates,there were significantly more males,small-for-gestational-age infants,and cesarean deliveries.Only 5 of the 26 had no ident ifiable risk factor.Hyperinsulinism was diagnosed at a median age of 13 days(r ange,2 to 180 days)and resolved by a median age of 181 days(range,18 to 403 d ays).Diazoxide was effective in 19 of the 21 neonates treated.In the 11 neonat es tested,the AIRs to calcium,leucine,glucose,and tolbutamide resembled thos e in normal controls and differed from genetic hyperinsulinism due to KATP chann el and GDH mutations.Conclusions:We define a syndrome of prolonged neonatal hy perinsulinism that is responsive to diazoxide,persists for several months,and resolves spontaneously.AIR tests suggest that both the KATP channel and GDH hav e normal function.展开更多
文摘Objectives:To characterize the clinical features and insulin regulation in in fants with hypoglycemia due to prolonged neonatal hyperinsulinism.Study design:Data were collected on 26 infants with hypoglycemia due to neonatal hyperinsuli nism that later resolved.Acute insulin response(AIR)tests to calcium,leucine,glucose,and tolbutamide were performed in 11 neonates.Results were compared to children with genetic hyperinsulinism due to mutations of the adenosine triph osphate-dependent potassium(KATP)channel and glutamate dehydrogenase(GDH).R esults:Among the 26 neonates,there were significantly more males,small-for-gestational-age infants,and cesarean deliveries.Only 5 of the 26 had no ident ifiable risk factor.Hyperinsulinism was diagnosed at a median age of 13 days(r ange,2 to 180 days)and resolved by a median age of 181 days(range,18 to 403 d ays).Diazoxide was effective in 19 of the 21 neonates treated.In the 11 neonat es tested,the AIRs to calcium,leucine,glucose,and tolbutamide resembled thos e in normal controls and differed from genetic hyperinsulinism due to KATP chann el and GDH mutations.Conclusions:We define a syndrome of prolonged neonatal hy perinsulinism that is responsive to diazoxide,persists for several months,and resolves spontaneously.AIR tests suggest that both the KATP channel and GDH hav e normal function.
