Transient hyperinsulinism in neonates

Abstract

Congenital hyperinsulinusm is rare disease characterized high secretion of insulin by pancreatic beta cells leading to the development of hypoglycemia. Persistent and transient forms of hyperinsulinism are distinguished. Transient hyperinsulinism are the most common cause of severe hypoglycemia in newborns. The etiology of this disease is not known. There are risk factors for the development of transient hyperinsulinism: asphyxia at birth, prematurity, maternal diabetes, low or large weight by gestation. Hypoglycemia with hyperinsulinism is severe. Therefore, early diagnosis and therapy especially during the neonatal period, are necessary.The article describes 3 clinical cases of transient hyperinsulinism in children with different gestational age and concomitant pathology. All children recevied insulinostatic therapy with diazoxide with a positive effect: euglycemia without glucose requirement . In all children, therapy was completed subsequently. At the time of publication of the article, the physical and psychomotor development of children is normal.

Hypoglycemia in Children: Major Endocrine-Metabolic Causes and Novel Therapeutic Perspectives

“…Hyperinsulinism can be transient, congenital or syndromic. Transient hyperinsulinism is a common cause of severe hypoglycemia in newborns and it can be found in children of diabetic mothers, IUGR, SGA or it can be secondary to perinatal stress [ 22 ]. The disease course is usually self-limiting and it is defined by resolution prior to 6 months of age [ 23 ].…”

A Newborn With Severe and Refractory Hypoglycemia

“…Transient HI is a common cause of severe hypoglycemia in the early neonatal period. 4 Although the usual risk factors predisposing to HI were not present in utero (maternal diabetes, isolated large for gestational age, reduced glycogen stores as seen in prematurity, small for gestational age or subnormal liver enzyme function as seen in asphyxia and also prematurity), there were other pointers suggestive of fetal and newborn stress, such as maternal pre-eclampsia and a risk for early-onset sepsis. Transient HI requiring therapy to maintain blood glucose ≥3.5 mmol/L can persist from a few days to weeks, to even a few months of life.…”

“…4 Additionally, a poor response to diazoxide is known to be classically associated with persistent forms of HI. 4 Comparison of clinical characteristics to predict transient vs persistent HI at an early stage has shown no difference in time of onset, degree of hypoglycemia, or symptomatology. A birth weight of 90th centile or more did have a positive correlation with more persistent forms of HI.…”

“…Persistent HI is defined by the requirement of therapy for more than 4 months to maintain a normoglycemic state. 4 Additionally, a poor response to diazoxide is known to be classically associated with persistent forms of HI. 4 Comparison of clinical characteristics to predict transient vs persistent HI at an early stage has shown no difference in time of onset, degree of hypoglycemia, or symptomatology.…”