RESUMEN
Persistent severe hypoglycemia in the neonatal period is a rare condition, and hyperinsulinism is the most common cause. Its diagnosis is of great importance as it may be exceedingly difficult to control. Nesidioblastosis is a term used to describe the presence of numerous abnormal clusters of insulin secreting cells histopathologically, Its aetiology is unknown, that there may be a genetic component with an autosomal recessive inheritance pattern Is suggested by its familial occurance. We report its occurance in 3 sibs of a Jordanian family. The first is a baby girl FTND, birth weight 4.1 kg., died at the age of 6 days due to severe hypoglycemia, the second is baby boy delivered by cesarean section because of a large fetus, birth weight 5 kg., died at the age of 31 days with histological evidence of nesidioblastosis, the third is a baby girl delivered at 36/40, birth weight 3.7 kg., hypoglycemic investigated and treated by 95% pancreatectomy, well and alive. Our report gives another evidence of Its mode of Inheritance and confirms the importance of early recognition and efficient treatment In preventing irreversable brain damage which is likely to result in survivors with subsequent mental retardation