Persistent hyperinsulinaemic hypoglycaemia of infancy (nesidioblastosis): a report from Kuwait

We report nine Bedouin children from Kuwait with persistent hyperinsulinaem ic hypoglycaemia (PHHI) seen over a 13-year period in two regional hospital s. The incidence of PHHI in this inbred community is high (1:20,000); five of them came from two families. All the children presented with seizures as sociated with severe and recurrent hypoglycaemia, eight presenting in the n eonatal period and one at the age of 2 months. One child died soon after bi rth. All the others received diazoxide initially, which achieved remission in one while two siblings remain dependent on the drug. Long-acting somatos tatin analogue (octreotide) was successfully used in one child. Four childr en underwent pancreatectomy, two showed diffuse and two had localized nesid ioblastosis. Two children achieved normal neurodevelopmental milestones, fo ur suffered mental retardation of varying degrees and three died. Early dia gnosis and prompt treatment are essential to avoid the neurological damage associated with hypoglycaemia. In some cases, this condition is due to an a utosomal recessive pattern of inheritance and it is therefore important to offer genetic counselling to families with one or more affected siblings.