title: Persistent hyperinsulinaemic hypoglycaemia of infancy (nesidioblastosis): a report from Kuwait.
abstract: We report nine Bedouin children from Kuwait with persistent hyperinsulinaemic hypoglycaemia (PHHI) seen over a 13-year period in two regional hospitals. 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 associated with severe and recurrent hypoglycaemia, eight presenting in the neonatal period and one at the age of 2 months. One child died soon after birth. All the others received diazoxide initially, which achieved remission in one while two siblings remain dependent on the drug. Long-acting somatostatin analogue (octreotide) was successfully used in one child. Four children underwent pancreatectomy, two showed diffuse and two had localized nesidioblastosis. Two children achieved normal neurodevelopmental milestones, four suffered mental retardation of varying degrees and three died. Early diagnosis and prompt treatment are essential to avoid the neurological damage associated with hypoglycaemia. In some cases, this condition is due to an autosomal recessive pattern of inheritance and it is therefore important to offer genetic counselling to families with one or more affected siblings.
authors.affiliation: Department of Paediatrics of Sabah, Hospital, Kuwait.
authors.forename: D G
authors.initials: DG
authors.lastname: Ramadan
authors.forename: M H
authors.initials: MH
authors.lastname: Badawi
authors.forename: M
authors.initials: M
authors.lastname: Zaki
authors.forename: Z
authors.initials: Z
authors.lastname: el Mazidi
authors.forename: E A
authors.initials: EA
authors.lastname: Ismail
authors.forename: H
authors.initials: H
authors.lastname: el Anzi
mesh_terms: D018912:Arabs; D002657:Child Development; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006946:Hyperinsulinism / Q000453:epidemiology* / Q000235:genetics / Q000628:therapy; D007003:Hypoglycemia / Q000453:epidemiology* / Q000235:genetics / Q000628:therapy; D007223:Infant; D007231:Infant, Newborn; D007730:Kuwait / Q000453:epidemiology; D008297:Male; D010182:Pancreatic Diseases / Q000453:epidemiology* / Q000235:genetics / Q000628:therapy; D011379:Prognosis; D016896:Treatment Outcome