Hussain Khalid, Aynsley-Green Albert, Stanley Charles A
The London Centre for Paediatric Endocrinology and Metabolism, Great Ormond Street Hospital for Children NHS Trust WC1N 3JH and the Institute of Child Health, University College London, WC1N 1EH United Kingdom.
Pediatr Endocrinol Rev. 2004 Nov;2 Suppl 1:163-7.
Congenital hyperinsulinism of infancy is the commonest cause of persistent and recurrent hypoglycaemia in the neonatal and infancy period. It is a heterogeneous disorder with respect to clinical presentation, histology, molecular biology and genetics. The biochemical profile is one of hypoketotic, hypofattyacidemic hypoglycemia. To prevent permanent brain damage from hypoglycemia, the treatment of infants with congenital hyperinsulinism must be prompt and aggressive. The drugs used in the medical therapy for congenital hyperinsulinism are diazoxide, octreotide, glucagon and nifedipine.
婴儿先天性高胰岛素血症是新生儿期和婴儿期持续性和复发性低血糖最常见的原因。在临床表现、组织学、分子生物学和遗传学方面，它是一种异质性疾病。生化特征是低酮血症、低脂血症性低血糖。为防止低血糖造成永久性脑损伤，对先天性高胰岛素血症婴儿的治疗必须迅速且积极。用于先天性高胰岛素血症药物治疗的药物有二氮嗪、奥曲肽、胰高血糖素和硝苯地平。
