胰岛素泵联合生长激素治疗Mauriac综合征一例报道

Insulin pump combined with growth hormone in the treatment of Mauriac syndrome:a case report

Mauriac综合征是一组表现为侏儒、肝大、肥胖的青少年型糖尿病综合征,又名糖尿病假性侏儒症。患者常为青春期前起病,症状严重者多为婴幼儿时发病。由于患者自身胰岛功能缺陷,Ins分泌受损,机体处于能量负平衡状态,最终导致血糖控制不佳,生长发育迟缓。

Mauriac syndrome is a group of adolescent diabetic syndrome characterized by dwarfism,hepatomegaly and obesity,also known as diabetic pseudodwarfism.This disease usually starts before puberty,and the severe cases may development early in infants and children.Due to the defect of islet function,impaired insulin secretion,poor blood glucose control,and long-term negative energy balance,the patient’s growth and development are seriously delayed.