地中海贫血患儿内分泌功能及生育问题

Endocrine function and fertility in children with thalassemia

早期输血治疗、高血清铁蛋白水平、螯合治疗依从性差及脾切除是重型地中海贫血患者内分泌系统并发症的主要危险因素。生长发育迟缓,出现下丘脑-垂体-性腺轴紊乱,表现为青春期停滞、延迟和性腺机能减退;常合并甲状旁腺功能减退(HPT)、亚临床甲状腺功能减退症;胰腺功能受损,临床表现葡萄糖耐量试验异常及症状性糖尿病。

Early blood transfusion,high serum ferritin level,poor compliance with chelation therapy and splenectomy are the main risk factors for endocrine complications in patients with severe thalassemia. Endocrine system complications are manifested in the following aspects:firstly,growth retardation and hypothalamus-pituitary-gonad axis disorder,manifested as puberty stagnation,delay and hypogonadism;secondly,hypoparathyroidism (HPT) and subclinical hypothyroidism. finally,impaired pancreas function,characterized by abnormal glucose tolerance test and symptomatic diabetes mellitus.