自身免疫性多腺体病综合征Ⅲ型患儿临床特征分析

目的了解儿童自身免疫性多腺体综合征(APS)的诊断和治疗,探讨自身免疫性甲状腺疾病(AITD)对糖尿病代谢控制的影响。方法入选者为1993 2004年在儿童医院诊断治疗的1型糖尿病合并甲状腺疾病患儿16例,男性3例,女性13例。对病人的病史、家族史、体检及内分泌相关检查的结果进行分析。抗体检查:GAD-Ab、IAA、ICA、TG-Ab、TPO-Ab、TRAb和肾上腺皮质细胞质抗体(ACC)。采用t检验对1型糖尿病(T1DM)合并AITD病人分为甲状腺高功能和低功能者的糖化血红蛋白(HbA1C)进行比较。结果自身免疫性多腺体病综合征Ⅲ型(APSⅢ)患儿的第一腺体疾病发病年龄为7岁4月~13岁11月,平均年龄10.56岁,中位年龄10.65岁。T1DM合并弥漫性毒性甲状腺肿(GD)或桥本甲状腺炎(HT)者分别为4例和12例。甲状腺功能亢进者6例,功能低下者10例。T1DM和AITD发病间隔为0~10年;甲状腺病临床表现可以不突出。有内分泌疾病家族史者占25%。GD控制前3例病人的HbA1C分别为10%、12%和14%,1例已经结束疗程GD控制良好者的HbA1C为7.8%。2例合并HT并甲状腺高功能者的HbA1C分别为11.8%和10.2%。而HT甲状腺功能减低者的糖尿病代谢控制相对较好,HbA1C为6.3%~10%。经t检验显示高功能组和低功能组病人的HbA1C差异有统计学意义(P=0.003),胰岛素用量差异无统计学意义。结论本组患儿女性多于男性;HT较GD为多;多伴有内分泌疾病家族史。甲状腺功能状态影响糖尿病的治疗。T1DM病人宜定期进行甲状腺自身抗体和甲状腺功能检查。

Clinical Features of Autoimmune Polyglandular Syudrome Type Ⅲ in Children

Objective To analyze the clinical features of 16 cases with T1DM and AIDT and to evalute the effect of AITD on T1DM.Methods General data of 16 cases were analyzed including family history,physical signs, hormones and antibodies.T test was used to compare the insulin and HbA_1C between 2 groups.Results The onset age of the patients was 7～14 years old,median age was 10.56.3 cases were male and 13 female,The interval between two diseases onset was 0～10 years.T1DM combinded with GD or HT were 4 and 12 cases,respectively.4 patients had family history.The levels of HbA_1C of GD were 10%,12% and 14%,respectively before alleviation;GD combinded with hyperparathyroidism were 11.8% and 10.2%,GD combinded with hypoparathyroidism was 6.3%～10%.There were significantly difference between the two groups above metion(P=0.003).Conclusion There are more famales than males,more HT than GD in APS.Most of APS have family history.It is imporfant to check the thyroid function and antibodies early and regularly for T1DM patients,especially for girls.