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唐氏综合征患儿多重抗体介导的甲状腺功能亢进症的管理

Management of multiple-antibody-mediated hyperthyroidism in children with Down's syndrome.

作者信息

Bhowmick S K, Grubb P H

机构信息

Department of Pediatrics, US Air Force Medical Center, Keesler AFB, Miss 39534-2519, USA.

出版信息

South Med J. 1997 Mar;90(3):312-5. doi: 10.1097/00007611-199703000-00009.

Abstract

During a period of 7 years at our institution, four girls and one boy with Down's syndrome, ages 9 to 16 years, were examined and treated for hyperthyroidism. Two patients had Graves' disease and they responded to propylthiouracil (PTU) with a predictable clinical course resulting in remission within 4 years. The remaining three patients included in this report had hyperthyroid profiles similar to those of the two with Graves' disease except for their antibody panels. These patients, in addition to the elevated thyroid-stimulating immunoglobulin (TSI) level observed in Graves' disease, also had significantly elevated antimicrosomal antibody (AMA) and antithyroglobulin antibody (ATGA) at the time of diagnosis. Elevated TSI level was again present in two patients who had a recurrence of hyperthyroidism after PTU therapy was discontinued. Treatment of these three patients was best done with the continuation of PTU therapy at a lower dose and the addition of thyroxine as soon as mild hypothyroidism developed. Treatment with PTU and thyroxine was continued until the TSI level was no longer elevated. Levels of AMA and ATGA remained elevated long after the TSI level became normal. All three patients eventually had hypothyroidism and continue to require thyroxine replacement.

摘要

在我们机构的7年时间里,对4名年龄在9至16岁的患有唐氏综合征的女孩和1名患有唐氏综合征的男孩进行了甲状腺功能亢进症的检查和治疗。两名患者患有格雷夫斯病,他们对丙硫氧嘧啶(PTU)有反应,临床病程可预测,4年内病情缓解。本报告中纳入的其余三名患者除抗体检测结果外,甲状腺功能亢进的特征与两名格雷夫斯病患者相似。这些患者除了在格雷夫斯病中观察到的甲状腺刺激免疫球蛋白(TSI)水平升高外,在诊断时抗微粒体抗体(AMA)和抗甲状腺球蛋白抗体(ATGA)也显著升高。在停止PTU治疗后甲状腺功能亢进复发的两名患者中,TSI水平再次升高。这三名患者的最佳治疗方法是继续使用较低剂量的PTU治疗,并在出现轻度甲状腺功能减退时尽快添加甲状腺素。继续使用PTU和甲状腺素治疗,直到TSI水平不再升高。TSI水平恢复正常很久之后,AMA和ATGA水平仍持续升高。所有三名患者最终都出现了甲状腺功能减退,并且继续需要甲状腺素替代治疗。

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