Peculiarities of Graves' disease in children and adolescents with Down's syndrome
Peculiarities of Graves' disease in children and adolescents with Down's syndrome
复制标题
DOI：
10.1530/eje-09-0751
复制
发表时间：
2010-03-01
影响因子：
5.8
通讯作者：
Arrigo, Teresa
中科院分区：
文献类型：
--
作者：
De Luca, Filippo;Corrias, Andrea;Arrigo, Teresa
目的：比较两组儿童Graves病（GD）的临床表现和病程，两组儿童分别包括28例唐氏综合征（DS）患者和109例非DS对照者。根据整个随访期间的临床变化以及TSH、游离甲状腺素和TSH受体自身抗体血清水平的变化，确定两组中GD随时间的演变。DS组女性患病率（50 vs 81.6%; chi（2）= 12.0，P < 0.0005）和GD表现时的平均年龄（9.9 ± 4.4 vs 11.5 ± 3.5岁，P < 0.05）显著低于对照组。DS患者对他巴唑治疗的临床反应性明显更好，第一个周期停药后复发率较低（7.1 vs 31.2%; chi（2）= 7.4，P < 0.005），确定性停药后持续缓解率较高（46.4 vs 26.7%; chi（2）= 4.1，P < 0.05）。DS组无1例需要手术或放射性碘消融治疗，而对照组有11%的患者需要非药物治疗（χ 2 = 3.8，P < 0.05）。21.4%的DS患者和3.7%的对照者有桥本甲状腺炎（HT）的病史（χ 2 = 10.4，P < 0.005）。在DS组和对照组中，GD与其他自身免疫性疾病的相关性分别为32.1%和12.8%（χ 2 = 5.94，P < 0.025）。结论：DS儿童和青少年GD具有以下特点：（1）发病较早;（2）无性别优势;（3）临床病程较轻;（4）HT前体发生率较高; v）更频繁地与其他自身免疫性疾病相关联。
Objective: To compare the presentation and clinical course of Graves' disease (GD) in two pediatric populations consisting of 28 patients with Down's syndrome (DS) and 109 controls without DS respectively.Design and methods: The evolution over time of GD was determined in both groups according to the clinical changes and the variations in TSH, free thyroxine, and TSH receptor autoantibodies serum levels during the entire follow-up.Results: Female prevalence (50 vs 81.6%; chi(2) = 12.0, P < 0.0005) and average age at GD presentation (9.9 +/- 4.4 vs 11.5 +/- 3.5 years, P < 0.05) were significantly lower in DS group than in controls. Clinical responsiveness to methimazole therapy was significantly better in DS patients, as demonstrated by both the lower relapse rates after the first cycle withdrawal (7.1 vs 31.2%; chi(2) = 7.4, P < 0.005) and the higher persistent remission rates after definitive therapy withdrawal (46.4 vs 26.7%; chi(2) = 4.1, P < 0.05). Moreover, in DS group, no patients needed surgery or radioiodine ablation, whereas non-pharmacological treatment was necessary in 11% of controls (chi(2) = 3.8, P < 0.05). Antecedents of Hashimoto's thyroiditis (HT) were documented in 21.4% of DS patients and in 3.7% of controls (chi(2) = 10.4, P < 0.005). Association with other autoimmune diseases was detected in 32.1% of DS cases and in 12.8% of controls (chi(2) = 5.94, P < 0.025).Conclusions: GD in DS children and adolescents is characterized by several peculiarities: i) earlier presentation; ii) no gender predominance; iii) less severe clinical course; iv) higher frequency of documented HT antecedents; v) more frequent association with other autoimmune diseases.