Department of Pediatrics, University of Messina, 98124 Messina, Italy.
Eur J Endocrinol. 2010 Mar;162(3):591-5. doi: 10.1530/EJE-09-0751. Epub 2009 Dec 2.
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.
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.
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).
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.
比较由 28 例唐氏综合征（DS）患儿和 109 例无 DS 对照患儿组成的两组儿科人群中格雷夫斯病（GD）的表现和临床病程。
根据整个随访过程中 TSH、游离甲状腺素和 TSH 受体自身抗体血清水平的临床变化和变化，确定两组 GD 的时间演变。
DS 组女性患病率（50%对 81.6%；chi(2)=12.0，P<0.0005）和 GD 发病时的平均年龄（9.9+/-4.4 岁对 11.5+/-3.5 岁，P<0.05）明显低于对照组。DS 患者对甲巯咪唑治疗的临床反应明显更好，表现在第一次停药后复发率较低（7.1%对 31.2%；chi(2)=7.4，P<0.005）和停药后持续缓解率较高（46.4%对 26.7%；chi(2)=4.1，P<0.05）。此外，DS 组无患者需要手术或放射性碘消融，而对照组有 11%需要非药物治疗（chi(2)=3.8，P<0.05）。在 21.4%的 DS 患儿和 3.7%的对照组中发现了桥本甲状腺炎（HT）的病史（chi(2)=10.4，P<0.005）。在 32.1%的 DS 病例和 12.8%的对照组中发现了与其他自身免疫性疾病的关联（chi(2)=5.94，P<0.025）。
唐氏综合征儿童和青少年的 GD 具有以下特点：i）发病较早；ii）无性别优势；iii）临床病程较轻；iv）HT 病史发生率较高；v）与其他自身免疫性疾病的关联更频繁。
