Immunological Profile and Predisposition to Autoimmunity in Girls With Turner Syndrome.
Immunological Profile and Predisposition to Autoimmunity in Girls With Turner Syndrome.
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特纳综合征女孩的自身免疫性的免疫学特征和自身免疫性的倾向。
DOI：
10.3389/fendo.2018.00307
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发表时间：
2018
影响因子：
5.2
通讯作者：
Malecka-Tendera E
中科院分区：
文献类型：
--
作者：
Gawlik AM;Berdej-Szczot E;Blat D;Klekotka R;Gawlik T;Blaszczyk E;Hankus M;Malecka-Tendera E
特纳综合征（TS）患者患自身免疫性疾病（AD）的风险是一般女性人群的两倍，是男性人群的四倍。TS中AD发病率增加的原因仍在讨论中。我们假设存在特定的体液、细胞和调节性T细胞（Treg）免疫特征，其易患AD、免疫紊乱和免疫调节紊乱。该研究包括37名患有TS的女孩，没有感染迹象。对照组包括11名没有激素紊乱的健康女孩。从所有参与者中收集了AD和免疫系统疾病的病史。水平：免疫球蛋白IgG、伊加、IgM、总淋巴细胞、淋巴细胞亚群CD 3+、CD 4+、CD 8+、CD 19+、自然杀伤细胞、Treg细胞（CD 4 + CD 25 + CD 127 − FOXP 3+），抗炎细胞因子（白细胞介素-10、转化生长因子-β）、抗核抗体、谷氨酸脱羧酶在每个参与者中测定GAD 65抗体、抗甲状腺过氧化物酶抗体和抗甲状腺球蛋白抗体。TS组和对照组的平均年龄和BMI相当（11.9 ± 4.1 vs. 12.5 ± 4.0岁; 19.2 ± 3.4 vs. 19.7 ± 4.6，p > 0.05）。对照组的平均hSDS显著更高（−2.2 ± 0.9 vs. −0.4 ± 1.5，p < 0.0001）。AD和复发性中耳炎合并并发症之前在9例（24.3%）和10例（27.0%）TS女孩中得到证实。TS组的IgG水平显著低于对照组（p = 0.02），%CD 4显著低于对照组（p < 0.001），CD 4：CD 8比值显著低于对照组（p < 0.001）。TS女孩和健康对照组之间的平均Treg%无差异。然而，将具有共存自身免疫的TS组与其余参与者之间的Treg%进行比较，观察到统计学显著差异（2.09 ± 0.5 vs. 2.77 ± 1.6，p = 0.048）。与其他患有TS的女孩和对照组相比，iXq患者的CD4%较低，抗TPO Ab和抗TG Ab阳性的频率更高（p = 0.001，p < 0.001，p = 0.01）。TS易患AD，特别是如果与共存的iXq相关。我们的初步研究结果表明，TS患者可能会提出一个特定的配置文件的体液和细胞免疫标志物，不同于健康的女孩。
The risk of autoimmune diseases (AD) in patients with Turner Syndrome (TS) is twice higher than in the general female population and four times higher than in the male population. The causes of the increased incidence of AD in TS are still under discussion. We hypothesized the presence of a specific humoral, cellular, and regulatory T cell (Treg) immunity profile which predisposes to AD, disorders of immunity, and disorders of immune regulation. The study encompassed 37 girls with TS and with no signs of infection. The control group included 11 healthy girls with no hormonal disorders. A medical history focused on AD and immunity disorders was taken from all participants. The levels of: immunoglobulins IgG, IgA, IgM, total lymphocytes, lymphocytes subpopulations CD3+, CD4+, CD8+, CD19+, natural killer cells, Treg cells (CD4+ CD25+ CD127− FOXP3+), anti-inflammatory cytokines (interleukin-10, transforming growth factor-β), anti-nuclear antibodies, glutamic acid decarboxylase (GAD65 Abs), anti-thyroid peroxidase (anti-TPO Ab), and anti-thyroglobulin (anti-TG Ab) autoantibodies were determined in each participant. The mean age and BMI in the TS group and in controls were comparable (11.9 ± 4.1 vs. 12.5 ± 4.0 years; 19.2 ± 3.4 vs. 19.7 ± 4.6, p > 0.05). Mean hSDS was significantly higher in controls (−2.2 ± 0.9 vs. −0.4 ± 1.5, p < 0.0001). AD and recurrent otitis media with complications were previously confirmed in 9 (24.3%) and 10 (27.0%) girls with TS. The TS group had significantly lower levels of IgG (p = 0.02), lower%CD4 (p < 0.001) and a significantly lower CD4:CD8 ratio than the controls (p < 0.001). There were no differences in mean Treg% between girls with TS and healthy controls. However, comparing Treg% between the TS group with coexisting autoimmunity and the remaining participants, a statistically significant difference was observed (2.09 ± 0.5 vs. 2.77 ± 1.6, p = 0.048). Patients with iXq had lower CD4% and more frequently had positive anti-TPO Ab and anti-TG Ab compared to the remaining girls with TS and controls (p = 0.001, p < 0.001, p = 0.01). TS predisposes to AD, especially if associated with coexisting iXq. Our preliminary findings show that patients with TS may present a specific profile of humoral and cellular immunity markers, different from healthy girls.
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影响因子：
3.2
作者：
McCarthy, Kateri;Bondy, Carolyn A
通讯作者：
Bondy, Carolyn A
影响因子：
12.8
作者：
Bakalov VK;Gutin L;Cheng CM;Zhou J;Sheth P;Shah K;Arepalli S;Vanderhoof V;Nelson LM;Bondy CA
通讯作者：
Bondy CA
DOI：
10.1111/j.1651-2227.1991.tb11733.x
发表时间：
1991-01-01
期刊：
ACTA PAEDIATRICA SCANDINAVICA
影响因子：
--
作者：
SPADONI, GL;ROSSI, P;BOSCHERINI, B
通讯作者：
BOSCHERINI, B
影响因子：
5.8
作者：
El-Mansoury, M;Bryman, I;Landin-Wilhelmsen, K
通讯作者：
Landin-Wilhelmsen, K
影响因子：
3.6
作者：
Aversa T;Lombardo F;Valenzise M;Messina MF;Sferlazzas C;Salzano G;De Luca F;Wasniewska M
通讯作者：
Wasniewska M