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首页> 外文期刊>Archives of disease in childhood >Antibody deficiency and autoimmunity in 22q11.2 deletion syndrome.
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Antibody deficiency and autoimmunity in 22q11.2 deletion syndrome.

机译：22q11.2缺失综合征的抗体缺乏和自身免疫。

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BACKGROUND: Although severe T cell immunodeficiency in DiGeorge anomaly is rare, previous studies of humoral function in these patients have found no antibody abnormalities but have not examined the response to polysaccharide antigens. Isolated cases of autoimmunity have been reported. Several patients with 22q11.2 deletion attending our immunology clinic suffered recurrent sinopulmonary infection or autoimmune phenomena. AIMS: To investigate humoral immunodeficiency, particularly pneumococcal polysaccharide antibody deficiency, and autoimmune phenomena in a cohort of patients with 22q11.2 deletion. METHODS: A history of severe or recurrent infection and autoimmune symptoms were noted. Lymphocyte subsets, immunoglobulins, IgG subclasses, specific vaccine antibodies, and autoantibodies were measured. Subjects were vaccinated with appropriate antigens as indicated. RESULTS: Of 32 patients identified, 26 (81%) had severe or recurrent infection, of which 13 (50%) had abnormal serum immunoglobulin measurements and 11/20 >/=4 years old (55%) had an abnormal response to pneumococcal polysaccharide. Ten of 30 patients (33%) had autoimmune phenomena; six (20%) were symptomatic. CONCLUSIONS: Humoral immunodeficiency is more common than previously recognised in patients with 22q11.2 deletion. Normal T cell function and immunoglobulin levels do not exclude poor specific antibody responses. Patients should be referred for formal immunological assessment of cellular and humoral immune function.
机译：背景：尽管在DiGeorge异常中严重的T细胞免疫缺陷很少见，但这些患者的体液功能先前的研究未发现抗体异常，但未检查对多糖抗原的反应。已有独立的自身免疫病例报道。前往我们免疫学诊所就诊的22q11.2缺失的几例患者反复出现肺肺感染或自身免疫现象。目的：研究一组22q11.2缺失患者的体液免疫缺陷，特别是肺炎球菌多糖抗体缺乏和自身免疫现象。方法：记录有严重或反复感染的病史和自身免疫症状。测量了淋巴细胞亚群，免疫球蛋白，IgG亚类，特异性疫苗抗体和自身抗体。如图所示，用适当的抗原给受试者接种疫苗。结果：在确定的32例患者中，有26例（81％）患有严重或反复感染，其中13例（50％）血清免疫球蛋白检测异常，11/20> / = 4岁（55％）对肺炎球菌有异常反应多糖。 30名患者中有10名（33％）患有自身免疫现象;有六个（20％）有症状。结论：在22q11.2缺失患者中，体液免疫缺陷比以前公认的更为普遍。正常的T细胞功能和免疫球蛋白水平不能排除较差的特异性抗体反应。应转诊患者进行细胞和体液免疫功能的正式免疫学评估。

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