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Severe atopic dermatitis and transient hypogammaglobulinemia in children.

机译:小儿特应性皮炎严重和短暂性低球蛋白血症。

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We sought to describe the clinical outcomes of eight pediatric patients diagnosed with atopic dermatitis (AD) and hypogammaglobulinemia through retrospective review of medical records. All patients presented with severe facial AD. The mean and median ages of diagnosis of hypogammaglobulinemia were 6.2 months and 6.5 months, respectively, with a mean immunoglobulin G (IgG) level of 156 mg/dL. Seven of the eight patients identified in our search demonstrated simultaneous improvement in AD and serum IgG levels within 2 years of initial presentation, suggesting a diagnosis of transient hypogammaglobulinemia. The remaining patient demonstrated normalization by age 6, but no IgG levels had been measured between initial presentation and age 6. The five patients who were tested for specific antibody response to tetanus and Haemophilus influenzae type b vaccination all produced protective responses. All eight patients initially presented with high serum IgE levels. On initial evaluation, three patients had leukocytosis (white blood cell count >18,000 cells/μL), and six had peripheral blood eosinophilia. Three patients outgrew their AD by age 5, and five had clinically good to excellent control of their AD at their last visit, coincident with normalization of IgG levels. Although severe AD and immunoglobulin deficiency may rarely be associated with complex immunodeficiency disorders, our observations suggest that, with careful immunologic monitoring and diligent skin care, most children who present with severe AD and hypogammaglobulinemia exhibit improvement in dermatitis and serum IgG levels within 2 years of onset without major complications.
机译:我们试图通过回顾性医疗记录来描述八名被诊断为特应性皮炎(AD)和低球蛋白球蛋白血症的儿科患者的临床结局。所有患者均出现严重的面部AD。诊断为低血球蛋白血症的平均年龄和中位年龄分别为6.2个月和6.5个月,平均免疫球蛋白G(IgG)水平为156 mg / dL。在我们的研究中确定的八名患者中,有七名在首次就诊后的两年内表现出AD和血清IgG水平同时改善,提示诊断为短暂性低血球蛋白血症。其余患者在6岁时表现出正常化,但在初次就诊至6岁之间未测量到IgG水平。测试了对破伤风和b型流感嗜血杆菌疫苗的特异性抗体反应的五名患者均产生了保护性反应。所有八名患者最初都表现出较高的血清IgE水平。初步评估时,三名患者有白细胞增多症(白细胞计数> 18,000个细胞/μL),六名患者外周血嗜酸性粒细胞增多。五岁时三名患者的AD消失,五名患者在最后一次就诊时对AD的临床控制良好,与IgG水平正常相符。尽管严重的AD和免疫球蛋白缺乏症可能很少与复杂的免疫缺陷疾病有关,但我们的观察结果表明,经过仔细的免疫学监测和勤奋的皮肤护理,大多数患有严重AD和低球蛋白血症的儿童在2年内表现出皮炎和血清IgG水平的改善。起病无重大并发症。

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