Effect Of Cytokines In Host Defense And Inflammation

1)水疱方案被用于评估几个罕见免疫缺陷患者群体的体内炎症，包括STAT 3缺陷、CGD等。 我们继续招募CGD受试者（目前n=4），以检查该患者人群体内炎症的发生和进展。 2)干扰素-β轴是FDA批准的CGD疗法，但它在一些患者中引起显著的流感样症状，并且轶事报告表明它可能不会使所有患者受益。 在2014财年，我们完成了对2名受试者的多个样本的微阵列评价，这些受试者完成了我们的NIH方案#10-I-0123慢性肉芽肿病中特定基因突变受试者对IFN-γ的生化反应评估。 患者在研究持续时间内接受几次剂量，并在每次剂量和洗脱期测定mRNA和蛋白质表达。 表达分析确定了一个治疗相关的签名，将用于制定一个新的临床协议在下一个财政年度。

1) The blister protocol is being used to evaluate inflammation in vivo in several populations of patients with rare immunodeficiencies including STAT3 deficiency, CGD, and others. We continue to accrue subjects with CGD (currently n=4) in order to examine the development and progression of inflammation in vivo in this patient population. 2) Interferon-γ prophyaxis is an FDA-approved therapy for CGD however it causes significant flu-like symptoms in some patients and anecdotal reports suggest that it might not benefit all patients. During FY14, we completed microarray evaluation of multiple samples from 2 subjects who completed our NIH Protocol #10-I-0123 Assessment of the Biochemical Response to IFN-gamma in Subjects with Specific Gene Mutations in Chronic Granulomatous Disease. Patients received several doses over the study duration and mRNA and protein expression was determined at each dose and during washout phases. Expression analysis identified a treatment-related signature that will be used to develop a new clinical protocol over the next FY.