The Immune Basis For The Gastrointestinal Complications

这是一项影响CVID患者亚群的胃肠道疾病的自然史研究。有体重减轻或腹泻记录的受试者将与无胃肠道症状的CVID患者进行比较；受试者将接受上、下内窥镜检查以评估组织学并获得用于LPMC纯化的标本；对LPMC和PBMC进行免疫分型和细胞因子分泌检测；将测量肠道吸收；脾脏大小将被确定。我们计划招募多达20名受试者。目的是了解T细胞异常导致特发性炎症性肠病，可以折磨这些患者。细胞因子异常的特定模式应该建议在这种情况下进行特定的治疗。

This is a natural history study of the gastrointestinal disease that affects a subset of CVID patients. Subjects with documented weight loss or diarrhea will be compared with CVID patients without gastrointestinal symptoms; subjects will have upper and lower endoscopy to evaluate histology and obtain specimens for LPMC purification; LPMC and PBMC will be immunophenotyped and tested for cytokine secretion; intestinal absorption will be measured; spleen size will be determined. We plan to recruit up to 20 subjects. The goal is to understand the T cell abnormality contributing to the idiopathic inflammatory bowel disease that can afflict these patients. Specific patterns of cytokine abnormalities should suggest specific therapies to test in this condition.