The Immune Basis For The Gastrointestinal Complications

这是一项对影响部分CVID患者的胃肠道疾病的自然病史研究。有记录的体重减轻或腹泻的受试者将与没有胃肠道症状的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LPMC纯化标本；LPMC和PBMC将进行免疫表型和细胞因子分泌测试；将测量肠道吸收；将确定脾大小。我们计划招收多达20名受试者。目的是了解导致特发性炎症性肠病的T细胞异常，这种疾病可能会困扰这些患者。细胞因子异常的特定模式应建议在这种情况下测试特定的治疗方法。

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.