FAMILIAL OCCURRENCE OF COMPLEMENT DYSFUNCTION IN CROHNS-DISEASE - CORRELATION WITH INTESTINAL SYMPTOMS AND HYPERCATABOLISM OF COMPLEMENT

FAMILIAL OCCURRENCE OF COMPLEMENT DYSFUNCTION IN CROHNS-DISEASE - CORRELATION WITH INTESTINAL SYMPTOMS AND HYPERCATABOLISM OF COMPLEMENT
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DOI:
10.1136/gut.26.2.151
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发表时间:
1985-01-01
期刊:
GUT
影响因子:
24.5
通讯作者:
BINDER, V
BINDER, V
中科院分区:
医学1区
文献类型:
--
作者:
ELMGREEN, J;BOTH, H;BINDER, V

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研究了克罗恩病先证者和其一级亲属的补体。对照组包括24名健康志愿者和24名溃疡性结肠炎或消化性溃疡患者。在21名先证者中有8名和33名亲属中有6名显示出通过旁路途径产生的趋化活性低于正常水平,两组的频率与对照组有显著差异(P < 0.005),患者和亲属的发现之间有很强的联系。正如先前在克罗恩病患者中所显示的,低正常世代与亲属中补体C3利用率降低有关。循环补体C3c裂解产物水平升高提示克罗恩病先证者补体参与。所有亲属血浆C3c均正常,6例补体功能障碍患者均无胃肠道症状或炎症性肠病史。克罗恩病患者中观察到的补体异常并不仅仅反映了粘膜炎症或补体的过度分解代谢。
Complement was studied in Crohn''s disease probands with early onset and in their 1st degree relatives. Controls included 24 healthy volunteers and 24 patients with ulcerative colitis or peptic ulcers. Subnormal generation of chemotactic activity by the alternative pathway was shown in 8 of 21 probands and in 6 of 33 relatives, a frequency in both groups significantly different from controls (P < 0.005), with a strong connection between findings in patients and relatives. As previously shown in patients with Crohn''s disease, the subnormal generation was related to decreased utilization of complement C3 in relatives. Raised levels of circulating complement C3c split products suggested complement involvement in Crohn''s disease probands. Plasma C3c was normal in all relatives, and none of the 6 cases with complement dysfunction had gastrointestinal symptoms or a history of inflammatory bowel disease. Complement abnormality seen in Crohn''s disease patients does not simply reflect mucosal inflammation or hypercatabolism of complement.