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家族性冷球蛋白血症与C4缺乏症。

Familial cryoglobulinemia and C4 deficiency.

作者信息

Berliner S, Weinberger A, Zamir R, Hazaz B, Pinkhas J

出版信息

Scand J Rheumatol. 1984;13(2):151-4. doi: 10.3109/03009748409100379.

Abstract

The coexistence of cryoglobulinemia and C4 deficiency was found in one family. Twelve subjects were examined, most of them being asymptomatic. Cryoglobulins were found in 9 members and its type was defined in 6 of them, 3 having mixed IgM-IgG and 3 having monoclonal IgM cryoglobulins. The concentration of the third component of the complement system was normal in 10 subjects, while the concentration of the fourth one was found to be below the lower limit of normal in 9 subjects. Only C4 deficiency--and not cryoglobulinemia--was HLA-linked. There was no evidence of HBs antigenemia. The results of this study are discussed in the light of the pertinent literature.

摘要

在一个家族中发现了冷球蛋白血症与C4缺乏症并存的情况。对12名受试者进行了检查,他们中的大多数人没有症状。在9名成员中发现了冷球蛋白,其中6人的冷球蛋白类型得以确定,3人有混合性IgM-IgG冷球蛋白,3人有单克隆IgM冷球蛋白。补体系统第三成分的浓度在10名受试者中正常,而第四成分的浓度在9名受试者中低于正常下限。只有C4缺乏症(而非冷球蛋白血症)与HLA相关联。没有乙肝表面抗原血症的证据。结合相关文献对本研究结果进行了讨论。

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