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[人类白细胞抗原系统与迟发性皮肤卟啉症]

[The HLA system and porphyria cutanea tarda].

作者信息

Köstler E, Gebhardt B, Seebacher C

出版信息

Dtsch Z Verdau Stoffwechselkr. 1984;44(2):95-100.

PMID:6592073
Abstract

Familiar occurrence of porphyria cutanea tarda (P. C. T.) has led to search for genetic markers of this disease. One of the three contradictory studies in literature showed significant increased frequencies of HLA-A3 and -B7 in the P. C. T. These results are important for the hypothesis that a disorder of iron metabolism linked to the HLA system (HLA-A3) supports a manifestations of the disease. Our investigations don't support generally this hypothesis. In 90 P. C. T.-patients significantly higher frequencies of any HLA-A, -B and -C-locus were not to be found. But we found a statistic guaranteed higher frequency of HLA-A9 in the subgroup (n = 34) of alcoholic induced P. C. T. An association between HLA-A9 and liver diseases is reported somewhere else. The higher percentage of HLA-A3 incidence in this group was not significant. Significant higher frequency of HLA-A9 possibly refers to liver diseases by P. C. T. HLA-A3--association is imagineable with iron-over loaded disorder in this group.

摘要

迟发性皮肤卟啉症(PCT)的常见发生促使人们寻找该疾病的基因标记。文献中三项相互矛盾的研究之一表明,PCT患者中HLA - A3和 - B7的频率显著增加。这些结果对于铁代谢紊乱与HLA系统(HLA - A3)相关支持该疾病表现这一假说很重要。我们的研究总体上不支持这一假说。在90例PCT患者中,未发现任何HLA - A、 - B和 - C位点的频率显著更高。但我们发现在酒精性诱发的PCT亚组(n = 34)中,HLA - A9的频率有统计学意义的升高。在其他地方报道了HLA - A9与肝脏疾病之间的关联。该组中HLA - A3发生率较高的百分比无统计学意义。HLA - A9频率显著更高可能与PCT所致肝脏疾病有关。在该组中,HLA - A3与铁过载紊乱之间的关联是可以想象的。

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