先天性因子 XIII 缺乏症中的纤维蛋白交联
Fibrin cross-linking in congenital factor XIII deficiency.
作者信息
Rodeghiero F, Barbui T
出版信息
J Clin Pathol. 1980 May;33(5):434-7. doi: 10.1136/jcp.33.5.434.
Homozygous patients with factor XIII deficiency are devoid of immunologically identifiable A protein, the active enzymatic component. Quantitative studies of transamidase activity of the factor are available in only a few cases, and the fibrin cross-linking pattern is not well known. The present paper deals with the quantitative estimation of factor XIII transamidase activity (dansylcadaverine system), factor XIII molecular subunits, and the corresponding fibrin cross-linking pattern in seven homozygous patients with factor XIII deficiency. The results indicate that transamidase activity was present in all patients, and the range was 0.5-1.7%. The pattern of fibrin stabiisation showed an absence of cross-linking in two patients, the presence of gamma-gamma-dimers (traces) in four, and gamma-gamma-dimers plus incomplete alpha-polymers (traces) in one patient. In conclusion, the homozygous patients reported here were not completely devoid of functioning factor XIII.
患有因子 XIII 缺乏症的纯合子患者缺乏免疫学上可识别的 A 蛋白，即活性酶成分。仅在少数病例中对该因子的转酰胺酶活性进行了定量研究，并且纤维蛋白交联模式尚不清楚。本文探讨了 7 例患有因子 XIII 缺乏症的纯合子患者中因子 XIII 转酰胺酶活性（丹磺酰尸胺系统）、因子 XIII 分子亚基以及相应的纤维蛋白交联模式的定量评估。结果表明，所有患者均存在转酰胺酶活性，范围为 0.5 - 1.7%。纤维蛋白稳定模式显示，两名患者不存在交联，四名患者存在γ-γ-二聚体（微量），一名患者存在γ-γ-二聚体加不完全α-聚合物（微量）。总之，本文报道的纯合子患者并非完全缺乏有功能的因子 XIII。
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