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首页> 外文期刊>Archives of pathology & laboratory medicine >Factor XIII: Congenital deficiency factor XIII, acquired deficiency, factor XIII A-subunit, and factor XIII B-subunit
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Factor XIII: Congenital deficiency factor XIII, acquired deficiency, factor XIII A-subunit, and factor XIII B-subunit

机译：因子XIII：先天性缺陷因子XIII，获得性缺陷，因子XIII A亚基和因子XIII B亚基

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摘要

Factor XIII (FXIII) is a transglutaminase consisting of 2 catalytic A subunits and 2 noncatalytic B subunits in plasma. The noncatalytic B subunits protect the catalytic A subunits from clearance. Congenital FXIII deficiency may manifest as a lifelong bleeding tendency, abnormal wound healing, and recurrent miscarriage. Acquired FXIII deficiency, with significant reductions in FXIII levels, has been reported in several medical conditions. The routine screening tests for coagulopathies-prothrombin time, activated partial thromboplastin time, and thrombin time-do not show abnormalities in cases of FXIII deficiency. A quantitative, functional, FXIII activity assay that detects all forms of FXIII deficiency should be used as a first-line screening test. Treatment consists of recombinant FXIII or FXIII concentrate. If these are unavailable, then fresh-frozen plasma and cryoprecipitates may be used. Factor XIII has a long half-life; therefore, the patients can lead near-normal lives with regular replacements. Patients with acquired FXIII deficiency with inhibitors need immunosuppressive therapy in addition to factor replacements.
机译：因子XIII（FXIII）是一种转谷氨酰胺酶，由血浆中的2个催化性A亚基和2个非催化性B亚基组成。非催化性B亚基保护催化性A亚基免于清除。先天性FXIII缺乏症可能表现为终生出血趋势，伤口愈合异常和反复流产。据报道，在几种医疗条件下，获得性FXIII缺乏症，其FXIII水平明显降低。在FXIII缺乏的情况下，常规的凝血病-凝血酶原时间，活化的部分凝血活酶时间和凝血酶时间的筛查测试未显示异常。可以将检测所有形式的FXIII缺乏症的定量，功能性FXIII活性测定作为一线筛查测试。处理由重组FXIII或FXIII浓缩物组成。如果这些不可用，则可以使用新鲜冷冻的血浆和冷沉淀物。第十三因子的半衰期很长;因此，患者可以定期更换以过着接近正常的生活。获得性FXIII缺乏症且伴有抑制剂的患者除因子替代外还需要免疫抑制治疗。

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