Acquired activated protein C resistance is associated with lupus anticoagulants and thrombotic events in pediatric patients with systemic lupus erythematosus.
Acquired activated protein C resistance is associated with lupus anticoagulants and thrombotic events in pediatric patients with systemic lupus erythematosus.
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获得性活化蛋白 C 抵抗与系统性红斑狼疮儿科患者的狼疮抗凝剂和血栓事件相关。
DOI：
10.1182/blood.v97.4.844
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发表时间：
2001
期刊：
影响因子：
20.3
通讯作者：
Maureen E. Andrew
中科院分区：
文献类型：
--
作者：
C. Male;L. Mitchell;James Julian;P. Vegh;Penny Joshua;M. Adams;M. David;Maureen E. Andrew
获得性活化蛋白C抵抗（APCR）已被假设为抗磷脂抗体（APLA）引起血栓事件（TE）的可能机制。然而，获得性APCR与APLA相关的现有证据有限。更重要的是，获得性APCR与TE的关联尚未得到证实。本研究的目的是确定在系统性红斑狼疮（SLE）儿科患者中，（1）获得性APCR是否与APLA的存在相关，（2）APCR是否与TE相关，以及（3）APCR和APLA之间是否存在相互作用与TE相关。对59名连续的、非选择的SLE儿童进行了一项横断面队列研究。主要临床结局为经客观影像学检查证实的症状性TE。实验室检查包括狼疮抗凝剂（LA）、抗心磷脂抗体（ACLA）、APC比率、蛋白S、蛋白C和凝血因子V Leiden。结果显示，59例患者中有10例（17%）发生TE。58例患者中有18例（31%）存在获得性APCR。获得性APCR与LA的存在显著相关，但与ACLA无关。获得性APCR也与TE显著相关。APCR和LAs在与TEs的关联中存在显著的交互作用。同时存在APCR和LA与TE的最高风险相关。蛋白S和蛋白C浓度与APLA、APCR或TE的存在无关。获得性APCR的存在是鉴定TE高风险的LA阳性患者的标志物。获得性APCR可能反映了LA对蛋白C途径的干扰，这可能代表了LA相关TE的机制。（血。2001;97：844-849）
Acquired activated protein C resistance (APCR) has been hypothesized as a possible mechanism by which antiphospholipid antibodies (APLAs) cause thrombotic events (TEs). However, available evidence for an association of acquired APCR with APLAs is limited. More importantly, an association of acquired APCR with TEs has not been demonstrated. The objective of the study was to determine, in pediatric patients with systemic lupus erythematosus (SLE), whether (1) acquired APCR is associated with the presence of APLAs, (2) APCR is associated with TEs, and (3) there is an interaction between APCR and APLAs in association with TEs. A cross-sectional cohort study of 59 consecutive, nonselected children with SLE was conducted. Primary clinical outcomes were symptomatic TEs, confirmed by objective radiographic tests. Laboratory testing included lupus anticoagulants (LAs), anticardiolipin antibodies (ACLAs), APC ratio, protein S, protein C, and factor V Leiden. The results revealed that TEs occurred in 10 (17%) of 59 patients. Acquired APCR was present in 18 (31%) of 58 patients. Acquired APCR was significantly associated with the presence of LAs but not ACLAs. Acquired APCR was also significantly associated with TEs. There was significant interaction between APCR and LAs in the association with TEs. Presence of both APCR and LAs was associated with the highest risk of a TE. Protein S and protein C concentrations were not associated with the presence of APLAs, APCR, or TEs. Presence of acquired APCR is a marker identifying LA-positive patients at high risk of TEs. Acquired APCR may reflect interference of LAs with the protein C pathway that may represent a mechanism of LA-associated TEs. (Blood. 2001;97:844-849)
与活化蛋白 C 抑制剂相关的广泛静脉和动脉血栓形成。
影响因子：
20.3
作者：
Zivelin,A;Gitel,S;Griffin,JH;Xu,X;Fernandez,JA;Martinowitz,U;Cohen,Y;Halkin,H;Seligsohn,U;Inbal,A
通讯作者：
Inbal,A
与磷脂抗体的存在相关的血栓素质可能是由于游离蛋白 S 水平低所致。
DOI：
10.1016/0002-9343(92)90679-6
发表时间：
1992
期刊：
The American journal of medicine
影响因子：
--
作者：
Parke,AL;Weinstein,RE;Bona,RD;Maier,DB;Walker,FJ
通讯作者：
Walker,FJ
缺血性中风中激活的蛋白 C 抵抗并非由于因子 V 精氨酸 506--> 谷氨酰胺突变。
影响因子：
8.3
作者：
Fisher,M;Fernandez,JA;Ameriso,SF;Xie,D;Gruber,A;Paganini-Hill,A;Griffin,JH
通讯作者：
Griffin,JH