因子 VIII 水平和出血情况与血友病 A 妊娠携带者的因子 8 (F8) 突变有关：一项多中心回顾性队列研究。
Factor VIII levels and bleeding according to factor 8 (F8) mutation in pregnant carriers of haemophilia A: a multicentre retrospective cohort study.
机构信息
Oxford Haemophilia and Thrombosis Centre, Churchill Hospital, Oxford, UK.
Oxford Haemophilia and Thrombosis Centre and Oxford NIHR BRC, Churchill Hospital, Oxford, UK.
出版信息
Br J Haematol. 2021 Apr;193(2):397-400. doi: 10.1111/bjh.17254. Epub 2021 Mar 14.
This retrospective cohort study investigated the association between factor 8 (F8) genotype severity and factor VIII (FVIII) levels during pregnancy for 52 women (64 pregnancies) who were heterozygous carriers of mild, moderate or severe haemophilia A. There were no significant differences in FVIII levels for carriers of mild, moderate or severe haemophilia A at baseline [mean (SD) level: mild, 0·78 (0·22); moderate, 0·83 (0·33); severe, 0·70 (0·25) iu/ml; P = 0·81] or in the third trimester [mean (SD) level: mild, 1·42 (0·28); moderate, 1·47 (0·41); severe, 1·37 (0·49) iu/ml; P = 0·80). Post-partum haemorrhage rates were higher for carriers of severe haemophilia A (13/24; 54·2%) compared to carriers of mild haemophilia A (four of 14; 28·6%).
这项回顾性队列研究调查了 52 名（64 次妊娠）携带轻度、中度或重度血友病 A 杂合子的女性在怀孕期间因子 8（F8）基因型严重程度与因子 VIII（FVIII）水平之间的关系。在基线时，轻度、中度和重度血友病 A 携带者的 FVIII 水平没有显著差异[平均（SD）水平：轻度，0.78（0.22）；中度，0.83（0.33）；重度，0.70（0.25）iu/ml；P=0.81]或在孕晚期[平均（SD）水平：轻度，1.42（0.28）；中度，1.47（0.41）；重度，1.37（0.49）iu/ml；P=0.80]。重度血友病 A 携带者（24 例中有 13 例；54.2%）产后出血发生率高于轻度血友病 A 携带者（14 例中有 4 例；28.6%）。
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