切换至 "中华医学电子期刊资源库"

中华产科急救电子杂志 ›› 2015, Vol. 04 ›› Issue (01) : 38 -43. doi: 10.3877/cma.j.issn.2095-3259.2015.01.009

所属专题: 文献

专家论坛

妊娠合并血友病
陈焱1, 程蔚蔚1,()   
  1. 1. 200030 上海交通大学医学院附属国际和平妇幼保健院产科
  • 收稿日期:2014-09-10 出版日期:2015-02-18
  • 通信作者: 程蔚蔚

Pregnancy complicated with hemophilia

Yan Chen1, Weiwei Cheng1()   

  • Received:2014-09-10 Published:2015-02-18
  • Corresponding author: Weiwei Cheng
引用本文:

陈焱, 程蔚蔚. 妊娠合并血友病[J]. 中华产科急救电子杂志, 2015, 04(01): 38-43.

Yan Chen, Weiwei Cheng. Pregnancy complicated with hemophilia[J]. Chinese Journal of Obstetric Emergency(Electronic Edition), 2015, 04(01): 38-43.

血友病是一种凝血功能障碍的X染色体隐性遗传性疾病,主要由于凝血因子Ⅷ或Ⅸ缺乏所致。妊娠合并血友病患者在整个孕期和分娩过程中均面临着出血的风险。本文就妊娠合并血友病的产前诊断、孕期管理、分娩方式、治疗及新生儿处理等作一介绍。

Hemophilia is a recessive X-chromosome genetic diseases with coagulation disorders, due to clotting factor Ⅷ or Ⅸ deficiency. Pregnant women with hemophilia could be at risk of bleeding throughout gestation and during delivery. This article introduced the prenatal diagnosis, obstetrical management, delivery mode, therapy and newborn treatment of hemophilia.

表1 血友病A/B的临床分型[2]
表2 血友病A的鉴别诊断实验[2]
图1 APTT纠正试验
[1]
Lyon MF. Sex chromatin and gene action in the mammalian X-chromosome[J]. Am J Hum Genet, 1962, 14:135-148.
[2]
丁秋兰,王学锋,王鸿利,等. 血友病诊断和治疗的专家共识[J]. 临床血液学杂志,2010, 23(1):49-53.
[3]
Ljung RC. Prenatal diagnosis of haemophilia[J]. Haemophilia, 1999, 5(2):84-87.
[4]
Kadir RA, Economides DL, Braithwaite J, et al. The obstetric experience of carriers of haemophilia[J]. Br J Obstet Gynaecol, 1997, 104(7):803-810.
[5]
Ludlam CA, Pasi KJ, Bolton-Maggs P, et al. A framework for genetic service provision for haemophilia and other inherited bleeding disorders[J]. Haemophilia, 2005, 11(2):145-163.
[6]
Kadir RA. Women and inherited bleeding disorders: pregnancy and delivery[J]. Semin Hematol, 1999, 36(3 Suppl 4):28-35.
[7]
Lavery S. Preimplantation genetic diagnosis: new reproductive options for carriers of haemophilia[J]. Haemophilia, 2004, 10(Suppl 4):126-132.
[8]
Giangrande PL. Management of pregnancy in carriers of haemophilia[J]. Haemophilia, 1998, 4(6):779-784.
[9]
Kadir RA, Economides DL, Braithwaite J, et al. The obstetric experience of carriers of haemophilia[J]. Br J Obstet Gynaecol, 1997, 104(7):803-810.
[10]
Yang MY, Ragni MV. Clinical manifestations and management of labor and delivery in women with factor IX deficiency[J]. Haemophilia, 2004, 10(5):483-490.
[11]
Ljung R, Lindgren AC, Petrini P, et al. Normal vaginal delivery is to be recommended for haemophilia carrier gravidae[J]. Acta Paediatr, 1994, 83(6):609-611.
[12]
Kulkarni R, Lusher JM. Intracranial and extracranial hemorrhages in newborns with hemophilia: a review of the literature[J]. J Pediatr Hematol Oncol, 1999, 21(4):289-295.
[13]
MacLean PE, Fijnvandraat K, Beijlevelt M, et al. The impact of unaware carriership on the clinical presentation of haemophilia[J]. Haemophilia, 2004, 10(5):560-564.
[14]
Kulkarni R, Lusher JM. Intracranial and extracranial hemorrhages in newborns with hemophilia: a review of the literature[J]. J Pediatr Hematol Oncol, 1999, 21(4):289-295.
[15]
Chalmers EA. Neonatal coagulation problems[J]. Arch Dis Child Fetal Neonatal Ed, 2004, 89(6):F475-F478.
[16]
Kulkarni R, Lusher J. Perinatal management of newborns with haemophilia[J]. Br J Haematol, 2001, 112(2):264-274.
[17]
Kulkarni R, Lusher JM, Henry RC, et al. Current practices regarding newborn intracranial haemorrhage and obstetrical care and mode of delivery of pregnant haemophilia carriers: a survey of obstetricians, neonatologists and haematologists in the United States, on behalf of the National Hemophilia Foundation's Medical and Scientific Advisory Council[J]. Haemophilia, 1999, 5(6):410-415.
[18]
Klinge J, Auberger K, Auerswald G, et al. Prevalence and outcome of intracranial hemorrhage in hemophiliacs-a survey of the paediatric group of the German Society for Thrombosis and Haemostasis (GTH)[J]. Eur J Paediatr, 1999, 158 (Suppl 3):S162-S165.
[19]
Buchanan GR. Factor concentrate prophylaxis for neonates with hemophilia[J]. J Pediatr Hematol Oncol, 1999, 21(4):254-256.
[20]
Rodriguez V, Schmidt KA, Slaby JA, et al. Intracranial haemorrhage as initial presentation of severe hemophilia B: case report and review of Mayo Clinic Comprehensive Hemophilia Center experience[J]. Haemophilia, 2005, 11(1):73-77.
[21]
Lorenzo JI, López A, Altisent C, et al. Incidence of factor Ⅷ inhibitors in severe haemophilia: the importance of patient age[J]. Br J Haematol, 2001, 113(3):600-603.
[22]
van der Bom JG, Mauser-Bunschoten EP, Fischer K, et al. Age at first treatment and immune tolerance to factor Ⅷ in severe hemophilia[J]. Thromb Haemost, 2003, 89(3):475-479.
[23]
Rivard GE, Lillicrap D, Poon MC, et al. Can activated recombinant factor Ⅶ be used to postpone the exposure of infants to factor Ⅷ until after 2 years of age?[J]. Haemophilia, 2005, 11(4):335-359.
[24]
Gilchrist GS, Wilke JL, Muehlenbein LR, et al. Intrauterine correction of factor Ⅷ (FⅧ) deficiency[J]. Haemophilia, 2001, 7(5):497-499.
[25]
Madoiwa S, Yamauchi T, Hakamata Y, et al. Induction of immune tolerance by neonatal intravenous injection of human factor Ⅷ in murine hemophilia A[J]. J Thromb Haemost, 2004, 2(5):754-762.
[26]
Russell KE, Olsen EH, Raymer RA, et al. Reduced bleeding events with subcutaneous administration of recombinant human factor IX in immune-tolerant hemophilia B dogs[J]. Blood, 2003, 102(13):4393-4398.
[1] 孙长鲛, 许平平, 谢兰兰, 李慧敏, 杨凯, 蔡谞, 陈连旭. 合并血友病患者全膝关节置换术的手术挑战[J]. 中华关节外科杂志(电子版), 2022, 16(06): 771-775.
[2] 尚宏喜, 贺敬龙, 郝敏, 丁强, 孙炜, 刘安庆, 谢伟. 全膝关节置换术在重度血友病性关节炎的疗效分析[J]. 中华关节外科杂志(电子版), 2021, 15(06): 753-757.
[3] 郎虓, 程蔚蔚. 妊娠期铁缺乏与贫血[J]. 中华产科急救电子杂志, 2022, 11(04): 202-207.
[4] 黎静, 钟梅. 妊娠合并重度特发性血小板减少性紫癜的诊治[J]. 中华产科急救电子杂志, 2019, 08(02): 72-75.
[5] 周琼, 曾万江. 妊娠期溶血的管理[J]. 中华产科急救电子杂志, 2018, 07(02): 108-112.
[6] 黄婷婷, 刘淮. 妊娠合并溶血性贫血的诊断及治疗[J]. 中华产科急救电子杂志, 2016, 05(02): 97-101.
[7] 熊中堂, 江庆萍, 刘少颜, 彭娟. 11例妊娠合并白血病患者的临床分析[J]. 中华产科急救电子杂志, 2015, 04(04): 238-242.
[8] 甘旭培, 徐先明. 妊娠合并再生障碍性贫血[J]. 中华产科急救电子杂志, 2015, 04(01): 32-37.
[9] 周小芳, 樊尚荣. 妊娠合并缺铁性贫血[J]. 中华产科急救电子杂志, 2015, 04(01): 23-27.
[10] 盛晴, 赵扬玉. 妊娠相关性血小板减少症[J]. 中华产科急救电子杂志, 2015, 04(01): 15-18.
[11] 唐加明. 妊娠合并血栓性血小板减少性紫癜[J]. 中华产科急救电子杂志, 2015, 04(01): 10-14.
[12] 张晓红, 王大鹏. 妊娠合并白血病[J]. 中华产科急救电子杂志, 2015, 04(01): 5-9.
[13] 罗纯苑, 刘杭鑫, 李玥, 麦艳梅, 石凌波. 一例产后获得性血友病A报告及总结分析[J]. 中华临床实验室管理电子杂志, 2023, 11(02): 114-117,123.
阅读次数
全文


摘要