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Semin Thromb Hemost 2023; 49(04): 371-381
DOI: 10.1055/s-0042-1758117
Review Article

Planning Pregnancy and Birth in Women with Inherited Bleeding Disorders

A. Kinga Malinowski
1   Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynaecology, Lunenfeld-Tanenbaum Research Institute, Sinai Health System, Toronto, Canada
2   Department of Obstetrics and Gynaecology, University of Toronto, Toronto, Canada
,
Rezan Abdul-Kadir
3   Department of Obstetrics and Gynaecology and Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free NHS Foundation Hospital, London, United Kingdom
4   Institute for Women's Health, University College London, London, United Kingdom
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Abstract

Inherited bleeding disorders are characterized by a diverse clinical phenotype within and across specific diagnoses. von Willebrand disease (VWD), hemophilia A, and hemophilia B comprise 95 to 97% of inherited bleeding disorders, with the remaining 3 to 5% attributed to rare bleeding disorders, including congenital fibrinogen disorders, factor deficiencies (affecting FII, FV, FV + FVIII, FVII, FX, FXI, and FXIII), and platelet function defects. The pregnancy, birth, and the puerperium may be adversely influenced in the setting of an inherited bleeding disorder depending on its type and clinical phenotype. Obstetric hemostatic challenges may sometimes also unmask the presence of a previously unknown inherited bleeding disorder. This review aims to address the approach to pregnancy and birth in the context of an inherited bleeding disorder and highlights the significance of multidisciplinary input into the care of these women.

Keywords

bleeding disorders - pregnancy - delivery - postpartum hemorrhage


Publication History

Article published online:
11 November 2022

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  • References

  • 1 Presky KO, Kadir RA. Women with inherited bleeding disorders - challenges and strategies for improved care. Thromb Res 2020; 196: 569-578
    CrossrefPubMedGoogle Scholar
  • 2 World Federation of Hemophilia. WFH report on the annual global survey 2017. 2018 Accessed September 25. 2022 at: http://www1.wfh.org/publications/files/pdf-1714.pdf
    PubMedGoogle Scholar
  • 3 Peyvandi F, Garagiola I, Menegatti M. Gynecological and obstetrical manifestations of inherited bleeding disorders in women. J Thromb Haemost 2011; 9 (Suppl. 01) 236-245
    CrossrefPubMedGoogle Scholar
  • 4 Lavee O, Kidson-Gerber G. Update on inherited disorders of haemostasis and pregnancy. Obstet Med 2016; 9 (02) 64-72
    CrossrefPubMedGoogle Scholar
  • 5 O'Brien SH. Evaluation and management of heavy menstrual bleeding in adolescents: the role of the hematologist. Blood 2018; 132 (20) 2134-2142
    CrossrefPubMedGoogle Scholar
  • 6 Kadir RA, Economides DL, Sabin CA, Owens D, Lee CA. Frequency of inherited bleeding disorders in women with menorrhagia. Lancet 1998; 351 (9101): 485-489
    CrossrefPubMedGoogle Scholar
  • 7 Elbaz C, Sholzberg M. An illustrated review of bleeding assessment tools and common coagulation tests. Res Pract Thromb Haemost 2020; 4 (05) 761-773
    CrossrefPubMedGoogle Scholar
  • 8 Azzam HA, Goneim HR, El-Saddik AM, Azmy E, Hassan M, El-Sharawy S. The condensed MCMDM-1 VWD bleeding questionnaire as a predictor of bleeding disorders in women with unexplained menorrhagia. Blood Coagul Fibrinolysis 2012; 23 (04) 311-315
    CrossrefPubMedGoogle Scholar
  • 9 Rodeghiero F, Tosetto A, Abshire T. et al; ISTH/SSC Joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost 2010; 8 (09) 2063-2065
    CrossrefPubMedGoogle Scholar
  • 10 Elbatarny M, Mollah S, Grabell J. et al; Zimmerman Program Investigators. Normal range of bleeding scores for the ISTH-BAT: adult and pediatric data from the merging project. Haemophilia 2014; 20 (06) 831-835
    CrossrefPubMedGoogle Scholar
  • 11 Deforest M, Grabell J, Albert S. et al. Generation and optimization of the self-administered bleeding assessment tool and its validation as a screening test for von Willebrand disease. Haemophilia 2015; 21 (05) e384-e388
    CrossrefPubMedGoogle Scholar
  • 12 Pavord S, Rayment R, Madan B. et al. Management of inherited bleeding disorders in pregnancy: green-top guideline no. 71 (joint with UKHCDO). BJOG 2017; 124 (08) e193-e263
    CrossrefPubMedGoogle Scholar
  • 13 Chi C, Kadir RA. Inherited bleeding disorders in pregnancy. Best Pract Res Clin Obstet Gynaecol 2012; 26 (01) 103-117
    CrossrefPubMedGoogle Scholar
  • 14 Szecsi PB, Jørgensen M, Klajnbard A, Andersen MR, Colov NP, Stender S. Haemostatic reference intervals in pregnancy. Thromb Haemost 2010; 103 (04) 718-727
    Google Scholar
  • 15 Sharief LT, Lawrie AS, Mackie IJ, Smith C, Peyvandi F, Kadir RA. Changes in factor XIII level during pregnancy. Haemophilia 2014; 20 (02) e144-e148
    CrossrefPubMedGoogle Scholar
  • 16 Kouides PA. Present day management of inherited bleeding disorders in pregnancy. Expert Rev Hematol 2016; 9 (10) 987-995
    CrossrefPubMedGoogle Scholar
  • 17 Chi C, Lee CA, Shiltagh N, Khan A, Pollard D, Kadir RA. Pregnancy in carriers of haemophilia. Haemophilia 2008; 14 (01) 56-64
    CrossrefPubMedGoogle Scholar
  • 18 Ramsahoye BH, Davies SV, Dasani H, Pearson JF. Obstetric management in von Willebrand's disease: a report of 24 pregnancies and a review of the literature. Haemophilia 1995; 1 (02) 140-144
    CrossrefPubMedGoogle Scholar
  • 19 Kadir RA, Lee CA, Sabin CA, Pollard D, Economides DL. Pregnancy in women with von Willebrand's disease or factor XI deficiency. Br J Obstet Gynaecol 1998; 105 (03) 314-321
    CrossrefPubMedGoogle Scholar
  • 20 Martín-Salces M, Jimenez-Yuste V, Alvarez MT, Quintana M, Hernández-Navarro F. Review: factor XI deficiency: review and management in pregnant women. Clin Appl Thromb Hemost 2010; 16 (02) 209-213
    CrossrefPubMedGoogle Scholar
  • 21 Bolton-Maggs PH, Perry DJ, Chalmers EA. et al. The rare coagulation disorders–review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation. Haemophilia 2004; 10 (05) 593-628
    CrossrefPubMedGoogle Scholar
  • 22 Wilson RD, De Bie I, Armour CM. et al. Joint SOGC-CCMG opinion for reproductive genetic carrier screening: an update for all Canadian providers of maternity and reproductive healthcare in the era of direct-to-consumer testing. J Obstet Gynaecol Can 2016; 38 (08) 742-762.e3
    CrossrefPubMedGoogle Scholar
  • 23 Salomon LJ, Sotiriadis A, Wulff CB, Odibo A, Akolekar R. Risk of miscarriage following amniocentesis or chorionic villus sampling: systematic review of literature and updated meta-analysis. Ultrasound Obstet Gynecol 2019; 54 (04) 442-451
    CrossrefPubMedGoogle Scholar
  • 24 Pavord SR, Lester W. Inherited bleeding disorders in pregnancy. Fetal Matern Med Rev 2009; 20 (03) 205
    CrossrefPubMedGoogle Scholar
  • 25 Tongsong T, Khumpho R, Wanapirak C, Piyamongkol W, Sirichotiyakul S. Effect of umbilical cord bleeding following mid-pregnancy cordocentesis on pregnancy outcomes. Gynecol Obstet Invest 2012; 74 (04) 298-303
    CrossrefPubMedGoogle Scholar
  • 26 Manzanares S, Benítez A, Naveiro-Fuentes M, López-Criado MS, Sánchez-Gila M. Accuracy of fetal sex determination on ultrasound examination in the first trimester of pregnancy. J Clin Ultrasound 2016; 44 (05) 272-277
    CrossrefPubMedGoogle Scholar
  • 27 Mortarino M, Garagiola I, Lotta LA, Siboni SM, Semprini AE, Peyvandi F. Non-invasive tool for foetal sex determination in early gestational age. Haemophilia 2011; 17 (06) 952-956
    CrossrefPubMedGoogle Scholar
  • 28 Tsui NB, Kadir RA, Chan KC. et al. Noninvasive prenatal diagnosis of hemophilia by microfluidics digital PCR analysis of maternal plasma DNA. Blood 2011; 117 (13) 3684-3691
    CrossrefPubMedGoogle Scholar
  • 29 Ingerslev MD, Langhoff-Roos J, Soegaard K, Funding E, Diness BR. Prenatal genetic testing by late amniocentesis to guide delivery management in haemophilia carriers. Haemophilia 2017; 23 (05) e466-e468
    CrossrefPubMedGoogle Scholar
  • 30 Gordon MC, Narula K, O'Shaughnessy R, Barth Jr WH. Complications of third-trimester amniocentesis using continuous ultrasound guidance. Obstet Gynecol 2002; 99 (02) 255-259
    PubMedGoogle Scholar
  • 31 Hodor JG, Poggi SH, Spong CY. et al. Risk of third-trimester amniocentesis: a case-control study. Am J Perinatol 2006; 23 (03) 177-180
    Article in Thieme ConnectPubMedGoogle Scholar
  • 32 Pacheco LD, Costantine MM, Saade GR, Mucowski S, Hankins GD, Sciscione AC. von Willebrand disease and pregnancy: a practical approach for the diagnosis and treatment. Am J Obstet Gynecol 2010; 203 (03) 194-200
    CrossrefPubMedGoogle Scholar
  • 33 Mumford AD, Ackroyd S, Alikhan R. et al; BCSH Committee. Guideline for the diagnosis and management of the rare coagulation disorders: a United Kingdom Haemophilia Centre Doctors' Organization guideline on behalf of the British Committee for Standards in Haematology. Br J Haematol 2014; 167 (03) 304-326
    CrossrefPubMedGoogle Scholar
  • 34 Palla R, Peyvandi F, Shapiro AD. Rare bleeding disorders: diagnosis and treatment. Blood 2015; 125 (13) 2052-2061
    CrossrefPubMedGoogle Scholar
  • 35 Bolton-Maggs PH, Chalmers EA, Collins PW. et al; UKHCDO. A review of inherited platelet disorders with guidelines for their management on behalf of the UKHCDO. Br J Haematol 2006; 135 (05) 603-633
    CrossrefPubMedGoogle Scholar
  • 36 Peyvandi F, Palla R, Menegatti M. et al; European Network of Rare Bleeding Disorders Group. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders. J Thromb Haemost 2012; 10 (04) 615-621
    CrossrefPubMedGoogle Scholar
  • 37 Castaman G, Tosetto A, Rodeghiero F. Pregnancy and delivery in women with von Willebrand's disease and different von Willebrand factor mutations. Haematologica 2010; 95 (06) 963-969
    CrossrefPubMedGoogle Scholar
  • 38 Hawke L, Grabell J, Sim W. et al. Obstetric bleeding among women with inherited bleeding disorders: a retrospective study. Haemophilia 2016; 22 (06) 906-911
    CrossrefPubMedGoogle Scholar
  • 39 Siboni SM, Spreafico M, Calò L. et al. Gynaecological and obstetrical problems in women with different bleeding disorders. Haemophilia 2009; 15 (06) 1291-1299
    CrossrefPubMedGoogle Scholar
  • 40 Kadir RA, Economides DL, Braithwaite J, Goldman E, Lee CA. The obstetric experience of carriers of haemophilia. Br J Obstet Gynaecol 1997; 104 (07) 803-810
    CrossrefPubMedGoogle Scholar
  • 41 Biguzzi E, Siboni SM, Ossola MW, Zaina B, Migliorini AC, Peyvandi F. Management of pregnancy in type 2B von Willebrand disease: case report and literature review. Haemophilia 2015; 21 (01) e98-e103
    CrossrefPubMedGoogle Scholar
  • 42 Giles AR, Hoogendoorn H, Benford K. Type IIB von Willebrand's disease presenting as thrombocytopenia during pregnancy. Br J Haematol 1987; 67 (03) 349-353
    CrossrefPubMedGoogle Scholar
  • 43 McLaughlin D, Kerr R. Management of type 2B von Willebrand disease during pregnancy. Acta Haematol 2017; 137 (02) 89-92
    CrossrefPubMedGoogle Scholar
  • 44 Castaman G, Bertoncello K, Bernardi M, Rodeghiero F. Pregnancy and delivery in patients with homozygous or heterozygous R854Q type 2N von Willebrand disease. J Thromb Haemost 2005; 3 (02) 391-392
    CrossrefPubMedGoogle Scholar
  • 45 Dennis MW, Clough V, Toh CH. Unexpected presentation of type 2N von Willebrand disease in pregnancy. Haemophilia 2000; 6 (06) 696-697
    CrossrefPubMedGoogle Scholar
  • 46 Lak M, Peyvandi F, Mannucci PM. Clinical manifestations and complications of childbirth and replacement therapy in 385 Iranian patients with type 3 von Willebrand disease. Br J Haematol 2000; 111 (04) 1236-1239
    CrossrefPubMedGoogle Scholar
  • 47 Inocêncio G, Braga A, Azevedo S, Buchner G. Management of von Willebrand disease type 3 during pregnancy - 2 cases reports. Eur Rev Med Pharmacol Sci 2013; 17 (21) 2857-2859
    PubMedGoogle Scholar
  • 48 Ososki R, Lambing A, Kuriakose P. Management of pregnancy in type 3 von Willebrand disease with additional platelet dysfunction. Haemophilia 2015; 21 (05) e412-e415
    CrossrefPubMedGoogle Scholar
  • 49 James AH, Jamison MG. Bleeding events and other complications during pregnancy and childbirth in women with von Willebrand disease. J Thromb Haemost 2007; 5 (06) 1165-1169
    CrossrefPubMedGoogle Scholar
  • 50 Sharief LA, Kadir RA. Congenital factor XIII deficiency in women: a systematic review of literature. Haemophilia 2013; 19 (06) e349-e357
    CrossrefPubMedGoogle Scholar
  • 51 Frenkel E, Duksin C, Herman A, Sherman DJ. Congenital hypofibrinogenemia in pregnancy: report of two cases and review of the literature. Obstet Gynecol Surv 2004; 59 (11) 775-779
    CrossrefPubMedGoogle Scholar
  • 52 Gilabert J, Regañon E, Vila V. et al. Congenital hypofibrinogenemia and pregnancy, obstetric and hematological management. Gynecol Obstet Invest 1987; 24 (04) 271-276
    CrossrefPubMedGoogle Scholar
  • 53 Goodwin TM. Congenital hypofibrinogenemia in pregnancy. Obstet Gynecol Surv 1989; 44 (03) 157-161
    CrossrefPubMedGoogle Scholar
  • 54 Ness PM, Budzynski AZ, Olexa SA, Rodvien R. Congenital hypofibrinogenemia and recurrent placental abruption. Obstet Gynecol 1983; 61 (04) 519-523
    PubMedGoogle Scholar
  • 55 Edwards RZ, Rijhsinghani A. Dysfibrinogenemia and placental abruption. Obstet Gynecol 2000; 95 (6, Pt 2): 1043
    PubMedGoogle Scholar
  • 56 Mathonnet F, Guillon L, Detruit H. et al. Fibrinogen Poissy II (gammaN361K): a novel dysfibrinogenemia associated with defective polymerization and peptide B release. Blood Coagul Fibrinolysis 2003; 14 (03) 293-298
    CrossrefPubMedGoogle Scholar
  • 57 Takala T, Oksa H, Rasi V, Tuimala R. Dysfibrinogenemia associated with thrombosis and third-trimester fetal loss. A case report. J Reprod Med 1991; 36 (05) 410-412
    PubMedGoogle Scholar
  • 58 Yamanaka Y, Takeuchi K, Sugimoto M, Sato A, Nakago S, Maruo T. Dysfibrinogenemia during pregnancy treated successfully with fibrinogen. Acta Obstet Gynecol Scand 2003; 82 (10) 972-973
    CrossrefPubMedGoogle Scholar
  • 59 Funai EF, Klein SA, Lockwood CJ. Successful pregnancy outcome in a patient with both congenital hypofibrinogenemia and protein S deficiency. Obstet Gynecol 1997; 89 (5, Pt 2): 858
    CrossrefPubMedGoogle Scholar
  • 60 Miesbach W, Galanakis D, Scharrer I. Treatment of patients with dysfibrinogenemia and a history of abortions during pregnancy. Blood Coagul Fibrinolysis 2009; 20 (05) 366-370
    CrossrefPubMedGoogle Scholar
  • 61 Munoz J, Schering J, Lambing A. et al. The dilemma of inherited dysfibrinogenemia during pregnancy. Blood Coagul Fibrinolysis 2012; 23 (08) 775-777
    CrossrefPubMedGoogle Scholar
  • 62 Fadhlaoui A, Ben Salah N, Khrouf M. et al. Pregnancy and delivery in a patient with Glanzmann's thrombasthenia. [article in French]. Tunis Med 2011; 89 (12) 951-952
    PubMedGoogle Scholar
  • 63 George JN, Caen JP, Nurden AT. Glanzmann's thrombasthenia: the spectrum of clinical disease. Blood 1990; 75 (07) 1383-1395
    CrossrefPubMedGoogle Scholar
  • 64 Siddiq S, Clark A, Mumford A. A systematic review of the management and outcomes of pregnancy in Glanzmann thrombasthenia. Haemophilia 2011; 17 (05) e858-e869
    CrossrefPubMedGoogle Scholar
  • 65 Wijemanne A, Watt-Coote I, Austin S. Glanzmann thrombasthenia in pregnancy: optimising maternal and fetal outcomes. Obstet Med 2016; 9 (04) 169-170
    CrossrefPubMedGoogle Scholar
  • 66 Domschke C, Strowitzki T, Huth-Kuehne A, Staritz P, Sohn C, Schuetz F. Successful in vitro fertilization and pregnancy in Glanzmann thrombasthenia. Haemophilia 2012; 18 (05) e380-e381
    CrossrefPubMedGoogle Scholar
  • 67 Rachad M, Chaara H, Fdili Fz, Jayi S, Bouguern H, Melhouf MA. Glanzmann's thrombasthenia and pregnancy: report of a case and literature review. [article in French]. Pan Afr Med J 2011; 10: 61
    PubMedGoogle Scholar
  • 68 Macêdo MB, Brito JdeM, Macêdo PdaS, Brito JA. Primigravida with Bernard-Soulier syndrome: a case report. BMC Res Notes 2015; 8: 178
    CrossrefPubMedGoogle Scholar
  • 69 Mitrović M, Elezović I, Miljić P, Antić D. [Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: report of two cases]. Srp Arh Celok Lek 2014; 142 (5-6): 351-355
    CrossrefPubMedGoogle Scholar
  • 70 Noris P, Schlegel N, Klersy C. et al; European Hematology Association – Scientific Working Group on Thrombocytopenias and Platelet Function Disorders. Analysis of 339 pregnancies in 181 women with 13 different forms of inherited thrombocytopenia. Haematologica 2014; 99 (08) 1387-1394
    CrossrefPubMedGoogle Scholar
  • 71 Peitsidis P, Datta T, Pafilis I, Otomewo O, Tuddenham EG, Kadir RA. Bernard Soulier syndrome in pregnancy: a systematic review. Haemophilia 2010; 16 (04) 584-591
    CrossrefPubMedGoogle Scholar
  • 72 Barone I, Meccariello G, Cazzato L, Capalbo SF, Matteo M, Greco P. Management of platelet storage pool disease during pregnancy with recombinant factor VIIa. Eur J Obstet Gynecol Reprod Biol 2013; 170 (02) 576-577
    CrossrefPubMedGoogle Scholar
  • 73 Jewell M, Magann EF, Barr A, Baker R. Management of platelet storage pool deficiency during pregnancy. Aust N Z J Obstet Gynaecol 2003; 43 (02) 171-172
    CrossrefPubMedGoogle Scholar
  • 74 Denholm A, Smith MP, Patterson DM. Accurate diagnosis of high-affinity vWF-platelet disorders: a case study of pseudo von Willebrand disease. N Z J Med Lab Sci 2008; 62 (01) 7-9
    PubMedGoogle Scholar
  • 75 Grover N, Boama V, Chou MR. Pseudo (platelet-type) von Willebrand disease in pregnancy: a case report. BMC Pregnancy Childbirth 2013; 13: 16
    CrossrefPubMedGoogle Scholar
  • 76 O'Connor D, Lester W, Willoughby S, Wilde JT. Pregnancy in platelet-type VWD: a case series. Thromb Haemost 2011; 106 (02) 386-387
    PubMedGoogle Scholar
  • 77 Agarwal N, Willmott FJ, Bowles L, Pasi KJ, Beski S. Case report of Grey platelet syndrome in pregnancy. Haemophilia 2011; 17 (05) 825-826
    CrossrefPubMedGoogle Scholar
  • 78 Dolberg OJ, Ellis M. Successful pregnancy and delivery in a woman with gray platelet syndrome. Isr Med Assoc J 2011; 13 (02) 117-118
    PubMedGoogle Scholar
  • 79 Obeng-Tuudah D, Hussein BA, Hakim A, Gomez K, Abdul Kadir R. The presentation and outcomes of Hermansky-Pudlak syndrome in obstetrics and gynecological settings: a systematic review. Int J Gynaecol Obstet 2021; 154 (03) 412-426
    CrossrefPubMedGoogle Scholar
  • 80 Kadir R, Chi C, Bolton-Maggs P. Pregnancy and rare bleeding disorders. Haemophilia 2009; 15 (05) 990-1005
    CrossrefPubMedGoogle Scholar
  • 81 Peyvandi F, Bidlingmaier C, Garagiola I. Management of pregnancy and delivery in women with inherited bleeding disorders. Semin Fetal Neonatal Med 2011; 16 (06) 311-317
    CrossrefPubMedGoogle Scholar
  • 82 Naderi M, Eshghi P, Cohan N, Miri-Moghaddam E, Yaghmaee M, Karimi M. Successful delivery in patients with FXIII deficiency receiving prophylaxis: report of 17 cases in Iran. Haemophilia 2012; 18 (05) 773-776
    CrossrefPubMedGoogle Scholar
  • 83 Lebreton A, Casini A, Alhayek R, Kouteich KL, Neerman-Arbez M, de Moerloose P. Successful pregnancy under fibrinogen substitution in a woman with congenital afibrinogenaemia complicated by a postpartum venous thrombosis. Haemophilia 2015; 21 (01) e108-e110
    CrossrefPubMedGoogle Scholar
  • 84 Huq FY, Kadir RA. Management of pregnancy, labour and delivery in women with inherited bleeding disorders. Haemophilia 2011; 17 (Suppl. 01) 20-30
    CrossrefPubMedGoogle Scholar
  • 85 Reynen E, James P. Von Willebrand disease and pregnancy: a review of evidence and expert opinion. Semin Thromb Hemost 2016; 42 (07) 717-723
    Article in Thieme ConnectPubMedGoogle Scholar
  • 86 Shakur H, Roberts I, Fawole B. et al; WOMAN Trial Collaborators. Effect of early tranexamic acid administration on mortality, hysterectomy, and other morbidities in women with post-partum haemorrhage (WOMAN): an international, randomised, double-blind, placebo-controlled trial. Lancet 2017; 389 (10084): 2105-2116
    Google Scholar
  • 87 Kadir RA, Davies J, Winikoff R. et al. Pregnancy complications and obstetric care in women with inherited bleeding disorders. Haemophilia 2013; 19 (Suppl. 04) 1-10
    CrossrefPubMedGoogle Scholar
  • 88 Winikoff R, Scully MF, Robinson KS. Women and inherited bleeding disorders - a review with a focus on key challenges for 2019. Transfus Apheresis Sci 2019; 58 (05) 613-622
    CrossrefPubMedGoogle Scholar
  • 89 Dunkley S, Curtin JA, Marren AJ, Heavener RP, McRae S, Curnow JL. Updated Australian consensus statement on management of inherited bleeding disorders in pregnancy. Med J Aust 2019; 210 (07) 326-332
    CrossrefPubMedGoogle Scholar
  • 90 Lee CA, Chi C, Pavord SR. et al; UK Haemophilia Centre Doctors' Organization. The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors' Organization. Haemophilia 2006; 12 (04) 301-336
    CrossrefPubMedGoogle Scholar
  • 91 Chi C, Lee CA, England A, Hingorani J, Paintsil J, Kadir RA. Obstetric analgesia and anaesthesia in women with inherited bleeding disorders. Thromb Haemost 2009; 101 (06) 1104-1111
    Article in Thieme ConnectPubMedGoogle Scholar
  • 92 Dunkley SM, Russell SJ, Rowell JA. et al; Australian Haemophilia Centre Directors' Organisation. A consensus statement on the management of pregnancy and delivery in women who are carriers of or have bleeding disorders. Med J Aust 2009; 191 (08) 460-463
    CrossrefPubMedGoogle Scholar
  • 93 Lyon MF. Sex chromatin and gene action in the mammalian X-chromosome. Am J Hum Genet 1962; 14: 135-148
    PubMedGoogle Scholar
  • 94 Huq FY, Kulkarni A, Agbim EC, Riddell A, Tuddenham E, Kadir RA. Changes in the levels of factor VIII and von Willebrand factor in the puerperium. Haemophilia 2012; 18 (02) 241-245
    CrossrefPubMedGoogle Scholar
  • 95 Gupta SN, Kechli AM, Kanamalla US. Intracranial hemorrhage in term newborns: management and outcomes. Pediatr Neurol 2009; 40 (01) 1-12
    CrossrefPubMedGoogle Scholar
  • 96 Rooks VJ, Eaton JP, Ruess L, Petermann GW, Keck-Wherley J, Pedersen RC. Prevalence and evolution of intracranial hemorrhage in asymptomatic term infants. AJNR Am J Neuroradiol 2008; 29 (06) 1082-1089
    CrossrefPubMedGoogle Scholar
  • 97 James AH, Hoots K. The optimal mode of delivery for the haemophilia carrier expecting an affected infant is caesarean delivery. Haemophilia 2010; 16 (03) 420-424
    CrossrefPubMedGoogle Scholar
  • 98 Ljung R. The optimal mode of delivery for the haemophilia carrier expecting an affected infant is vaginal delivery. Haemophilia 2010; 16 (03) 415-419
    CrossrefPubMedGoogle Scholar
  • 99 Davies J, Kadir RA. Mode of delivery and cranial bleeding in newborns with haemophilia: a systematic review and meta-analysis of the literature. Haemophilia 2016; 22 (01) 32-38
    CrossrefPubMedGoogle Scholar
  • 100 Sabir H, Stannigel H, Schwarz A, Hoehn T. Perinatal hemorrhagic shock after fetal scalp blood sampling. Obstet Gynecol 2010; 115 (2, Pt 2): 419-420
    CrossrefPubMedGoogle Scholar
  • 101 Sheldon WR, Blum J, Vogel JP, Souza JP, Gülmezoglu AM, Winikoff B. WHO Multicountry Survey on Maternal and Newborn Health Research Network. Postpartum haemorrhage management, risks, and maternal outcomes: findings from the World Health Organization Multicountry Survey on Maternal and Newborn Health. BJOG 2014; 121 (Suppl. 01) 5-13
    Google Scholar
  • 102 El-Refaey H, Rodeck C. Post-partum haemorrhage: definitions, medical and surgical management. A time for change. Br Med Bull 2003; 67: 205-217
    CrossrefPubMedGoogle Scholar
  • 103 Menard MK, Main EK, Currigan SM. Executive summary of the reVITALize initiative: standardizing obstetric data definitions. Obstet Gynecol 2014; 124 (01) 150-153
    CrossrefPubMedGoogle Scholar
  • 104 Hamm RF, Wang EY, Bastek JA, Srinivas SK. Assessing reVITALize: Should the definition of postpartum hemorrhage differ by mode of delivery?. Am J Perinatol 2017; 34 (05) 503-507
    PubMedGoogle Scholar
  • 105 Tardy B, Chalayer E, Kamphuisen PW. et al; SSC Subcommittee on Control of Anticoagulation of the ISTH. Definition of bleeding events in studies evaluating prophylactic antithrombotic therapy in pregnant women: a systematic review and a proposal from the ISTH SSC. J Thromb Haemost 2019; 17 (11) 1979-1988
    CrossrefPubMedGoogle Scholar
  • 106 Peyvandi F, Menegatti M, Siboni SM. Post-partum hemorrhage in women with rare bleeding disorders. Thromb Res 2011; 127 (Suppl. 03) S116-S119
    CrossrefPubMedGoogle Scholar
  • 107 Klamroth R, Gröner A, Simon TL. Pathogen inactivation and removal methods for plasma-derived clotting factor concentrates. Transfusion 2014; 54 (05) 1406-1417
    CrossrefPubMedGoogle Scholar
  • 108 Trigg DE, Stergiotou I, Peitsidis P, Kadir RA. A systematic review: the use of desmopressin for treatment and prophylaxis of bleeding disorders in pregnancy. Haemophilia 2012; 18 (01) 25-33
    Google Scholar
  • 109 Peitsidis P, Kadir RA. Antifibrinolytic therapy with tranexamic acid in pregnancy and postpartum. Expert Opin Pharmacother 2011; 12 (04) 503-516
    CrossrefPubMedGoogle Scholar
  • 110 Wong H, Curry N. Do we need cryoprecipitate in the era of fibrinogen concentrate and other specific factor replacement options?. ISBT Sci Ser 2018; 13 (01) 23-28
    CrossrefPubMedGoogle Scholar
  • 111 Chi C, Huq FY, Kadir RA. Levonorgestrel-releasing intrauterine system for the management of heavy menstrual bleeding in women with inherited bleeding disorders: long-term follow-up. Contraception 2011; 83 (03) 242-247
    CrossrefPubMedGoogle Scholar