Document Detail


Pregnancy in women with von Willebrand's disease or factor XI deficiency.
MedLine Citation:
PMID:  9532993     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To assess the obstetric outcome in women with von Willebrand's disease or factor XI deficiency. SETTING: Haemophilia Centre and Haemostasis Unit, The Royal Free Hospital. POPULATION: Women with von Willebrand's disease (n = 31) and with factor XI deficiency (n = 11) registered at the Royal Free Hospital Haemophilia Centre who had had a pregnancy within the previous 17 years (1980-1996), including 84 in women with von Willebrand's disease and 28 in women with factor XI deficiency. METHODS: Women were interviewed and details of the obstetric history were obtained. The records of the Haemophilia Centre and the women's maternity records were also reviewed. RESULTS: Threatened miscarriage occurred in 33% and 14% of pregnancies with von Willebrand's disease and factor XI deficiency, respectively. Excluding recurrent miscarriages, 14/68 (21%) of pregnancies with von Willebrand's disease and one pregnancy with factor XI deficiency miscarried spontaneously. There was an increased incidence of primary and secondary post-abortal bleeding complications. Factor VIII and von Willebrand factor antigen and activity levels increased significantly in pregnancy in all women apart from those with severe von Willebrand's disease. Factor XI, however, did not show any significant change. No neonatal haemorrhagic complications in association with the birth process were reported, although ventouse and difficult forceps deliveries were avoided. Extensive perineal bruising and haematoma was reported in three women with von Willebrand's disease; two of these were associated with forceps delivery. The incidence of primary postpartum haemorrhage was 18.5% in von Willebrand's disease and 16% in factor XI deficiency. Blood transfusion was required in six cases of von Willebrand's disease and two cases of factor XI deficiency. Ten of fourteen instances of primary postpartum haemorrhage occurred when maternal factor levels were < 50 IU/dL with no prophylactic treatment for labour. The incidence of secondary postpartum haemorrhage was 20% in von Willebrand's disease and 24% in factor XI deficiency. None of the women who had prophylactic treatment during labour or the puerperium suffered any significant bleeding complications. There were three neonatal bleeding complications. CONCLUSION: Pregnancy, labour and the puerperium are associated with significant bleeding problems in women with von Willebrand's disease or factor XI deficiency, but these are largely preventable. Specialist obstetric care in close liaison with the haemophilia centre is essential to minimise maternal and neonatal complications.
Authors:
R A Kadir; C A Lee; C A Sabin; D Pollard; D L Economides
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  British journal of obstetrics and gynaecology     Volume:  105     ISSN:  0306-5456     ISO Abbreviation:  Br J Obstet Gynaecol     Publication Date:  1998 Mar 
Date Detail:
Created Date:  1998-04-24     Completed Date:  1998-04-24     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  7503752     Medline TA:  Br J Obstet Gynaecol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  314-21     Citation Subset:  AIM; IM    
Affiliation:
University Department of Obstetrics and Gynaecology, Royal Free Hospital, London.
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MeSH Terms
Descriptor/Qualifier:
Abortion, Spontaneous / etiology
Abortion, Threatened / etiology
Anesthesia, Conduction
Antigens / analysis
Birth Weight
Contusions / etiology
Delivery, Obstetric
Factor VII / analysis
Factor XI / analysis
Factor XI Deficiency / complications*
Female
Hemorrhage / etiology
Humans
Infant, Newborn
Postpartum Hemorrhage / etiology
Pregnancy
Pregnancy Complications, Hematologic / blood*
Pregnancy Outcome
Uterine Hemorrhage / etiology
von Willebrand Diseases / complications*
von Willebrand Factor / immunology
Chemical
Reg. No./Substance:
0/Antigens; 0/Von Willebrand antigen; 0/von Willebrand Factor; 9001-25-6/Factor VII; 9013-55-2/Factor XI

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