Objective To assess the obstetric outcome in women with von Willebrand's disease or factor XI Setting Haemophilia Centre and Haemostasis Unit, The Royal Free Hospital.Population Women with von Willebrand's disease (n = 3 1) 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)(1981)(1982)(1983)(1984)(1985)(1986)(1987)(1988)(1989)(1990)(1991)(1992)(1993)(1994)(1995)(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 recwent 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 VIJI 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 185% 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. SDecialist obstetric care in close liaison with the haemophilia centre is essential to minimise maternal deficiency.and neonatal complications.

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Objective To determine the prevalence of von Willebrand disease in women presenting with menorrhagia.Design Systematic review of studies evaluating the prevalence of von Willebrand disease in women with menorrhagia. Setting Hospital outpatient clinics (mainly gynaecological) and population surveys.Population Women presenting with menorrhagia.Methods Relevant studies were extracted from MEDLINE search, bibliographies of identified articles and published proceedings of meetings and conferences. Main outcome measures Number of women with von Willebrand disease.Results Eleven studies were included, totalling 988 women with menorrhagia. One hundred and thirty-one women were diagnosed to have von Willebrand disease with prevalences in individual studies ranging from 5% to 24%. The overall prevalence was 13% (95% CI 11-15.6%). The prevalence was higher in the European studies-18% (95% CI 15 -23%) compared with that in North American studies-10% (95% CI 7.5 -13%). This difference (P ¼ 0.007) is likely to be the result of differences in the studies, which include method of recruitment of study population, method of assessing menstrual blood loss ethnic composition of study population, criteria for diagnosis and use of race-and ABO blood group-specific values for von Willebrand factor. Conclusions The prevalence of von Willebrand disease is increased in women with menorrhagia and is the underlying cause in a small but significant group of women with menorrhagia across the world. Testing for this disorder should be considered when investigating women with menorrhagia, especially those of Caucasian origin, those with no obvious pelvic pathology or with additional bleeding symptoms.

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SummaryTo assess variations of coagulation factors in women, 123 women were included in a cross-sectional study of the effect of age, ethnic origin, blood group and menstrual cycle on surface induced coagulation time (activated partial thromboplastin time) and plasma levels of Factor VIII clotting assay, von Willebrand factor antigen, von Willebrand factor activity and factor XI. The effect of menstrual cycle was further assessed in a longitudinal study including 39 Caucasian women, 20 of whom were using combined oral contraceptives. Activated partial thromboplastin time was longer in women with blood groups B or O, and plasma levels of factor VIII clotting assay, von Willebrand factor antigen and von Willebrand factor activity were significantly higher in black women. Fibrinogen, von Willebrand factor antigen and von Willebrand factor activity concentrations showed strong cyclic variations with peak values in the luteal phase. This pattern was dampened for von Willebrand factor antigen and von Willebrand factor activity but completely disappeared for fibrinogen with the use of combined oral contraceptives. There was a cyclical pattern for factor VIII clotting assay in pill users, evidence of which was not evident in non-pill users. There were strong associations between the levels of von Willebrand factor antigen and von Willebrand factor activity and age, with levels rising by an average of 0.17 and 0.15 U/ml, respectively, for each 10 year increase in age. In conclusion, there are great inter- and intraindividual variations in coagulation markers in women due to different physiological conditions such as age, ethnicity, blood group and phases of the menstrual cycle. However, there were no significant associations between coagulation markers and weight, alcohol consumption or smoking status.

In the setting of routine antenatal screening, an increased NT measurement is a marker of a high-risk pregnancy even in karyotypically normal fetuses. In addition, the increased incidence of structural abnormalities makes the close follow-up of these pregnancies imperative and should include specialized fetal echocardiography.

a Background The levonorgestrel-releasing intrauterine system (LNG-IUS) is used commonly by gynaecologists as a contraceptive and to treat menorrhagia. However, its efficacy has not been examined in women with inherited bleeding disorders. Design A prospective pilot study.Setting A teaching hospital in north London with a designated haemophilia centre.Population Female patients with a known inherited bleeding disorder.Methods Sixteen women with subjective and objective menorrhagia caused by inherited bleeding disorders (13 von Willebrand's Disease, two factor XI deficiency and one Hermansky -Pudlak syndrome), who had previously undergone unsuccessful medical treatment were followed up for nine months after LNG-IUS insertion. Bleeding was measured by pictorial chart and haemoglobin concentration. Results All women reported that their periods were improved, pictorial chart scores were lower and 56% became amenorrhoeic. None reported side effects. Conclusion The LNG-IUS is well tolerated and effective and improves quality of life.

Objective To determine the value of early pregnancy sonography in detecting fetal abnormalities in an unselected obstetric population.Design Prospective cross-sectional study. All women initially underwent transabdominal sonography and when the anatomical survey was considered to be incomplete, transvaginal sonography was also performed (20.1 %). Nuchal translucency was measured and karyotyping was performed as appropriate.Setting University Department of Obstetrics and Gynaecology.Participants 6634 sequential unselected women (mean maternal age 29-9 years, range 13-50; mean gestational age 12& weeks, range 11+0-14+6), carrying 6443 live fetuses participated in this study.Main outcome measure Detection rate of fetal anomalies and the associated cost per case detected in early pregnancy. ResultsThe incidence of anomalous fetuses was 1.4% (926443) including 43 chromosomal abnormalities. The detection rate for structural abnormalities was 59.0% (37/63, 95% CI 46.5-72.4) and the specificity was 99.9% in early pregnancy. When the first and second trimester scans were combined, the detection for structural abnormalities was 8 1.0% (5 1/63, 95% CI 67.7-89.2). Seventy-eight percent (31/40) of chromosomal abnormalities (excluding three cases of XXY) were diagnosed at 11-14 weeks, either because of a nuchal translucency greater than or equal to the 99th centile for gestational age (43%; 17/40,95% CI 27-4-60.4), or due to the presence of structural abnormalities (35%; 14/40, 95% CI 21-2-52.8). Sixty-five percent (15/23) of cases of trisomy 21 were also diagnosed either because of having a nuchal translucency greater than or equal to the 99th centile (57.0%; 13/23) or due to the presence of a structural abnormality (9.0%; 2/23). Overall, the detection rate of structurally abnormal fetuses was 59% (37/63) in early pregnancy and 81% in combination with the second trimester scan. The cost per abnormality diagnosed in early pregnancy is estimated to be f6258 per structurally abnormal fetus, f7470 per chromosomal abnormality and f4453 per anomalous fetus. ConclusionThe majority of fetal structural and chromosomal abnormalities can be detected by sonographic screening at 11-14 weeks, but the second trimester scan should not be abandoned.