R. A. Kadir

Frequency of inherited bleeding disorders in women with menorrhagia

BACKGROUND Although menorrhagia is a common gynaecological symptom, a specific cause is identified in less than 50% of affected women. We investigated the frequency of inherited bleeding disorders in women with menorrhagia. METHODS Women referred for investigation of menorrhagia whose pelvis was normal on clinical examination and who had an estimated menstrual blood loss of more than 80 mL were studied. A detailed menstrual history and history about other bleeding symptoms was taken. The activated partial thromboplastin time, factor VIII activity, von-Willebrand-factor antigen and activity, and factor XI (FXI) were measured in all patients; further tests were done when results were at or outside the limits of the assays. FINDINGS 150 women were screened. An inherited bleeding disorder was diagnosed in 26 (17%) patients: the disorders were von Willebrands disease of mild (15) or moderate severity (three), mild FXI deficiency (four), mild von Willebrands disease and FXI deficiency (one), combined von Willebrands disease, FXI deficiency, and factor X deficiency (one), carriage of haemophilia-A gene (one), and platelet dysfunction (one). The frequency of von Willebrands disease and FXI deficiency were 13% (95% CI 7.9-18.8%) and 4% (1.5-8.5%), respectively. Menorrhagia since menarche was noted in 11 (8.9%) of 123 women without a bleeding disorder compared with 13 (65%) of 20 women with von Willebrands disease (p=0.001) and four (66.7%) of six women with FXI deficiency (p<0.001). INTERPRETATION Inherited bleeding disorders are found in a substantial proportion of women with menorrhagia and a normal pelvis examination. We suggest that such patients should be investigated for these disorders-especially von Willebrands disease-before invasive procedures are done.

Pregnancy in women with von Willebrand's disease or factor XI deficiency

Objective To assess the obstetric outcome in women with von Willebrands disease or factor XI deficiency.

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

Summary.  The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. These guidelines have been provided for healthcare professionals for information and guidance and it is also intended that they are readily available for women with bleeding disorders.

von Willebrand disease in women with menorrhagia: a systematic review

Objective  To determine the prevalence of von Willebrand disease in women presenting with menorrhagia.

The value of sonography in early pregnancy for the detection of fetal abnormalities in an unselected population.

Objective To determine the value of early pregnancy sonography in detecting fetal abnormalities in an unselected obstetric population.

The use of levonorgestrel‐releasing intrauterine system for treatment of menorrhagia in women with inherited bleeding disorders

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.

The obstetric experience of carriers of haemophilia

Objective To review the obstetric problems, pregnancy outcome and management of carriers of haemophilia.

Quality of life during menstruation in patients with inherited bleeding disorders

Menorrhagia is a common and major problem for patients with inherited bleeding disorders, especially vWD. Quality of life during menstruation was assessed in 99 patients with inherited bleeding disorders including vWD (n = 57), carriers of haemophilia A (n = 17), carriers of haemophilia B (n = 7) and FXI deficiency (n = 18), and comparison was performed with an age‐matched control group (n = 69). A questionnaire was used that included four main sections: (i) general health, (ii) health and daily activities, (iii) dysmenorrhea and (iv) quality of life during the menstrual period. Although patients with inherited bleeding disorders felt that their health (in general) was very good, they had significantly poorer quality of life on all the scales used than controls. Thirty‐nine per cent reported having cut down on the amount of time spent on work and other activities as a result of their menstruation; 47% felt that they accomplished less than they would like during this period, 38% felt that they were limited in the kind of work and other activities that they could do, and 40% found that it took extra effort to perform their work. Fifty‐one per cent experienced moderate, severe or very severe dysmenorrhoea. Quality of life was statistically poorer in patients with vWD, menstrual scores> 100 according to the pictorial blood assessment chart (PBAC), those who had periods ≥8 days and those who experienced flooding or passage of clots. In conclusion, menstruation has a negative effect on the quality of life in patients with inherited bleeding disorders especially in those with objectively confirmed menorrhagia.

First trimester sonographic detection of chromosomal abnormalities in an unselected population

Objective To investigate the role of first trimester sonography in detecting chromosomal abnormalities in an unselected obstetric population.

Antifibrinolytic therapy with tranexamic acid in pregnancy and postpartum

Objective: The aim of this study is critically to review the available evidence regarding the use, efficacy and safety of tranexamic acid in the management of hemorrhage during pregnancy and for prevention and treatment of postpartum hemorrhage. Research design and methods: We performed a systematic search of electronic literature (PubMed, Embase, CINAHL, Scopus, Cochrane, DARE) to review all studies looking at the use of tranexamic acid during pregnancy and puerperium. We did a metanalysis on three randomized controlled trials that evaluated reduction in blood loss in women undergoing cesarean sections with the use of tranexamic acid. Results: An electronic search yielded 34 articles, the studies dating from 1976 to 2010, five randomized controlled trials, seven observational studies, and twenty-two case reports. Meta-analysis showed that the estimate of the combined effect of tranexamic acid compared with placebo was a difference of 32.5 ml reduction in blood loss (95% CI −4.1 – 69.13; p = 0.08). Tranexamic acid was also used successfully to prevent and treat bleeding in observation studies and case reports. Pulmonary embolism was reported in two cases; however, the possible involvement of tranexamic acid in these thrombotic episodes could neither be confirmed nor excluded. Conclusions: The clinical studies suggest that tranexamic acid reduces the amount of blood loss after delivery during cesarean sections and vaginal deliveries, and reduces the requirement for blood transfusion. Tranexamic acid seems to be safe and effective in the prevention and management of bleeding during pregnancy. Further investigation and larger clinical trials with better design and methodological quality are required to confirm these findings.