von Willebrand disease in women with menorrhagia: a systematic review.

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.

DDAVP nasal spray for treatment of menorrhagia in women with inherited bleeding disorders: a randomized placebo-controlled crossover study.

To assess DDAVP (1-deamino-8-d-arginine vasopressin; desmopressin) nasal spray in the management of menorrhagia in patients with inherited bleeding disorders, 39 women (aged 18-50 years) with menorrhagia were recruited and were randomized to start 2 months' therapy with placebo or DDAVP (300 micro g) spray in a double-blind crossover study. Twenty-eight and 24 completed first and second period of treatment, respectively. Menstrual loss was assessed using the pictorial blood assessment chart (PBAC) during each treatment period.

Ocular manifestation of vitamin A deficiency among Orang asli (Aborigine) children in Malaysia.

This study determined the prevalence of ocular manifestation of vitamin A deficiency in Orang Asli (Aborigine) children. Night blindness was found in 16.0% of the children, conjunctiva xerosis in 57.

Obstetrical and gynaecological bleeding: a common presenting symptom.

Excessive haemorrhage is one of the commonest symptom when women present to obstetricians and gynaecologists. In the majority of cases the cause is related to the primary obstetric and gynaecological pathology and is easily identified. However, less commonly, the bleeding is unexpected and due to an undiagnosed underlying coagulation defect, the commonest of which is von Willebrand (vWD) disease.

Ultrasonographic markers for chromosomal abnormalities in women with negative nuchal translucency and second trimester maternal serum biochemistry.

Objective: To analyze the value of second trimester ultrasound examination among those women whose fetuses were indicated to be at low risk of chromosomal anomalies on the basis of both first trimester nuchal translucency measurement and second trimester biochemical screening.

Methods: A retrospective study of 5500 pregnancies carried out at the fetal medicine unit, Royal Free Hospital. During a period of over 3 years 5500 pregnancies underwent a first trimester scan and nuchal translucency measurement which enabled the detection of 62% (20 of 32) of all chromosomal anomalies.

Reproductive choices of women in families with haemophilia.

To assess women's experiences in pregnancy and attitudes towards their reproductive choices, a structured questionnaire was sent to all obligate and potential carriers of haemophilia (A and B), aged 14-60 years, registered with our haemophilia centre. One hundred and ninety-seven of 545 (36%) returned completed questionnaires. Clinical details, including type and severity of the disease in the family and results of DNA analysis for carrier detection, were obtained from patient notes.

Variations in coagulation factors in women: effects of age, ethnicity, menstrual cycle and combined oral contraceptive.

To 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.

Women and inherited bleeding disorders: pregnancy and delivery.

The most common inherited bleeding disorders in women are von Willebrand disease (vWD), carriership of hemophilia A and B, and factor XI (FXI) deficiency. Pregnancy and delivery are associated with major concerns and particular risks in women with these disorders. An increased awareness among clinicians of these disorders and their obstetric complications, a multidisciplinary approach to management, close collaboration between obstetricians and hemophilia centers, and the availability of management guidelines are essential to minimize maternal and neonatal complications.

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. 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.

Assessment of menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders.

Menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders were assessed in this study. One hundred and sixteen women, including 66 with von Willebrand's disease (vWD), 30 carriers of haemophilia and 20 with factor XI (FXI) deficiency were interviewed and their gynaecological history obtained. Their case records were also reviewed and menstrual loss was objectively assessed using a pictorial blood assessment chart (PBAC).

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.

The significance of choroid plexus cysts, echogenic heart foci and renal pyelectasis in the first trimester.

Objective: To determine the significance of certain soft ultrasonographic markers for chromosomal abnormalities in the first trimester.

Design: This was a prospective cross-sectional study.

Setting: University Department of Obstetrics and Gynaecology, London, UK.

Factor XI deficiency in women.

Factor XI (FXI) deficiency is an uncommon autosomally transmitted coagulopathy found predominantly in Jewish kindreds. It is associated with variable bleeding tendency that usually manifests after trauma, surgery, or other challenges to hemostasis. Therefore, women with FXI deficiency are at risk of excessive bleeding during their menstrual periods, childbirth, and after surgery.

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

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.

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.

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.

Methods: 2281 women (mean maternal age 30 years [range 16-47]; mean gestational age 12(+3) weeks [range 11-14]) underwent transabdominal scanning to assess fetal structure and, if anatomical survey was considered to be incomplete (31% of cases), transvaginal sonography was also performed. Measurement of nuchal translucency was included and karyotyping performed as considered appropriate.

Prenatal diagnosis of supernumerary chromosome derivative (22) due to maternal balanced translocation in association with diaphragmatic hernia: a case report.

An aneuploid fetus was detected prenatally by cordocentesis at 27 weeks' gestation following ultrasonographic diagnosis of severe fetal growth retardation and a large diaphragmatic hernia. The fetal karyotype was revealed to be 47,XX,der(22)t(11;22)(q23.3;q11.

The obstetric experience of carriers of haemophilia.

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

Design: Retrospective review of haemophilia and maternity unit records.

Setting: Haemophilia Comprehensive Care Centre.