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The monthly challenge of menstruation as well as the haemostatic challenge of childbirth postpartum renders more females than males symptomatic with von Willebrand disease. Among vWD patients, the obstetrical and gynaecological morbidity is certainly more pronounced in Type 2,3 patients compared to Type 1 patients, but even in the latter group there is a high proportion of menorrhagia with associated anaemia, loss of time from work/school and the use of hysterectomy for ultimate control of bleeding. Despite the well known adage of the "gestational palliation" of vWD, there is a high proportion of postpartum haemorrhage in Type 1 patients also especially after the first 24 h after delivery. This may occur despite normalization of the factor VIIIc level in the third trimester, particularly in Type 2,3 patients. With the increasing availability of intranasal/subcutaneous DDAVP that could be readily administered at home for menorrhagia, there recently has been ongoing efforts internationally to determine the prevalence of vWD in females presenting with menorrhagia with a prevalence of 17% combined from two studies of 180 patients total. Issues remain regarding the optimal dose/schedule of intranasal/subcutaneous DDAVP for menorrhagia and the relative efficacy of antifibrinolytic agents. The proper role of oral contraceptives and danazol also deserves further study in vWD patients with menorrhagia. In sum, a comprehensive care approach in females with vWD is warranted analogous to the successful model of care of male haemophiliacs with the intent to (a) reduce unnecessary surgical interventions for menorrhagia, (b) improve the quality of life during menses and (c) optimize peri-partum management.
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PMID:Females with von Willebrand disease: 72 years as the silent majority. 987 12

The transition of childhood to adulthood includes many changes to nearly all parts of the body and that is certainly true of blood and the coagulation system. Some disorders, like iron deficiency anemia, develop as the result of rapid growth. Approximately 10% of American adolescents are anemic and the prevalence is far greater in high-risk populations, such as urban, indigent African-American adolescents, in which 40-50% of young women are anemic. Adolescents at greater-than-average risk for developing iron deficiency anemia, such as athletes involved in lengthy, intense physical activities and pregnant adolescents, should be screened for anemia. Other blood problems are inherited but the first manifestations may not emerge until adolescence, as in the case of an adolescent girl discovered to have von Willebrand's disease during the evaluation of excessive menstrual bleeding. Besides iron deficiency anemia and von Willebrand's disease, this review focuses on management of other common hematologic disorders seen in adolescent patients, including immune thrombocytopenic purpura, hemophilia, thrombocytosis, and hypercoagulable disorders.
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PMID:Anemia and coagulation disorders in adolescents. 1061 33

Type 1 von Willebrand disease (vWD) is generally regarded clinically as 'mild' and the obstetrical-gynaecological features have not been fully described. We administered a patient questionnaire and provider survey of the medical and quality of life aspects of childbirth and menstruation to 99 type 1 vWD patients and compared the patients presently menstruating (n=81) to a cohort of 150 menstruating females in the general population. The following measurements had a statistically higher proportion in the vWD group: number of tampons/towels used for a typical menstrual cycle (P=0. 002); percentage reporting that clothes are stained by menses (P = 0. 001); past or present history of anaemia (P = 0.001); childbirth-related bleeding (P=0.001); and childbirth-related bleeding necessitating RBC transfusion (P=0.002). Quality of life assessment of the impact of menses in both of the above cohorts was measured by a Likert scale using seven quality of life parameters. Compared to the control group, the vWD patients had a significantly higher score, with P-values of < 0.0001 for each parameter. Hormonal interventions for menorrhagia in the vWD patients were < or = 50% effective. Menorrhagia resulted in red blood cell transfusions in 6% of patients, dilatation and curettage in 17% and hysterectomy in 13%. Despite the common connotation of type 1 vWD as clinically 'mild', childbirth and the monthly challenge to haemostasis presented by menstruation result in a substantial degree of morbidity in females with type 1 vWD. These results support the rationale for ongoing international efforts to increase awareness of vWD as a cause for menorrhagia and to improve the quality of life in females with known vWD.
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PMID:Gynaecological and obstetrical morbidity in women with type I von Willebrand disease: results of a patient survey. 1112 90

Careful review of the literature regarding clinical signs caused by hypothyroidism in dogs has shown that some assumptions regarding the relation of hypothyroidism to other conditions are based on anecdotal evidence. Cutaneous manifestations are present in most hypothyroid dogs, but the specific abnormalities and breed variations remain to be clearly defined. Decreased metabolic rate manifested by obesity and lethargy is also common. Neurologic manifestations, although uncommon, clearly occur in hypothyroid dogs. Cardiac abnormalities seem to be common, but their clinical significance is questionable. The only consistent hematologic abnormality that occurs in hypothyroid dogs is anemia; evidence for acquired von Willebrand's disease or other bleeding disorders is negligible. Reproductive dysfunction secondary to hypothyroidism is unlikely to occur in male dogs, and there is no evidence to support abnormalities in female dogs. The relation of megaesophagus, laryngeal paralysis, ocular abnormalities, and gastrointestinal disorders with hypothyroidism remains to be established. Future research into canine hypothyroidism may serve to convert dogma into a more clear understanding of the manifestations and pathophysiologic findings of this common endocrinopathy.
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PMID:Conditions associated with canine hypothyroidism. 1157 Jan 33

The impact of von Willebrand disease in females is pronounced in terms of menorrhagia and postpartum haemorrhage. There is a very high proportion of von Willebrand disease patients with menorrhagia and associated anaemia, impairment of quality of life, including loss of time from work or school, and a high rate of the use of hysterectomy for ultimate control of the bleeding. The 'early' detection of von Willebrand disease in females may avert these complications. Consequently, there have recently been ongoing international efforts to determine the prevalence of von Willebrand disease in females presenting with menorrhagia, providing a prevalence of 7-20% combined from three studies including a total of 300 patients. Issues remain regarding the optimal dose/schedule of intranasal or subcutaneous desmopressin use for menorrhagia and the relative efficacy of anti-fibrinolytic agents. The proper role of oral contraceptives deserves further study in von Willebrand disease patients with menorrhagia as recent studies have paradoxically demonstrated a lower response rate in type 1 than type 2 or 3 von Willebrand disease. Despite the well-known adage of the 'gestational palliation' of von Willebrand disease, there is also a high proportion of postpartum haemorrhage in type 1 patients, especially after the 24 hour post-delivery period. This may occur despite a normalization of the factor VIIIc level in the third trimester, particularly in type 2 and 3 patients. The care-giver must be aware that haemorrhage can occur up to 5 weeks postpartum. In sum, studies over the past decade have documented a substantial impact of menses and childbirth on von Willebrand disease patients. These results should serve as a basis for interventional studies to reduce the morbidity of menstruation and childbirth.
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PMID:Obstetric and gynaecological aspects of von Willebrand disease. 1168 6

Two women aged 39 and 30 years were investigated for possible coagulation disorders because of menorrhagia, anaemia and postoperative haemorrhages. These investigations revealed that they had type 1 Von Willebrand's disease. During the treatment with desmopressin, factor VIII and Von Willebrand factor (VWF) activity normalised. About one third of the patients referred to a gynaecologist for menorrhagia have an inherited bleeding disorder, of which type 1 Von Willebrand's disease is the most prevalent. Once a gynaecological cause of the menorrhagia has been excluded, or in the case of an increased risk on the basis of the medical history, a limited haemostatic investigation can establish or exclude an inherited coagulation disorder. For women with a coagulation disorder the menorrhagia can be treated. Surgical interventions can be carried out safely following treatment with desmopressin or factor VIII/VWF concentrates.
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PMID:[An inherited hemostatic disorder as the cause of menorrhagia]. 1193 74

A 2.5-year-old spayed female German Shepherd Dog was referred for evaluation of progressive anemia, lethargy, and weight loss. Seventeen days earlier, the dog had received a whole blood transfusion to manage hemorrhage after ovariohysterectomy. Mild fever, splenomegaly, and thrombocytopenia were also identified. Von Willebrand disease and Babesia gibsoni infection were diagnosed. Because of the serologic cross-reactivity of B gibsoni and B canis in the immunofluorescent antibody assay for IgG antibodies against these organisms, polymerase chain reaction amplification of parasite DNA was required to identify the infecting Babesia sp. The source of the B gibsoni infection was traced to an apparently healthy American Pit Bull Terrier blood donor. Despite resolution of clinical signs in the dog of this report, a series of antiparasitic treatments failed to eliminate the B gibsoni infection. Screening of potential blood donor dogs for Babesia spp is becoming increasingly important in the United States.
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PMID:Transfusion-associated Babesia gibsoni infection in a dog. 1268 86

Menorrhagia is one of the most important and frequent complications in women with congenital von Willebrand disease (vWD). Three cases of menorrhagia with vWD (type 1; 1 case, type 2A; 2 cases) were successfully treated with tranexamic acid at dose of 3 grams daily in four divided doses for the first 5 days of the menstrual cycle. All patients had severe menorrhagia lasted for more than 10 days with iron deficiency anemia of hemoglobin levels of 6.5-8.4 g/dl. Common dosage of tranexamic acid of 1 gram daily in 4 divided doses on days 1-5 of their menstrual cycles did not correct their menorrhagia. The treatment was then changed to the daily dose of 3 grams in 4 divided doses on days 1-5 of their menstrual cycles. Thereafter, their menorrhagia became well-controlled with improvement of their anemia up to hemoglobin of 11.5-12.4 g/dl. High dose of tranexamic acid has been administered safely in all patients for 3-5 years without significant complications. Oral high-dose administration of tranexamic acid is very convenient and useful for treatment of menorrhagia in the patients with vWD.
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PMID:High dose of tranexamic acid for treatment of severe menorrhagia in patients with von Willebrand disease. 1291 7

von Willebrand disease (vWD) is the most common congenital bleeding disorder in the USA, affecting 1-3% of the population. Previously characterizing the bleeding symptoms in females with type 1 vWD, we evaluated 42 males with type 1 vWD, mean age 16 years (1-64), of whom 24 (57%) presented with bleeding symptoms. The most common initial symptom was postoperative bleeding (26%). The most common bleeding symptoms ever were epistaxis (53%), bruising (50%), postoperative bleeding (47%), haematomas (29%) and oral bleeding (29%). Of postoperative bleeding, ear/nose/throat (44%), dental (17%) and circumcision bleeding (22%) occurred at a median 10 years of age, despite a previous bleeding or family history in 89%. Complications included anaemia in five (12%), neurological sequelae after subdural haematoma and tonsillectomy in two (5%), transfusion-associated hepatitis C in two (5%) and degenerative joint disease after traumatic haemarthroses in one (2%). The bleeding time (BT) was prolonged in 83%, and the ristocetin cofactor (vW:RCoF) and factor VIII (FVIII:C) decreased in 64% and 43%, respectively. Haemarthroses and haematoma formation were associated with a longer activated partial thromboplastin time (APTT) (P < 0.05), and anaemia with a lower FVIII:C (P < 0.05). In 81%, a haemostatic response occurred with 1-8 deamino-d-arginine vasopressin (DDAVP), although, in 13%, surgical intervention was also required to achieve haemostasis. Postoperative bleeding could have been avoided in 89%, if a preoperative past bleeding history or family history had been obtained, and, in at least 94%, if a preoperative BT and APTT had also been performed. The failure to avoid postoperative bleeding and related complications in patients with vWD by taking a personal and family bleeding history constitutes a major public health problem.
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PMID:Bleeding manifestations in males with von Willebrand disease. 1496 5

The aim of the present study was to draw an outline of the clinical epidemiology of bleeding gastrointestinal angiodysplasias. The study includes a report of a case and of our series of patients with bleeding gastrointestinal angiodysplasias admitted, between 1993 and 2003, to a ward of Internal Medicine where digestive endoscopy is also performed. A review of the literature is also provided. An 80-year-old cirrhotic woman with aortic stenosis, was referred to our Department because of anemia due to melena. In spite of 13 hospitalizations during which numerous diagnostic procedures including endoscopy, X-ray studies, arteriography, labeled red blood cells scanning and laparotomy with intraoperative ileoscopy, the site and nature of the bleeding lesion remained unidentified. Her red blood cell requirement progressively increased from 6 U in 1993 to 24 U in 1994 to 40 U as of September 1995. Enteroscopy disclosed duodeno-jejunal angiodysplasia. The patient subsequently received 35 additional red blood cell units during 7 new admissions. Between 1993 and 2003, 24 patients were identified. They were mainly women and their average age was 77 years. Angiodysplasias were localized in the large bowel in 92% of cases. Comorbidities included: heart disease (79%), chronic liver disease (29%) and chronic renal failure (21%). One fourth of patients were under anticoagulant drugs or had a hemostatic blood disorder. All patients received blood transfusions and endoscopic treatment was performed in approximately half of the cases. The most relevant updates are related to the pathogenic relationship between aortic stenosis, von Willebrand's disease and bleeding gastrointestinal angiodysplasias, the hemostatic alterations associated with liver cirrhosis or with chronic renal failure and the diagnosis and treatment of bleeding gastrointestinal angiodysplasias. A better understanding of the clinical epidemiology of bleeding gastrointestinal angiodysplasias may facilitate their diagnosis and contribute to an effective clinical management.
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PMID:[Bleeding gastrointestinal angiodysplasias: our experience and a review of the literature]. 1531 67


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