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  2. Discover a treatment that can help manage perioperative bleeding for adults with VWD.

  1. May 20, 2019 · 1 INTRODUCTION. Von Willebrand disease (VWD), the most frequent autosomal inherited bleeding disorder, is caused by quantitative or qualitative defects of von Willebrand factor (VWF), an adhesive protein that binds platelets to exposed sub-endothelium and carries factor VIII (FVIII) in circulation. 1, 2 As a consequence, in addition to the defect of VWF, also FVIII, the protein deficient in ...

    • Giancarlo Castaman, Paula D James
    • 17
    • 2019
    • Prevalence
    • Diagnosis
    • Complications
    • References

    Von Willebrand disease (VWD) occurs with equal frequency among men and women, affecting up to 1% of the general population.1,2However, women are more likely to experience symptoms of VWD because of...

    In a CDC survey of women with VWD: [Read articleexternal icon] 1. There was an average of 16 years between the onset of their bleeding symptoms and diagnosis of a bleeding disorder. 2. Women reported an average of 6 bleeding symptoms before a diagnosis of VWD was made. Common symptoms included menorrhagia, bruising, nosebleeds, and bleeding from surgery, injury, and delivery. Menorrhagia was the most commonly reported symptom. 3. To obtain the diagnosis of VWD, tests were carried out on average twice (range 1–20 times). 4. 38% of women reported that they were first diagnosed by a Hemophilia Treatment Center (HTC) doctor. Among women diagnosed by a provider other than an HTC doctor, 42% were diagnosed by a hematologist, 28% by an internist or a family doctor and 19% by a gynecologist.

    In a CDC study of 102 women with all types of VWD compared to 88 controls: [Read articleexternal icon] 1. The most commonly reported bleeding symptoms among women with VWD were: 1.1. Heavy menstrual bleeding (95%) 1.2. Bleeding after minor injuries (92%) 1.3. Excessive gum bleeding (76%) 2. 74% of women with VWD reported excessive bleeding from several sites (e.g. nose, gum and uterus) or following several procedures or injuries (e.g. dental, surgical, childbirth and minor injury) compared to 6% of controls. 3. 41% of women with VWD reported a diagnosis of migraine headaches compared with 13% of controls. 4. 37% of women with VWD reported a diagnosis of arthritis compared with 15% of controls. 5. 37% of women with VWD compared with 10% of controls indicated that their menstrual period limited routine work, social activities and had a negative effect on life. 6. More women with VWD than controls in this study had undergone hysterectomy (25% vs. 9%). In studies of 42 men and 38 women...

    Rodeghiero F, Castaman G, Dini E. Epidemiological investigation of the prevalence of von Willebrand’s disease. Blood. 1987 Feb;69(2):454-9.
    Werner EJ, Broxson EH, Tucker EL, et al. Prevalence of von Willebrand disease in children: a multiethnic study. J Pediatr 1993 Dec; 123(6): 893-8.
    Ragni MV, FA Bontempo, C Hassett. Von Willebrand disease and bleeding in women. Haemophilia 1999; 5:313-317.
    Ziv O, Ragni MV. Bleeding manifestations in males with von Willebrand disease. Haemophilia 2004; 10:162-8.
  2. Von Willebrand disease (VWD) is a bleeding disorder in which your blood has trouble clotting. It is inherited, meaning it is passed down from parents to their children. People with von Willebrand disease sometimes experience heavier-than-normal bleeding from injury, surgery, and, in women, menstrual flow and childbirth.

    • Types
    • Causes
    • Symptoms
    • Diagnosis
    • Treatment
    • Prevention
    VWD Type 1:is the most common form. People with type 1 have lower levels of VWF to normal.
    VWD type 2:is characterized by a defect in the structure of VWF. The protein does not function properly, causing lower than normal activity.
    VWD type 3:It is usually the most severe. People with Type 3 VWD may have to bleed in joints and muscles, without being afflicted by an injury.

    Like hemophilia, von Willebrand’s disease is a genetic disorder that gets passed from parents to children. The son of a man or a woman with von Willebrand’s disease has a 50% chance of inheriting the disease gene. In types 1 and 2, the child has inherited the disease gene from one parent. In class 3, the son has inherited disease genes from both parents.

    Many people suffering from this disease have almost no symptoms or are mild or moderate. Symptoms usually begin in childhood and continue throughout life. But when they do occur, the severity varies from person to person. Many only notice the symptoms when they take aspirin or similar drugs that interfere with clotting. Common symptoms include: 1. Easy bruising. 2. Frequent or prolonged nosebleeds. 3. Prolonged bleeding gums and minor cuts. 4. Heavy or prolonged bleeding during menstruation. 5. Bleeding in the digestive system. 6. Prolonged bleeding after injury, surgery, or invasive dental procedures.

    The doctor will proceed with a physical examination, he asked about their symptoms and medical history. Von Willebrand’s disease can be challenging to diagnose. Low levels of von Willebrand factor and bleeding do not always mean that the person has the condition. Probably because it is a bleeding disorder, blood tests focusing on the following criteria are a need: 1. Bleeding time – in this disease is prolonged, particularly after taking an aspirin. 2. Factor VIII antigen – which indirectly measures levels of VWF in their blood; Thanks to this disease, it gets reduced. 3. Willebrand factor multimers – examines the different structural types of VWF in the blood; due to this condition, it gets reduced.

    Most affected people do not need surgery and if required will depend on the type VW suffering and its severity. It is only necessary if you have a dental or surgical procedure that may cause bleeding. The aim of the treatment focuses on correcting the prolonged bleeding time and if the blood clotting disorder is present. To achieve this, both von Willebrand factor and factor VIII activity procoagulant should get raised to normal levels in the plasma. You should get an average concentration of von Willebrand factor that is functionally normal in patients with qualitative abnormalities of the protein. There are two trends in the treatment of this disease: The replacement therapy. In some patient’s replacement therapy can induce the formation of inhibitory antibodies that can complicate the treatment of bleeding episodes making it impossible to correct the abnormality when the titer inhibitors are very high. Induce the release of compound tissue stores using Desamino-8-Arginine Vasopre...

    There are no guidelines for preventing disease. It is advised to consult a genetic counselor to help review detailed family history and discuss risks and tests available for von Willebrand disease.

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  4. Bleeding disorders, such as leukemia, platelet disorders, clotting factor deficiencies, or (less common) von Willebrand disease Pregnancy complications (miscarriage) Common causes of dysmenorrhea (menstrual pain) include: 6 , 9

  5. Sep 19, 2012 · Prevention of von Willebrand's Disease. Von Willebrand's disease is inherited and this is the reason why it simply cannot be prevented. In case parents have a child suffering from this condition, they may have genetic counseling, especially if they plan to have more children.

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