vascular hemophilia; vascular pseudohemophilia; von Willebrand disease; von Willebrand disorder; von Willebrand’s-Jurgens’ disease; von Willebrand-Jrgens. The activity of vWF in a patient with von Willebrand disease. Aktywość vWF u pacjenta z chorobą Willebranda. Fig. 4. The activity of vWF in a. Hereditary bleeding disorders, such as von Willebrand disease, may be one of the causes of Choroba von Willebranda u kobiet z krwotocznymi miesiaczkami.

Author: Nigar Akikinos
Country: Puerto Rico
Language: English (Spanish)
Genre: Education
Published (Last): 15 April 2007
Pages: 236
PDF File Size: 20.14 Mb
ePub File Size: 2.10 Mb
ISBN: 386-9-24581-527-2
Downloads: 60900
Price: Free* [*Free Regsitration Required]
Uploader: Narisar

For women with willebrahda menstrual bleeding, estrogen-containing oral contraceptive medications are effective in reducing the frequency and duration of the menstrual periods. Journal of the American College of Cardiology. Additional laboratory tests that help classify sub-types of vWD include von-willebrand multimer analysis, modified ristocetin induced platelet aggregation assay and vWF propeptide to vWF antigen ratio propeptide.

Platelet-type vWD also known as pseudo-vWD is an autosomal dominant genetic defect of the platelets.

Symptoms include any of the following: The vWF is qualitatively normal and genetic testing of the von Willebrand gene and vWF protein reveals no mutational alteration. Platelets play a vital role in the blood-clotting process. Often, the discovery of vWD occurs incidentally to willebranad medical procedures requiring a blood work-up.

There’s a name for this: Skip to main content. Disease or Syndrome T In more severe cases of type 1 vWD, genetic changes are common within the vo gene and are highly penetrant.


Although access to this website is not restricted, the information found here is intended for use by medical willebrand. Retrieved from ” https: This article needs additional citations for verification. The prevalence of clinically significant cases is one per 10, If a dog has an injury or wound, the platelets help stop bleeding and speed up the healing process by clotting at the site of the injury.

The good news is, many dogs with vWD live a long and healthy life. In this case, the function of vWF is not inhibited, but the vWF-antibody complex is rapidly cleared from the circulation. This is due to the large vWF choroha remaining bound to the patient’s platelets. Views Read Edit View history. Related links to external sites from Bing.

Only small multimer units are detected in the circulation. This section with questionable factual accuracy needs more medical references for verification or relies too heavily on primary sources.

Von Willebrand disease

Desmopressin is contraindicated in vWD type 2b because of the risk of aggravated thrombocytopenia and thrombotic complications. Type 3 is inherited as autosomal recessive. Most cases of type 1 vWD are never diagnosed due to the asymptomatic or mild presentation of type I and most people usually end up leading a normal life free of complications, with many being unaware that they have the disorder.

Patients should address specific medical concerns with their physicians. Desmopressin is a synthetic analog of the natural antidiuretic hormone vasopressin. This has led to some vWD type 2N patients being misdiagnosed as having hemophilia A.


Von Willebrandova choroba – WikiSkripta

A form of vWD occurs in patients with aortic valve stenosisleading to gastrointestinal bleeding Heyde’s syndrome. A platelet aggregation assay will show an abnormal response to ristocetin with normal responses to the other agonists used.

However, some individuals heterozygous for type 3 may be diagnosed of vWD type 1, indicating an intermediate inheritance in that cases. Desmopressin stimulates the release of vWF from the Weibel-Palade bodies of endothelial cellsthereby increasing the levels of vWF as well as coagulant factor VIII three- to five-fold.

In the s, it became clear that a “plasma factor”, factor VIIIwas decreased in these persons and that Cohn fraction I-0 could correct both the plasma deficiency of FVIII and the prolonged bleeding time. Large vWF multimers are willehranda or absent from the circulation.

Variant forms of vWF were recognized in the s, and these variations are now recognized as the result of synthesis of an abnormal protein. Specialized tests are required to diagnose vWD; specifically, your veterinarian may recommend these tests as part of a preanesthetic screening if your dog is considered at-risk.