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.
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During the s, molecular and cellular studies distinguished hemophilia A and vWD more precisely. Other coagulation factor assays may be performed depending on the results of a coagulation screen.
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.
Von Willebrand disease
Overview Platelets play a vital role in the blood-clotting process. Patients should address specific cboroba concerns with their physicians.
Von Willebrand factor is mainly active in conditions of high blood flow and shear stress. The genetic causes of milder forms of low vWF are still under investigation, and these forms may not always be caused by an abnormal vWF gene. An acquired form can sometimes result from other medical conditions. The ristocetin cofactor activity is decreased and high molecular weight large vWF multimers are present in the circulation.
Deficiency of vWF, therefore, shows primarily in organs with extensive small vesselssuch as skingastrointestinal tractand uterus.
It affects both male and female dogs equally, and while any dog can have vWD, there are several breeds that are at greater risk:. This section with questionable factual accuracy needs more medical references for verification or relies too heavily on primary sources. 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.
Patients with von Willebrand disease typically display a normal prothrombin time and a variable prolongation of partial thromboplastin time. Please review the contents of the article and add the appropriate references wiillebranda you can. Von Willebrand factor antigen vWF: This is due to the large vWF multimers remaining bound to the patient’s platelets.
The testing for vWD can be influenced coroba laboratory procedures. In milder cases of type 1 vWD, a complex spectrum of molecular pathology may exist in addition to polymorphisms of the vWF vpn alone.
Von Willebrand’s Disease in Dogs
The ristocetin cofactor assay is normal. Occasionally, type 2 also inherits recessively. It can arise from failure to secrete vWF into the circulation or from vWF being cleared more quickly than normal. Causal mutations for type 2 were identified in dogs of the breeds German Wirehaired PointerGerman Shorthaired Pointerand Chinese Crested ; and for type 3 in dogs of the breeds KooikerhondjeScottish Terrier and Shetland Sheepdog.
Genetic screening is offered for known breeds. Patients with this subtype are unable to use desmopressin as a treatment for bleeding, because it can lead to unwanted platelet aggregation and aggravation of thrombocytopenia.
Evaluation Indications Personal or Family History of significant bleeding see symptoms as above and Planned for surgical procedure with moderate to high risk of bleeding or Current bleeding symptoms or abnormal lab results Complete history and examination See Bleeding Disorder Symptoms suggestive of Bleeding Diathesis as listed above Medication causes of Bleeding Disorder e.
Hematology and Oncology – Coagulopathy Pages. Most cases are hereditary, but acquired forms of vWD have been described. These images are a random sampling from a Bing search on the term “Von Willebrand Disease. Platelet-type vWD also known as pseudo-vWD is an autosomal dominant genetic defect of the platelets. vin
Von Willebrand Disease
In pigs, the causal mutation for vWD type 3 has also been identified. Vob play a vital role in the blood-clotting process. Infusion of platelet concentrates is recommended for correction of hemorrhage associated with platelet-type vWD.
Type 2M vWD is a qualitative defect of vWF characterized by its decreased ability to bind to GPIb receptor on the platelet membrane and normal capability at multimerization. VWD can also affect dogs, pigs, and chooba. In dogs affected by type 1 vWD, the causal mutation was the same across all breeds and the same mutation was also detected in some human vWD type 1 patients.
In these strains, the vWF gene has been knocked out. Women may experience heavy menstrual periods and blood loss during childbirth. When treatment is necessary, it may include: Severe internal bleeding and bleeding into joints are uncommon in all but the most severe type, vWD type 3.
For the normal function of the coagulation factor, see von Willebrand factor. Differential Diagnosis See Bleeding Disorder.
This is a ” gain of function ” defect. The prevalence of vWD is about one in individuals. Diseases of clotting D50—69,74— Mice affected by vWD type 3 were produced by genetic engineering to obtain a small sized model for the human disease. Variant forms of vWF were recognized in the s, and these variations are now recognized as the result of synthesis of an abnormal protein.