Willebrand Juergens Syndrome

Willebrand-Jürgens Syndrome: Causes

Pathogenesis (disease development) Willebrand-Jürgens syndrome refers to a defect or deficiency of von Willebrand factor (vWF). For the various forms of Willebrand-Jürgens syndrome, see “Introduction.” Etiology (Causes) Biographic causes Genetic burden from parents, grandparents. Disease-related causes (= acquired). Blood, blood-forming organs – immune system (D50-D90). Autoimmunological diseases, unspecified Neoplasms – tumor diseases (C00-D48). Malignant lymphoma … Willebrand-Jürgens Syndrome: Causes

Willebrand-Jürgens Syndrome: Therapy

General measures Individuals with Willebrand-Jürgens syndrome should obtain an identification card and carry it with them at all times! Injections should be administered intravenously and/or subcutaneously. There should always be very careful hemostasis after injury/surgery. Review of permanent medication due topossible effect on the existing disease. Vaccinations The vaccinations recommended by the STIKO should be … Willebrand-Jürgens Syndrome: Therapy

Willebrand-Jürgens Syndrome: Drug Therapy

Therapeutic target Hemostasis or hemorrhage prophylaxis. Therapy recommendations See specifics for the following agents depending on the type of Willebrand-Jürgens syndrome. Caution. Acetylsalicylic acid (ASA) is contraindicated in patients with Willebrand-Jürgens syndrome. Type Active ingredients Special features 1 + 2 Desmopressin For light bleeding 3 Desmopressin Continuous infusion during surgery (OP) Factor VIII/von Willebrand factor … Willebrand-Jürgens Syndrome: Drug Therapy

Willebrand-Jürgens Syndrome: Symptoms, Complaints, Signs

The following symptoms and complaints may indicate Willebrand-Jürgens syndrome: Main symptoms Tendency to bleed for long periods of time; manifests primarily after surgery (e.g., tooth extraction) Tendency to roßflächige hematomas (bruises). Menorrhagia – bleeding is prolonged (> 6 days) and increased Frequent recurrent (always recurring) epistaxis (nosebleeds). Hemarthros – bleeding into the joint. Mucosal bleeding … Willebrand-Jürgens Syndrome: Symptoms, Complaints, Signs

Willebrand-Jürgens Syndrome: Medical History

Medical history (history of illness) represents an important component in the diagnosis of von Willebrand-Jürgens syndrome. Family history What is the general health status of your relatives? Are there any diseases in your family that are common? Are there any hereditary diseases in your family? Social history What is your profession? Current medical history/systemic history … Willebrand-Jürgens Syndrome: Medical History

Willebrand-Jürgens Syndrome: Complications

The following are the most important diseases or complications that may be contributed to by Willebrand-Jürgens syndrome: Cardiovascular system (I00-I99) Aortic valve stenosis (Heyde syndrome) – narrowing of the aortic valve (heart valve between the left ventricle(heart chamber) and the aorta (main artery)); often associated with homozygous occurrence of von Willebrand-Jürgens syndrome. Musculoskeletal system and … Willebrand-Jürgens Syndrome: Complications

Willebrand-Jürgens Syndrome: Examination

A comprehensive clinical examination is the basis for selecting further diagnostic steps: General physical examination – including blood pressure, pulse, body weight, height; furthermore: Inspection (viewing). Skin, mucous membranes, and sclerae (white part of the eye) [extensive hematoma (bruising)]. Measurement of joint mobility and range of motion of the joint (according to the neutral zero … Willebrand-Jürgens Syndrome: Examination

Willebrand-Jürgens Syndrome: Test and Diagnosis

Laboratory parameters of 1st order – obligatory laboratory tests. Small blood count Differential blood count Coagulation parameters – bleeding time [↑], PTT [↑], Quick [normal]. Determination of clotting factors: VWF (von Willebrand factor; synonyms: Clotting factor VIII-associated antigen or von Willebrand factor antigen, vWF-Ag). VIII (hemophilia A) IX (Hemophilia B) Laboratory parameters 2nd order – … Willebrand-Jürgens Syndrome: Test and Diagnosis