Von Willebrand Disease: diagnosis and management
| Autor: | Michael Williams, Jesal Patel |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities medicine.medical_specialty Bleeding episodes Antifibrinolytic biology medicine.drug_class business.industry Excessive Bruising medicine.disease Haemophilia Laboratory testing Surgery Von Willebrand factor hemic and lymphatic diseases Pediatrics Perinatology and Child Health biology.protein medicine Von Willebrand disease Intensive care medicine business Prophylactic treatment |
| Zdroj: | Paediatrics and Child Health. 25:354-359 |
| ISSN: | 1751-7222 |
| DOI: | 10.1016/j.paed.2015.05.005 |
| Popis: | Von Willebrand Disease is a common cause of excessive bruising and bleeding in children. Its diagnosis can be challenging, not only because of the various subtypes of the disorder but because of the considerable overlap between VWD and normal individuals. Laboratory diagnosis requires a range of quantitative and qualitative tests of the VWF protein, with targeted gene analysis increasingly used to confirm the diagnosis of type 2 and type 3 VWD. Bleeding Assessment Tools may be helpful in directed laboratory testing but are often less so in young children who have had limited haemostatic challenges. The diagnosis and management of affected children is best undertaken by specialist Haemophilia Centres and, as with all bleeding disorders, will often require a multidisciplinary approach. Treatment for VWD may require the use of antifibrinolytic drugs, vasopressin or VWF-containing plasma concentrates: treatment is often on-demand for individual bleeding episodes but there are specific indications for the use of prophylactic treatment in some children. Given its prevalence and presenting symptoms, VWD should always be considered in the assessment of children suspected of non-accidental injury. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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