Choroba von Willebranda u dzieci

*Anna Klukowska¹, Paweł Łaguna¹, Katarzyna Skrzypczyk-Smalisz¹, Renata Koziołek²

Streszczenie
Aim:The objective of the study was to evaluate the number of patient registered in Out-patient Bleeding Disorders Department and the type od disease, to analyse the bleeding symptoms and type and course of surgical procedure in children with vWD.
Material and methods: All children with von Willebrand disease registered in Out-patient Bleeding Disorders Department were evaluated. The diagnose was based on laboratory tests: factor VIII level, activity of von Willebrand factor (vWF:RCo) and antigen of von Willebrand factor (vWF:Ag). Analised surgery procedures were done in Otolaryngological Department.
Results: The disease was diagnosed in 221 cases. In 154 children type 1 was found, in 34 type 2 and type 3 in 33 children. Among all the patients typical symptoms for the vWD were predominately observed: mouth and nose mucose bleedings. The image of the disease was differentiated according to type of vWD. Bleedings were not registered in more than half patients in type 1 but in all patients with type 3 were present. Moreover haemarthroses appeared in 29% of all children with type 3 disease. Age when the disease was diagnosed was related to the type of vWD. In total 76 different surgical procedure were performed. No bleeding complications were noted.
Conclusions: 1. In von Willebrand disease type 3 the bleedings are leading symptoms whereas type 1 is often asymptomatic and the diagnosis is made by chance. 2. Surgical procedure can be done safely in patients with vWD when proper treatment is given during and after surgical period.

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