Review Dental Treatment of Patients with Congenital Bleeding Disorders

Authors: J. Papež;  K. Chleborád;  T. Dostálová
Authors‘ workplace: Stomatologická klinika dětí a dospělých 2. LF UK a FNM, Praha
Published in: Česká stomatologie / Praktické zubní lékařství, ročník 114, 2014, 6, s. 109-115
Category: Review


The commonest congenital bleeding disorders in childhood are hemophilia, von Willebrand disease and deficiency of factor XI. Disease is characterized by a deficient of coagulability. It is a gonosomal recessive condition. There are two main types of hemophilia: Hemophilia A is characterized by a deficiency of factor VIII (FVIII) and hemophilia B is characterized by a deficiency of factor IX (FIX). Hemophilia is an X-linked hereditary disorder and affects predominantly males because of their hemizygous (karyotyp 46, XY). Females – heterozygot (karyotyp 46, XX) are transmitter of gene for hemophilia and they are rarely affected unless there is an unequal lyonisation of the X – chromosome. There are two other common diseases which are needed to mention: von Willebrand disease and factor XI deficiency. Von Willebrand disease is the commonest congenital bleeding disorder on the other hand deficiency of factor XI is not so common. Both of them have similar clinical manifestation a treatment such as hemophilia. The provision of dental treatment in patients with congenital bleeding disorders has often been neglected. In past most treatments were carried out under general anesthesia and usually the only one treatment plan was extraction followed by provision of dentures. Since the introduction of coagulation factor, use new local hemostatic agents, improvement of operative methods, minimal invasive therapy and an increasing interest in the preventive of dental problems started new era in dental care for patients with congenital bleeding disorders. Nowadays use doctor during the intervention, replacement therapy to increase level of FVIII, FIX, von Willebrand factor and factor XI prophylactic factor with combination fibrin glue, antifibrinolytic agents and other agents which make treatment faster, safe and more comfortable. Point of this article is to provide other dentists general informatics about healing patients with congenital bleeding disorders.

hemophilia A – hemophilia B – von Willebrand disease – factor XI deficiency – orthodontic treatment – anesthesia – periodontal disease – oral surgical procedures – dental extraction – fibrin glue


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Maxillofacial surgery Orthodontics Dental medicine
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