Haemophilia is a rare, X-linked congenital bleeding disorder, characterised by a deficiency of coagulation factor VIII (FVIII), known as haemophilia A, or factor IX (FIX), known as haemophilia B. These factor deficiencies are the result of pathogenic variants in the F8 and F9 clotting factor genes1. Haemophilia is predominantly manifested in males, while female individuals are typically heterozygous carriers of a mutated gene2.
Haemophilia is characterised by spontaneous bleeding episodes that most often occur in muscles (haematomas) or joints (haemarthrosis). The severity of the disease is categorised based on the level of residual FVIII or FIX activity in the plasma: severe (< 1 international unit (IU)/dl), moderate (1–5 IU/dl) or mild (5–40 IU/dl)1. Spontaneous bleeding is common in severe haemophilia, whereas in moderate or mild haemophilia, bleeding is most usually initiated by a provoking factor such as injury, surgery or tooth extraction. Carriers usually show no symptoms3.
Both FVIII and FIX deficiency result in reduced or absent activity of the Xase complex preventing the formation of activated factor X (FXa). The manifestation of haemophilia A and B is virtually indistinguishable on the basis of symptoms4.
In the context of dental considerations, observation of an unexpected bleeding episode following dental procedure, even more commonly following oral injury or in course of tooth eruption in children, may attract attention for the presence of inherited bleeding disorders (IBD). The role of dentists in the recognition of these conditions is of utmost relevance1,5,6,7.
It has been reported in anecdotally that elderly individuals with haemophilia who underwent dental extractions during childhood had experienced formation of substantial, friable clots in the dental socket in the absence of treatment. Moreover, heavy bleeding returned after a temporary cessation following tooth extraction (“re-bleeding”)3. This phenomenon can be attributed to the fact that patients with haemophilia (PWH) exhibit relatively normal initiation and amplification phases of coagulation. Consequently, these patients are able to form an initial platelet plug at the site of bleeding; however, they are unable to generate a sufficient thrombin burst at the platelet surface that is necessary to stabilise the initial platelet plug into a firm fibrin clot8,9.
Haemophilia is one of the most prevalent IBD, yet these patients represent a minority of the general population10. Consequently, dentists may not acquire confident knowledge and treatment practice11,12. The risk of blood-borne infections resulting from earlier plasma-derived factor concentrates decreased the eagerness of dentists to provide care for PWH13. Nevertheless, effective oral hygiene practices and regular dental examinations are of paramount importance in preventing dental diseases, a fact that assumes even greater significance in the context of the haemophilia patient population7,14. Prolonged bleeding following dental treatment in PWH has the potential to result in serious, and occasionally life-threatening complications. Consequently, it is advised that invasive procedures should be avoided wherever feasible. It is imperative that dentists are aware of the level of risk associated with each procedure, in order to minimise the possibility of complications arising from bleeding15,16. Moreover, establishment of an effective professional contact between dentists and haematologists are supposed to facilitate the planning of preventive measures and to discuss optimal haemostatic management. This collaborative approach is crucial in ensuring the minimization of complications in patients.
Previous studies, including our recently published survey, reported that PWH had difficulties accessing dental services11,17,18,19,20,21,22. Most studies focused on the patients’ perspective, except of a single report from the UK incorporating the views of dentists, too11.
The objective of this study was to examine the attitudes and treatment practices of Hungarian dentists in treating PWH. We focused on primary care, both government funded and private practices, as the majority of patients do not require advanced level care and can be treated in general dental practice (GDP) by adhering to established protocols. Furthermore, dentists working in primary care are the first and most appropriate point of contact for patients within the current Hungarian health insurance system.
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