Abstract

Background: Haemophilia is a disease of genetic origin, which causes a defect in blood coagulation. Under normal conditions, in the case of leakage from the blood vessels, the blood forms a clot that reduces or blocks the bleeding. This process involves the activation of several plasma proteins in a cascade-like species. Two of these proteins, produced in the liver, factor VIII and factor IX, are deficient or present a functional defect in people with haemophilia. Because of this deficit, the haemophiliacs easily suffer external and internal bleeding. Surgical treatment of these patients is to be observed, and often their treatment is delayed due to unclear guidelines and risks in treating these patients. The aim is to provide clear guidelines in the case of surgical treatment of these patients. Methods: In this study, we have considered all the guidelines that refer to the gold-maxillofacial surgery, focusing on the literature of the last 10 years. Results: Surely, this collection of guidelines will favor the choice of the clinician towards safer and predictable protocols. This study does not want to create a guideline but evaluates the literature of the last 10 years, and highlights the latest for the treatment of these patients., with the aim of informing the pathology and at the same time making the surgical maneuvers safer. Conclusions: Despite the research of literature has produced few results, it was nevertheless possible to draw up a guideline thanks to additional information extrapolated from textbooks and other scientific articles. According to the guidelines, it is possible to proceed to the treatment of these patients, if with appropriate therapy in a safe and risk-free manner.

Highlights

  • Haemophilias are hereditary coagulopathies whose basic anomaly consists of the quantitative or qualitative alteration of one or more plasma proteins in the coagulation system

  • As already mentioned in the introduction, haemophilia is a recessive hereditary disorder that causes a serious deficiency in blood coagulation due to the total or partial lack of factor VIII

  • In haemophilia type A the deficit can be quantitatively variable, so as to induce different clinical pictures: with functional levels of factor VIII up to 2% (i.e., A severe) haematomas are frequent and, in childhood, the spontaneous hemarthrosis; with factor VIII levels ranging from 2 to

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Summary

Introduction

Haemophilias are hereditary coagulopathies whose basic anomaly consists of the quantitative or qualitative alteration of one or more plasma proteins in the coagulation system. Haemophilia A has an incidence estimated at 1: 20,000–100,000 male births per year, of which the 30% of cases without familiarity (largely attributable to new mutations). In the case of leakage from the blood vessels, the blood forms a clot that reduces or blocks the bleeding. This process involves the activation of several plasma proteins in a cascade-like species. IX, are deficient or present a functional defect in people with haemophilia Because of this deficit, the haemophiliacs suffer external and internal bleeding. The aim is to provide clear guidelines in the case of surgical treatment of these patients

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