RESEARCH PAPER
Epidemiological analysis of 22 patients with congenital bleeding diathesis hospitalized in the Department of Maxillofacial Surgery in Lublin, Poland, 2008–2013
1
Maxillofacial Surgery Department, Medical University, Lublin, Poland
2
Haematooncology Department, Medical University, Lublin, Poland
Corresponding author
Jolanta Wojciechowicz
Maxillofacial Surgery Department, Medical University of Lublin, Staszica 11, 20-081 Lublin, Poland
Maxillofacial Surgery Department, Medical University of Lublin, Staszica 11, 20-081 Lublin, Poland
J Pre Clin Clin Res. 2016;10(1):45-49
KEYWORDS
ABSTRACT
Introduction:
Haemophilia A and B are congenital bleeding disorders caused by coagulation factor VIII or IX deficiency. Haemophilia A and B occur in men, like all qualities dependent on genes linked to gender, whereas women are asymptomatic carriers of the gene. The most dangerous manifestations of severe haemophilia include spontaneous haemorrhages into the joints, muscles and body cavities, haematuria and potentially very dangerous intracranial haemorrhages are also quite frequent. The most common manifestations in the region of the maxillofacial skeleton and oral cavity are recurring bleedings from the nose and the gums after dental extractions, as well as extensive haemorrhages from wounds after surgical procedures.
Objective:
The aim of the study was the analysis of epidemiologic case records of 22 patients affected by various types of haemophilia, admitted to Maxillofacial Surgery Department at Medical University in Lublin, Poland, during 2008–2013.
Results:
The most numerous group constituted patients diagnosed with Haemophilia A, followed by patients with von Willebrand disease and Haemophilia B, aged 21–40 years. The main cause of hospital admission was the need to perform complex oral cavity sanation. The patients were treated with factor VIII or Factor IX, FEIBA or Heamate P, depending on the type and severity of bleeding diathesis.
Haemophilia A and B are congenital bleeding disorders caused by coagulation factor VIII or IX deficiency. Haemophilia A and B occur in men, like all qualities dependent on genes linked to gender, whereas women are asymptomatic carriers of the gene. The most dangerous manifestations of severe haemophilia include spontaneous haemorrhages into the joints, muscles and body cavities, haematuria and potentially very dangerous intracranial haemorrhages are also quite frequent. The most common manifestations in the region of the maxillofacial skeleton and oral cavity are recurring bleedings from the nose and the gums after dental extractions, as well as extensive haemorrhages from wounds after surgical procedures.
Objective:
The aim of the study was the analysis of epidemiologic case records of 22 patients affected by various types of haemophilia, admitted to Maxillofacial Surgery Department at Medical University in Lublin, Poland, during 2008–2013.
Results:
The most numerous group constituted patients diagnosed with Haemophilia A, followed by patients with von Willebrand disease and Haemophilia B, aged 21–40 years. The main cause of hospital admission was the need to perform complex oral cavity sanation. The patients were treated with factor VIII or Factor IX, FEIBA or Heamate P, depending on the type and severity of bleeding diathesis.
REFERENCES (16)
1.
Knobe K, Berntorp E. New treatments in hemophilia: insights for the clinician. Ther Adv Hematol. 2012; 33: 165–175.
2.
Windyga J. Haemophilias- advances in diagnosis and management. Acta Haematol Pol. 2010; 41(2): 183–199.
3.
Windyga J, Solano M, Trujill, Hafeman A. BAX326 (RIXUBIS): a novel recombinant factor IX for the control and prevention of bleeding episodes in adults and children with hemophilia B. Ther Adv Hematol. 2014; 5(5): 168–180.
4.
Anderson JAM, Brewer A, Creagh D, Hook S, Mainwaring J, McKenan A, Yee TT, Yeung CA. Guidance on the dental management of patients with haemophilia and congenital bleeding disorders. British Dental J. 2013; 215(10): 497–504.
5.
Windyga J, Chojnowski K,Klukowska A, Łętowska M, Mital A, Podolak-Dawidziak M, Zdziarska J, Zawilska J. Polish guidelines for the management of inherited bleeding disorders caused by coagulation factor deficiency. Acta Haematol Pol. 2008; 39(3): 537–564.
6.
Windyga J. How will haemophilia be treated in future? Acta Haematol Pol. 2008; 39(4): 651–660.
7.
Israels S, Schwetz N, Boyar R, McNicol A. Bleeding Disorders: Characterization. Dental Considerations and Management. J Can Dent Assoc. 2006; 72: 9.
8.
Van Bladel ER, Roest M, de Groot PG, Schutgens R. Up-regulation of platelet activation in hemophiliA. www.haematologica.org/content/96/6/888, 2011; 96(6): 888–895.
9.
Salem K, Eshghi P. Dental health and oral health-related quality of life in children with congenital bleeding disorders. Haemophilia. 2013; 19: 65–70.
10.
Ministerstwo Zdrowia. Narodowy program leczenia chorych na hemofilie i pokrewne skazy krwotoczne na lata 2012–2018. (in Polish).
11.
Sumera A. Polskie Stowarzyszenie Chorych na Hemofilię. Biuletyn informacyjny 2012; 2(43). (In Polish).
12.
Zanon E, Brandolin B, Saggiorato G, Bacci Ch. Complex dental extractions in a patient with severe haemophilia A and inhibitors treated with activated prothrombin complex concentrate. Blood Transfus. 2012; 10: 225–7.
13.
Wojciechowicz J, Maślanko G, Dobieżańska B, Tomaszewski T. Therapeutic procedures of congenital haemorrhagic diathesis in patients hospitalised at the Maxillo-Facial Surgery Departament of Medical University of Lublin During 1981–2007. Pol J Environ Stud. 2008; 17, 6A, 1: 120–125.
14.
Peisker A, Raschke G-F, Schultze-Mosgau S. Management of dental extraction in patients with Haemophilia A and B: A report of 58 extractions. Med Oral Patol Oral Cir Bucal. 2014; 1,19 (1): 55–60.
15.
Gupta A, Epstein J, Cabay R. Bleeding Disorders of Importance in Dental Care and Related Patient Management. J Can Dental Assoc. 2007; 73(1).
16.
Sokołowska B, Walter-Croneck A, Gromek T, Dmoszynska A. Acquired hemophilia and life- threatening bleeding -Difficult problem that affects not only males but also females. Acta Haematol Pol. 2011; 42(3): 583–591.
| eISSN: | 1898-7516 |
| ISSN: | 1898-2395 |
We process personal data collected when visiting the website. The function of obtaining information about users and their behavior is carried out by voluntarily entered information in forms and saving cookies in end devices. Data, including cookies, are used to provide services, improve the user experience and to analyze the traffic in accordance with the Privacy policy. Data are also collected and processed by Google Analytics tool (more).
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
