Can Hemophilia Impact on the Oral Health Conditions of Children and Adolescents? A Systematic Review and Metanalysis


  • Leandro Tavares da Silva
  • Juliana Feltrin de Souza
  • Letícia Maíra Wambier
  • José Vítor Nogara Borges Menezes
  • Cassius de Carvalho Torres-Pereira
  • Fabian Calixto Fraiz


Blood Coagulation Disorders, Study Characteristics, Oral Health


Objective: To analyze if the oral health conditions in children and adolescents are associated with hemophilia (PROSPERO-42020168192). Material and Methods: The search strategy was performed in PubMed, Scopus, Lilacs/BBO, Web of Science, Cochrane, and Grey literature databases. Two independent researchers assessed the risk of bias in these studies by the Newcastle-Ottawa Scale. For the meta-analysis, the clinical conditions data were extracted as numerical variables according to their indexes, such as dental caries experience (dmft/DMFT), gingival condition (Modified Gingival Index - IGM), and oral hygiene (Plaque Index - PI). The quality of the evidence of the meta-analysis was evaluated by the GRADE tool (GRADEproGDT). Results: From a total of 431 studies, 27 were included, and 10 were included in the meta-analysis. The studies presented a moderate risk of bias, ranging from 2 to 7 points. The dental caries experience in primary (-0.62; CI95%: -1.68–0.43) and permanent dentitions (-0.05; CI95%: -0.69–0.59), gingival condition (-0.12; CI95%: -0.27–0.03), and oral hygiene (0.36; CI95%: -0.06–0.77) did not differ between the groups. Conclusion: Based on studies with very weak evidence, there were no differences in the oral health conditions of children and adolescents with and without hemophilia.


Srivastava A, Brewer A, Mauser-Bunschoten Eea. Guidelines for the management of haemophilia. Haemophilia 2013; 19(1):e1-47.

World Federation of Hemophilia. Report on the Annual Global Survey 2018. Canada; 2019.

Montalvão S, Sternick G, Flores T, Saraiva A, Rezende S. Manual de testes de hemostasia. Brasília: Ministério da Saúde 2010. [In Portuguese].

Othman NA, Sockalingam SN, Mahyuddin A. Oral health status in children and adolescents with haemophilia. Haemophilia 2015; 21(5):605-11.

Leite RB, Nascimento RNM, Soares RSC, Marssoni ACLT, Barboza CAG, Gomes RCB. Knowledge of dentists in the care of patients with hereditary coagulopathies. RFO UPF 2018; 23(2):150-5.

Babu NSV, Shah M, Patel P. Oral health status in children with haemophilia - a comparative study. J Haem Pract 2016; 3(2):43-7.

Baskirt EA, Albayrak H, Ak G, Erdem AP, Sepet E, Zulfikar B. Dental and periodontal health in children with hemophilia. J Coagul Disord 2010; 2(2):1-4.

Schaffer R, Duong MT, Wachter B, Arana E, Frances D. Access to dental care for people with bleeding disorders: survey results of hemophilia treatment centers in the U.S. Spec Care Dentist 2016; 36(6):295-9.

Boyd D, Kinirons M. Dental caries experience of children with haemophilia in Northern Ireland. Int J Paediatr Dent 1997; 7(3):149-53.

Jangra B, Goswami M. Assessment of dental caries experience and periodontal health status among children with haemophilia in New Delhi, India - a case control study. Oral Health Prev Dent 2017; 15(2):131-7.

Zaliuniene R, Aleksejuniene J, Peciuliene V, Brukiene V. Dental health and disease in patients with haemophilia - a case-control study. Haemophilia 2014; 20(3):e194-e8.

Azhar S, Yazdanie N, Muhammad N. Periodontal status and IOTN interventions among young hemophiliacs. Haemophilia 2006; 12(4):401-4.

Kozma A, Clevet O, Traila A, Ursu E, Jinca C, Oancea R, et al. The impact of replacement therapy on dental health in haemophilia. Rom J Leg Med 2019; 27(2):200-4.

Kumar M, Pai KM, Kurien A, Vineetha R. Oral hygiene and dentition status in children and adults with hemophilia: A case-control study. Spec Care Dentist 2018; 38(6):391-4.

Mazzoni A, Pignatari SSN. Prevalência de cárie e aspectos periodontais em crianças e adolescentes portadores de hemofilia tipo A e B. Rev Paul Odontol 2009; 31(2):27-30. [In Portuguese].

Mielnik-Blaszczak M. Evaluation of dentition status and oral hygiene in Polish children and adolescents with congenital haemorrhagic diatheses. Int J Paediatr Dent 1999; 9(2):99-103.

Dogan C, Sasmaz I, Antmen B, Seydaoglu G, Yazicioglu I. Physical and perceptional oral health status of children with hemophilia A. Haemophilia 2010; 16:51.

Hermida Bruno ML, Álvarez Loureiro L, Lewis W, Gabriel L, Boggia Tutaglio B, Segovia A, et al. Situación de salud oral de niños uruguayos portadores de coagulopatías hereditarias: Centro Hospitalario Pereira Rossell, Montevideo, Uruguay. Univ Odontol 2011; 30(64):31-5. [In Spanish].

Salem K, Seyyedkhamesi S, Aminian M. Evaluation of oral and dental health status in hemophilic children and adolescents in the city of Rasht. J Pediatr Res 2018; 5(4):182-6.

Sonbol H, Pelargidou M, Lucas VS, Gelbier MJ, Mason C, Roberts GJ. Dental health indices and caries-related microflora in children with severe haemophilia. Haemophilia 2001; 7(5):468-74.

Yazicioglu I, Deveci C, Çiftçi V, Antmen B, Doğan MC. Parent’s report on oral health-related quality of life of children with haemophilia. Haemophilia 2019; 25(2):229-35.

Zaliuniene R, Aleksejuniene J, Brukiene V, Peciuliene V. Do hemophiliacs have a higher risk for dental caries than the general population? Medicina 2015; 51(1):46-56.

Moher D, Liberati A, Tetzlaff J, Altman D, PRISMA Group. Preferred reporting items for systematic reviews and meta-analysis: the PRISMA statement. J Clin Epidemiol 2009; 62(10):1006-12.

Lo C, Mertz D, Loeb M. Newcastle-Ottawa Scale: comparing reviewers' to authors' assessments. BMC Med Res Methodol 2014; 14:45.

Wells G, Shea B, O’Connell D, Peterson J, Welch V, Losos M, Tugwell P: The Newcastle-Ottawa Scale (NOS) for assessing the quality of nonrandomised studies in meta-analyses. 2013. Available from:

Guyatt G, Oxman A, Schunemann Hea. GRADE guidelines: a new series of articles in the Journal of Clinical Epidemiology. J Clin Epidemiol 2011; 64(4):380-2.

Schunemann H, Hill S, Guyatt Gea. The GRADE approach and Bradford Hill's criteria for causation. J Epidemiol Community Health 2011; 65(5):392-5.

Higgins JPT, Green S. Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0. The Cochrane Collaboration: London, UK, 20 2011.

Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ 2021; 372:n71.

Al-Kubaisi AHMA, Al-Alouse WS. Dental caries experience among patients with congenital coagulation disorders. J Fac Medd Baghdad Jol 2006; 48(1):17-20.

Salem K, Eshghi P. Dental health and oral health-related quality of life in children with congenital bleeding disorders. Haemophilia 2013; 19(1):65-70.

Daneswari V, Reddy A. Oral hygiene status and treatment needs among haemophilic children in Telangana State and Andhra Pradesh State. Ann Med Res 2017; 7(3):28-31.

Mythri H, Jagdish GR, Chandu GN. Dental caries experience among haemophilic children. RJPBCS 2013; 4(4):1233-8.

Reddy KS, Reddy NV, Niharika P, Reddy MA, Danaeswari V, Noorjahan M. Oral health status and treatment needs among hemophilic children in Hyderabad, Telangana, India. Int J Clin Pediatr Dent 2019; 12(1):30-2.

Evangelista LM, Lima CCB, Idalino RCL, Lima MDM, Moura LFAD. Oral health in children and adolescents with haemophilia. Haemophilia 2015; 21(6):778-83.

Rodrigues MJ, Luna ACA, Leal LC, Lira SS, Marques KM. Prevalence of caries and associated factors in hemophiliac children. Rev Bras Hematol Hemoter 2008; 30(2):114-9.

Rodrigues LV, Camelo Moreira MDS, de Oliveira CR, de Medeiros JJ, Lima Neto EA, Gondim Valenca AM. Factors associated with toothache in patients affected by hereditary coagulopathies. Pesqui Bras Odontopediatria Clin Integr 2017; 17(1):e3699.

Rajantie H, Alapulli H, Makipernaa A, Ranta S. Oral health care in children with haemophilia in Helsinki, Finland. European Archives of Paediatric Dentistry 2013; 14(5):339-43.

Vujkov S, Bajkin B, Blagojevic D, Petrovic B, Konstantinidis N, Rajic N, et al. Oral health in children and young adults with hemophilia in Serbia. Haemophilia 2014; 20:128.

Jepsen S, Blanco J, Buchalla W, Carvalho JC, Dietrich T, Dörfer C, et al. Prevention and control of dental caries and periodontal diseases at individual and population level: consensus report of group 3 of joint EFP/ORCA workshop on the boundaries between caries and periodontal diseases. J Clin Periodontol 2017; 44(Suppl 18):S85-S93.

Chaffee BW, Rodrigues PH, Kramer PF, Vítolo MR, Feldens CA. Oral health-related quality-of-life scores differ by socioeconomic status and caries experience. Community Dent Oral Epidemiol 2017; 45(3):216-24.

Petersen PE, Ogawa H. Strengthening the prevention of periodontal disease: the WHO approach. J Periodontol 2005; 76(12):2187-93.

Fejerskov O. Concepts of dental caries and their consequences for understanding the disease. Community Dent Oral Epidemiol 1997; 25(1):5-12.

Sanders AE, Slade GD, Lim S, Reisine ST. Impact of oral disease on quality of life in the US and Australian populations. Community Dent Oral Epidemiol 2009; 37(2):171-81.

Medina E, Pailaquilén R. Systematic Review and its Relationship with Evidence-Based Practice in Health. Rev Latino-Am Enfermagem 2010; 18(4):824-31.

Kalsi H, Nanayakkara L, Pasi K, Bowles L, Hart D. Access to primary dental care for patients with inherited bleeding disorders. Haemophilia 2012; 18(4):515-5.

Stoffman J, Andersson N, Branchford B, Batt K, D’Oiron R, Escuriola, et al. Common themes and challenges in hemophilia care: a multinational perspective. Hematology 2019; 24(1):39-48.




How to Cite

da Silva, L. T. ., de Souza, J. F. ., Wambier, L. M. ., Menezes, J. V. N. B. ., Torres-Pereira, C. de C. ., & Fraiz, F. C. . (2022). Can Hemophilia Impact on the Oral Health Conditions of Children and Adolescents? A Systematic Review and Metanalysis. Pesquisa Brasileira Em Odontopediatria E Clínica Integrada, 22, e210152. Retrieved from



Systematic Reviews (and Meta-Analysis)