|Year : 2016 | Volume
| Issue : 1 | Page : 76-81
Dental health in children with congenital bleeding disorders in and around Davangere: A case-control study
NB Nagaveni1, Shruthi Arekal1, P Poornima1, Suresh Hanagawady2, Sneha Yadav1
1 Department of Pedodontics and Preventive Dentistry, College of Dental Sciences, Davangere, Karnataka, India
2 Founder, Karnataka Hemophilia Society, Davangere, Karnataka, India
|Date of Web Publication||2-Feb-2016|
N B Nagaveni
Department of Pedodontics and Preventive Dentistry, College of Dental Sciences, Davangere, Karnataka
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Aim: The present study was carried out to investigate the dental and some other aspects of oral health status of young patients with congenital bleeding disorders (CBDs) and compared with controls. Materials and Methods: Decayed, missed, filled tooth surfaces (DMFS-dmfs) in permanent and primary teeth scores, simpliﬁed oral hygiene index, occlusion, occurrence of hypoplasia, fluorosis other hard tissue and soft tissue findings of 50 CBD patients at the age range of 4-15 years and 50 of other children as control were compared. Data were analyzed by Chi-square and Student's unpaired t-test. Results: Patients were signiﬁcantly more caries-free with less decayed teeth in primary-permanent dentition (P < 0.05) and with lower scores for overall hygiene. Conclusion: By this, it can be concluded that children with CBD have a significantly lower prevalence of dental caries and better oral hygiene compared with matched, healthy controls.
Keywords: Congenital bleeding disorders, dental caries, hemophilia, oral hygiene
|How to cite this article:|
Nagaveni N B, Arekal S, Poornima P, Hanagawady S, Yadav S. Dental health in children with congenital bleeding disorders in and around Davangere: A case-control study. J Indian Soc Pedod Prev Dent 2016;34:76-81
|How to cite this URL:|
Nagaveni N B, Arekal S, Poornima P, Hanagawady S, Yadav S. Dental health in children with congenital bleeding disorders in and around Davangere: A case-control study. J Indian Soc Pedod Prev Dent [serial online] 2016 [cited 2020 Aug 6];34:76-81. Available from: http://www.jisppd.com/text.asp?2016/34/1/76/175522
| Introduction|| |
Congenital bleeding disorder (CBD) patients constitute a minor but significant part of the population. The disorder, especially in its severe forms, has been associated with mortality and morbidity, as numerous impacts on overall health have been detected.  Among the CBD, hemophilia is the most common bleeding disorder seen worldwide, which is sex-linked in nature,  in which there is a deficiency of factor VIII (hemophilia A) and a deficiency of factor IX (hemophilia B). In the past, hemophiliacs or patients with other CBD tended to die at a young age, but with the development of safe and effective treatments, a person with these disorders can now have a normal life expectancy. 
Oral care of these patients is not of primary importance in developing countries, as they have tended to receive less oral health care, or of lower quality, than the general population, yet they may have oral problems that can affect their systemic health.  The two main dental diseases affecting every person including those with CBD are dental caries and gingivitis.  However, the potential problem in such patients is bleeding in the oral cavity; poor oral health is considered the major risk factor in children with CBD, as the nature of many oral diseases, as well as dental treatments, encompasses bleeding-associated procedures.  One group of workers said that the most common site of oral bleeding was the frenum of the lip and the tongue. Thus, the dentist may be the first to diagnose a patient with hemophilia.  Thus, the purpose of the present study is to examine different aspects of dental health or disease in children with CBD and to compare them to matched controls from the general population.
| Materials and Methods|| |
The present cross-sectional observational study was conducted by the Department of Pedodontics and Preventive Dentistry, to study the dental caries experience and oral health status among children with CBD. The study was conducted among 100 children of 3-18 years age group. Fifty children with CBD who were referred to hemophilia society and 50 children who were referred to the Department of Pedodontics in the same age and gender distribution were selected as the control group.
The two-stage sampling procedure was adopted to select the sample. In the first stage, equal numbers of children with CBD and children without CBD were selected randomly from the hemophilia society and from the Department of Pedodontics, Davangere, respectively. Probability proportional to the size was used to ensure that all would have an equal chance to be selected in the study. In the second stage, 3-18 years children were selected from each group. The study group consisted of patients with CBDs including deficiency of factors: VII, VIII, IX, XIII, and Von Willbrand factor.
Prior to the clinical examination, parents and subjects were informed about the procedure. The research protocol of the study was reviewed and approved by the Ethical Committee of the Institution before commencing the study.
Parameters examined were dental caries, malocclusion and other hard and soft tissue findings. Teeth affected by dental caries and teeth restored/extracted as sequelae of dental caries were assessed using decayed, missed, filled tooth (dmft) decayed missing filled surfaces (dmfs), DMFT and DMFS index  for primary and permanent teeth respectively. Oralh ygiene assessment was done by using simpliﬁed oral hygiene index (OHIS) (Greene and Vermillion).  Moreover, the presence of any anomalies such as microdontia, macrodontia, supernumerary teeth, fusion and others, enamel hypoplasia, and fluorosis were also assessed. Prevalence of dental trauma, both hard tissue and soft tissue and other soft tissue findings on the tongue, buccal mucosa, the floor of the mouth, lips, gingival, etc., were compared between both groups.
A survey proforma was created to gather data from the sample. The children were examined by a single examiner under natural light (Type III examination). Diagnosis of dental caries was made by using WHO criteria. Dental caries status was recorded by means of DMF index, that is, dmft for primary dentition and DMFT for permanent dentition. The response obtained was tabulated and the results were expressed as frequency distributions and computed in percentages. The observations were statistically analyzed using the Chi-square test, Student's unpaired t-test.
| Results|| |
In this study, two-stage sampling procedure was adopted to select the sample. In the first stage, equal numbers of children with CBD and children without CBD were selected randomly from the hemophilia society and from the Department of Pedodontics, Davangere, respectively. Probability proportional to the size was used to ensure that all would have an equal chance to be selected in the study. In the second stage, 3-18 years children were selected from each group. The study group consisted of patients with CBDs including deficiency of factors: VII, VIII, IX, XIII, and Von Willbrand factor.
Factor VIII deficiency was seen in 35 children, whereas factor VII deficiency was seen in only one child, factor IX deficiency was seen in eight children, factor XIII deficiency was seen in three children, and deficiency of Von Willbrand factor was seen in three children. Of 50, 18 children had the positive family history, out of which 14 children had their mother's brother been affected by the same CBD whereas four children had their own brothers affected by the same CBD. Thirty-two children had a negative family history with none of the family member suffering by CBD. When the subjects were compared according to the age in both the groups, 10 and nine subjects of CBD group and control group respectively fell in the age group of 3-6 years, 29 and 31 subjects of CBD group and control group respectively fell in the age group of 6-12 years whereas 11 and 10 subjects of CBD group and control group respectively fell in the age group of 12-18 years. The mean age score for test group was 9.72 ± 3.85 and for control group it was 9.58 ± 3.10 with a t = 0.20 and P = 0.842. Thus, there was no statistical difference in age.
When parents were asked about the knowledge regarding oral hygiene parents of 36 children had knowledge and awareness regarding oral hygiene and parents of 14 children did not have, whereas in control group, parents of 22 children had knowledge and awareness regarding oral hygiene and parents of 28 children did not have the knowledge and awareness regarding oral hygiene.
DMFT and DMFS index was used to compare the caries experience between both the groups and there was a statistically significant difference along with dmfs and dmft scores [Table 1] and [Graph 1][Additional file 1]. Thus, it showed that the overall caries experience was less in children with CBD than the controls. When we compared oral hygiene, excellent oral hygiene was not found in any case, and test group showed a better oral hygiene compared to control group [Table 2] and [Graph 2][Additional file 2]. However, no statistical significant difference was found between test group and control when the parameters of malocclusion were considered [Table 3] and [Graph 3][Additional file 3].
|Table 1: Comparison of dental caries characteristics of the study and control group|
Click here to view
When the dental anomalies such as supernumerary teeth, enamel hypoplasia, fluorosis, and trauma were considered again there was no statistical significant difference found between the groups. The supernumerary tooth was seen in 2% of the subjects in the test group only. Enamel hypoplasia was seen in 10% and 6% of the subjects of test and control group, respectively. Fluorosis was seen in 2% and 4% of the subjects of test and control group, respectively. The in the cadence of trauma was seen in 10% and 8% of the subjects of test and control group, respectively.
Soft tissue examination showed that there was an ulcer present on the dorsum surface of tongue in 2% of the subjects in both the groups. Buccal mucosa also showed an ulcer in 2% of the subjects in test group only. When the floor of the mouth was considered, there were no such findings in both the groups. There was a lip ulcer which was seen in 6% and 4% of the subjects of test and control group respectively. Other soft tissue changes like gingivitis were seen in 14% of the subjects of both groups.
| Discussion|| |
Inherited tendency to bleed is an uncommon disorder which is termed as CBD. ,, The most common is hemophilia A, hemophilia B affecting approximately 1 in 7500 males and Von Willbrand's disease. Children with these disorders are unable to produce one or more of the essential clotting proteins. ,,,, Hemophilia A is a deficiency of factor VIII, hemophilia B or Christmas disease is a deficiency of factor IX, Von Willebrand's disease caused by a deficiency of von Willebrand factor a protein that mediates platelets adhesion. Other factor deficiencies, such as those of factors II, V, and XIII (one case per 1 million) and factor VII (one case per 500,000) are rare and extremely uncommon. 
While these CBD may not directly target oral tissues, oral health can be influenced as a consequence of general health problems and oral care of these patients is not of primary importance in developing countries, as they have tended to receive less oral health care, or of lower quality, than the general population, yet they may have oral problems that can affect their systemic health. 
Therefore, it is important that they also maintain good oral health as it can be influenced as a consequence of general health problems.  Management of patients with CBD in dentistry causes considerable problems. Unsurprisingly, it has been reported that patients with CBD have difficulties in accessing professional dental care either due to disease-specific risks or due to patient-related barriers, both potentially contributing to deteriorating oral health and consequently increasing the need for more invasive dental treatments. 
Understanding the oral health and treatment needs of patients with CBD is the off key importance to the population's health and is necessary for targeting patients with CBD for primary oral health prevention as well as for implementing timely secondary prevention, both reducing dental treatment needs and their related risks in this vulnerable segment of the population. Different workers reported that the prevalence of caries in both the primary and permanent dentition was lower in children with CBD than the general population. 
However, very few studies have been conducted with regard to dental caries and oral health in children with CBD. ,
The present research, therefore, is an attempt to study the prevalence of dental caries and oral health in children with CBD and to compare them to matched controls from the general population. A total of 100 children between the age group of 3-18 years, 50 children with CBD who were referred to hemophilia society and 50 children who were referred to the department of pedodontics in the same age and gender distribution were selected as the control group. The data so collected were tabulated and analyzed. The distributions of study group according to the type of CBD are presented in [Table 1]. Of 50 patients who had CBD, 35 (75%) patients had factor VIII deficiency, 1 (2%) patient had factor VII deficiency, 8 (16%) patients had factor IX deficiency, 3 (6%) patients had factor XIII deficiency, 3 (6%) patients had deficiency of von Willbrand factor deficiency.
When we compared the overall ﬁndings of dental health between children with CBD and controls, children with CBD showed better dental health, i.e., less overall caries experience and lower dental treatment needs were observed in these children. In control group the mean DMFS and DMFT scores were 2.96 ± 4.08 and 1.18 ± 1.36 and mean dmfs and dmft scores were 7.86 ± 7.83 and 3.02 ± 3.03 which was significantly greater than in the test group who showed mean DMFS and DMFT scores as 1.08 ± 1.67 and 0.56 ± 0.78 and mean dmfs and dmft scores as 1.52 ± 3.96 and 0.64 ± 1.63. This fact per se reﬂects the supportive care that CBD patients have received at a young age from the CBD care centre, including exposure to topical ﬂuoride, obligatory dental visits, regular education of patients and parents.
A study which also showed that there was a statistically significant difference in the caries prevalence between CBD and healthy nonhemophilic children. They said that the overall dental health was better in deciduous dentitions, i.e., less overall caries experience and lower dental treatment needs were observed in children with hemophilia as compared to their healthy counterparts. No difference was found when permanent dentitions were compared between the hemophiliacs and controls.  Results similar to those of the present study have been reported in studies from England, Ireland, Germany and Egypt. ,,, However, a poorer dental situation in CBD patients compared with controls is found in Poland, Turkey and India. , Inconsistency in the level of provided health care in different communities is probably the main causative factor.
Another study  which was contrary to the present study where there was significantly higher DMFT and DMFS values in the hemophilic group when compared to control group. Moreover, they thought that the higher index results in children with hemophilia were a consequence of neglected or insufficient tooth brushing. When caries are considered, children with hemophilia must be seen as a high-risk group. They also checked for gingival index (GI) values which were again significantly higher in the hemophilia group than in the control group. They said that the factor that increased the GI values affected the high prevalence of caries in the study group. They concluded that data gained from the questionnaire and clinic examinations of the study group disclosed the need for and necessity of preventive treatments and dental education in patients with hemophilia.
A study  showed that the overall ﬁnding was better in deciduous dentitions, i.e., less overall caries experience and lower dental treatment needs were observed in children with hemophilia as compared to their healthy counterparts. However, no differences were observed when permanent dentitions were compared between the hemophiliacs and controls. This was also supported by Salem et al.  in their study. In contrary, the present study showed there was a significant difference in both the dentition with respect to caries experience, i.e., overall hemophiliacs showed better dental health than controls.
With regard to OHIS which was done only on those subjects who had their permanent incisors and first molars erupted also showed a significant difference. Out of 50 subjects, 29 subjects showed good oral hygiene in hemophilia group. In control group, OHIS was done on 25 subjects in which 21 showed good oral hygiene whereas four subjects showed fair oral hygiene. Same results have been seen in one more study. 
When other variables which included occlusion, crowding, spacing, crossbite and other malocclusion were assessed, there was no statistical significant between test and control group.
An important issue in CBD is oral bleeding. The highly vascular oral cavity is a common site for hemorrhage in this group of patients. Mouth lacerations are a common cause of bleeding in children with all severities of CBD.  Spontaneous and stimulatory bleeding were reported mainly during the time of eruption and shedding of primary teeth or subsequent to oral lacerations especially in the tongue region. In the present study, 72% of parents answered positively about the awareness of dental health, and their main concern was with bleeding in during the time of eruption and shedding of the teeth.
History of oral bleeding, including how, which area and when was obtained. Gum bleeding spontaneously or by tooth brushing was not the main complaint in almost all participants, and this was in line with their SOHI. A number of studies reported lower oral hygiene/plaque scores among CBD, although their gingival situation was similar. , In a study, where authors checked the bleeding incidence during dental management of patients with CBD, found that there was a low incidence (3.4%) of bleeding complications after oral procedures. Even considering only oral surgery, the rate of bleeding events remained a low 3%. ,,
The present study indicated that there was a statistically significant difference in caries experience and oral hygiene between CBD and their counterparts. But no significant difference was seen when we considered occlusion, crowding, spacing, crossbite and other malocclusion.
| Conclusion|| |
There are certain limitations noticed in this study; this was a survey where only a minimum samples were considered, which may not be generalized. The comparison between the CBD in the hemophilia society and patients who visited the department with some complaint may not be appropriate. In spite of these limitations, this study has hinted on the overall dental health in subjects with CBD and controls and also showed that the subjects with CBD showed better oral health. Further research is required to compare overall dental health in subjects with CBD and controls on large samples so that it can be generalized.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Salem K, Eshghi P. Dental health and oral health-related quality of life in children with congenital bleeding disorders. Haemophilia 2013;19:65-70.
Kabil N, ElAlfy MS, Metwalli N. Evaluation of the oral health situation of a group of Egyptian haemophilic children and their re-evaluation following an oral hygiene and diet education programme. Haemophilia 2007;13:287-92.
Zaliuniene R, Aleksejuniene J, Peciuliene V, Brukiene V. Dental health and disease in patients with haemophilia - A case-control study. Haemophilia 2014;20:e194-8.
Harrington B. Primary dental care of patients with haemophilia. Haemophilia 2000;6 (Suppl 1):7-12.
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;7:468-74.
Klein H, Palmer CE, Kuntson JW. Studies on dental caries. I. Dental status and dental needs of elementary school children. JADA 1938;25:1703-5.
Greene JC, Vermillion JR. The oral hygiene index: A method for classifying oral hygiene status. JADA 1960;61:172-9.
Durham TM, Hodges ED, Harper J, Green JG, Tennant F. Management of traumatic oral-facial injury in the hemophiliac patient with inhibitor: Case report. Pediatr Dent 1993;15:282-7.
Zanon E, Martinelli F, Bacci C, Zerbinati P, Girolami A. Proposal of a standard approach to dental extraction in haemophilia patients. A case-control study with good results. Haemophilia 2000;6:533-6.
Gupta A, Epstein JB, Cabay RJ. Bleeding disorders of importance in dental care and related patient management. J Can Dent Assoc 2007;73:77-83.
Adewumi A, Sakhalkar V. Dental management of a patient with factor X deficiency. J Can Dent Assoc 2009;75:461-4.
Gómez-Moreno G, Cañete-Sánchez ME, Guardia J, Castillo-Naveros T, Calvo-Guirado JL. Orthodontic management in patients with haemophilia. About two clinical cases. Med Oral Patol Oral Cir Bucal 2010;15:e463-6.
Madan N, Rathnam A, Bajaj N. Treatment of an intraoral bleeding in hemophilic patient with a thermoplastic palatal stent - A novel approach. Int J Crit Illn Inj Sci 2011;1:79-83.
Rayen R, Hariharan VS, Elavazhagan N, Kamalendran N, Varadarajan R. Dental management of hemophiliac child under general anesthesia. J Indian Soc Pedod Prev Dent 2011;29:74-9.
Rodrigues LV, Moreira Mdos S, de Oliveira CR, de Medeiros JJ, Lima Ede A Neto, Valença AM. Tooth loss and associated factors in patients with coagulopathies in the State of Paraíba, Brazil. Rev Bras Hematol Hemoter 2013;35:319-24.
Dogan MC, Yazicioglu I, Antmen B. Anxiety and pain during dental treatment among children with haemophilia. Eur J Paediatr Dent 2013;14:284-8.
Peisker A, Raschke GF, 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;19:e55-60.
Zwain AM, Al-Ameen MM, Al-Alousi WS. Oral health status and caries related microflora among children with congenital coagulation disorders (Comparative study). J Babylon Univ Pure Appl Sci 2012;20:145-149.
Boyd D, Kinirons M. Dental caries experience of children with haemophilia in Northern Ireland. Int J Paediatr Dent 1997;7:149-53.
Ziebolz D, Stuhmer C, Hornecker E, Zapf A, Mausberg RF, Chenot JF. Oral health in adult patients with congenital coagulation disorders - A case control study. Haemophilia 2011;17:527-31.
Mielnik-Blaszczak M. Evaluation of dentition status and oral hygiene in Polish children and adolescents with congenital haemorrhagic diatheses. Int J Paediatr Dent 1999;9:99-103.
Sudhanshu S, Shashikiran ND. Prevalence of dental caries and treatment needs among CBD Children of Kota city, Rajasthan. Ann Essences Dent 2010;2:18-21.
AlpkiliÇ Baskirt E, Albayrak H, Ak G, Pinar Erdem A, Sepet E, Zulﬁkar B. Dental and periodontal health in children with hemophilia. J Coagul Disord 2009;1:7-10.
Sanders BJ, Shapiro AD, Hock RA, Weddell JA, Belcher CE. Management of the medically compromised patients: Hematologic disorders, cancer, hepatitis, and AIDS. In Dentistry for the child and Adolescent , 8 th
Edition, McDonald RE, Avery DR, Dean JA, Mosby: St. Louis. p. 559-64.
Azhar S, Yazdanie N, Muhammad N. Periodontal status and IOTN interventions among young haemophilia. CBD 2006;12:401-4.
Noor N, Maxood A, Mumtaz R. Dental management of haemophilic pediatric patients. Pak Oral Dent J 2012;32:66-70.
[Table 1], [Table 2], [Table 3]