ISSN: 2277-5536 (Print); 2277-5641 (Online)

Prevalence of Orofacial Complications in Thalassemic Patients from Solapur, Maharashtra State, India. Dama S.B.** and Dama L.B.* *Department of Zoology, D. B. F. Dayanand College of Arts and Science, Solapur -413002, (M.S.), India. **Department of Zoology, Dr. Babasaheb Ambedkar Marathwada University, Aurangabad-431001(M.S.), India. (Corresponding Author: Dama L. B.:- E-mail: southraj@gmail.com) ABSTRACT The orofacial complications in thalassemia patients, skull and face deformities can be closely related to patient's age. Early diagnosis and blood transfusion caused less prevalence of rodent face. The aim of the present study is to surveying the prevalence of orofacial complications in thalassemic patients from Solapur District, Maharashtra State, India. Present observational survey study, one hundred twenty five thalassemic childrens with age 6 months to 18 Years, coming for to get blood transfusion from different parts of Solapur district during June 1 st , 2009 to May 1st, 2013. The questionnaires included general information, medical and dental history. The prevalence of orofacial complications in thalassemia was: rodent face- male (73.97%), female (75.00%) total (74.4%); saddle nose- male (79.45%), female (90.38%) total (84.00%); maxillary protrusion- male (79.45%), female (82.69%) total (80.8%); Upper anterior teeth spacing- male (52.05%), female (53.84%) total (52.8%); Anterior open bite- male (69.86%), female (78.84%) total (73.6%); deep bite- male (90.41%), female (1.92%) total (53.6%); Mucosal discolorationmale (0.00%), female (1.92%) total (0.8%); respectively. In this study, Upper anterior teeth spacing and deep bite was observed, more than the normal population especially in older patients, which is due to rotation of mandible and protrusion of maxilla leading to over growth of upper anterior teeth of maxilla. Skull and face deformities can be closely related to the patient's age. Early diagnosis and blood transfusion caused less prevalence of rodent face. KEY WORDS: Inherited blood disorder, Mortality, Orofacial Complications, Thalassemia. INTRODUCTION Thalassemia is a serious inherited blood disorder. It results in excessive destruction of red blood cells, which leads to anemia. It is not infectious and cannot be passed from one individual to the other by personal or any other contact, or through blood transfusion, food or air (Wikipedia, 2008). An estimated 40 million Indians are Thalassemia carriers and over 10,000 Thalassemia major patients are born every year. Survival of a Thalassemia major depends upon repeated lifelong blood transfusions and iron chelation. The disease can be prevented by creating awareness, genetic counseling and screening at pre-nuptial or pre-conceptual stage followed by antenatal diagnosis (National Thalassemia Welfare Society, 2013). Without treatment, affected children have severe failure to thrive and shortened life expectancy. Treatment with a regular transfusion program, chelation therapy, bone marrow transplantation and medication aimed at reducing transfusion iron overload, allows for normal growth and development and extends life expectancy into the third to fifth decade (GeneReviews, 2009). Recent data indicate that about 7% of the Worlds population is a carri er of a hemoglobin disorder and that 300,000-500,000 children are born each year with the severe homozygous states of these diseases (WHO/March of dime, 2006). The carrier rate for beta thalassemia gene varies from 1 to 3% in Southern India to 3% to 15% in Northern India. Pusaksrikit et al. (1987), finds the occlusion of the teeth in thalassemic patients. Drew and Sach (1997), studied the management of thalassemia induced skeletal facial deformity. Effects of thalassemia major on components of the craniofacial complex studied by Bassimitci (1996). Agha (2000) evaluates the maxillofacial anomalies in Beta thalassemia major. Hypoparathyroidism and intracranial calcification in b-thalassemia major observed by Zafeiriou et al. (2011). Abu Alhaija et al. (2004), observed the cephalometric measurements and facial deformities in subjects with beta-thalassaemia major. Singh and Venketasubramanian (2004), studied the recurrent cerebral infarction in beta thalassaemia major. Amini et al. (2007), studied the craniofacial morphology of Iranian children with TM. Salehi et al. (2007), studied the prevalence of Orofacial complications in Iranian Patients with -Thalassemia Major. In 2008, Ashraf (2007), evaluates the oro-maxillofacial changes in major thalassemia. Verma et al.(2007), studied the intracranial calcification in beta thalassemia Major. Certain communities in India, such as Punjabis and Sindhis from Northern India, Kutchis, Bhanushalis, Lohanas from Gujarat, Neobuddhists, Kolis and Agris, Lingayats from Maharashtra have a higher carrier rate ( Nikam et al., 2011; 2012a, b, c, d) The present study, to find out the prevalence of orofacial complications in thalassemic children from Solapur District, Maharashtra State, India. MATERIALS AND METHODS Thalassemia patients The geographical regions of Solapur District, Thalassemia analyzed (Figure -1) in the framework research work: i) Akkalkot ii) Barshi iii) Karmala iv) Madha v) Malshirus vi) Mangalveda vii) Mohol viii) North Solapur ix) Pandharpur x) South Solapur xi) Sangola xii) Solapur City. All individuals were non-related and their selection DAV International Journal of Science Volume-2, Issue-1 January, 2013

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depended on their well-defined phenotypes, transfusion-dependency, and geographical origins. Present observational survey study, one hundred twenty five (Male =73, Female =52) clinically proved by their medical reports, cases of thalassemic childrens with age 6 months to 18 Years, coming for to get bl ood transfusion from different parts of Solapur district, Maharashtra State during June 1st 2009 to May 1st, 2013. The entire survey study was carried out under the observations of Medical officer from Thalassemia transfusion centre, Indian Red Cross Society, Gopabai Damani Blood Bank, Solapur Maharashtra, India. The study population consisted of one hundred twenty five, cases of Thalassemia children attending for regular blood transfusions in the 5 blood banks and hospitals collaborating in this multicentre study were carried out, with prior written consent from the parents/ guardians. Provided questionnaires included all information (General, medical and dental). Clinical orofacial examination of the patients was performed by means of disposable mirror, probe and flash light and sterilized gauze. Data were analyzed through the standard statistical methods. A B

Figure- 1. Map of India; Red Color indicates Maharashtra State. B- Map of Solapur Solapur District, the geographical area of thalassemia in the framework of this research work. RESULTS AND DISCUSSION The prevalence of orofacial complications (Table 1) in Thalassemia patients was: rodent face (Figure 2) male (73.97%), female (75.00%) total (74.4%); saddle nose (Figure 3) male (79.45%), female (90.38%) total (84.00%); maxillary protrusion (Figure 4) male (79.45%), female (82.69%) total (80.8%); Upper anterior teeth spacing (Figure 5) male (52.05%), female (53.84%) total (52.8%); Anterior open bite (Figure 6) male (69.86%), female (78.84%) total (73.6%); deep bite (Figure 7) male (90.41%), female (1.92%) total (53.6%); Mucosal discoloration (Figure 8) male (0.00%), female (1.92%) total (0.8%); respectively. Present study, orofacial complications are seen, mainly dental and facial abnormalities include spacing of teeth, open bite, protrusion of maxilla, saddle nose and rodent face maxillary protrusion, anterior teeth spacing, anterior open bite, and deep bite and mucosal discoloration. In this study, Upper anterior teeth spacing and deep bite was observed, more than the normal population especially in older patients, which is due to rotation of mandible and protrusion of maxilla leading to over growth of upper anterior teeth of maxilla. The anterior open bite was slightly more than normal population. Regular and repeated blood-infusion keeping the hemoglobin in an appropriate level (at least 10g/dl), along with iron removal can prevent face and skull deformities. There was not any relationship between the complications and sex. The goal for future research in thalassemia is not only to introduce new strategies of diagnosis and treatment of thalassemia but also to discover ways to prevent transfusion-induced malaria, HCV, HIV, HBsAg, VDRL and other complications in thalassemic patients. Current work deals with specific complications in thalassemia patients such as orofacial complications, consigunity, ethnicity, hyperthyroidism, Alloimmunization, Hb level at pre and post blood transfusions, anthropologic measurements, hematological study and transfusion-induced or transmitted pathogens, complications in liver, spleen, alloimmunization etc. Efforts should be towards promoting awareness of thalassemia amongst the public and health professionals, and promoting the establishment and improvement of policies aimed at the prevention and medical treatment of thalassemia. DAV International Journal of Science Volume-2, Issue-1 January, 2013

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Table-1. Showing the sex wise prevalence of orofacial complications in thalassemic patients.
Parameter Rodent face Sex M F T M F T M F T M F T M F T M F T M F T Yes SCT 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 No SCT 1 0 1 1 0 1 1 0 1 1 0 1 1 0 1 1 0 1 1 0 1 Yes BTI 9 3 12 11 3 14 11 4 15 5 4 9 10 4 14 1 0 1 0 0 0 No BTI 3 1 4 1 1 2 1 0 1 7 0 7 2 0 2 11 4 15 12 4 16 Yes BTMi 5 4 9 6 3 9 6 3 9 3 2 5 6 2 8 0 0 0 0 0 0 No BTMi 1 0 1 0 1 1 0 1 1 3 2 5 0 2 2 6 4 10 6 4 10 Yes TM 40 32 72 41 41 82 41 36 77 30 22 52 35 35 70 6 1 7 0 1 1 No TM 14 12 26 13 3 16 13 8 21 24 22 46 19 9 28 48 43 91 54 43 97 Yes (%) 54 (73.97) 39 (75) 93 (74.4) 58 (79.45) 47 (90.38) 105 (84) 58 (79.45) 43 (82.69) 101 (80.8) 38 (52.05) 28 (53.84) 66 (52.8) 51 (69.86) 41 (78.84) 92 (73.6) 66 (90.41) 1 (1.92) 67 (53.6) 0 (0) 1 1.92) 1 (0.8) No (%) 19 (26.02) 13 (25) 32 (25.6) 15 (20.54) 5 (9.61) 20 (16) 15 (20.54) 9 (17.30) 24 (19.2) 35 (47.94) 24 (32.87) 59 (47.2) 22 (30.13) 11 (21.15) 33 (26.4) 7 (9.58) 51 (98.07) 58 (46.6) 73 (100) 51 (8.07) 124 (99.2)

Saddle nose

Maxillary protrusion Maxillary anterior teeth spacing Anterior bite Deep bite open

Mucosal discoloration

Figure-2. Prevalence of orofacial complications in thalassemia patients: Rodent face.

Figure -3. Prevalence of orofacial complications in thalassemia patients: Saddle nose.

Figure-4. Prevalence of orofacial complications in thalassemia patients: Maxillary protrubution DAV International Journal of Science Volume-2, Issue-1 January, 2013

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The orofacial complications in patients with thalassemia major were similar to findings of other studies (Drew and Sach, 1997; Bassimitci et al., 1996; Agha Hoseini and Shabandy, 2000). The results of orofacial complications in thalassemic patients showed that the rate of rodent face, maxillary protrusion, Anterior open bite, and saddle nose increased with age. This was quite predictable because the maxilla is one of the most common bones affected by thalassemia (Kliegman and Behromon, 2002; Faiez, 2013). The evaluation of oro-maxillofacial changes in major thalassemia observations were similar to results reported by Salehi (2007) and Ashraf et al (2008). Overall, it indicated a reduction in complications during last decades, which was due to early diagnosis, treatment, and regular follows up. The orofacial complications, the data was corroborated with the reports of Kreme (1986). Bimaxillary protrusion and other abnormalities are also frequently seen in thalassemia major cases. Pratima Raju et al. (2002) are evaluating the oral manifestations as correlates in TM cases in current dental practice in central part of India. This study will be helpful in further defining the orofacial complications in thalassemic patients.

Figure- 5. Prevalence of orofacial complications in thalassemia patients: Maxillary anterior teeth spacing.

Figure -6. Prevalence of orofacial complications in thalassemia patients: Anterior open bite

Figure-7. Prevalence of orofacial complications in thalassemia patients: Deep bite.

Figure-8. Prevalence of orofacial complications in thalassemia patients: Mucosal discoloration. DAV International Journal of Science Volume-2, Issue-1 January, 2013

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ACKNOWLEDGEMENTS The authors thank Dr. P.K. Joshi, Secretary and Dr. Rajiv Pradhan, Joint Secretary for their excellent technical assistance as well as the doctors and technicians of Smt. Gopabai Damani Blood Bank (Indian Red Cross Society) Solapur for their help in blood collection during this study. The authors also thanks to Head, Department of Zoology, Dr. Babasaheb Ambedkar Marathwada University, Aurangabad- 431001, Maharashtra, India. REFERENCES Abu Alhaija E.S., Hattab F.N. and Al-Omari M.A. (2004). Cephalometric measurements and facial deformities in subjects with beta-thalassemia major. Eur. J. Orthod. 24: 9-19. Agha Hoseini F. and Shabandy M. (2000). Evaluation of maxillofacial anomalies in B-tha-lassemia major [PhD Thesis]. Dental School, Tehran University of Medical Sciences, Iran. Amini F., Jafari A., Eslamian L. and Sharifzadeh S. (2007). A cephalometric study on craniofacial morphology of Iranian children with beta-thalassemia major. Orthod Craniofac Res. 10: 36-44. Ashraf Sayyedi., Fereydoun Pourdanesh., Bahador Sarkari. and Sayyed Hesamedin Nabavizadeh. (2008). Evaluation of oro-maxillofacial Changes in major thalassemia. Inside Dentistry. 4(2). Bassimitci S., Yucel-Eroglu E. and Akalar M. (1996). Effects of thalassemia major on components of the craniofacial complex. Brit. J. Orthodo. 23: 157162. Drew S.J. and Sach S.A. (1997). Management of thalassemia induced skeletal facial deformity. J. Oral Maxfac Surg. 55(1): 1331-1339. Faiez N. Hattab (2013). Patterns of physical growth and dental development in Jordanian children and adolescents with thalassemia major. J. Oral Sci. 55(1): 71-77. GeneReviews (2009). http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgibook=gene&part=a-thal. Kliegman R.M. and Behromon R.E. (2002). Nelson Essential of Pediatrics. 4th ed. Saunders Co. 662-663. Kremer B.H. and Gotzfried H.F. (1986). Orofacial changes in beta-thalassemia major. Dtsch Z Mund Kiefer Gesichtschir. 10(5): 382-390. Nikam S.V., Dama S.B., Saraf S.A., Jawale C.S., Kirdak R.V., Chondekar R.P., Patil S.S., Deshmukh P.S., Nikam S.V., Dama S.B., Jawale C.S, Deshmukh P.S., Patil S.S., Tembhurkar V.R. and Dama L.B. (2012a). Height and weight correlation in thalassemic patients from Solapur District, Maharashtra, India. DAV Int. J. Sci. 1(1): 54-57. Nikam S.V., Dama S.B., Dama L.B. and Saraf S.A. (2012b). Geographical distribution and prevalence percentage of thalassemia from Solapur district, Maharashtra, India. DAV Int. J. Sci. 1(2): 91-95. Nikam S.V., Dama S.B., Chondekar R.P., Dama L.B. and Jawale C.S. (2012c). Mortality In Thalassemic Patients From Solapur District, Maharashtra State, India. Bangladesh J. Med. Sci. 11(3): 03. Nikam S.V., Dama S.B (Kinagi), Patil S.S (Kadadi) and Dama L.B. (2012d). Literacy Status in Thalassemic Patients from Solapur District, Maharashtra, India: A Statistical Study. Trends Life Sci. 1(1):22-24. Pratima Raju., Minal Chaudhary. and Vijay Wadhwan (2002). Evaluation of Oral manifestations as correlates in Thalassemia major cases in current dental practice in Central part of India . Internet J. Hematol. 6(2). Pusaksrikit S. Isarangkura P. and Hathirat P. (1987). Occlusion of the teeth in thalassemic patients. Birth Defects Orig Artic Ser 23: 429-433. Salehi M.R., Farhud D.D., Tohidast T.Z. and Sahebjamee M. (2007). Prevalence of orofacial complications in iranian patients with -thalassemia major. Ir. J. Publ. Health. 36 (2): 43-46. Shaikh F.I. and Dama L.B. (2011). Prevalence of red cell alloimmunization in repeatedly transfused patients with Thalassemia in Solapur District, Maharashtra State, India . UGC-Sponsored Proc. Nat. Workshop Seminar BioResources Bio-Industries Economic Zool. 1-3. Singh R. and Venketasubramanian N. (2004). Recurrent cerebral infarction in beta thalassaemia major. Cerebrovasc Dis. 17(4): 344-345. Verma S. Saxena., A.K. and Marwaha R.K. (2007). Intracranial calcification in beta thalassemia Major. Ind. Pediatr. 44: 856-858. WHO/March of dime (2006). Report of a joint meeting, May 5-15. Wikipedia (2008). URL: http://en.wikipedia.org/w/index.php?title=Thalassemia& action=edit. National Thalassemia Welfare Society (2013). URL: http://www.thalassemiaindia.org/ Zafeiriou D., Athanasiou M., Katzos G., Economou M. and Kontopoulos E. (2011). Hypoparathyroidism and intracranial calcification in b-thalassemia major. J Pediatr. 138: 411.

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