Abstract
Hemoglobinopathies are the most common genetic disorders reported in the World. The aim of our study was to establish the spectrum of the different haemoglobinopathies presenting in a tertiary medical college in western Odisha to help develop management, prevention and control programmes in the community. Patients who had any form of haemoglobinopathy underwent HPLC analysis, With regard to the different haemoglobin fractions our study revealed that HbSS (sickle cell disease) is the most common abnormal haemoglobin in Western Odisha, followed by HbAS (Sickle cell trait).Both beta thalassemia major and minor were associated with sickle cell haemoglobinopathy, more commonly with sickle cell disease. Castes like kulita, harijan, aghariya and chasa presented with a higher incidence of different haemoglobin-opathies. HbE was found to be very uncommon. HbF was found to have a higher level in all haemoglobinopathies
Keywords: Haemoglobinopathies, western Odisha, high-performance liquid chromatography, sickle cell disease, sickle cell trait.
References
- Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;86:480-87.
- Weatherall D. The inherited disorders of haemoglobin: an increasingly neglected global health burden. Indian J Med Res.2011;134(4):493–97
- Serjeant GR, Serjeant BE, editors. Sickle cell disease, 3rd ed. Oxford: Oxford Univ Press; 2001
- Shukla RM, Solanki BR. Sickle cell trait in central India. Lancet 1985;1:297-8.
- Kamle M, Chaturvedi P. Epidemiology of sickle cell disease in a rural hospital in central India. Indian Pediatr 2000;37:391-6
- Moorchung N, Phillip J, Sarkar R, Prasad R, Dutta V. Is high pressure liquid chromatography an effective screening tool for characterisation of molecular defects in hemoglobinopathies?. Indian Journal of Pathology and Microbiology. 2013;56(1):36.
- Gaston M, Smith J, Gallagher D, Flournoy-Gill Z, West S, Bellevue R et al. Recruitment in the Cooperative Study of Sickle Cell Disease (CSSCD). Controlled Clinical Trials. 1987;8(4):131-140.
- Platt O, Brambilla D, Rosse W, Milner P, Castro O, Steinberg M et al. Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death. New England Journal of Medicine. 1994;330 (23): 1639-1644.
- Wierenga K, Hambleton I, Lewis N, Unit S. Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study. The Lancet. 2001;357(9257):680-683.
- Serjeant G. Sickle-cell disease. The Lancet. 1997;350(9079):725-730.
- Kar, B., Kulozik, A., Sirr, S., Satapathy, R., Kulozik, M., Serjeant, B. and Serjeant, G. (1986). SICKLE CELL DISEASE IN ORISSA STATE, INDIA. The Lancet, 328(8517), pp.1198-1201
- .Spectrum of hemoglobinopathies in the state of Orissa, India: a ten years cohort study.Balgir RS ,The Journal of the Association of Physicians of India [01 Dec 2005, 53:1021-1026]
- Clinico-Haematological Profile of Thalassemia, Intermedia Patients,Seema Tyagi et al Int J Hum Genet, 3(4): 251-258 (2003)
- Newborn Screening for Sickle Cell Disease: 4 Years of Experience From California's Newborn Screening Program FE Shafer et al. J Pediatr Hematol Oncol 18 (1), 36-41. 2 1996.
- Steinberg M. Predicting clinical severity in sickle cell anaemia. British Journal of Haematology. 2005;129(4):465-481.
Corresponding Author
Dr Chandan Kumar Das
Associate Professor, Department of Medicine, IMS & SUM Hospital, Bhubaneswar
Email: This email address is being protected from spambots. You need JavaScript enabled to view it. 9937063390
DOI: https://dx.doi.org/10.18535/jmscr/v5i8.128
