Detection of Haemoglobin S Among Libyan Children in Sebha Medical Centre
Keywords:
Haemoglobin S, Sickle cell anaemia, Libyan childrenAbstract
Background: Sickle cell anaemia is caused by a single-base mutation leading to substitution of amino acid valine for glutamic acid at the sixth position of the β-globin chain. In the homozygous (Hb SS) both genes are abnormal, whereas in the heterozygous (Hb AS) only one chromosome carries the gene. Clinical presentation of sickle cell disease occurs frequently in children admitted in the Department of Paediatrics in Sebha Medical Centre.
Objective: The main aim of this study, was to apply the alkaline cellulose acetate electrophoresis technique to investigate the presence of haemoglobin S in blood collected from these children.
Materials and Methods: Blood samples were collected in EDTA anticoagulant tubes from 19 child (males = 13 and females = 6), suspected with sickle cell anaemia, and used for haematology and haemoglobin alkaline electrophoresis (pH 8.4) analysis.
Results: The haematological indices results were: total haemoglobin (X= 7.27 ± 1.35 g/dl), RBC (X= 2.56 ± 0.53 x 106/mm3), MCV (X= 88.74 ± 8.05 fl), MCH (X= 29.87 ± 2.40 pg) and Total leukocytes count (TLC) (X=13.69±6.31 x 106/mm3). The results of haemoglobin fractions as separated by alkaline electrophoresis were: 7 (36.8%) children had a haemoglobin fraction migrated in the position of Hb A, and 8 (42.1%) children had a single abnormal fraction migrated in the position of Hb S. Whereas, 4 (21.0%) children had two fractions migrated in the position of Hb A and S.
Conclusion: We conclude that a large prospective study would lead to better understanding of the distribution of these genetic disorder among Libyan populations, at least in this region of the country. Consequently, better genetic counselling and more efficient way of utilising the health care resources as well as improving family planning programmes could be achieved.
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References
Simmons, A. Hemoglobin: In Technical Haematology (3rd edition). J.B. Lippincott Company. Philadelphia, 1980:19 – 47.
Bauer, J.D. Hemoglobin, porphyrin, and iron metabolism. In Kaplan, L.A., Pesce, A.J. (ed.), Clinical chemistry; theory, analysis, correlation. St Louis. The C.V. Mosby company,1984: 611-655.
Besa, E.C. Hemoglobin and Hemoglobinopathies. In: Besa, E.C., Catalano, P.M., Kanta, J.A., Jeffereries, L.C., (ed.). Hematology. Baltimore, 1992:125-150.
Malpas, J.S., Murphy, M. Disease of the blood : In Clinical Medicine (2nd edition). Kumar, P.J., Clark, M.L. Bailliere Tindall. London,1990: 295-348.
Simpore, J., Pignatelli, S., Barlati, S. Biological and clinical presentation of patients with hemoglobiopathies attending an urban hospital in Ouagadougou: confirmation of the modification of the balance between Hb s and Hb C in Burkina Faso. Hemoglobin; 2002; 26(2):121-127.
Chotray, G.P., Dash, B.P., Ranjit, M. Spectrum of haemoglobinopathies in Orissa, India. Hemoglobin, 2004;28(2):117-122.
Al-Riyami, A.A., Suleiman, A.J., Afifi, M. A community-based study of common hereditary blood disorders in Oman. East Mediterranean Health Journal, 2001;7(6):1004-1011.
Wajcman. Diagnosis and screening of sickle cell disease. Review Practice, 2004;30(14):1543-1547.
Jain, R.C. Sickle cell and thalassaemic genes in Libya. Haemoglobinopathies in Libya. Trans R Soc Trop Med Hyg, 1985;79(1):132-3.
Helena laboratories: Hemoglobin electrophoresis procedure. Helena laboratories, Beaumont, Texas, USA.
Tietz, N.W. Clinical Guide to laboratory Tests. W.B. Saunders Company. Philadelphia, 1983:174 – 343.
Hughes-jones. Lecture notes on haematology (3rd edition). Blackwell scientific publications. Oxford,1980:149-158.
Stephens, A. Haemoglobinopathies, Screening and diagnosis. The Biochemical Science, 2005:139-144.
Fairbanks, V.F. Hemoglobin, haemoglobin derivatives, and myoglobin. In N.W. Tietz (ed.), Fundamentals of clinical chemistry. W.B. Saunders Company. Philadelphia, 1976: 401-454.
Tulip Group Customers. Sickle Haemoglobinopathies. The CRUX (II)IX; May/Jun, 2005:1-8.
Husquinet, H., Parent, M.T., Schoos-Barbette, S., et al. Hemoglobin in D-Los Angeles [beta 121 (Gh40Glu---Gin] in the province of Liege, Belgium. Hemoglobin, 1986;10(6):587-92.
Fucharoen, S., Changtrakun, Y., Surapot, S., et al. characterization of Hb D-Punjab [beta 121 (Gh40Glu---Gin] in Thailand. Hemoglobin, 2002;26(3):261-9.
Del Guidice, R.E., Doering, K.M., Teran, A. Evaluation of SICKLEQUIK, a differential solubility test for hemoglobin S. American Journal of Medical Technology, 1979;45(4):287-9.
Jain, R.C. Haemoglobinopathies in Libya. Journal of Tropical Medicine and Hygiene, 1979;82(6):128-132.
Merghoub, T., Sanchez-Mazas, A., Tamouza, R., et al. Haemoglobin D-Ouled Rabah among Mozabites: a relevant variant to trace the origin of Berber population. European Journal of Human Genetics, 1997;5(6):390-6.
Pathak, K., Kishore, S., Shivkumar, V.B., et al. Study of haemoglobin S percentage and haematological parameters in sickle cell trait. Indian Journal of Pathology and Microbiology, 2003;46(3):420-4.
El-Hazmi, M.A., Jabbar, F.A., Al-Faleh, F.Z., et al. The haematological, biochemical and clinical-presentation of haemoglobin S in Saudi Arabia. Haematological & clinical expression. Tropical and Geographical Medicine, 1987;39(2):157-62.
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