Title: A Study of Free radical activity in thalassaemia major patients in South Bengal
Authors: Prof Tamal Kanti Ghosh MD, PhD, MBA, Prof C.R. Maity MD,MNAMS,PhD, Prof Nabendu Chaudhuri, MD, MNAMS, PhD, D,Sc, Dr Reena Ghosh MD
DOI: http://dx.doi.org/10.18535/jmscr/v3i9.55
Abstract
Thalassaemias are commonest single gene disorder, the production of normal heamoglobin is partly or completely suppressed due to defective synthesis of one or more globin chains. Loss of AHSP, precipitation free α-Hb iron that generate ROS thereby causing peroxidative RBC membrane and tissue damage leding to Increased MDA(Malondialdehyde) & RBC SOD levels .
Two hundred and fifty one diagnosed thalassaemic subjects from 6months-40 years of age attending thalassaemia care centres in Burdwan were investigated for Plasma MDA /SOD and RBC SOD. The control group shows SOD levels 5.98±1.72U/ml (RBC) 3.79±1.42 U/ml (Plasma) & Plasma MDA 6.86±1.72 nmol/ml. In beta Thal patients RBC-SOD was 13.76±2.22 U/ l and Plasma-SOD 08.100±1.88 U/ l, Plasma MDA 15.12±2.28 nmol/ml was observed.
Conclusion: Plasma SOD & MDA in thalassaemia major patients is raised due to generation of production of Reactive Oxygen Species (ROS) as a consequence to formation of hemichromes heamolysis of Red Blood Cells and Iron overload.
Keywords: Thalassaemia, MDA,SOD, Reactive Oxygen Species (ROS)
References
1. Kihm AJ, Kong Y, Hongw, Russell JE, et al : An abundant erythroid protein that Stabilize free -haemoglobin. Nature; 417 : 758 – 763; 2002.
2. Amer J., Gold farb. A., Fibach E.: Flow cytometric measurement of reactive oxygen species production by normal and thalassaemia red blood cells. Eur. J. Haematol ; 70 : 84 – 90; 2003.
3. Giardini O, Cantani A, Don francesco A, Martino F. et al : Biochemical and clinical effects of vitamin E administration in homozygous beta-thalassaemia. Acta vitaminol Enzymol ; 7 : 55, 1985.
4. Lardi S, Ferchichi S, Khelil AH et al Oxidant, antioxidant status and metabolic data in patients with beta thalassaemia. Clinica Chimica Acta : Vol 338, Issue 1–2. 79–86; 2003.
5. Paoleth F, Mocali A. Detormination of Super oxide dismutase activity of purely chemical system based on NADPH (H) Oxidation Methodsin onzymology Vol 186/(18) : 209 – 219. 1990
6. Gardenes HW. Fee Rad Bio Med 7 : 85–80 (1994)
7. An gastiriotis M, Modell B Global Epidemiology of Haemoglobin disorders Annals of New York Academy of Sciences ; 850 : 25–69.
8. Cunningham MJ, Macklin EA, New feld EJ, Cohen A. R., Complications of beta thalassaemia major in North America Blood; 104 : 34 – 39 : 2004.
9. Liverca MA, TessorlereL, Pintaudi AM Chae Oxidative stress and antioxidant. Status of -thalassaemia major : iron overload and depletim of lipid – soluble antioxidants : Blood No 1988 : 3608 – 14 ; 1996.
10. Yenchitsomanus Pand Wasi P : Increased erythrocyte superoxide dismutase activities in beta O-thalassaemia/haemoglobin E and in HbH diseases ; J. Clinical pathol ; 36 (3) : 329-33, 1983.