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Lipid Profile in Children with Beta – Thalassemia Major in Babylon Dakhel Ghani Omran College of science for Women, Department of Biology, University of Babylon Abstract The aim of the present study was to investigate the lipid pattern in children with beta thalassemia major in Babylon province. Forty transfusion dependent beta – thalassemia major patient, 20 males and 20 Females (mean age 9±5 years) that undergo periodical blood transfusion and desferoxame as a chelating agent, were studied. Twenty controls of matched age and gender were also included in this study. Beta thalassemia major patients showed significantly lower total cholesterol, high density lipoprotein cholesterol, and low density lipoprotein cholesterol when compared with control. Serum triglycerides levels of beta–thalassemia major patients were found to be significantly higher than levels in healthy children . Results mentioned above revealed that lipid profile changed in patients with beta–thelassemia major. Many factors such as iron over load, liver injury, hormonal disturbances and aging might cause these changes . الخالصة صممتتهذهمملدذاسة ابم ذسمتممرذاستلحام اهذاستاصم ذ م ذت ممااا ذاسمةه ذونذذ م ذافالممامذاستصممارانذمتم مذاسمنبممعتعاذتممنذ ممو ذرالمماذ ذا مم) ذاستصمارانذراملاذاستم مذتامرذلتمهذ20ذل م اذوذ20 ذالمنذتمنذاف مارذواسمل و ذ40ذشت هذهملدذاسة ابم ذ.اسكرا ةذ ذتتا ظ ذمارلذ ذ ام ذ ذو ملسمذلم ذ تم. ذ ممن ذبمات ذس تةاممةDesferal ت ماستلا ذ م ذت مثذاسمنبممعتعاذم اما ه ذاسممة ذمصمو ةذةو مم ذتماذ ممن ذاسممذ ذ ذ.ذب ذ14-4ذالنذب عتاذتنذ نذاست بانذ تتتو ذبعا ةذذل اوتهذا تا ذافالامذرانذ20ت و ذتنذ ذ ذت ااا ذاسكوسبل ومذاسك ذواسر ولا ماهذاسةه عم ذ اسعم ذاسكما م ذP< 0.05 ذرا هذ لائجذهلاذاسمترذا خلاضاذت وعاذمتبلوىذ ذ ذ.وذق ا ذاسكما ذتقا ذمافالامذافصتا ذ ذ ذافالامذاستصمارانذماستقا م ذتماذتتتو م ذاسبمعا ةذP< 0.05 ذسوتظذا للا اذت وعاذ ذقع ذاسك ذوسبل ومذاسمنم ذمتبلوىذ ذ ذ. ذوانذاس واتمملذاسلم ذلبممر ذ.ذانذاس لممائجذاستبلخ صم ذتممنذهمملدذاسة ابم ذلرممانذانذاسممةهونذللم م ذ م ذافالممامذاستصممارانذماسمنبممعتعا ذ ذ.هلدذاسلحا اهذمصو ةذ ئعب ذت ااذثماةةذل اثذاستةاةذولتا ذ باجذاسكرةذواضا ا ذاسا تو اهذواسلقة ذماس ت ذ Introduction Thalassemia are the most common heterogeneous group of genetic disorders in which the production of normal hemoglobin (Hb) is partly or completely suppressed because of defective synthesis of one or more globin chains, that very widely in severity from asymptomatic forms to sever or even fatal entities (Crisaru, et al., 1990; Melody, et al., 2004). Patients with beta thalassemia major may go through several complications as the transfusion– related infection like HBV and HIV (Chen et al., 2006). Iron overload complication are also noticed that includes endocrinopathies, heart and liver diseases, and chelation therapy complications (Ferrara et al . , 2004) . Thalassemic patients are also subjected to peroxidative tissue injury. It has been documented that circulating low density lipoprotein–C (LDL–C in thalassemic patients show marked oxidative leading to pathogenesis . Free radical production is increased in patients with iron over load. Iron–over loaded patients have elevated plasma levels of thiobarbituric acid reactants and increased hepatic levels of aldehyde protein adducts , indicating lipid peroxideation (Canata, et al., 2001) . During the past years many scientific evidences have raised the advers effect of abnormal blood lipid levels, like total cholesterol and other lipids and lipoproteins, on atherosclerotic disease (Ginsberg 1994). 1899 Journal of Babylon University/Pure and Applied Sciences/ No.(5)/ Vol.(18): 2010 At this point it should be mentioned that the relation ships between blood lipids and atherosclerosis might be influenced by several other life style–related factors, like glucose intolerance, blood pressure levels dietary habits and smoking habits (William and Waikan , 2001) . Selimoglu, et al , (2002) suggested that both serum iron and triglycerides are involved in the pathogenesis of LDL–C oxidation. The present study examined the lipid profile of thalassemic patients in Babylon province . Materials and Methods -Patients:The study population included 40 patients with beta thalassemia major (20 females and 20 males) that undergo periodical blood transfusion and desferoxame as over four month in thalassemia center in Babylon. The tested group aged from 4-14 years . The diagnoses of beta-thalassemia major were made based on the clinical, hematological and hemoglobin electrophoresis profiles which is indicative sign of the presence of beta-thalassemia major. Twenty controls (10 Males and 10 females) of matched age and gender were also included in this study. All patients were transfusion dependent at a rate one to tow times monthly. Also , all of them are treated with desaferal . -Blood Collection:Blood samples were drawn from the thalassemic patients (whom are free from HBV , HCV and HIV) in plain tubes and allowed to clot and centrifuged to obtain serum . Methods Serum lipid profile was determined after 12-14 h of fasting including : total cholesterol (TC), triglycerides (TG) , high density lipoprotein cholesterol (HDL-C) and low density lipoprotein cholesterol (LDL-C), and these parameters were evaluated by using analytical kits from Biomagreb company . Statistical analysis All quantitative data were expressed as means S.D. student two sample t-test was used to explain the significance and p0.05 was obtained as a lower limit of significance . Results The results of various lipid analyses of controls and thalassemic children are explained in the table. Beta-thalassemia major patients had significantly (P<0.05) lower total cholesterol (T-C), high density lipoprotein cholesterol (HDL-C) and low density lipoprotein (LDL-C) compared with control. However serum triglycerides level of beta-thalassemia major males and females patients were significantly higher (P<0.05) than control males and females . 1900 Table : Serum lipids (triglycerides , total cholesterol , high density lipoprotein cholesterol , and low density lipoprotein cholesterol) levels of males and females children affected with beta-thalasseuia major . Parameter Patients Control Male Male Female Signficancy Female 92±18 84±80 Triglycerides (g /dl) 148±37 P> 0.05 136±41 109±22 179±31 168±45 Cholesterol (g/dl) P> 0.05 124±32 34±13 53±5 49±7 HDL – C (g/dL) P> 0.05 38±14 58±23 136±38 100±7 LDL-C (g/dl) P> 0.05 62±28 -Values are means + SD Discussion The present study investigated the distribution of some blood lipids in the blood of Babylon children with beta-thalassemia major, it was found that the majority of the participants had low total cholesterol levels HDL-C and LDL-C levels in addition triglycerides levels were substantially high. Papanastasiou, et al. (1996) have shown that total cholesterol , HDL and LDLcholesterol was significantly decreased , while triglycerides were significantly increased in the thalassemic patients compared to the control subjects . They also found a positive correlation between age and triglycerides levels. The present study also observed similar results regarding blood lipids levels among patients and healthy controls from general population. Other study on beta thalassemia major in patients from Italy found that these patients disclosed significantly lower total cholesterol, LDL cholesterol, HDL-cholesterol and higher triglycerides concentration (Papanastasiou et al., 1996). It appears, therefore, that many factors such as iron over load (high ferritin level), liver injury (disturbances of ratio between AST and ALT) and hormonal disturbances affects lipid pattern among patients with major form of beta thalassemia . Some authors suggested that accelerated erythropoiesis and increased uptake of LDL by macrophages and histocytes of the reticuloendothelial system are the main determinants of low plasma cholesterol levels in beta thalassemia (Maioli, et al., 1989; Maioli, et al., 1997). In addition, total serum phospholipids their fractions and cholesterol were significantly lower among patients with thalassemia major (Giardini, et al., 2004). These changes were referred to hepatic damage and iron over load furthermore, some serum lipid polyunsaturated fatty acids were significantly decreased among patients with beta thalassemia major as compared to normal controls. Since these alterations are a sign of lipid peroxidation, the causes of this phenomenon were studied . These differences on blood lipids and lipoprotein levels could also attribute to the adherence of a heal their lifestyle by people with beta thalassemia, which could include consumption of healthy food since childhood . However, other report (Maioli, et al., 1984) showed that adolescents with beta thalassemia minor have significantly lower cholesterol levels than patients with beta thalassemia major. The investigator suggested that this has been related to their disorder and not influenced by age, gender, hemoglobin or ferritin levels at this point 1901 Journal of Babylon University/Pure and Applied Sciences/ No.(5)/ Vol.(18): 2010 it should be noted that the extrapolation of these findings into other populations with beta thalassemia major might be under scrutiny since thalassemia is genetically oriented and various expressions of the related polymorphisms may be involved in the distribution of blood lipids and lipoprotein levels . Increased susceptibility of red-blood cells lipids to autooxidation in thalassemia major has been documented (Canatan, et al., 2001; Hartman et al., 2002). Since autooxidation may be initiated by free radicals which are constantly formed in the normal red cells, and may be especially prevalent when unstable hemoglobin is present (Thein, 1992) . This study shows similar results in lipid profile of thalassemic patients that were observed by other studiy, where significant decrease in total cholesterol and HDL-C were detected (Low , 2005) . Triglycerides lipase activities (both hepatic and extra hepatic) were significantly lower in thalassamic patients. Christina et al (2004) speculated that the decreased levels of these enzymatic activities could play a role in determining the decrease of HDL-cobserved in thalassmic patients. The present findings are in agreement with those found by other studies (Pongana and Pongana, 2002; Flavio, 2005) as there is significant increase in plasma triglycerides. However other researchers did not find such differences (Maioli, et al., 1999) they also noted an increase in triglycerides with increasing ferritin values and positive correlation of the patients triglycerides with both age and oxidative LDL-C antibodies (Kattwasser and Gottschalk , 1999) . A decrease in the amount of stored iron only known cause for a low serum ferritin result (Bishop et al., 2000) ; while increased stored iron (over load) associated with raised serum ferritin levels (e.g. massive blood transfusion). Also liver disease or inflammation with result in high serum ferritin (Barton , 2007) . Because of the regular blood trans fusions , approximately all beta thalasemic patients had abnormally very high levels of serum ferritin, indicating that these patients have iron over load, probably due to multiple blood transfusion, increased dietary iron absorption or in inadequate chelating therapy with desferral (Williamy and Waikn, 2001). In this study triglycerides levels could be correlated positively with the serum feiritin levels these result might support the hypothesis that both serum iron and triglycerides are involved in the pathogenesis of LDL-C oxidation (Selimoglu, et al. , 2002). The present data showed that LDL-C was significantly decreases in its values and it is in agreement with those found by other workers (Giardini, et al., 2004) . Moreover Maioli, et al., (1997) showed that LDL-C was influenced by both diagnosis and ferritin levels . In the present study we elicited a significant positive correlation between ferritin and triglycerides a marker of disturbed lipid profile with abnormal iron over load and inadequate chelating therapy with desferal. From this study , we conclude that a higher erythroid bone marrow activity with the enhanced cholesterol consumption be the dominant mechanism implicated in the lipid abnormalities of thalassemia major patients . References Barton, J.C. (2007). 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