Lipids profile in children and adolescents with β-thalassemia major

ABSTRACT Introduction: Patients with β-thalassemia major (β-TM) are at risk of developing abnormal lipid profiles. Lipid abnormalities, in turn, have a potential role in the pathogenesis of some clinical aspects of thalassemia Objectives: To evaluate lipid levels and atherogenic indices in β-TM patients compared to healthy children and for any association between lipid levels, lipid peroxidation and inflammatory biomarkers Methods: This case-control study was carried out on 79 patients with β-TM, aged 6 to 16 years, registered at the Basrah Center for Hereditary Blood Diseases and 85 age- and sex-matched apparently healthy children and adolescents. Complete blood count, serum lipids and ferritin, liver function tests, C-reactive protein (CRP), high-sensitivity CRP (hs-CRP) and serum malondialdehyde (MDA) were evaluated Results: Patients with β-TM had significantly lower hemoglobin (Hb), total cholesterol (TC), high-density lipoprotein (HDL), low-density lipoprotein (LDL) and non-high-density lipoprotein (non-HDL) and significantly higher very low-density lipoprotein (VLDL), triglycerides (TGs), LDL/HDL ratio, MDA, hs-CRP, total serum bilirubin (TSB), alanine aminotransferase (ALT) and aspartate aminotransferase (AST) than the control group (p < 0.05). A bivariate analysis revealed that TC has a significant positive association with Hb. The TG has a significant negative association with Hb and a positive association with serum ferritin. Furthermore, MDA, TG, TSB, ALT and AST were significantly positively correlated with serum ferritin (p < 0.05) Conclusions: Atherogenic dyslipidemia, defined as a high low-density lipoprotein cholesterol (LDL-C/high-density lipoprotein cholesterol (HDL-C) ratio and high TG level, is common among pediatric β-TM patients, it is associated with iron overload and places patients at an increased cardiovascular risk.

Lipids profile in children and adolescents with ß-thalassemia major.

INTRODUCTION: Patients with ß-thalassemia major (ß-TM) are at risk of developing abnormal lipid profiles. Lipid abnormalities, in turn, have a potential role in the pathogenesis of some clinical aspects of thalassemia OBJECTIVES: To evaluate lipid levels and atherogenic indices in ß-TM patients compared to healthy children and for any association between lipid levels, lipid peroxidation and inflammatory biomarkers METHODS: This case-control study was carried out on 79 patients with ß-TM, aged 6 to 16 years, registered at the Basrah Center for Hereditary Blood Diseases and 85 age- and sex-matched apparently healthy children and adolescents. Complete blood count, serum lipids and ferritin, liver function tests, C-reactive protein (CRP), high-sensitivity CRP (hs-CRP) and serum malondialdehyde (MDA) were evaluated RESULTS: Patients with ß-TM had significantly lower hemoglobin (Hb), total cholesterol (TC), high-density lipoprotein (HDL), low-density lipoprotein (LDL) and non-high-density lipoprotein (non-HDL) and significantly higher very low-density lipoprotein (VLDL), triglycerides (TGs), LDL/HDL ratio, MDA, hs-CRP, total serum bilirubin (TSB), alanine aminotransferase (ALT) and aspartate aminotransferase (AST) than the control group (p < 0.05). A bivariate analysis revealed that TC has a significant positive association with Hb. The TG has a significant negative association with Hb and a positive association with serum ferritin. Furthermore, MDA, TG, TSB, ALT and AST were significantly positively correlated with serum ferritin (p < 0.05) CONCLUSIONS: Atherogenic dyslipidemia, defined as a high low-density lipoprotein cholesterol (LDL-C/high-density lipoprotein cholesterol (HDL-C) ratio and high TG level, is common among pediatric ß-TM patients, it is associated with iron overload and places patients at an increased cardiovascular risk.

Red Blood Cell and Serum Magnesium Levels Among Children and Adolescents With Sickle Cell Anemia.

Patients with sickle cell anemia (SCA) can acquire many biochemical abnormalities, including altered magnesium levels. However, the roles of magnesium in the pathogenesis and management of SCA need to be determined. The aim of this work was to evaluate magnesium levels among pediatric patients with SCA in Basra, Iraq. The study employed a case-control design and examined 87 patients with SCA (3-15 years old) who had attended the Basra Center for Hereditary Blood Diseases while in a steady state and 90 apparently healthy control subjects. Complete blood count, red blood cell (RBC), and serum magnesium, calcium, potassium, sodium, zinc, and copper levels were measured in all subjects. The results revealed significantly lower RBC and serum magnesium levels among the patients with SCA (3.62 ± 0.42 and 1.35 ± 0.19 mg/dL, respectively) than those among the control subjects (4.47 ± 0.55 and 1.87 ± 0.27 mg/dL, respectively). In addition, compared to the control subjects, the patients with SCA had significantly lower serum levels of potassium, sodium, and zinc; significantly higher serum levels of copper; and significantly higher Ca/Mg and Na/Mg ratios. Among the SCA patients, the RBC magnesium level was significantly negatively associated with the frequencies of vaso-occlusive crises (r = - 0.423, P < 0.001) and disease-related hospitalization (r = - 0.225, P < 0.05). To conclude, the RBC magnesium level, but not the serum magnesium level, is significantly associated with vaso-occlusive crises and hospitalization. Therefore, screening and management of low RBC magnesium levels in SCA patients are required.

The prevalence of autoantibodies among relatives for type 1 and 2 diabetic patients.

OBJECTIVE: To estimate the prevalence of islet cells autoantibodies among non-diabetic relatives of type 1 and type 2 diabetic patients. METHODS: The study was conducted at the College of Medicinal Technology, Basra, Iraq, from December 2010 to December 2012, and comprised both diabetics and non-diabetics. The participants were first divided into two groups: type 1 diabetics; and type 2 diabetics. Both the groups had 2 sub-groups each; their non-diabetic relatives and non-relatives as controls. Laboratory investigations were done to estimate glutamic acid decarboxylase and islet cell antigen-2 autoantibodies by enzyme-linked immunosorbent assay for all groups. Significant differences were assessed with chi-square test. RESULTS: Of the 300 subjects, 100(33.3%) were diabetics and 200(66.6%) non-diabetics. Among diabetics, 40(40%) had type 1 while 60(60%) had type 2 diabetes. Among type 1 diabetics, 27(67.5%) tested positive for glutamic acid decarboxylase autoantibodies compared to 15(25%) in type 2 diabetics, 6(15%) among relatives of type 1, 7(11.7%) among relatives of type 2 diabetic patients, and none among controls. Besides, 16(40%) of type 1 diabetic patients tested positive for islet cell antigen-2 autoantibodies compared to 8(13.3%) in type 2, and none in the sub-groups. CONCLUSIONS: The relatives of both types of diabetic patients showed immunity to islet cell autoantibodies which confirmed the effect of genetic factors in disease pathogenesis and may be important in disease prediction.

Evaluation of Serum Leptin Levels and Growth in Patients with ß-Thalassaemia Major.

Background. Iron deposition in the body can damage the endocrine glands of patients with ß-thalassaemia major (ß-TM). Leptin plays a key role in the regulation of appetite, body fat mass, and endocrine function. Objectives. This study aimed to evaluate the relationship between serum leptin and growth and pubertal development in patients with ß-TM, as well as whether serum leptin can predict growth retardation and delayed puberty in these patients. Methods. Fifty ß-TM patients (aged 8-20 years) and 75 age-matched healthy controls were recruited. Anthropometric data and sexual maturity ratings were assessed. Serum leptin was measured by ELISA. Results. Serum leptin levels were significantly lower in patients with ß-TM than in healthy individuals (P < 0.001). Leptin levels were also significantly reduced in female patients with short stature (P < 0.002) and in patients who displayed delayed puberty (P = 0.032) compared to those with normal stature who had reached puberty. The sensitivity of leptin for predicting short stature and delayed puberty among patients was 84.6% and 92.3%, respectively. Conclusion. Low serum leptin is sensitive to predict short stature and significant in ß-TM females only. This link could thus be used as a guide for further therapeutic or hormonal modulation.

Antioxidant status in acute asthmatic attack in children.

OBJECTIVES: To determine the oxidant - antioxidant imbalance in asthmatic children, by measuring the levels of malondialdehyde (MDA) as an oxidant marker of lipid peroxidation as well as antioxidant compounds like vitamin C, vitamin E and uric acid and to investigate whether their concentrations are associated with more severe asthma. METHODS: This case controlled prospective study was conducted on 219 children aged 1-12 years, attending Basra Maternity and Children Hospital. Included were 98 asthmatic children during acute attack and 121 nonasthmatic, apparently healthy children. Serum malondialdehyde (MDA) as an oxidant marker of lipid peroxidation, and vitamin C, vitamin E and uric acid (as antioxidants) were estimated in asthmatic children during acute attack and compared with non-asthmatic children. RESULTS: Asthmatic children during exacerbation of their asthma have significant lower serum levels of antioxidant compounds like vitamin C, vitamin E and uric acid (p < 0.001) and significantly high malondialdehyde as compared with the controls. MDA was significantly elevated (P < 0.001), while that of vitamin C, vitamin E and uric acid were significantly decreased with increasing severity of asthmatic attack (P < 0.001). A significant negative correlation between MDA with vitamin C (P < 0.05, r = - 0.44) was observed in severe asthmatic attacks. CONCLUSION: Asthmatic patients during acute attack suffer a high degree of reactive oxygen species formation causing considerable oxidative stress that is indicated by the high level of oxidants (MDA) and low level of antioxidants.