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.

Co-Inheritance of α-thalassemia gene mutation in patients with sickle cell Disease: Impact on clinical and hematological variables.

BACKGROUND: Sickle cell disease (SCD) is a monogenic, phenotypically highly variable disease with multisystem pathology. The phenotypic heterogeneity of SCD is attributed to environmental and genetic factors such as fetal hemoglobin and co-inheritance of α-thalassemia. OBJECTIVES: To look for different types of α-thalassemia gene mutations among SCD patients and evaluate the influence of the co-inheritance of α-thalassemia on clinical and hematological variables. METHODS: This cross-sectional analytical study included 765 SCD patients, and 150 patients (with low mean corpuscular volume (MCV), low mean corpuscular hemoglobin (MCH) and normal serum ferritin levels) were tested for α-thalassemia gene mutations. Multiplex PCR and reverse hybridization and sequencing for both α genes using the Vienna Lab Strip Assay PCR study were performed using conventional PCR technology. RESULTS: Out of 150 patients tested for α-thalassemia gene mutations, 141 patients were found to have one or more of the mutational types, representing 18.4% of all studied SCD patients. The most common mutations found were the -3.7 deletion (76.6%), followed by the -4.2 deletion (12.1%), mutant α2polyA-1 (Saudi type) (9.2%), and --MED double gene deletion (7.8%). Acute painful episodes did not differ significantly in sickle cell anemia (SCA) patients with or without α-thalassemia, although the co-inheritance of α-thalassemia has a protective role against many disease-related complications. However, this role was not observed with other types of SCD. The means of red blood cell count, hemoglobin, and hematocrit were significantly higher, while the MCV, MCH, reticulocyte count, and hemoglobin A2 percentage were significantly lower in patients with α-thalassemia gene mutations than in those without α-thalassemia gene mutations (P < 0.05). CONCLUSIONS: The co-inheritance of α-thalassemia and SCA confers protection against many disease-related complications and is associated with improved hematological indices. However, this protection was not noticed in patients with other types of SCD.

Evaluation of the expression of red blood cell CD36, interleukin-6 and interleukin-8 in sickle cell anemia pediatric patients.

Sickle cell anemia (SCA) is a complex multisystem disease characterized by acute and chronic inflammation, with alterations in inflammatory cytokines and adhesion molecules. This case-control study was carried out to assess the levels of CD36, immature reticulocytes, interleukin (IL)-6 and IL8 in SCA patients (in crisis and the steady state) and healthy controls. It included 90 children who were 2-18 years old; 60 with SCA and 30 healthy controls. Complete blood count, total reticulocyte count, reticulocyte subpopulations, immature reticulocyte fraction (IRF), percentage of CD36-positive red blood cells (RBCs), IL-6 and IL-8 levels were evaluated. The total white blood cell (WBC) and neutrophil counts, CD36-positive RBCs percentage, IRF, IL-6 and IL-8 levels were significantly higher in crises than in the steady state (P < 0.05). We also found that patients with SCA had significantly higher reticulocyte, WBC and neutrophil counts, fetal hemoglobin, CD36-positive RBCs percentage, IRF, and IL-6 and IL-8 levels than healthy children (P < 0.05). A significant positive linear correlation was reported between IL-6 and neutrophils during crises (Spearman correlation coefficient = 0.397, P = 0.03). These findings suggest that the levels of adhesion molecules and inflammatory markers and IRF, as evidenced by CD36-positive RBCs, IL-6 and IL-8, are elevated in SCA patients, both during steady state and crises, although these elevations are more marked during crises. Further knowledge about these cytokines and adhesion molecules will help in understanding the pathogenesis and improve therapy of SCA.

Nutritional status of children and adolescents with haemophilia in Basra, Iraq.

INTRODUCTION: Patients with haemophilia are at increased risk of being overweight or obese, which in turn may have a profound effect on morbidity and quality of life. AIMS: To assess the nutritional status of paediatric patients with haemophilia and identify possible risk factors that may adversely affect their nutritional status. METHODS: A case-control study was performed on 63 haemophilia patients and 135 control subjects aged 5-18 years. For all participants, weight, height and body mass index Z (BMIZ) score were assessed. Physical activity (PA) was assessed using the International Physical Activity Questionnaire and the Children's Physical Activity Questionnaire for participants ≥15 years and 5-14 years old, respectively. RESULTS: The frequency of overweight and obesity was 36.29% among the control group compared with 23.81% among patients, while thinness was higher in patients with haemophilia (19.05%) compared with 9.63% in the control group, P = .042. Low PA was reported in 22.73% of haemophilia patients aged 5-14 years compared with 2.06% in the control group, P < .001. However, no difference in PA was reported among participants aged ≥15 years. There were no significant differences in the frequency of nutritional problems among patients with respect to age, type of haemophilia, disease severity and hepatitis C seropositivity. Regression analysis revealed a negative association between paternal educational level and high BMIZ in patients ≥15 years, P = .028. CONCLUSIONS: Paediatric patients seem to have lower BMIZ than the control group. In addition, most of the studied factors were not found to predict either high or low BMIZ among studied patients.

Microcytosis in children and adolescents with the sickle cell trait in Basra, Iraq.

BACKGROUND: Microcytic anemia, the most common form of anemia in children and adolescents, is a heterogeneous group of diseases that is acquired or inherited. We assessed the frequency and causes of microcytosis in children and adolescents with the sickle cell trait (SCT). METHODS: This descriptive study included 95 subjects (49 males and 46 females) with SCT who attended Basra Center for Hereditary Blood Diseases for evaluation. Investigations included complete blood count, high performance liquid chromatography, capillary electrophoresis, and measurement of serum ferritin and transferrin levels. RESULTS: SCT subjects had a low hemoglobin (Hb) concentration (9.79±1.75 g/dL), low mean corpuscular volume (MCV, 67.43±9.22), low mean corpuscular Hb (21.15±3.64), and a normal red cell distribution width (RDW, 14.00±2.30). Among 95 SCT subjects, 81 (85.26%) had microcytosis, 12 (12.63%) had normal MCV, and 2 (2.11%) exhibited macrocytosis. Sixty-three (77.78%) SCT subjects with microcytosis were iron deficient, and 18 (22.22%) had normal iron levels. The mean serum ferritin and HbA2 levels were significantly lower, while the RDW, sickle Hb, and serum transferrin levels were significantly higher in patients with microcytosis and iron deficiency compared to non-iron deficient subjects (P<0.05). Correlation coefficients did not reveal a significant association between the MCV and iron status of SCT subjects (P>0.05). CONCLUSION: Despite the frequent occurrence of iron deficiency in SCT subjects, co-inheritance of alpha-thalassemia seemed to be the cause of low MCV in non-iron deficient individuals with microcytosis. Genetic analysis is required to understand the genetic basis of this phenomenon.

Sero-epidemiological study of visceral leishmaniasis in Basrah, Southern Iraq.

OBJECTIVES: To study selected epidemiological aspects of visceral leishmaniasis, assess direct agglutination test (DAT) as a diagnostic method and the sero-epidemiological prevalence of the disease among apparently healthy children in Basrah, Iraq. METHODS: This prospective study included 146 children suspected of visceral leishmaniasis who were admitted to Basrah Maternity and Children Hospital and Basrah General Hospital from November 2004 till November 2005 and 37 serum samples that were collected from patients with different diseases considered in the differential diagnosis of visceral leishmaniasis. In addition, 1000 apparently healthy children were randomly selected for the sero-epidemiological survey. Direct agglutination test was done for all of them. RESULTS: Out of 146 suspected visceral leishmaniasis cases, 124 (84.9%) were proved by the examination of bone marrow aspirate, 132 (91.1%) were positive by direct agglutination test (DAT) and only 3 (2%) were positive by immunochromatographic strip test. The sensitivity and specificity of DAT were (100%), with a cut-off point of 1:800. In the in-patient group children less than 2 years of age were mainly affected. The highest frequency of disease was reported in July, 24 cases (18.8%). Sand flies were recorded in the environment of all sero-positive cases (100%) in each group, stray dogs and wild canines were present in (75.7% and 15.5%) in in-patients group compared to (69% and 22.5%) in sero-epidemiological group, respectively. Low maternal education was present in a significantly higher frequency among sero-positive cases. CONCLUSIONS: Direct agglutination test can be used as a screening tool for visceral leishmaniasis on a wide range in endemic areas, with a high sensitivity and specificity.

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.