الفهرس | Only 14 pages are availabe for public view |
Abstract The thalassemias are a group of autosomal recessive blood disorders characterized by defects in the synthesis of one or more of the hemoglobin chains. The consequent globin-chain imbalance predisposes those affected to hemolysis and impaired erythropoiesis. The result is a spectrum of disorders ranging from asymptomatic individual carriers, through to patients who may require life-long regular blood transfusions. Prx2, a typical 2‐cysteine (Cys) peroxiredoxin, is part of the cyto‐protective systems involved in the tight control of the levels of reactive oxygen species (ROS) generated during cell’s life span or related to cellular stress such as in iron‐overload. Since ROS might also function as a second messenger through transient oxidation of cysteine residues in signaling targets, it has been proposed that the redox state of cysteine residues of Prxs might control the redox state of partner proteins possibly through on‐off switching mechanism of proteins sensitive to redox conditions. We aimed to evaluate serum peroxiredoxin-2 concentrations in children with betathalassemia and to explore its possible relations with the severity of clinical features and iron overload. After An approval from Menoufia faculty of medicine ethical committee was taken before beginning of the study and informed consent was taken from the guardians of each participant. Our study conducted on 90 children, forty of them with beta thalassemia major and twenty with thalassemia intermedia as study group, and their results was compared with thirty apparently healthy children as control group in hematology unit of Pediatric department, Menoufia University hospitals. Our results showed that Consanguinity and family history were significantly higher in thalassemia major and thalassemia intermedia than control group with no significant difference between thalassemia major and thalassemia intermedia. There was no significant difference regarding age and sex. Weight Z score and BMI Z score were significantly lower in thalassemia major and thalassemia intermedia than control group with no significant difference between thalassemia major and thalassemia intermedia. Height percentile was significantly lower in thalassemia major and thalassemia intermedia than control group. Also weight percentile and BMI percentile were significantly lower in thalassemia intermedia and thalassemia major than control group with no significant difference between thalassemia major and thalassemia intermedia. There was no significant difference between study groups regarding weight, height, BMI and height Z score. Splenectomy, iron chelation therapy, pallor and jaundice were significantly higher in thalassemia major than thalassemia intermedia. The age of start blood transfusion starts earlier in thalassemia major than thalassemia intermedia. Abdominal examination (splenomegaly and splenectomy scar) was significantly higher in thalassemia major than thalassemia intermedia. However, there was no significant difference between β thalassemia major and intermedia regarding history of complication and dark urine. Iron, transferrin saturation and ferritin were significantly higher in thalassemia major than thalassemia intermedia |