الفهرس 
ThalassemiaOnly 14 pages are availabe for public view
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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