الفهرس 
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Abstract
Summary
Beta-thalassemias (β-thalassemias) are a group of inherited blood
disorders caused by reduced or absent synthesis of the beta
chains of hemoglobin resulting in variable phenotypes ranging from
severe anemia to clinically asymptomatic individuals.
Repeated transfusion produces chronic iron overload which causes many complications including cardiac, pulmonary, hepatic and frequently endocrine complications.
The aim of this work is to study the pathophysiology of iron overload in children with chronic hemolytic anemias ( thalassemia intermedia, thalassemia major ).
The studied children included sixty patients with chronic hemolytic anemias divided into three groups; Thalassemia intermedia (n=30), thalassemia major (n=30) and th.]irty children serve as control.
All patients were subjected to detailed history taking, complete physical examination including; general examination, anthropometric measurement, and complete systemic examination.
All cases were investigated including; complete blood picture, blood film, reticulocytic count, serum ferritin and serum soluble transferrin receptors (sTfR.) (ELISA).
This study revealed the following results:
There is no statistically significant difference between all studied groups of hemolytic anemias as regard general charachteristic data (age, sex and), but there is high positive consanguinity among diseased groups.
There was a statistically significant short stature and underweight in studied groups specially thalassemia major group.
There was an increase in splenectomy rate; 6 in thalassemia intermedia (30%), 11 in thalassemia major (55%) and 6 in sickle cell anemia (30%)) and increased incidence of splenomegaly; 11 in thalassemia intermedia (55%), 9 in thalassemia major (45%) and 9 in sickle cell anemia(45%) .
There was an increased incidence of hepatomegaly in all studied groups; 13 in thalassemia intermedia (65%), 15 in thalassemia major (75%)
There was a statistically significant low values of hemoglobin among all studied groups specially thalassemia major group that had the lowest hemoglobin concentrations than thalassemia intermedia .
There was no statistically significant difference in the serum ferritin level among the studied groups, that it was found to be high among all patients with chronic hemolytic anemias, however, the highest values were found among patients with thalassemia major.
There was no statistically significant difference in the serum soluble transferrin rceptor (sTfR) level among the studied groups, that it was found to be high however, the highest values were found among patients with thalassemia major.
There was a statically negative correlation between sTfR levels and hemoglobin levels among all studied groups.
There is a significant positive correlation between sTfR and serum ferritin among diseased groups explained by the presence of a high degree of ineffective erythropoisis (reflected by elevated sTfR) that cause hepcidin depression which leads to increased iron absorption and iron overload (reflected by elevated serum ferritin).