الفهرس 
ThalassemiaOnly 14 pages are availabe for public view
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Abstract
β
thalassemia is a hereditary chronic hemolytic anemia that typically requires lifelong regular transfusion therapy to keep hemoglobin levels close to normal and allow adequate tissue oxygenation. The chronic administration of large amount of blood together with extravascular hemolysis and increased iron absorption in the intestinal system leads to iron deposition into the heart, liver, lung etc. in patients with β-TM.
It can be reversible only if early intensive chelation therapy has been initiated. Also, the iron induced cardiomyopathy is silent in young patients with β-TM and conventional echocardiography is not sensitive for early diagnosis of the preclinical stage of cardiac involvement.
Tissue hypoxia in β-thalassemia together with high level of extracellular non-transferrin-bound iron (NTBI) which is very toxic and labile plasma iron (LPI) or ‘free iron’ which is a directly chelatable component of NTBI. It is highly toxic as it can catalyze and form harmful free hydroxyl radicals which results in modification of the N terminal of human serum albumin representing the primary binding site for metals decreasing its binding capacity for metals and resulting in the formation of ischemia-modified HAS (IMA).
The aim of the present study is to measure the level of ischemia modified albumin (IMA) as a marker of tissue hypoxia and enhanced oxidative strees in β-thalassemia major Egyptian patients, compare its level with healthy individual and correlate it with serum ferritin and echocardiographic finding.
This work was a case control study, that was conducted at Hematology Clinic children’s hospital, Ain shams university hospitals.
Sixty patients with β- thalassemia major were selected from those who were attending the clinic served as a patients group and sixty age and sex matched healthy individuals served as a control group.
All studied patients were subjected to a full clinical assessment and were investigated by serum level of ischemia modified albumin (IMA) and echocardiography using conventional M mode echocardiography.
The control group were investigated by ischemia modified albumin (IMA) and echocardiography.
Our study showed that no significant difference between patients group and control group as regard age and gender.
Upon comparing the serum level of IMA between patients whose rang was (38-400) ng/ml and control whose range was (8-65) ng/ml there was a significant increase in the level of IMA among patients compared to control group (p=0.000) and cut off value of IMA between patients and control is 38ng/ml.
The patients had significant higher IVSD, LVIDD, LVPWD, IVSS, LVIDS, AOD, LAD, PAP (p=0.000) and significant lower EF% (p=0.000) and FS% (p=0.008) in comparison with control. There was significant positive correlation between IMA and serum ferritin (r=0.663) (p=0.000) also positive correlation between IMA and both LVIDD (r=0.385) (p=0.002) and LVIDS (r=0.411) (p=0.001) while there was negative correlation between IMA and both EF(r=- 0.383) (p=0.003) and FS (r=-0.356) (p=0.005).
Cut off value of IMA between normal and abnormal values of LVIDD, LVIDS, EF and FS is 190ng/ml (68.3%) of our patients had pulmonary hypertension which was significantly higher in splenectomized group of patients (P=0.004).
Also we detected a positive correlation between serum ferritin and both LVIDD (r= 0.269) (p=0.037) and LVIDS (r= 0.308) (p= 0.017) and the negative correlation between serum ferritin and both EF (r= -0.364) (p= 0.004) and FS (r= -0.336) (p= 0.009).
The cut off value of serum ferritin between normal and abnormal values of LVIDD and LVIDS is 2952ng/ml.
The cut off value of serum ferritin between normal and abnormal values of EF and FS is 2644 ng/ml.
Our study showed that higher level of IMA (p=0.007) and serum ferritin (p=0.000) were related to low adherence to chelation therapy.