الفهرس 
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Abstract
This study was carried out at the Pediatric Haematology Clinic and Echo cardio-graphic unit of the Pediatric Hospital, Zagazig University, during the period from June 2012 till November 2013.
This study aimed to asses the occurance of pulmonary arterial hypertention in B- thalassemia patients after spleenectomy, identifying the possible clinical predictors for development of this complication .
This study included 75 B thalassemia patients; these patients were on conventional therapy, each according to their clinical status in accordance with the WHO regimen
All patients received regular blood transfusion therapy before the age of one year, these patients are classified into 2 groups:
group (I):
Fifty patients were diagnosed as spleenectomized B- thalassemia major, their ages ranged from 1.5-13 years, they were 28 males and 22 females.
24 patients received. Desferrioxamine (desferal), 2 Desferrioxamine (desferal) + Deferiprone (kelfer), 8 Deferiprone (Ferriprox), 14 Deferasirox (Exjade) and 2 only received Deferasirox (Exjade) + hydra.
group (II):
Twenty five age and sex matched children with B. thalassemia major but not spleenectomized were used as a control group, they were on regular blood transfusion and iron chelating agents as follow 4 patients received Desferrioxamine (desferal), 5 received (desferal) + ferriprox, 4 received ferriprox, 1 received hydra, 1 received kelfer and 10 received Exjade.
All Cases and control were subjected to clinical assessment, laboratory evaluations and echocardiographic examination.
Mean age of our patients was11.09±2.65 for cases and 11.5±4.46 years for control group., with sex distribution of cases 56% male and 44% female and 64% males and 36% female for control group . There were significant increase in WBCS, platelets count, SGPT, serum bilirubin, HBA2, HBF and serum ferritin in cases than control group while HBA1 was significantly lower in cases than controls.
The study also showed significant decrease in frequency of blood transfusion after spleenectomy. Highest percentage of cases received desferal but highest percentage of controls received exjade and there was significant difference between both.
Pulmonary artery pressure was increased in cases than controls (ESPAP, EDPAP, MPAP, RPAP, LPAP) but EDPAP only was significantly increased.
Combination of iron chelation leads to more decrease in serum ferretin (desferal + kelfer) in cases and (desferal + ferriprox) in control group.
In our study there was positive correlation between serum ferritin and MPAP. Also there was significant WBCS among our cases no significant relation between EDPAP and different iron chelating agents.
But there was highly significant positive correlation between EDPAP and platelets count and a significant relation with serum bilirubin and SGPT among cases but no significant correlation among control group.
This study also showed significant positive correlation between MPAP with serum ferritin and serum bilirubin among cases but no significant correlation among control group.
. There was significant positive correlation between ESPAP and serum bilirubin and SGPT among cases but no significant correlation among control group.
Conclusion
Although spleenectomy in B thalassemia patients decreases the need for blood transfusion, it increases the risk of pulmonary hypertension.
Combined oral and subcutaneous iron chelating agents are more effective than a single agent in decreasing serum ferritin level.
Elevated serum ferritin, platelet count, serum bilirubin and SGPT may be used as indirect indicators of elevated pulmonary artery pressure.