الفهرس 
Aim of the WorkOnly 14 pages are availabe for public view
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Abstract
SUMMARY AND CONCLUSION B
eta Thalassemia is considered the most common chronic hemolytic anemia in Egypt (Shawky and Kamal, 2011).
Regular transfusion and chelation treatment in
β-thalassemia major βTM is improving patients’ quality of live, but creates a state of iron overload with the consequence of iron deposition in parenchymal tissues. Especially, elevated cardiac iron concentration is an early marker for the risk of cardiomyopathy and arrhythmia.
This work was done to assess exocrine and endocrine pancreatic function in beta thalassemia major patients in relation to disease duration, frequency of blood transfusion, iron overload especially cardiac iron overload, iron chelating therapy, compliance to chelation and find out if decline changes in exocrine and endocrine pancreatic function can reflect hepatic or cardiac iron deposition.
Cross sectional study was conducted from April 2012 to April 2013 on 44 children and adolescents suffering from beta thalassemia major βTM from those regularly attending the Hematology clinic, Children’s Hospital, Ain Shams university and sequentialy collected and 44 Children and adolescents comparable in age and sex to the patients group was taken as control after taking their parents consent and will be subjected to biochemical investigations (serum amylase and lipase) with exclusion of patients with any systemic disease rather than beta thalasemia major.
The studied children were subjected to full medical history laying stress on symptoms and complications of iron overload.
The children and adolescents with βTM group were subjected to the following laboratory investigations. Complete blood count (CBC), hemoglobin level and platelet count, serum ferritin, liver functions tests and exocrine pancreatic functions in form of S. amylase and S. lipase The triglycrides• glucose (TyG) index, oral glucose tolerance test. Magnetic resonance imaging T2*study of both heart and liver also was done.
In our study, cardiac iron overload among 35.7% of studied patients with cardiac T2* less than 20ms. Also about 21.4% of our patients have cardiacT2* less than 10ms so heavily loaded with iron in cardiac βTM patients with cardiac complication in the current study had low cardiac T2* than other thalasemic patients (Pvalue =0.028).
Higher median serum ferrritin was found among patients with low cardiac T2* (P value= 0.034).
Thalasemic patients with cardiac T2* more than 20 have serum ferritin <3256 ng /dl, TYG index <27800, serum amylase >46 IU/L, serum lipase >56.IU/L and fasting blood glucose more than 118mg/dl with positive predictive value of 77and negative predictive value of 65. In the current study, hepatic siderosis was detectedin (59%) of studied patients with liverT2*ranged from (4 - 9) ms.
Also a significant correlation between LIC and serum ferritin (p value=0.010) among our studied βTM patient Thalasemic Patients with splenctomy in our studied group show no significant correlation with LIC and cardiacT2∗ A significant difference between patients and control as regards serum amylase and lipase (p value=0.003) and (p value=0.056) respectively. With also inverse correlation between serum amylase and lipase with triglyceride index among βTM patients (r= -0.375* P= 0.014) and (r=-0.368* P = 0.017) respectively.
We classified our studied patients according to fasting blood glucose into (3 groups), 45.5% of studied patients with normal FBG (less than 100), 9.5% Patients with glucose equal to or above 126 ng/dL were considered diabetic and about 45.5% of studied patients had impaired fasting glucose (fasting blood glucose between 100 ng/dL and 125 ng/dL.
In the current study thalasemic patients with diabetes show low serum amylase (p value= 0.0005) and lipase
(p value= 0.0007) also low cardiac T2* (p value = 0.0006) and LIC (p value = 0.0006) than other βTM patients.
Inverse correlation between triglyceride index and cardiacT2* (r=-0.376 P=0.014) and LIC (r= -0.629 P=0.000) was found that reflects insulin resistance among studied patients with low LIC and cardiac T2*.
High serum triglycerides level among diabetic patients (154-222) with significant correlation between serum triglycerides and LIC (r= -0.629 P=0.000) and cardiacT2*
(r= -0.542 P= 0.000).
So, the endocrine and exocrine pancreatic function might become an equivalent predictor, to cardiac siderosis especially, as pancreatic iron seems to be found predominantly in the exocrine tissue however, further studies have to be done to verify results on a larger patient scale