الفهرس | Only 14 pages are availabe for public view |
Abstract Summary The thalassaemias are common genetic disorders worldwide and they constitute a major problem for patients, health providers and society. Beta thalassemia patients usually require regular blood transfusions to survive, however the chronic transfusion, combined with extravascular haemolysis and increased intestinal absorption of iron, leads to significant haemosiderosis of all organs, including the heart. Cardiac complications such as heart failure and arrhythmias are the major causes of death in Beta Thalassemia patients. Iron cardiomyopathy is reversible, if chelation starts in time. Once heart failure develops, the prognosis is frequently poor.The new parameters of cardiac function, derived from two-dimensional speckle-tracking echocardiography could be useful for an early diagnosis of cardiac involvement in patients with Thalassemia major. Currently cardiac MRI has been known as a noninvasive technique of choice for monitoring iron overload in the heart , also identifies patients at high risk of heart failure and arrhythmia from myocardial siderosis in thalassemia major and is superior to serum ferritin and liver iron That study aim to detect early myocardial disease in beta thalassemia and to assess the effect of iron load on the Summary -117- myocardium by speckle tracking echocardiography correlating with the efficacy of iron chelation and other risk factors. This cross sectional study included 30 transfusion dependant β-thalassemia patients aged between 11-20 years with mean 14.77 ±2.45 years a male to female ratio 1.3:1 recruited from the Pediatric Hematology Unit, Children Hospital, Ain Shams University. All included patients were subjected to detailed medical history{including transfusion, chelation, hepatitis C virus history with calculation of mean serum ferritin in last 2years}, clinical examination, laboratory investigations including CBC, liver function tests, markers of hemolysis (total bilirubin, indirect bilirubin), Radiological investigation included Echocardiography (Tissue Doppler and Speckle Tracking),MRI T2*. Patients were divided into two groups according to mean serum ferritin in the last 2 years: group 1: included patients with mean serum ferritin less than 2500ng/dl in last 2 years prior evaluation. group 2: included patients with mean serum ferritin more than or equal 2500 ng/dl in last 2 years prior evaluation. There were no significant differences between both groups as regard age & sex distribution, age at diagnosis, duration of the disease,chelation history ,compliance to it and splenectomy . However patients with mean serum Summary -118- ferritin > 2500 ng/mL in last 2 years prior evaluation showed significantly higher percentage of patients with positive family history and significantly shorter interval between transfusion. There was a significantly positive correlation between mean s.ferritin in the last 2 years and the disease duration and a significantly negative correlation between mean s ferritin and interval between transfusion. There were no significant differences between both groups as regard laboratory data although patients with mean serum ferritin > 2500 ng/mL in last 2 years prior evaluation showed significantly higher level of liver transaminases(ALT). There were no significant differences between both groups as regard M mode echocardiographic parameters. Patients with mean serum ferritin > 2500 ng/mL in last 2 years prior evaluation showed significantly lower longitudinal strain by speckled echo and there was a significantly negative correlation between them . There was no significant difference between both groups as regard percentage of patients with cardiac affection by speakled and those with left ventricular dysfunction. Patients with mean serum ferritin > 2500 ng/mL in last 2 years prior evaluation showed lower cardiac T2* and Summary -119- higher liver iron concentration yet results did not reach statistical significance. There were no significant differences between both splenectomized and non splenectomized patients nor between patients on mono and those on combined chelation therapy as regard echocardiographic data (M mode,speckle),affection by speckled and cardiac MRI T2*. There were no significant differences between both male and female patients as regard echocardiographic data (M mode,speckle),affection by speckled and cardiac MRI T2*. There were no significant differences between patients with cardiac affection by speckled and those without as regard M mode echocardiographic data / |