الفهرس 
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Abstract
B-Thalassemia is an inherited hemoglobin disorder caused by impaired synthesis of the b-globin chain and resulting in chronic hemolytic anemia (Modell & Berdoukas, 1984). Depending on clinical severity, 2 forms thalassemia major (TM) and thalassemia intermedia (TI) are distinguished (Zurlo et al., 1989).
TM is rapidly fatal unless adequate transfusions, in conjunction with intensive iron chelation therapy, are started early enough (Modell & Berdoukas, 1984) .In contrast, TI is generally characterized by a mild clinical picture, better prognosis, and better chance for survival, and patients require therapeutic intervention only later in life (Ehlers et al., 2006).
Cardiac complications are a main feature of the clinical spectrum of b-thalassemia. They are the leading cause of death and have been well documented only in TM (Shawky & Kamal, 2012). The prominent finding in this condition is left ventricle (LV) dysfunction which is attributed mainly to iron overload and leads gradually to cardiac failure and cardiogenic death (Marshall et al., 2006).
Although TM and TI share common basic pathophysiological mechanisms, cardiac involvement may be different in the latter because these patients live longer and generally have low hemoglobin levels and lower iron loads (Shawky & Kamal, 2012).
By definition, the patients who spontaneously maintain hemoglobin at or above 7g/dl, even at the price of bone marrow hyperplasia, characterized as thalassemia intermedia (Aessopos et al., 2009). The main reasons of early death in such patients concern the cardiac complications such as systolic and diastolic disorders caused by hemosiderosis (Aessopos & Berdoukas, 2009).
The clinical picture of TI is dominated by the multiple long-term effects of chronic anemia and tissue hypoxia and their compensatory reactions, including enhanced erythropoiesis and increased iron absorption (Kanavakis et al., 2007). The former is expressed as bone marrow expansion and extramedullary hematopoiesis, leading in turn to bone deformities and to spleen and liver enlargement (Isner et al., 1982).
The main reasons of early death in thalassemia intermedia patients concern the cardiac complications such as systolic and diastolic disorders caused by hemosiderosis (Challenor et al., 2004).
The studies conducted so far indicated that the end-systolic and end-diastolic dimension index and also the whole heart index and systolic dimension of thalassemia intermedia patients have a significant increase compared with control group ,it happens while the patients did not receive blood transfusion and did not show any clinical symptoms of cardiac disorder (Kanavakis et al ., 2007).
In the absence of regular treatment of thalassemia intermedia patients the diastolic performance of the left ventricle is maintained stable, while the pressure of pulmonary arteries continues to rise which is the main cause of death in these patients (Isner et al., 1982).
This case-control study was conducted on 46 thalassemic patients (mean age: 9.7 ± 4.6years) that include 15 females (32.6%) and 31 (67.4%) males, and 46 healthy individuals (mean age: 10.3 ± 4.1 years) that include 29 (63 %) males and 17 (37%) females as a control group from 1st of October, 2016 to 30 of September, 2017 in the Pediatric haematology Clinics or inpatients of Pediatric Department during the period of the study.
We performed a clinical analysis and reviewing the data of 46 children having thalassemia intermedia, with special consideration for the age, sex, height, weight, age at diagnosis, age of first blood transfusion, number of blood transfusion during the period of illness, and both groups were subjected to complete clinical examinations, investigations including; Hb level and Serum ferritin level, chest X-ray, ECG and echocardiography test by 2D, Mmode, and Doppler methods and parameters such as Aorta/Left atrium diameter (AO/LA), Shortening fraction (SF), Ejection fraction (EF), Left ventricular end-systolic dimension (LVESD), Left ventricular , end-diastolic dimension (LVEDD), Left ventricular posterior wall dimension in systole (LVPWS), Interventricular septal dimension in systole (IVSS), Left ventricular posterior wall dimension in diastole (LVPWD), Interventricular septal dimension in diastole (IVSD), Left ventricular mass index (LVMI), Myocardial performance index (MPI), E/A velocity ratio (E/A), Peak A velocity (Peak A), Peak E velocity (Peak E), and finally data was analyzed by SPSS software.
We found that the systolic and diastolic performance of thalassemia intermedia patients is affected earlier in comparison with control group in some parameters including LVMPI, LVMI, RVMPI, EF, FS, IVSD, IVSS, LPWDS and LVPWDD. For more evaluation, more quantitative and comprehensive research suggested by evaluation of specific effective factors to psychosocial health of these patients.
we found that the inter ventricular septum in systole IVSS (mm), inter ventricular septum in diastole IVSD (mm), left ventricle posterior wall dimension in diastole LVPWDD (mm), left ventricle end diastolic dimension LVEDD (mm), aortic diameter AO (mm) and left atrial diameter LA (mm) showed a significant weak correlation with the serum ferritin level; this is mean increase the serum ferritin level will be associated with this echocardiographic parameter affection
Evaluation of cardiac function in patients with thalassemia intermedia is an area of active research especially in the field searching for methods that help for detection of cardiac function in thalassemia intermedia cases to prevent development of the cardiac complications. In future we may have better preventive, diagnostic and therapeutic options for these cardiac complications.
Study Limitation:
• Age group: we conduct only the pediatric age group thalassemia intermedia, and as we know the most of complication appear in older age.
• Number of patient: we conduct only46 patient in our study.
• Lack of material potential: availability of other facilities like T* and MRI as echocardiography is not able to replace them even echocardiographic abnormalities are found.
Recommendation:
Based on this study, we recommended informing pediatricians to:
1- know the importance of the follow up of cardiac function (systolic and diastolic) in patients with thalassemia intermedia for early detection and treatment of cardiac complication even if they don’t do not require frequent transfusions.
2- we recommend following up this patient regularly by investigation such as
a- serum ferritin level: to prevent iron overload that can be the cause of reduction of cardiac muscular contractility, progressive systolic dysfunction, and development of heart failure.
b- HB level to prevent the chronic anemia that is cause of ventricular chambers enlargement, increased cardiac output, and reduced total vascular resistance.
3- Evaluation of myocardial iron overload by cardiovascular magnetic resonance T2* in thalassemia patient for early diagnosis of myocardial iron overload and institution of chelation therapy and thus mitigate progressive subclinical deterioration of cardiac performance.