RESUMEN
Early detection of myocardial dysfunction is essential for the management of patients with thalassemia. Four-dimensional echocardiography imaging technique may be useful for detecting subclinical cardiovascular disease. To evaluate the 4-dimensional echocardiographic strain in children with beta thalassemia major with no cardiac manifestation and correlate it with other echocardiographic parameters. This is a prospective cross-sectional cohort Study included 200 children, 1-18 years-old. They were divided into: One hundred children with p-Thalassemia major with no clinical cardiac manifestations and 100 healthy children as a control group. They were subjected to the following investigations: Complete blood count, serum ferritin and Four-dimensional echocardiographic strains (Longitudinal, Circumferential, Radial and Area strains). There was no significant difference between the two groups as regard mitral annulus systolic velocity (S wave), E/A ratio and iso-volumic acceleration, but there was a significant difference as regard to ejection fraction, left ventricle mass, sphericity index and myocardial performance index. The mean values of Left Ventricular Strains (Longitudinal, Circumferential, Radial and Area strains) were significantly lower in patients with thalassemia (- 14.86 ± 12.13, - 8.01 ± 3.829, 33.13 ± 10.61, - 19.45 ± 6.866) than controls (- 19.13 ± 1.502, - 16.32 ± 1.34, 37.28 ± 4.209, - 22.94 ± 3.06) than controls respectively with a positive correlation with 2-Dimensional strain. Strain parameters of the left ventricle obtained by four-dimensional.
RESUMEN
BACKGROUND: Mobilizing hematopoietic stem cells may be a promising intervention for the treatment of idiopathic dilated cardiomyopathy (IDCM) in infant and children. So the aim of the work is to evaluate the efficacy of granulocyte-colony stimulating factor (G-CSF) as a therapeutic modality in pediatric IDCM. METHODS: A randomized clinical trial was conducted on 40 pediatric patients with IDCM. They were subjected to history taking, clinical examination, serum lactate dehydrogenase (LDH), total creatinine phosphokinase (CPK), creatinine phosphokinase isoenzyme B (CK-MB) isoenzyme, and peripheral blood CD34(+) cell assessment before and at day 7 after subcutaneous G-CSF injection for 5 consecutive days. Echocardiography was done before and 1, 3 and 6 months after therapy. RESULTS: Clinical improvement in the form of regression of patients Modified Ross heart failure (MRHC) classification classes. Increased percentage of CD34(+) mobilized cells from the bone marrow, and significant increase in blood counts especially white blood cells 7 days after G-CSF injection. Significant improvement was found in echocardiographic data evaluating systolic function of the heart [Ejection fraction, Fractional shortening and systolic velocity at mitral annulus (Sm)]. CONCLUSIONS: Administration of G-CSF may be beneficial in improving systolic functions of the heart in pediatric IDCM and more studies with a large number of patients are needed.
