Assessment of the effect of combination therapy with deferiprone and desferrioxamine on cardiac complications in major thalassemia

Cardiac complications in major thalassemia are the most debilitating problem among them. Iron chelating with desferrioxamine, may be difficult or inadequate. The aim of this research is to evaluate the effect of combination therapy with the two drugs "deferiprone and desferrioxamine" on cardiac complications in major thalassemia. In a clinical trial study [before and after], 47 [24 men and 23 women] patients with major thalassemia referred to Booali research center, 10 to 35 years old, all of whom were undertaken combination therapy with deferiprone and desferrioxamine, enrolled in this study. Echocardiography was performed for all patients before and after at least 6 months of combination therapy. Echocardiographic systolic and diastolic function indices including LVEF, EPSS, CI and E/A, EF slope and MPI, as well as presence of arrhythmia were obtained. The data were analyzed by paired T Test and Pearson correlation. There were significant changes in ferritin, systolic function and MPI, indicating statistically significant improvement. Ferritin decreased to 3306 +/- 1347ng/ml from 4489 +/- 1607ng/ml [p=0.000]. LVEF increased to 69.7 +/- 2.6% from 67.6 +/- 3.9% [p=0.04]. EPSS decreased to 2.9 +/- 1.3mm from 3.9 +/- 2.1mm [p=0.004]. Cardiac Index increased to 3.6 +/- 0.18 from 3.5 +/- 0.3 [p=0.02]. MPI decreased to 0.33 +/- 0.05 from 0.37 +/- 0.06 [p=0.03]. Arrhythmia and diastolic function were also slightly improved which were statistically nonsignificant. The results showed that combination therapy with the two drugs deferiprone and desferrioxamine led to decrease ferritin level and improve systolic function in patients with major thalassemia

[Therapeutic effects of carvedilol on systolic function in children with dilated cardiomyopathy and heart failure]

Heart Failure [HF] and Dilated Cardiomyopathy [DCMP] are two common heart diseases among children. Carvedilol is the third generation of beta-blocker and although it has been approved in adults, very little is known about its safety, efficacy and dosing profile in children. This study is to evaluate the efficacy and safety of Carvedilol in children with HF or DCMP. This is a randomized double-blind study. We studied 30 patients with HF or DCMP for 6 months. 16 patients [2 DCMP and 14 HF] underwent standard medical therapy in addition to Carvedilol and the remained ones [2 DCMP and 12 HF] received the same regimen, except the placebo instead of Carvedilol. We visited all patients on a monthly basis program and echocardiographically, evaluated them for their systolic function indices including LVEF [Left Ventricular Ejection Fraction], EPSS [E Point Septal Separation], LVESV [Left Ventricular End Systolic Volume] and LVEDV [Left Ventricular End Diastolic Volume]. Data were analyzed by t-test and Mann-Whitney test. After 6 months of therapy, most of systolic function indices showed a more significant improvement in the Carvedilol group, including increase of LVEF [P=0.033] and decrease of EPSS [P=0.008] and LVESV [P=0.019]. No significant side-effects were observed in our patients. Pearson correlation coefficient between some variables showed further improvement of LVEF and EPSS in males. Also, response of cardiomyopathic patients to Carvedilol was more than patients with heart failure. The Carvedilol is an effective and safe remedy for treatment of children with HF and DCMP

[Prevalence of CHD in imperforated anus in patients referring to Booali Cina hospital from 1996 to 2003. [Is echocardiography required prior to surgery?]]

Imperforated anus is a disease that requires surgery in the first days of life therefore, association with other diseases or anomalies is of great importance. This study aims to determine the prevalence of Congenital Heart Disease [CHD] in patients with imperforated anus. This is a descriptive study on patients with imperforated anus referring to Booali hospital of Sari, IRAN from 1996 to 2003. Previous patients medical records was reviewed and physical examination and concurrently echocardiography were done for new patients. The data were subjected to descriptive statistical analysis using SPSS software. Ninty patients with imperforated anus were entered into the survey. Echocardiography revealed that 26 patients including 14 Female [57.2%] and 12 Male [42.8%] had CHD. 22 of CHD patients [84.6%] had imperforated anus[IA] of high type and 4 [15.3%] had IA of low type. CHD types consisted of : 7 patients [26.9%] ASD+PDA, 6 patients [23%] ASD+PDA+TR, 4 patients [15.3%] PDA, 4 patients [15.3%] MR, 1 patient [3.8%] TF, 2patients [7.6%] VSD and 2 patients [7.6%] ASD. Considering the high prevalence of CHD in IA, it seems that echocardiography is necessary in all those with IA particularly to predict outcome of surgery and prevent probable complications