Predictors of iron overload toxicity in multi-transfused beta-thalassemic children

Introduction: There are certain risk factors or predictors that can be used for early detection of cardiac iron overload to improve the long-term gains in beta-thalassemic children. Objective: The aim of the study was to consider the predictive abilities of some of the clinical attributes of the beta-thalassemia patients regarding cardiac iron overload to identify at risk patients. Materials and Methods: This current observational study was conducted in the Department of Pediatrics, VIMSAR, Burla from November 2016 to October 2018. A total of 105 thalassemic children were enrolled in the study after satisfying the inclusion criteria (multi-transfused beta-thalassemia children in the age group of 6–14 years). All the relevant data were collected and correlation-regression statistics were done using computer-based software. Results: Serum ferritin has weak negative correlation with left ventricular end diastolic diameter (LVEDD) (r=–0.511, p=0.000), good negative correlation with ejection fraction (EF) (r=–0.604, p=0.000), and weak positive correlation with left ventricular end systolic diameter (LVESD) (r=0.084, p=0.393). Number of units of packed red cell transfusion has strong negative correlation with EF (r=–0.785, p=0.000), weak negative correlation with LVEDD (r=–0.297, p=0.005), and weak positive correlation with LVESD (r=0.413, p=0.000). Corrected logistic regression equation, i.e., cardiac iron overload=1.997 (age in years) –3.119 (gender) –0.078 (units of packed red blood cells [PRBC]) +0.003 (serum ferritin in ng/ml) –0.149 (LVEDD in mm) –0.235 (weight in kg) –10.928 with prediction of 94.3%. Conclusions: Age of the patient, serum ferritin level, and number of units of PRBCs transfused, LVEDD and weight of the child are good predictors of myocardial iron overload among childhood beta-thalassemic and hence can be used as indices for monitoring of onset of cardiac iron overload

Serum ferritin as a diagnostic marker for cardiac iron overload among beta-thalassemia major children

Introduction: Beta-thalassemia major is the most common chronic hemolytic anemia. It is a well-comprehended fact that the toxic effects of iron overload particularly the cardiomyopathy are the major complication that roots from beta-thalassemia major children. Therefore, timely diagnosis is crucial to optimize the long-term gain. Objective: The objective of the study is to find the cutoff level of serum ferritin for early diagnosis of cardiac iron overload. Materials and Methods: This study was an observational analytical cross-sectional diagnostic study which was conducted from November 2016 to October 2018. With due approval of Institutional Ethics Committee and after taking proper informed consent from the parents and/or legal heir, 105 thalassemic children were enrolled in the study by simple consecutive sampling after satisfying the pre-defined inclusion and exclusion criteria. In this study, two-dimensional Doppler echocardiography was used to detect cardiac iron overload. Serum ferritin levels were estimated, and cutoff values were calculated for each of the echocardiographic parameters of cardiac iron overload, i.e. ejection fraction (EF), left ventricular end-diastolic diameter (LVEDD), and left ventricular end-systolic diameter (LVESD) by receiver operating characteristic curve analysis. Sensitivity (Sn), specificity (Sp), positive predictive value, and negative predictive value were calculated with considering p<0.05 as statistically significant. Results: The mean age of the study participants was 9±3 years. Cutoff value of serum ferritin for detecting abnormality in EF was 3286 ng/ml with Sn of 76.1% and Sp of 88.1%. Similarly, for detecting abnormal LVEDD, cutoff value of serum ferritin was 4640 ng/ml with Sn of 70.1% and Sp of 98.6%, and for LVESD, it was 3286 ng/ml with Sn of 90% and Sp of 70.5%. Conclusion: The serum ferritin level can be used as a reliable marker of myocardial iron overload among childhood beta-thalassemia and hence can be used as an important screening tool

Pulmonary arterial hypertension in children with sickle cell anaemia

Background:Pulmonary hypertension(PHT) is a life threatening complication which occurs in adults with sickle cell disease(SCD). But there is paucity of knowledge about prevalence of pulmonary hypertension in Paediatric SCD population.Objective:To find out the prevalence of pulmonary hypertension in children with SCD.Methods:552 children age between 5 to 14 years were enrolled after written informed consent was obtained from parents/guardian. Children with Congenital heart disease, pulmonary stenosis, Rheumatic heart disease were excluded from the study. PHT in all cases of with SCD children was screened by using 2D Echocardiography in the department of cardiology. PHT is defined as mean pulmonary artery pressure (mPAP) ≥ 25 mmHg at rest. All the data were validated and analyzed with SPSS version 25.Results:Overall prevalence of pulmonary hypertension in our study is 20.4%. Of these, 6.5% subjects have mild and 13.9% subjects have moderate pulmonary hypertension. Low haemoglobin (p=0.021), high number of crisis (p=0.000), high number of blood transfusion (p=0.000), presence of loud second heart sound (p=0.000) and presence of Parasternal heave (p=0.000) were significantly associated with pulmonary hypertension. The prevalence of pulmonary hypertension in children with SCD demonstrated an increase in trend with age.Conclusion:High pulmonary artery pressures do occur in children with SCD. Screening by echocardiography can lead to early detection and intervention