ABSTRACT
Background: Congenital heart disease (CHD) is the most common birth defect and accounts for significant global morbidity and mortality. Relatively little is known about the epidemiology of CHD in Jordan or the manner in which CHD is identified. Methods: A retrospective medical record review was conducted for all neonates who had an abnormal echocardiogram performed at a tertiary referral hospital. All included neonates had echocardiography performed by the same pediatric cardiologist at the discretion of the treatment team. Descriptive statistics were used to describe CHD incidence, types of CHD identified, and mechanism of identification. Results: The incidence of congenital heart disease was 17.8 per 1,000 live births. This rose to 24.6 per 1,000 if patent ductus arteriosus in preterm infants was included. The most common identified abnormalities were PDA, atrial septal defects, persistent pulmonary hypertension, septal hypertrophy, and ventricular septal defects. Most children were evaluated either for a murmur heard on exam or as a part of screening due to other comorbidities or risk factors. Less than 1% of children had a prenatal diagnosis. There was a higher rate of persistent pulmonary hypertension during the COVID-19 pandemic than before (p < 0.001). Conclusions: There is a high incidence of CHD in Jordan. Increased prenatal and perinatal screening for CHD may allow for earlier detection.
ABSTRACT
INTRODUCTION: Beta thalassemia major is the commonest inherited hematological disorder worldwide which needs lifelong sufficient supportive management. Hematopoietic stem Cell transplantation (HSCT) is the only curative treatment available till now. AIM: To evaluate the outcome of children who underwent allogenic hematopoietic stem Cell transplantation as a curative approach for Thalassemia Major, treated at Queen Rania AL- Abdullah children Hospital (QRCH). METHODS: A retrospective review of the medical files was conducted for all children (< 15 years) who had thalassemia major and received HSCT between January, 2010 and January, 2019. The following variables were studied for all patients: age , gender, Pesaro classifications, the count of infused raw bone marrow stem cell (CD34), engraftment time, outcome and complications. RESULTS: A total of 34 children were transplanted for thalassemia major, at an average of 4 cases per year. All underwent allogenic raw bone marrow transplantation from matched related donors. Thirteen patients (38.2%) were males and twenty one (61.2%) were females. The age ranged between 2 and 15 years, with a median age of 6.5 years. According to Pesaro classification, 31 patients were class 2 (91.2%) and 3 patients were class 3 (8.8%) while no single case met the criteria for class 1 Pesaro classification. The median CD34 count was 3.5 million/Kg of recipient weight (range, 1.5*106-7*106 /kg). The median time for neutrophil engraftment was 15.5 days. At a median follow up of 5 years (range 1- 9.5), 33 patients were alive. One patient died before 100 days post transplantation due to grade IV acute gastrointestinal Graft Versus Host Disease (GVHD). Three patients had secondary graft failure (8.8%). Six patients (17.5 %) developed mild grade 1-2 skin GVHD while another patient developed hemorrhagic cystitis due to BK virus and cytomegalovirus (CMV) which reactivated simultaneously, and was successfully managed. CONCLUSION: The outlook for Thalassemia major has dramatically changed after HSCT, with a considerable success in Jordan and results comparable to international data.
