BCL11A Polymorphism in Egyptian Children with ß-Thalassemia: Relation to Phenotypic Heterogeneity.

Fetal hemoglobin (HbF) is a potent genetic modifier of ß-thalassemia phenotype. B-cell lymphoma 11A ( BCL11A ) gene results in significant silencing of HbF. The aim of this study was to assess the prevalence of different BCL11A genotypes among a cohort of Egyptian children with ß-thalassemia and to correlate them to HbF and clinical severity score. Eighty-two children with ß-thalassemia (aged 12.95 ± 3.63 years) were recruited from the Pediatric Hematology Clinic, Ain Shams University. They were divided based on the clinical severity of ß-thalassemia into three subgroups: 20 mild (24.4%), 24 moderate (29.3%), and 38 severe (46.3%). Age, gender, age of diagnosis, initial HbF level, transfusion history, and history of splenectomy were assessed. Anthropometric measures, signs of anemia and hemosiderosis, and the severity score were determined. Laboratory investigations such as complete blood picture, ferritin, and single gene polymorphism genotyping of the rs11886868 were also performed. Our findings showed that 16 children had CC genotype (19.5%), 38 had TC genotype (46.3%), and 28 had TT genotype (34.1%) of the rs#. ß-thalassemia children with TT genotype had significantly higher severity scoring than the other two groups ( p < 0.001). Moreover, mean initial HbF was found to be lower in children with TT genotype followed by TC and CC genotypes ( p < 0.001). Increased γ-globin expression associated with BCL11A gene polymorphism is associated with better clinical severity of ß-thalassemia.

Postextubation Noninvasive Ventilation in Respiratory Distress Syndrome: A Randomized Controlled Trial.

OBJECTIVE: Successful extubation and prevention of reintubation remain primary goals in neonatal ventilation. Our aim was to compare three modalities of postextubation respiratory support-noninvasive positive pressure ventilation (NIPPV), nasal bilevel positive airway pressure (N-BiPAP), and nasal continuous positive airway pressure (NCPAP)-using the RAM cannula in preterm neonates with respiratory distress syndrome (RDS). Our secondary aim was to define the predictors of successful extubation. STUDY DESIGN: A total of 120 preterm neonates (gestational age ≤35 weeks) with RDS who had undergone primary invasive ventilation were randomized to receive either NIPPV, N-BiPAP, or NCPAP. The incidence of respiratory failure in the first 48 hours postextubation, total days of invasive and noninvasive ventilation, duration of hospitalization, and mortality were measured and compared among the three different noninvasive support modalities. RESULTS: There were no significant differences in the postextubation respiratory failure rates and the number of days of invasive as well as noninvasive ventilation among the three different support modalities (p > 0.05). The total number of days of mechanical ventilation and the duration of hospitalization were significantly higher in the N-BiPAP group than those in the NCPAP or NIPPV groups (p < 0.05). A gestational age of at least 29 weeks and a birth weight of at least 1.4 kg were predictive of successful extubation with a sensitivity of 98.2 and 85.3% and a specificity of 63.6 and 90.9%, respectively. CONCLUSION: Longer durations of mechanical ventilation and hospitalization were observed with N-BiPAP as a noninvasive mode of ventilation, but there was no significant difference in the extubation failure rates among the three modalities. Gestational age and birth weight were shown to be independent predictors of successful extubation of preterm neonates with RDS. KEY POINTS: · Successful extubation and reintubation prevention of preterms are primary goals in neonatal ventilation.. · NIPPV, N-BiPAP, and NCPAP could be used as postextubation noninvasive modes in preterm neonates.. · Gestational age and birth weight are independent predictors of successful extubation of preterms..

Gut priming with bovine colostrum and T regulatory cells in preterm neonates: a randomized controlled trial.

BACKGROUND: Necrotizing enterocolitis (NEC) and neonatal sepsis are still considered major problems, especially in formula-fed preterm neonates. This study aimed to investigate the effect of bovine colostrum on T regulatory cells, NEC, and late-onset sepsis in preterm neonates ≤34 weeks. METHODS: This prospective double-blind randomized controlled trial was conducted on 80 preterm infants who were randomly assigned to either the bovine colostrum group (n = 32) or control group (n = 48). T lymphocytes and their subsets, necrotizing enterocolitis, late-onset sepsis (LOS) and its severity, feeding tolerance, growth, length of hospital stay, and mortality were documented. RESULTS: The bovine colostrum group showed higher follow-up levels of CD4+CD25+ FOXP3+ T lymphocyte % (FOXP3 Tregs). FOXP3 Tregs and its difference in change levels between baseline and follow-up were considered as the most related factors to the bovine colostrum. Bovine colostrum group showed positive trends for reduction of sepsis severity and mortality with no significant difference in the incidence of NEC, LOS, and length of hospital stay. CONCLUSIONS: Preterm neonates who received bovine colostrum showed a higher FOXP3 Treg level. IMPACT: Bovine colostrum has no significant effect on the incidence of necrotizing enterocolitis. FOXP3 T regulatory cells and their increased level between baseline and follow-up is considered as the most influencing factors related to the bovine colostrum. Positive trends were noted for reduction of sepsis severity and concomitant mortality, but the study lacked the power to assess these outcomes.

Perioperative nutritional prehabilitation in malnourished children with congenital heart disease: A randomized controlled trial.

OBJECTIVE: The poor preoperative nutritional state of children with congenital heart disease (CHD) is often exacerbated postoperatively. The aim of this study was to evaluate the effect of perioperative 1- versus 2-wk nutritional prehabilitation programs on growth and surgical outcomes in malnourished children with CHD. METHODS: Forty malnourished infants scheduled for elective CHD surgery were randomized to receive either 1 or 2 wk of a nutritional prehabilitation program. Pre- and postoperative anthropometric parameters and feeding characteristics, feeding tolerance, duration of mechanical ventilation, intensive care unit (ICU) length of stay (LOS) and total hospital LOS were documented. RESULTS: The 2-wk prehabilitation group showed higher weight-for-age z-score and body mass index than the 1-wk group both preoperatively postnutritional, and postoperatively with significantly higher weight gain postoperatively. The 2-wk prehabilitation group had a shorter duration of postoperative mechanical ventilation, ICU LOS, and total hospital LOS. CONCLUSION: The 2-wk prehabilitation program was associated with better anthropometric measurements, shorter ICU LOS postoperatively, and shorter duration of hospitalization and mechanical ventilation. The preoperative nutritional status of children with CHD had a negative effect on ICU LOS and duration of mechanical ventilation.

Cerebral blood flow and serum neuron-specific enolase in early-onset neonatal sepsis.

BACKGROUND AND OBJECTIVES: Sepsis leads to systemic inflammatory response with cerebral blood flow (CBF) alteration and blood-brain barrier disruption that contribute to sepsis-associated encephalopathy (SAE). We aimed to evaluate cord blood neuron-specific enolase (cNSE) and CBF in early-onset neonatal sepsis (EONS) as predictors of SAE and to define short-term neurodevelopmental outcomes among survivors. METHODS: cNSE was measured in 200 neonates with antenatal risk factors for EONS, stratified into two groups: sepsis (n = 96) and no-sepsis (n = 104). Trans-cranial Doppler of peak systolic velocities (PSV), end diastolic velocities (EDV) and resistive indices (RI) of anterior (ACA) and middle (MCA) cerebral arteries recorded on day 1 postnatal. Griffiths mental developmental scale (GMDS) was assessed at 6 months. RESULTS: Increased cNSE, PSV, EDV, and decreased RI of both ACA and MCA were found in sepsis group compared to no-sepsis group (p < 0.001 for all). Patients with SAE (n = 34) had higher NSE, PSV, and EDV as well as lower RI of ACA and MCA compared to those without (p < 0.01 for all). SAE neonates had lower GMDS than those without. ACA RI of ≤0.61 was the best predictor of SAE. CONCLUSION: High CBF and cNSE could be useful markers for prediction of SAE. SAE impairs neurodevelopmental scales at 6 months.