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Aortic coarctation surgery in pediatric patients requires the placement of two arterial cannulas to monitor pressure gradients and surgical correction adequacy. Near-infrared spectroscopy (NIRS) monitoring provides insight into regional blood flow. This study aimed to investigate the correlation between NIRS values and invasive arterial pressures, exploring NIRS monitoring as a potential substitute for arterial cannulation. In a cohort of 21 consecutive pediatric patients undergoing aortic coarctation surgery, recordings of NIRS and invasive arterial pressure values were evaluated at various time intervals. Pearson correlation evaluated the relationship between NIRS values and invasively measured arterial pressures. Moderate to strong correlations were observed between the mean arterial pressure (MAP) of the upper and lower arteries and cerebral (rSO2-C) and somatic (rSO2-S) NIRS values 5 min after cross-clamp placement (r = 0.621, p = 0.003; r = 0.757, p < 0.001). Strong correlations were found 15 min after cross-clamp placement (r = 0.828, p = 0.002; r = 0.783, p = 0.004). Before transfer to the ICU, a strong correlation existed between the upper artery MAP and rSO2-C (r = 0.730, p < 0.001), but there was no correlation between the lower artery MAP and rSO2-S. These findings are promising, but further studies are required to validate it as a reliable substitute for invasive pressure monitoring in this patient population. [ABSTRACT FROM AUTHOR]

Background: The angiotensin-converting enzyme inhibitor (ACEI) enalapril is often administered to infants and young children with heart failure (HF) in various dosing regimens and formulations not adapted for their age. Methods: This prospective, two-center, open-label 8-week study evaluated an age-appropriate formulation of orodispersible minitablets (ODMTs) of enalapril (0.25 mg and 1 mg) in children aged 0 to 6 years with HF due to congenital heart disease. An age/weight-based dosing schedule was followed. Measures of echocardiographic parameters, blood pressure, heart rate, modified Ross score, and biochemistry were obtained over the 8-week period. The following two groups were assessed: ACEI-naïve and ACEI-pretreated patients. Results: In total, 53 children (age range of 0.05 to 4.8 years) were enrolled and 29 were ACEI-naïve. The average enalapril dose was 0.098 mg/kg (0.06–0.17 mg/kg) in the naïve group and 0.15 mg/kg (0.07–0.3 mg/kg) in pretreated patients. After 8 weeks, the modified Ross score and left ventricular diastolic dimension (LVD) z-score showed a significant decrease in both groups (p < 0.005). During 8 weeks follow-up, there were no difference in the z-scores for the systolic blood pressure (p = 0.071) or heart rate (p = 0.146). Conclusions: Pediatric patients treated with ODMTs of enalapril for 8 weeks had favorable improvements in LVD and HF symptoms. [ABSTRACT FROM AUTHOR]

Objective: The incidence of the 22q11.2 microdeletion among children who have at least two out of five major clinical criteria for 22q11.2 deletion syndrome. Design: Prospective study. Setting: University Children's Hospital in Belgrade, Serbia between 2005 and 2014. Participants: 57 patients with clinical characteristics of 22q11.2 deletion syndrome. Methods: Standard G-banding cytogenetic analysis was performed in all children, and the 22q11.2 genomic region was examined using fluorescence in situ hybridization (FISH). For patients with no deletion detected by FISH, multiplex ligationdependent probe amplification (MLPA) analysis was also done in order to detect cryptic deletions of this region and to analyze other genomic loci associated with phenotypes resembling the syndrome. A selected group of patients diagnosed to have 22q11.2 microdeletion by FISH underwent MLPA testing in order to characterize the size and position of deletion. Outcome Measure: The frequency of 22q11.2 microdeletion among children with at least two of the five major characteristics of 22q11.2 deletion syndrome (heart malformations, facial dysmorphism, T-cell immunodeficiency, palatal clefts and hypocalcemia/hypoparathyroidism) Results: Typical 22q11.2 microdeletion was detected in 42.1% of patients; heart malformation were identified in all of them, facial dysmorphism in 79.2%, immunological problems in 63.6%, hypocalcemia in 62.5% and cleft palate in 8.3%. Conclusions: A higher detection rate compared to one-feature criterion is obtained when at least two major features of 22q11.2 deletion syndrome are taking into consideration. The criteria applied in this study could be considered by centers in lowincome countries. [ABSTRACT FROM AUTHOR]