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Background and Aims The number of children being hospitalized for heart failure (HF) is increasing and, in recent years, mechanical circulatory support (MCS) is gaining more importance in the treatment end stage HF. The use of these complex technology implies changes in nursing assistance with equally complex aspects of care. The aim of this work was to describe our experience in the management of pediatric patients supported by different devises at the cardiology department at the Bambino Gesu Children’s Hospital. Methods Within the last 5 years, the MCS have been used in children with severe circulatory failure resistant to pharmacological therapy. ECMO was used in 83 pediatric patients (aged3.4±6.0years),Ventricular assist devices in 27, Berlin Heart EXCOR Pediatric was implanted in 23 children (aged5.5±6.8years), Heart Mate II in two children (13 ages), and Jarvik 2000 in 3 adolescents. These were patients with cardiomyopathy, fulminant myocarditis, end-stage congenital cardiac defects, and acute heart failure after congenital heart surgery. Results Nursing intensive care management is very complex and includes infection and anticoagulation monitoring, wound care: surgical site and cannulas/pedestal medication, nutritional support, pain management, psychological support, mobilization and the daily nursing management of the children. Conclusions Our experience shows that management of pediatric patients on MCS requires a highly specialized pediatric team. The goal of a nurse caring for a child on MCS is to be an active participant of all aspects of the patient’s care including a deep understanding of the patient’s physiology, of the indication for mechanical support, and of the most important complication that can occur.

Background Essential surfactant properties include transfer to gas-liquid interface, reduction of surface tension and film replenishment during respiratory cycles. Objective To compare component-specific film formation properties of infant and therapeutic surfactants. Design/methods Using a multiwell fluorescence assay, we compared maximal fluorescence (Max), time to reach Max (tMax) and phospholipid concentration for ½ maximal signal (½Max) for calfactant (CAL), poractant (POR), beractant (BER), colfosceryl palmitate (COL), with surfactant from immature infants with RDS. Dose-response studies were performed for addition of SP-B, albumin and budesonide. Results Max and ½Max values for CAL were higher/similar to those of rat surfactant. There were significant differences between CAL and other therapeutic surfactants for Max (CAL >COL >POR >BER) whereas ½Max were similar except for COL. In surfactant from 39 infant tracheal aspirates, ½Max was inversely correlated with SP-B content (p = 0.001). Addition of SP-B to samples with low endogenous content ( Conclusions This assay reveals differences in film formation efficiency for therapeutic surfactants reflecting differences in SP-B content and lipid composition. Film formation by infant surfactant is strongly influenced by SP-B content. The findings support the key physiological role of SP-B and the safety of surfactant as anti-inflammatory drug vehicle.

Background and aims We compared the effect of 3 parenteral nutrition (PN) regimens on the short-term growth of the preterm infant (PI). Methods We randomised 220 PI with BW -1. d-1[Non-Protein Energy NPE = 68 Kcal . kg . kg -1. d-1[NPE = 68 Kca . kg -1. d-1]) or HPHE (AA3.5, Glu 14, Fat 3.5 g . kg -1. d-1[NPE = 85 Kcal∙kg-1.d-1]). PN was started at 1 h of life and intakes were increased to reach targets on day 4. The primary outcome was weight gain from birth to 36 W PMA. Results Birth demographics and anthropometry were similar between groups. Administered AA, Glu and Fat intakes were significantly different among groups by study design. Growth data are in the Table below. There were no significant differences in the major complications of prematurity, except for Sepses occurring from day 4 to 28: SPSE 12/72 (12%), HPSE 9/74 (12%), HPHE 23/74 (31%), p = 0.01. SPSE (n = 72) HPSE (n = 74) HPHE (n = 74) pAge at nadir of body weight (days) 4.6 ± 2.3 4.1 ± 1.6 3.9 ± 1.5 0.07 Weight loss at nadir (% of BW) 12.5 ± 5.6 11.3 ± 4.7 11.8 ± 5.9 0.4 Time from Birth to the Regained BW (days) 12.3 ± 4.5 10.8 ± 4.3 10.7 ± 5.0 0.07 Weight Gain from birth to 36 W PMA (g∙kg-1?d-1) 15.6 ± 2.0 16.2 ± 2.3 15.7 ± 2.3 0.2 Conclusions Increasing AA Intake from 2.5 to 3.5 g·kg -1 ·d -1 with 68 or 85 Kcal . kg -1. d -1 of NPE did not improve (statistically and biologically) short-term growth. HPHE Infants had a significantly higher rate of sepsis. The alleged benefits of enhancing AA-Energy during PN for the PI should be carefully balanced against the odd of increasing infections.

Background/aims Alveolar capillary dysplasia (ACD) is characterised by pulmonary veins misalignment, capillary paucity and alveolar misdevelopment, and caused by FOXF1 mutations only in 40% of cases. Objectives were 1. to identify known and new gene defects and 2. to correlate them with molecular/cellular mechanisms. Methods We recruited a cohort of 23 pathology-confirmed cases. When DNA was available, genome-wide copy number variation was analysed through Array Comparative Genomic Hybridization (aCGH). Mutations were tested by direct sequencing of FOXF1 and candidate genes identified by aCGH; Molecular pathways were analysed by multi-channel immunofluorescence microscopy of ACD cases compared to human fetal/neonatal lung tissue at various development stages. Results Genomic deletions or mutations were identified in 57% of tested cases. Besides FOXF1, two of the genes involved stand out as potential candidates: MEOX2 and TBX4. ACD cases showed a markedly decreased expression of c-kit, a marker expressed in pulmonary small arteries and capillaries in fetal lung controls. In normal fetal lungs FOXF1 and TBX4 were prevalently expressed at the mesenchymal-epithelial border, and MEOX2 in pulmonary vascular smooth muscle cells (PVSMC). Their expression pattern and intensity were altered in all ACD cases, indicating that decreased FOXF-1 and/or its downstream transcription factor TBX4 disrupt lung micro vessel formation and homing to alveolar epithelium, and that a similar phenotype may derive from dysregulated PVSMC proliferation and angiogenesis related to MEOX2 insufficiency. Conclusion Genetic defects affecting the FOXF1 pathway affect the mesenchymal, endothelial and epithelial cross-talk leading to lung developmental disruption, pulmonary hypertension and hypoxic respiratory failure.

Background and Aims Limited data are available on the metabolic effects of intravenous fish oil (FO) in premature infant. We reported on the plasma cholesterol lowering effect of an intravenous lipid emulsion containing 10% FO or FMS (10% FO, 50% MCT, 40% soybean oil-SO) when compared with MS (50% MCT, 50% SO) (D’Ascenzo et al. JPediatr 2011). Aim of this study was to ascertain if the lower plasma cholesterol concentrations resulted from reduced biosynthesis or enhanced clearance. Methods Eighteen premature infants were randomised to receive FMS (n=9, GA 199±17 days, BW 983±138 g) or MS (n=9, GA 202±17 days, BW 992±178 g). The IV lipids were increased to 2.5 g∙kg –1 ∙d –1 in the first week of life. On day 7 patients received an oral dose of deuterated water for the measurement of cholesterol and palmitate biosynthesis. The incorporation of deuterium from water into cholesterol (cholesterol biosynthesis) was measured by Gas Chromatography and Combustion Isotope Ratio Mass Spectrometry (GC-C-IRMS). Fractional and Absolute Synthesis Rates (ASR) were obtained and compared by Mann-Whitney Test. Results Plasma free cholesterol concentrations were significantly lower in FMS than in MS (53±5 vs 64±9 mg/dl, p=0.01). ASR were 8.5±3.4 vs 8.4±2.6 mg/kg/d in FMS and MS respectively (p=0.93). Conclusions Intravenous FO in preterm infants has marked cholesterol lowering effect but cholesterol biosynthesis was not reduced. Measurements of cholesterol catabolism are in progress.

Background and Aim Whether pulmonary surfactant deficiency or dysfunction contributes to the pathogenesis of neonatal pneumonia is still debated. Aim of our study was to measure surfactant disaturated-phosphatidylcholine (DSPC) kinetics and surfactant specific proteins A and B (SP-A, SP-B) amount in term newborns with pneumonia using stable isotopes as tracers. Methods Twenty-seven term newborns (GA 39.0±1.5 weeks, BW 3165±602 g) requiring mechanical ventilation were studied. Twelve had severe pneumonia and 15 no lung disease. All newborns received intra-tracheally 2 mg/kg U 13 C-DPPC mixed with 2 mg/kg of exogenous surfactant. Isotopic enrichment of DSPC palmitate was measured from tracheal aspirates by mass spectrometry and kinetic data calculated. Surfactant proteins were measured by ELISA. Data were expressed as median (interquartile range) and comparisons were performed by Mann-Whitney test. p Results DSPC pool size (PS) was 9.3 mg/kg (3.1–30.2) in newborns with pneumonia and 38.0 mg/kg (24.9–124.6) in controls, p=0.016. DSPC half-life (HL) was 12.7 h (5.2–20.2) and 25.6 h (18.5–65.6) in newborns with pneumonia and in controls, respectively (p=0.004). Analysis for SP-A and SP-B are in progress. In newborns with pneumonia a correlation was found between DSPC kinetic parameters and oxygen requirement (DSPC PS and mean FiO2, R= –0.61, p=0.047; DSPC HL and mean FiO2, R= –0.54, p=0.086). Conclusions Surfactant DSPC kinetics was found to be markedly impaired in term newborns with pneumonia. Preliminary data suggest that these alterations correlate with disease severity; thus, studies on exogenous surfactant therapy and on the effect on surfactant metabolism are needed.