In patients with BAV, the root phenotype (aortic dilation predominantly at the sinuses, with normal or less dilated ascending tract) may be a marker of more severe aortopathy, warranting closer surveillance and earlier treatment. The more common ascending phenotype proved to be a more stable disease entity, generally with slower progression.

The thymus plays a fundamental role in establishing and maintaining central and peripheral tolerance and defects in thymic architecture or AIRE expression result in the development of autoreactive lymphocytes. Patients with partial DiGeorge Syndrome (pDGS) and Down Syndrome (DS) present alterations in size and architecture of the thymus and higher risk to develop autoimmunity. We sought to evaluate thymic architecture and thymocyte development in DGS and DS patients and to determine the extent to which thymic defects result in immune dysregulation and T cell homeostasis perturbation in these patients. Thymi from pediatric patients and age-matched controls were obtained to evaluate cortex and medullary compartments, AIRE expression and thymocyte development. In the same patients we also characterized immunophenotype of peripheral T cells. Phenotypic and functional characterization of thymic and peripheral regulatory T (Treg) cells was finally assessed. Histologic analysis revealed peculiar alterations in thymic medulla size and maturation in DGS and DS patients. Perturbed distribution of thymocytes and altered thymic output was also observed. DGS patients showed lower mature CD4+ and CD8+ T cell frequency, associated with reduced proportion and function of Tregs both in thymus and peripheral blood. DS patients showed increased frequency of single positive (SP) thymocytes and thymic Treg cells. However, Tregs isolated both from thymus and peripheral blood of DS patients showed reduced suppressive ability. Our results provide novel insights on thymic defects associated with DGS and DS and their impact on peripheral immune dysregulation. Indeed, thymic abnormalities and defect in thymocyte development, in particular in Treg cell number and function could contribute in the pathogenesis of the immunodysregulation present in pDGS and in DS patients.

The two most common BAV morphotypes are associated with aortic dilatation at two different tracts (RL at the root; RN at the tubular ascending tract) independently of valve function. Moreover, the determinants of aortic dilatation were at least in part different between the two morphotypes: this may provide stratification criteria for individualized methods of follow-up and treatment.

The quantity and quality of dietary lipids in infant formulae have a significant impact on health outcomes, especially when fat storing and/or absorption are limited (e.g., preterm birth and short bowel disease) or when fat byproducts may help to prevent some pathologies (e.g., atopy). The lipid composition of infant formulae varies according to the different fat sources used, and the potential biological effects are related to the variety of saturated and unsaturated fatty acids. For example, since lipids are the main source of energy when the normal absorptive capacity of the digestive tract is compromised, medium-chain saturated fatty acids might cover this requirement. Instead, ruminant-derived trans fatty acids and metabolites of n-3 long-chain polyunsaturated fatty acids with their anti-inflammatory properties can modulate immune function. Furthermore, dietary fats may influence the nutrient profile of formulae, improving the acceptance of these products and the compliance with dietary schedules.

The metabolic response to stress and infection is closely related to the corresponding requirements of energy and nutrients. On a general level, the response is driven by a complex endocrine network and related to the nature and severity of the insult. On an individual level, the effects of nutritional interventions are highly variable and a possible source of complications. This narrative review aims to discuss the metabolic changes in critically-ill children and the potential of developing personalized nutritional interventions. Through a literature search strategy, we have investigated the importance of blood glucose levels, the nutritional aspects of the different phases of acute stress response, and the reliability of the available tools to assess the energy expenditure. The dynamics of metabolism during stressful events reveal the difficult balance between risk of hypo- or hyperglycemia and under- or overfeeding. Within this context, individualized and accurate measurement of energy expenditure may help in defining the metabolic needs of patients. Given the variability of the metabolic response in critical conditions, randomized clinical studies in ill children are needed to evaluate the effect of individualized nutritional intervention on health outcomes.

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Introduction: Accurate assessment of resting energy expenditure (REE) can guide optimal nutritional prescription in critically ill children. Indirect calorimetry (IC) is the gold standard for REE measurement, but its use is limited. Alternatively, REE estimates by predictive equations/formulae are often inaccurate. Recently, predicting REE with artificial neural networks (ANN) was found to be accurate in healthy children. We aimed to investigate the role of ANN in predicting REE in critically ill children and to compare the accuracy with common equations/formulae. Study methods: We enrolled 257 critically ill children. Nutritional status/vital signs/biochemical values were recorded. We used IC to measure REE. Commonly employed equations/formulae and the VCO2-based Mehta equation were estimated. ANN analysis to predict REE was conducted, employing the TWIST system. Results: ANN considered demographic/anthropometric data to model REE. The predictive model was good (accuracy 75.6%; R2 = 0.71) but not better than Talbot tables for weight. After adding vital signs/biochemical values, the model became superior to all equations/formulae (accuracy 82.3%, R2 = 0.80) and comparable to the Mehta equation. Including IC-measured VCO2 increased the accuracy to 89.6%, superior to the Mehta equation. Conclusions: We described the accuracy of REE prediction using models that include demographic/anthropometric/clinical/metabolic variables. ANN may represent a reliable option for REE estimation, overcoming the inaccuracies of traditional predictive equations/formulae.

OBJECTIVES Our goal was to analyse a consecutive series of patients with solid organ tumours undergoing coronary artery bypass grafting (CABG) by defining the risk factors for early and long-term outcomes. METHODS Between 2005 and 2016, a consecutive series of 4079 patients underwent isolated CABG at our institution. Of 103 patients (2.5%) with active malignancy, we enrolled 82 patients (mean age 71 ± 7 years) with solid organ tumours, divided into 4 subgroups: lung (9 patients—11%), gastroenteric (16 patients—20%), urinary (48 patients—58%) and other solid tumours (9 patients—11%). A deterministic record linkage between the clinical database and the National Hospital Information System allowed identification of long-term survival rates and freedom from major adverse cardiovascular events (acute myocardial infarction, repeated admissions for percutaneous coronary intervention and heart failure). RESULTS The most common forms of cancer were prostate, colon and carcinoma of the lung. Compared to patients without cancer, patients with neoplasms were significantly older and had a higher rate of comorbidities, without significant differences among the cancer subgroups. The 30-day mortality rate was significantly higher in patients with cancer compared to those without cancer (4.9% vs 1.8%). However, on logistic regression analysis, cancer was an independent risk factor for postoperative pulmonary dysfunction but not for in-hospital death. The median follow-up time was 58 ± 12 months. The overall 5-year survival rate was 60% [95% confidence interval (CI) 47–71%], with a dismal 32% (95% CI 5–65%) survival rate among patients who had lung tumours only. The 5-year freedom from major adverse cardiovascular events was 64% (95% CI 52–74%), without significant differences among subgroups, and was comparable to that of the non-cancer population. Resolution of coronary heart disease allowed safe cancer surgical resection in 80% of the population. CONCLUSIONS Based on the results from the present study, CABG should not be denied to patients with solid organ tumours by claiming a worse prognosis or less graft durability. Further studies with larger numbers are warranted.