Adherence to dietary recommendations, nutrient intake adequacy and diet quality among pediatric cystic fibrosis patients: results from the greecf study

Nutrition is an important component of cystic fibrosis (CF) therapy, with a high-fat diet being the cornerstone of treatment. However, adherence to the dietary recommendations for CF appears suboptimal and burdensome for most children and adolescents with CF, leading to malnutrition, inadequate growth, compromised lung function and increased risk for respiratory infections. A cross-sectional approach was deployed to examine the degree of adherence to the nutrition recommendations and diet quality among children with CF. A total of 76 children were recruited from Aghia Sophia’s Children Hospital, in Athens, Greece. In their majority, participants attained their ideal body weight, met the recommendations for energy and fat intake, exceeding the goal for saturated fatty acids consumption. Carbohydrate and fiber intake were suboptimal and most participants exhibited low or mediocre adherence to the Mediterranean diet prototype. It appears that despite the optimal adherence to the energy and fat recommendations, there is still room for improvement concerning diet quality and fiber intake.info:eu-repo/semantics/publishedVersio

Body composition and lung function in children with cystic fibrosis and meconium ileus

The aim of this study was to explore whether history of meconium ileus (MI) at birth in children and adolescents with cystic fibrosis (CF) adversely affects body composition and lung function in later life. Data of children and adolescents with CF who underwent spirometry and DXA as part of their routine care were analyzed. Associations between MI (explanatory variable) and areal bone mineral density (total body less head—TBLH aBMD), lean tissue mass (LTM), and fat mass (FM) (outcomes) were assessed using general linear models. Potential relationships of TBLH aBMD, LTM, and FM with FEV1 (additional outcome) were also explored. One hundred and one subjects with CF (mean age 14 ± 3 years) were included, 19 (18.8%) of whom had history of MI. Negative associations were demonstrated between history of MI and FEV1 (P = 0.04), TBLH aBMD (P = 0.03), and FM (P < 0.01) but not between history of MI and LTM (P = 0.07) after adjustment for other variables. Lung function was positively associated with TBLH aBMD (P < 0.01) and LTM (P = 0.02) but not with FM (P = 0.20). Conclusion: Among children and adolescents with CF, those with history of MI have lower bone mineral density, FM, and lung function.What is Known:• Among children and adolescents with cystic fibrosis, those with history of meconium ileus in the neonatal period are at risk of having lower body mass index percentile and FEV1percent predicted.What is New:• Children and adolescents with cystic fibrosis and history of meconium ileus have decreased bone mineral density and fat mass compared to patients without such history.• Lower lung function in children with MI coexists with suboptimal bone mineral density. © 2017, Springer-Verlag Berlin Heidelberg

Inflammation and Infection in Cystic Fibrosis: Update for the Clinician

Inflammation and infection play an important role in the pathophysiology of cystic fibrosis, and they are significant causes of morbidity and mortality in CF. The presence of thick mucus in the CF airways predisposes to local hypoxia and promotes infection and inflammation. A vicious cycle of airway obstruction, inflammation, and infection is of critical importance for the progression of the disease, and new data elucidate the different factors that influence it. Recent research has been focused on improving infection and inflammation in addition to correcting the basic gene defect. This review aims to summarize important advances in infection and inflammation as well as the effect of new treatments modulating the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein. New approaches to target infection and inflammation are being studied, including gallium, nitric oxide, and phage therapy for infection, along with retinoids and neutrophil elastase inhibitors for inflammation

Nebulizer Care and Inhalation Technique in Children with Cystic Fibrosis

Nebulizers are used by the great majority of cystic fibrosis patients for delivery of cornerstone treatments. Inhalation technique and adequate disinfection and maintenance are important for optimizing medication delivery. In this study, inhalation technique and nebulizer disinfection/maintenance were assessed in cystic fibrosis patients by direct observation in clinic and completion of a scoring sheet. A total of 108 patients were recruited. The maximum inhalation technique score was attained by 30.5% and adequate inhalation technique score by 74.08% of patients. The inhalation technique score was best with the vibrating mesh nebulizer (p = 0.038), while patient age and number of nebulized medications did not affect ITS significantly (p > 0.05). Nebulizer disinfection/maintenance score was excellent in only 31.48%. Most families kept the nebulizer clean and used appropriate disinfection method, but only half of them replaced the nebulizer and nebulizer cup at the recommended time intervals. Nebulizer disinfection/maintenance score was positively affected by a number of nebulized medications and negatively by years of equipment use (p = 0.009 and p = 0.001, respectively). Even though inhalation technique and disinfection/maintenance practices were found to be adequate in a large proportion of cases, there is still a need for regular review and education. The type of nebulizer was associated with improved inhalation technique, but more data are required before making specific recommendations

Carotid Bifurcation Geometry and Atherosclerosis

Hemodynamic changes occurring at the initial segments of the arterial bifurcations appear to play an important role in the development of atherosclerotic plaque. Therefore, arterial geometry might be a potential marker for atherosclerosis. Considerable evidence suggests that geometry can influence local hemodynamics at the carotid bifurcation contributing to the development of atheroma. Bifurcation angle, differences in the area ratios including the flare, proximal curvature, sinus bulb width, and tortuosity of the internal or external carotid artery have been listed as potential contributory elements. These morphometric details have been studied not only in postmortem examination but also with the help of imaging modalities such as ultrasound, digital subtraction angiography, computed tomography angiography, and the assistance of computational models and magnetic resonance angiography. The establishment of certain anatomical and geometrical details in addition to traditional risk factors may help in the identification of patients at high risk of developing carotid artery disease. We reviewed the literature to highlight the evidence on the importance of various geometrical details in the development of carotid atheroma and to suggest areas of future research. © SAGE Publications

Viral Croup: Diagnosis and a Treatment Algorithm

Viral croup is a frequent disease in early childhood. Although it is usually self-limited, it may occasionally become life-threatening. Mild croup is characterized by the presence of stridor without intercostal retractions, whereas moderate-to-severe croup is accompanied by increased work of breathing. A single dose of orally administered dexamethasone (0.15-0.6mg/kg) is the mainstay of treatment with addition of nebulized epinephrine only in cases of moderate-to-severe croup. Nebulized budesonide (2mg) can be given alternatively to children who do not tolerate oral dexamethasone. Exposure to cold air or administration of cool mist are treatment interventions for viral croup that are not supported by published evidence, but breathing heliox can potentially reduce the work of breathing related to upper airway obstruction. In summary, corticosteroids may decrease the intensity of viral croup symptoms irrespective to their severity on presentation to the emergency department. Pediatr Pulmonol. 2014; 49:421-429. (c) 2013 Wiley Periodicals, Inc

Carotid Bifurcation Geometry as Assessed by Ultrasound is Associated with Early Carotid Atherosclerosis

Background: Atherosclerosis usually develops at geometrically susceptible areas, despite the impact of systemic risk factors on the entire vascular system. The aim of our study was to investigate whether carotid bifurcation geometry as assessed by ultrasound is associated with early carotid atherosclerosis, regardless of the presence of known atherosclerotic risk factors. Material and Methods: A nonrandomized prospective study was undertaken, including subjects of both genders, aged 50–60 years, without known cardiovascular family history, and symptoms and/or signs of cardiovascular disease. Clinical assessment and ultrasound of the carotid bifurcation evaluating geometrical characteristics, ultrasonic biopsy score (UBS), intima-media thickness (IMT), and the presence of plaque were recorded. Results: Two hundred one subjects (95 men/106 women; mean age: 55 years) provided 286 carotid bifurcations associated with atherosclerotic risk factors and 114 without (one poor imaging). While UBS in the right common carotid artery (CCA) was higher in subjects with atherosclerotic risk factors but free of plaque (P = 0.035), larger diameter of the bulb (dBULB) (P < 0.001), lower ratios of internal carotid artery diameter + external carotid artery diameter/diameter of CCA ([dICA + dECA]/dCCA) (P = 0.004), and (dICA2 + dECA2)/dCCA2 (P = 0.025) were independently associated with higher CCA IMT. The presence of plaque among the total 400 carotid bifurcations was associated in the left and right CCA with lower values of bulb diameter/dCCA (dBULB/dCCA) (P = 0.014) and (dICA + dECA)/dCCA (P = 0.001), respectively. Among subjects without atherosclerotic risk factors (n = 114), the presence of plaque in the right CCA was associated with lower values of (dBULB + dECA)/dCCA (P = 0.001) and that in the left with lower values of dBULB/dCCA (P = 0.001). Conclusions: Different dimensional ratios of the carotid bifurcation assessed by ultrasound are associated with the development of early carotid atherosclerosis independently from the presence of atherosclerotic risk factors. Carotid atherosclerotic process may be side dependent. © 2018 Elsevier Inc

Is routine ultrasound examination of the gallbladder justified in critical care patients?

Objective. We evaluated whether routine ultrasound examination may illustrate gallbladder abnormalities, including acute acalculous cholecystitis (AAC) in the intensive care unit (ICU). Patients and Methods. Ultrasound monitoring of the GB was performed by two blinded radiologists in mechanically ventilated patients irrespective of clinical and laboratory findings. We evaluated major (gallbladder wall thickening and edema, sonographic Murphy's sign, pericholecystic fluid) and minor (gallbladder distention and sludge) ultrasound criteria. Measurements and Results. We included 53 patients (42 males; mean age 57.6±2.8 years; APACHE II score 21.3±0.9; mean ICU stay 35.9±4.8 days). Twenty-five patients (47.2%) exhibited at least one abnormal imaging finding, while only six out of them had hepatic dysfunction. No correlation existed between liver biochemistry and ultrasound results in the total population. Three male patients (5.7%), on the grounds of unexplained sepsis, were diagnosed with AAC as incited by ultrasound, and surgical intervention was lifesaving. Patients who exhibited <2 ultrasound findings (30.2%) were managed successfully under the guidance of evolving ultrasound, clinical, and laboratory findings. Conclusions. Ultrasound gallbladder monitoring guided lifesaving surgical treatment in 3 cases of AAC; however, its routine application is questionable and still entails high levels of clinical suspicion. © 2012 Pavlos Myrianthefs et al