Ready-to-Use Supplemental Food for Nutritional Supplementation in Cystic Fibrosis.

Undernutrition is common in cystic fibrosis (CF) and is correlated with long-term outcomes, yet current nutritional interventions have not demonstrated consistent improvements in energy intake, and subsequently, growth. Development of novel nutritional interventions to increase energy intake is essential to improve clinical outcomes of individuals with CF. Ready-to-use supplemental food (RUSF) is a modifiable, inexpensive, palatable, safe, and nutrient-dense food for treatment or prevention of acute malnutrition in developing countries. Utilizing a linear-programming tool we identified 6 RUSF formulations with sufficient nutrient density (495 kcal/100 g), protein, and fat for children with CF. Palatability was established by a taste-trial and affirmed by a 2-wk tolerability assessment that demonstrated consistent consumption and tolerance of the RUSF. Although preliminary, this study demonstrates the potential for developing RUSF as a nutritional supplement for increasing energy intake in children with CF.

Reduction in Mortality Following Pediatric Rapid Response Team Implementation.

OBJECTIVE: To evaluate the effectiveness of a physician-led rapid response team program on morbidity and mortality following unplanned admission to the PICU. DESIGN: Before-after study. SETTING: Single-center quaternary-referral PICU. PATIENTS: All unplanned PICU admissions from the ward from 2005 to 2011. INTERVENTIONS: The dataset was divided into pre- and post-rapid response team groups for comparison. MEASUREMENTS AND MAIN RESULTS: A Cox proportional hazards model was used to identify the patient characteristics associated with mortality following unplanned PICU admission. Following rapid response team implementation, Pediatric Risk of Mortality, version 3, illness severity was reduced (28.7%), PICU length of stay was less (19.0%), and mortality declined (22%). Relative risk of death following unplanned admission to the PICU after rapid response team implementation was 0.685. CONCLUSIONS: For children requiring unplanned admission to the PICU, rapid response team implementation is associated with reduced mortality, admission severity of illness, and length of stay. Rapid response team implementation led to more proximal capture and aggressive intervention in the trajectory of a decompensating pediatric ward patient.

Official American Thoracic Society technical standards: flexible airway endoscopy in children.

BACKGROUND: Flexible airway endoscopy (FAE) is an accepted and frequently performed procedure in the evaluation of children with known or suspected airway and lung parenchymal disorders. However, published technical standards on how to perform FAE in children are lacking. METHODS: The American Thoracic Society (ATS) approved the formation of a multidisciplinary committee to delineate technical standards for performing FAE in children. The committee completed a pragmatic synthesis of the evidence and used the evidence synthesis to answer clinically relevant questions. RESULTS: There is a paucity of randomized controlled trials in pediatric FAE. The committee developed recommendations based predominantly on the collective clinical experience of our committee members highlighting the importance of FAE-specific airway management techniques and anesthesia, establishing suggested competencies for the bronchoscopist in training, and defining areas deserving further investigation. CONCLUSIONS: These ATS-sponsored technical standards describe the equipment, personnel, competencies, and special procedures associated with FAE in children.

Inhaled versus systemic antibiotics and airway inflammation in children with cystic fibrosis and Pseudomonas.

RATIONALE: Inhaled tobramycin has been shown to transiently clear Pseudomonas from lower airways in early cystic fibrosis (CF), but does not markedly reduce lung inflammation, a key factor in disease progression. OBJECTIVE: Test the hypothesis that systemic antibiotics are more effective than inhaled antibiotics for reducing lower airways inflammation. METHODS: Clinically stable CF children with recent Pseudomonas were randomized to receive 4 weeks of inhaled tobramycin or 2 weeks of systemic antibiotics (intravenous ceftazidime and tobramycin). Bronchoalveolar lavage fluid was obtained just before and 4-6 weeks after treatment. The primary outcome was change in % neutrophils in lavage fluid. RESULTS: Fifteen subjects (inhaled = 6, systemic = 9) completed the protocol. Three Systemic Group subjects could not have central venous access established and were treated with oral ciprofloxacin (plus inhaled tobramycin) for 2 weeks as an alternative "systemic" regimen, per protocol. Groups were well matched in age, markers of disease severity, and initial % neutrophils. The Systemic Group showed a modest median change in percent neutrophils (-7%) which was not statistically significant compared to inhaled (+5.4%, P = 0.07). However, the Systemic Group had significantly greater reductions in total cells (-50% vs. -3%, P < 0.01) and neutrophils (-74% vs. -10%, P = 0.02) per ml lavage fluid. Both groups had reduced bacterial quantity after treatment, but there was no significant difference between groups. CONCLUSIONS: In clinically stable children with CF, systemic antibiotics result in greater short-term reduction in lower airways inflammation than inhaled antibiotics.

Obesity, inflammation, and asthma severity in childhood: data from the National Health and Nutrition Examination Survey 2001-2004.

BACKGROUND: The prevalences of asthma and obesity in children have increased significantly during the past 2 decades. The basis for the relationship between pediatric asthma and obesity is not well established. OBJECTIVES: To explore the association between obesity and asthma severity in children and adolescents and to test whether obesity-induced inflammation, as characterized by serum C-reactive protein (CRP), is associated with increased severity of asthma. METHODS: Retrospective cohort analysis of interview, physical examination, and laboratory test data from participants younger than 20 years in 2 rounds of the National Health and Nutrition Examination Survey (2001-2002 and 2003-2004). We also performed generalized ordered logistic regression to evaluate the effect of body mass index (BMI) z score and CRP level on asthma severity, controlling for the impact of age, sex, race, income, insurance, and tobacco smoke exposure. RESULTS: Of the 77 million individuals younger than 20 years represented by this weighted sample, 19% met the study-defined criteria for asthma; most cases were defined as mild (11%) or moderate (6%); 2% had severe asthma. In multivariable models, elevated BMI z scores (odds ratio, 1.12; 95% confidence interval, 1.05-1.21) were associated with worse asthma severity. Elevated CRP level was associated with obesity (P < .001) and asthma severity (odds ratio, 1.33; 95% confidence interval, 1.16-1.52). CONCLUSIONS: Higher BMI z scores and elevated serum CRP levels are associated with increased asthma severity. These findings highlight the importance of controlling for inflammation when considering the role of obesity and provide support for the hypothesis that obesity-induced inflammation may contribute to greater asthma severity.

Intensive care management of the patient with cystic fibrosis.

Cystic fibrosis was previously thought to be a disease of childhood. With a better understanding of this condition along with improvements in therapy, patients with cystic fibrosis are now living well into adulthood. The aim of this article is to familiarize the intensive care unit physician with cystic fibrosis care, to discuss complications associated with cystic fibrosis specifically related to the intensive care unit, and to detail the current recommendations for the clinical management of the patient with cystic fibrosis. With advancing disease, the most severely affected organs are the lungs. Obstruction, infection, and inflammation contribute to the decline of pulmonary function, ultimately leading to death. Some patients may be eligible for lung transplantation, but choosing wisely will affect posttransplant survival. Because other organs are affected by the genetic defect and associated treatments, serious complications related to the liver, pancreas, intestines, and kidneys must be considered by the intensivist faced with a patient with cystic fibrosis. As practitioners, the fact that not all patients will survive and help our patients and families gracefully through the end-of-life process should be accepted.

Pulmonary complications of haematopoietic cell transplantation in children.

Paediatric haematopoietic cell transplantation has experienced significant advances in the last few decades. However, pulmonary complications are an important limitation to the efficacy of this intervention, contributing to post-transplantation morbidity and mortality. Such complications persist even in experienced centres and occur in adult and paediatric recipients. This review identifies the paediatric pulmonary complications that are commonly seen following haematopoietic cell transplantation and addresses both infectious and non-infectious aetiologies and their clinical manifestations, evaluation, and potential therapy. Ultimately, improvement in outcomes will require attention to immunosuppression as well as traditional diagnostic procedures and treatment. This article aims to review the current state of pulmonary complications post-transplantation, to examine the impact of our recent advances and changes in treatment, and to identify potential future therapies and hypothesise what role these might have on long-term survival.