Multifaceted quality improvement initiative to decrease pediatric asthma readmissions.

BACKGROUND: Asthma is the most common chronic disease of childhood and a leading cause of hospitalization in children. A primary goal of asthma control is prevention of hospitalizations. A hospital admission is the single strongest predictor of future hospital admissions for asthma. The 30-day asthma readmission rate at our institution was significantly higher than that of other hospitals in the Children's Hospital Association. As a result, a multifaceted quality improvement project was undertaken with the goal of reducing the 30-day inpatient asthma readmission rate by 50% within two years. METHODS: Analysis of our institution's readmission patterns, value stream mapping of asthma admission, discharge, and follow-up processes, literature review, and examination of comparable successful programs around the United States were all utilized to identify potential targets for intervention. Interventions were implemented in a stepwise manner, and included increasing inhaler availability after discharge, modifying asthma education strategies, and providing in-home post-discharge follow-up. The primary outcome was a running 12-month average 30-day inpatient readmission rate. Secondary outcomes included process measures for individual interventions. RESULTS: From a peak of 7.98% in January 2013, a steady decline to 1.65% was observed by July 2014, which represented a 79.3% reduction in 30-day readmissions. CONCLUSION: A significant decrease in hospital readmissions for pediatric asthma is possible, through comprehensive, multidisciplinary quality improvement that spans the continuum of care.

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.

Why exercise capacity does not improve after pulmonary valve replacement.

Optimal timing of pulmonary valve replacement (PVR) for pulmonary regurgitation is a debated topic. It is logical that maximal aerobic capacity (VO2peak) would decline when a PVR is needed, but a diminished VO2peak is not always present before PVR, and previous studies show no improvement in VO2peak after PVR. This study aimed to evaluate changes in resting spirometry from pre- to post-PVR sternotomy, to determine the limiting factors of VO2peak before and after PVR, and to determine whether changes in resting lung function after PVR may explain the lack of improvement in VO2peak after surgery. For 26 patients (age, 19.7 ± 7.8 years) with a history of right ventricular outflow tract revision, the study prospectively evaluated echocardiograms, resting spirometry, and maximal exercise tests before PVR and then an average of 15 months after PVR. Flow volume loops were reviewed by a pulmonologist and categorized as obstructive, restrictive, both obstructive and restrictive, or normal. Exercise tests were interpreted using Eschenbacher's algorithm to determine the primary factors limiting exercise. No change in VO2peak or spirometry after PVR was observed. Before PVR, many patients had abnormal resting lung functions (85 % abnormal), which was unchanged after PVR (86 5 % abnormal). The majority of the patients had a ventilatory limitation to VO2peak before PVR (66.7 %), whereas 28.5 % had a cardiovascular limitation, and 4.8 % had no clear limitation. After PVR, 65.2 % of the patients had a ventilatory limitation, whereas 30.4 % had a cardiovascular limitation, and 4.4 % had no clear limitation to VO2peak. Pulmonary function did not change up to 15 months after surgical PVR. The frequency of pulmonary limitation to VO2peak after PVR did not increase. The effect of pulmonary function on exercise-related symptoms must be considered in this patient population. Improved cardiac hemodynamics are unlikely to improve VO2peak in a primarily pulmonary-limited patient.

Diagnostic yield of bronchoscopy in children with leukemia or post hematopoietic stem cell transplant.

BACKGROUND: The utility of bronchoscopy with bronchoalveolar lavage (BAL) in immunocompromised children is not well understood. We aim to describe the bronchoscopy diagnostic yield and complications and to investigate factors associated with diagnostic yield. METHODS: This is a single-center, retrospective cohort study of 60 children with leukemia or post-hematopoietic stem cell transplant who had a bronchoscopy with BAL between 2017 and 2021. Comparisons were done with regression analysis. RESULTS: Of the 60 bronchoscopies performed, 46 (77%) revealed diagnostic information: 39 (65%) identified a pathogen, 14 (23.3%) found secretions/mucus plugging, and 6 (10%) found pulmonary hemorrhage. BAL results changed antimicrobial therapy in 27 (45%) cases. Bronchoscopies were performed in the intensive care unit (27/60) or operating room (33/60), with the former having a higher diagnostic yield (96% vs. 60%, p = 0.001). Half (50%) of bronchoscopies found a new infectious diagnosis. Respiratory symptoms (n = 58, 97%), supplemental oxygen use (n = 39, 65%), and antibiotic use (n = 56, 93%) before bronchoscopy were all common. The median volume of fluid instilled during bronchoscopy was 1.3 mL/kg (interquatile range [IQR]: 0.7, 2.6). None of these factors were associated with the diagnostic yield. Complications were rare and minor with only one child having self-resolved bleeding and four children, previously in room air requiring a nasal cannula. For the 27 (45%) children on mechanical ventilation when the bronchoscopy was performed, there was no difference in ventilator settings pre- and post-bronchoscopy. CONCLUSION: Bronchoscopies with BAL are useful, safe, and important in the diagnostic management of pulmonary complications in this cohort of children.

The US national registry for childhood interstitial and diffuse lung disease: Report of study design and initial enrollment cohort.

INTRODUCTION: Childhood interstitial and diffuse lung disease (chILD) encompasses a broad spectrum of rare disorders. The Children's Interstitial and Diffuse Lung Disease Research Network (chILDRN) established a prospective registry to advance knowledge regarding etiology, phenotype, natural history, and management of these disorders. METHODS: This longitudinal, observational, multicenter registry utilizes single-IRB reliance agreements, with participation from 25 chILDRN centers across the U.S. Clinical data are collected and managed using the Research Electronic Data Capture (REDCap) electronic data platform. RESULTS: We report the study design and selected elements of the initial Registry enrollment cohort, which includes 683 subjects with a broad range of chILD diagnoses. The most common diagnosis reported was neuroendocrine cell hyperplasia of infancy, with 155 (23%) subjects. Components of underlying disease biology were identified by enrolling sites, with cohorts of interstitial fibrosis, immune dysregulation, and airway disease being most commonly reported. Prominent morbidities affecting enrolled children included home supplemental oxygen use (63%) and failure to thrive (46%). CONCLUSION: This Registry is the largest longitudinal chILD cohort in the United States to date, providing a powerful framework for collaborating centers committed to improving the understanding and treatment of these rare disorders.

Adjusting ventilator settings to avoid air trapping in extremely premature infants reduces the need for tracheostomy and length of stay.

Despite the improving understanding of how lung mechanics and tidal volume requirements evolve during the evolution of bronchopulmonary dysplasia (BPD), clinical management continues to be heterogeneous and inconsistent at many institutions. Recent reports have examined the use of high tidal-volume low respiratory rate strategies in these patients once disease has been well established to help facilitate their eventual extubation and improve their long-term neurodevelopmental outcomes. In this retrospective observational research study, we describe how intentional adjustment of ventilator settings based on patient lung mechanics by an interdisciplinary BPD team improved the care of the at-risk population of infants, reduced the need for tracheostomies, as well as length of stay over a period of over 3 years. The team aimed to establish consistency in the management of these children using a high tidal volume, low-rate approach, and titrating PEEP to address the autoPEEP and bronchomalacia that is frequently observed in this patient population.

Pulmonary limitation to exercise after repair of D-transposition of the great vessels: atrial baffle versus arterial switch.

This study evaluated resting pulmonary function and its impact on exercise capacity after atrial baffle (BAFFLE) and arterial switch (SWITCH) repair of D-transposition of the great vessels (DTGV). Previously decreased exercise capacity in DTGV patients has been primarily attributed to cardiovascular limitations, whereas pulmonary limitations have largely been overlooked. Resting flow volume loops were compared for BAFFLE (n = 34) and SWITCH (n = 32) patients. Peak exercise variables were compared for BAFFLE (n = 30) and SWITCH (n = 25). Lung disease (restrictive and/or obstructive) was present in 53% of DTGV patients (BAFFLE 62% and SWITCH 44%; p = 0.14). BAFFLE patients had a normal breathing reserve, whereas that of SWITCH patients was decreased (27.3 ± 28.3 vs. 13.0 ± 19.2; p = 0.04). BAFFLE patients attained a lower percent of predicted peak oxygen pulse (82.7 ± 20.5% vs. 94.7 ± 19.3%; p = 0.04) and peak oxygen consumption (VO(2peak)) (26.6 ± 6.7 ml/kg/min vs. 37.3 ± 8.5 ml/kg/min; p < 0.01) than SWITCH patients. Patients after surgical repair for DTGV have an underappreciated occurrence of lung disease, even post-SWITCH. SWITCH patients have diminished breathing reserves, suggesting a pulmonary limitation to VO(2peak). BAFFLE patients have lower VO(2peaks), greater breathing reserves, and lower oxygen pulses than SWITCH patients, suggesting a cardiac limitation to peak aerobic capacity with probable secondary pulmonary limitations. Treating underlying lung disease in symptomatic patients after repair of DTGV may improve functional status.

Wheezing in preterm infants and children.

Wheezing is a common outcome of preterm birth. This article will review the mechanisms, epidemiology, and treatment of wheezing in preterm children with and without a history of bronchopulmonary dysplasia.

The Coordinators of Psychiatric Education (COPE) Residency In-Training Exam: a preliminary psychometric assessment.

OBJECTIVE: The Coordinators of Psychiatric Education (COPE) Residency In-Training Exam is a formative exam for Canadian psychiatric residents that was reconstructed using assessment best practices. An assessment of psychometric properties was subsequently performed on the exam to ensure preliminary validity and reliability. METHODS: An exam blueprint was developed based on the 2007 Royal College objectives for psychiatric training. A minimum pass level was established using a modified Angoff method. The exam was administered to all Canadian psychiatric residents in postgraduate years 2 to 5 with test reliability (Cronbach alpha) and item analysis performed. Exam validity was assessed through blueprint adherence and cross-year resident performance analysis. RESULTS: Four hundred two exams were suitable for analysis. The overall mean score for all residents was 69.6% (SD=8.5) with significant differences in total scores between each of the postgraduate year groups, with consistently better performance with increasing time in residency. Cronbach alpha was 0.79. CONCLUSION: The present study provides preliminary support that the reconstructed COPE Residency In-Training Exam demonstrates adequate reliability and validity, including showing the capacity to discriminate between levels of training.

Lung biopsy in children's interstitial and diffuse lung disease: Does it alter management?

INTRODUCTION: Pediatric patients with acute life-threatening consequences of interstitial and diffuse lung disease are often treated with empiric systemic corticosteroids, immune modulators, and/or broad antibiotic therapy. Histological evaluation of lung tissue represents the final necessary step in diagnosis-however, a definitive diagnosis may still remain elusive and medical therapies may not be changed following biopsy. We hypothesized that lung biopsy from pediatric patients with children's interstitial and diffuse lung disease (chILD) without a defined lesion on computed tomography (CT) imaging would guide diagnosis, but not substantially alter clinical management. METHODS: After IRB approval, patients who underwent a lung biopsy at a single large children's hospital between 2013 and 2018 were retrospectively reviewed. Patients without a defined lesion were included. Demographics, length of stay, oxygen-requirements, steroid, unique number of immune modulators, and antibiotics prebiopsy and postbiopsy were reviewed. Nonparametric data were compared by the Mann Whitney U and Kruskal Wallace tests and expressed as median with interquartile range. Decision tree alterations were analyzed by t test. P < .05 was significant. RESULTS: Sixty-four patients underwent lung biopsy during the period. Nineteen (30%) did not have a defined lesion on CT scan, and were included. A significant difference was seen between prebiopsy, 2 weeks, and 2 months postbiopsy prednisone dosing (P = .03), while the number of unique immune modulators, antibiotics, type of oxygen support and FiO2 were not significantly different before or after obtaining biopsy results. Pathology results provided additional information in 12 of 19 (63%) patients which resulted in management changes. CONCLUSIONS: Lung biopsy in chILD may guide clinical management, especially influencing the management of steroid dosing. Although on aggregate the number of antibiotics, immune modulators, mode of oxygen support and FiO2 did not differ significantly before and after biopsy, the pathologic evaluation provided diagnostic information that led to a variety of changes in therapeutic management in greater than half of the population.

ATS Core Curriculum 2020. Pediatric Pulmonary Medicine.

The American Thoracic Society Core Curriculum updates clinicians annually in adult and pediatric pulmonary disease, medical critical care, and sleep medicine, in a 3- to 4-year recurring cycle of topics. These topics will be presented at the 2020 International Conference. Below is the pediatric pulmonary medicine core, including pediatric hypoxemic respiratory failure; modalities in noninvasive management of chronic respiratory failure in childhood; surgical and nonsurgical management of congenital lung malformations; an update on smoke inhalation lung injury; an update on vaporizers, e-cigarettes, and other electronic delivery systems; pulmonary complications of sarcoidosis; pulmonary complications of congenital heart disease; and updates on the management of congenital diaphragmatic hernia.

Effect of fish oil-derived omega-3 polyunsaturated Fatty Acid supplementation on exercise-induced bronchoconstriction and immune function in athletes.

UNLABELLED: Exercise-induced bronchoconstriction (EIB) is a condition in which vigorous physical activity triggers acute airway obstruction in asthmatic and nonasthmatic individuals with hyperresponsive airways. Studies have shown that inflammatory mediators and contraction of airway smooth muscle are central components in the pathogenesis of EIB, and it has long been recognized that leukotrienes and prostaglandins play an important role in the EIB response. Clinical responses to current therapy, such as leukotriene modifiers and corticosteroids are heterogeneous, and even with optimal treatment there is a substantial burden of unaddressed disease. While daily medications such as leukotriene modifiers provide only modest protection against symptoms, prolonged use of several medications can result in reduced effectiveness or tachyphylaxis. Although the treatment of EIB almost exclusively involves pharmacotherapy, there is now convincing evidence that dietary modification has the potential to reduce the severity of this condition. Omega-3 polyunsaturated fatty acids, such as eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) in fish oils, compete with arachidonic acid as substrates for the formation of proinflammatory mediators, such as leukotrienes, prostaglandins, and cytokines. Studies have shown that 3 weeks of fish oil supplementation, rich in EPA and DHA, reduces exercise-induced airway narrowing, airway inflammation, and bronchodilator use in elite athletes and asthmatic individuals with EIB. Based on the evidence to date, fish oil supplementation may represent a potentially beneficial treatment intervention for athletes and asthmatic individuals with EIB. From this, it follows that physicians should pay more attention to what their asthma/EIB patients eat, and incorporate dietary assessment and nutritional counseling in their everyday practice. KEYWORDS: omega-3; polyunsaturated fatty acid; fish oil; exercise-induced bronchoconstriction.

Efficacy, cost effectiveness, and sustainability of a pediatric high risk asthma clinic.

AIM: At our institution, a pediatric High Risk Asthma clinic has been in operation for over 15 years, targeting children with poorly controlled, and difficult to treat asthma. This study evaluates the outcomes and cost-effectiveness of the High Risk Asthma clinic from 2000 through 2014. METHODS: A retrospective chart review was performed on all patients entering High Risk Asthma clinic from 2000-2014, and total hospitalizations and Emergency Department visits were tallied for the year prior to entering clinic and the year after. Costs incurred, and reimbursements obtained from payors were tallied to determine cost-effectiveness and sustainability. RESULTS: Consistent decreases in hospitalizations (51.2% decrease, P < 0.001) and Emergency Department visits (23.0% decrease, P = 0.048) were seen for patients entering High Risk Asthma clinic, with commensurate significant decreases in related costs. Reimbursements received for outpatient services were sufficient to offset operational costs of the High Risk Asthma clinic, when both clinic visit, and pulmonary function testing charges were included. CONCLUSIONS: A pediatric High Risk Asthma clinic model is efficacious in decreasing hospitalizations and Emergency Department visits for a difficult to treat population, and such a model can be cost-effective and sustainable.

Association of Antibiotics, Airway Microbiome, and Inflammation in Infants with Cystic Fibrosis.

RATIONALE: The underlying defect in the cystic fibrosis (CF) airway leads to defective mucociliary clearance and impaired bacterial killing, resulting in endobronchial infection and inflammation that contributes to progressive lung disease. Little is known about the respiratory microbiota in the early CF airway and its relationship to inflammation. OBJECTIVES: To examine the bacterial microbiota and inflammatory profiles in bronchoalveolar lavage fluid and oropharyngeal secretions in infants with CF. METHODS: Infants with CF from U.S. and Australian centers were enrolled in a prospective, observational study examining the bacterial microbiota and inflammatory profiles of the respiratory tract. Bacterial diversity and density (load) were measured. Lavage samples were analyzed for inflammatory markers (interleukin 8, unbound neutrophil elastase, and absolute neutrophil count) in the epithelial lining fluid. RESULTS: Thirty-two infants (mean age, 4.7 months) underwent bronchoalveolar lavage and oropharyngeal sampling. Shannon diversity strongly correlated between upper and lower airway samples from a given subject, although community compositions differed. Microbial diversity was lower in younger subjects and in those receiving daily antistaphylococcal antibiotic prophylaxis. In lavage samples, reduced diversity correlated with lower interleukin 8 concentration and absolute neutrophil count. CONCLUSIONS: In infants with CF, reduced bacterial diversity in the upper and lower airways was strongly associated with the use of prophylactic antibiotics and younger age at the time of sampling; less diversity in the lower airway correlated with lower inflammation on bronchoalveolar lavage. Our findings suggest modification of the respiratory microbiome in infants with CF may influence airway inflammation.

Effects of sildenafil on pulmonary hypertension and exercise tolerance in severe cystic fibrosis-related lung disease.

Cystic fibrosis (CF) patients with advanced lung disease are at risk for developing pulmonary vascular disease and pulmonary hypertension, characterized by progressive exercise intolerance beyond the exercise-limiting effects of airways disease in CF. We report on a patient with severe CF lung disease who experienced clinically significant improvements in exercise tolerance and pulmonary hypertension without changing lung function during sildenafil therapy.

Effect of treatment of cystic fibrosis pulmonary exacerbations on systemic inflammation.

RATIONALE: In cystic fibrosis (CF), pulmonary exacerbations present an opportunity to define the effect of antibiotic therapy on systemic measures of inflammation. OBJECTIVES: Investigate whether plasma inflammatory proteins demonstrate and predict a clinical response to antibiotic therapy and determine which proteins are associated with measures of clinical improvement. METHODS: In this multicenter study, a panel of 15 plasma proteins was measured at the onset and end of treatment for pulmonary exacerbation and at a clinically stable visit in patients with CF who were 10 years of age or older. MEASUREMENTS AND MAIN RESULTS: Significant reductions in 10 plasma proteins were observed in 103 patients who had paired blood collections during antibiotic treatment for pulmonary exacerbations. Plasma C-reactive protein, serum amyloid A, calprotectin, and neutrophil elastase antiprotease complexes correlated most strongly with clinical measures at exacerbation onset. Reductions in C-reactive protein, serum amyloid A, IL-1ra, and haptoglobin were most associated with improvements in lung function with antibiotic therapy. Having higher IL-6, IL-8, and α1-antitrypsin (α1AT) levels at exacerbation onset were associated with an increased risk of being a nonresponder (i.e., failing to recover to baseline FEV1). Baseline IL-8, neutrophil elastase antiprotease complexes, and α1AT along with changes in several plasma proteins with antibiotic treatment, in combination with FEV1 at exacerbation onset, were predictive of being a treatment responder. CONCLUSIONS: Circulating inflammatory proteins demonstrate and predict a response to treatment of CF pulmonary exacerbations. A systemic biomarker panel could speed up drug discovery, leading to a quicker, more efficient drug development process for the CF community.

Internet hand x-rays: A comparison of joint space narrowing and erosion scores (Sharp/Genant) of plain versus digitized x-rays in rheumatoid arthritis patients.

BACKGROUND: The objective of the study is to examine the reliability of erosion and joint space narrowing scores derived from hand x-rays posted on the Internet compared to scores derived from original plain x-rays. METHODS: Left and right x-rays of the hands of 36 patients were first digitized and then posted in standard fashion to a secure Internet website. Both the plain and Internet x-rays were scored for erosions and joint space narrowing using the Sharp/Genant method. All scoring was completed in a blind and randomized manner. Agreement between plain and Internet x-ray scores was calculated using Lin's concordance correlations and Bland-Altman graphical representation. RESULTS: Erosion scores for plain x-rays showed almost perfect concordance with x-rays read on the Internet (concordance 0.887). However, joint space narrowing scores were only "fair" (concordance 0.365). Global scores demonstrated substantial concordance between plain and Internet readings (concordance 0.769). Hand x-rays with less disease involvement showed a tendency to be scored higher on the Internet versions than those with greater disease involvement. This was primarily evident in the joint space narrowing scores. CONCLUSIONS: The Internet represents a valid medium for displaying and scoring hand x-rays of patients with RA. Higher scores from the Internet version may be related to better viewing conditions on the computer screen relative to the plain x-ray viewing, which did not include magnifying lens or bright light. The capability to view high quality x-rays on the Internet has the potential to facilitate information sharing, education, and encourage collaborative studies.

An infant with pulmonary interstitial glycogenosis: clinical improvement is associated with improvement in the pulmonary diffusion capacity.

Pulmonary interstitial glycogenosis (PIG) is an idiopathic interstitial lung disease of infants. The underlying pulmonary pathophysiology of PIG has not been well characterized. Herein we report a term-gestation infant who presented with persistent tachypnea and hypoxia. A chest CT scan demonstrated a diffuse ground glass appearance and lung biopsy demonstrated increased alveolar septae cellularity with glycogen-containing cells, consistent with a diagnosis of PIG. At 3 months of age, pulmonary function testing included: pre- and post-bronchodilator forced expiratory flows using the raised-volume technique and the ratio of pulmonary diffusing capacity for carbon monoxide to alveolar volume (DLCO /VA ). He was prescribed 5 days of oral prednisolone (2mg/kg/day) and pulmonary function testing (PFT) was repeated at 5, 13, and 20 months of age. Initial PFTs demonstrated reduced forced vital capacity (FVC: Z-score = -2.36) and an increased ratio of forced expiratory volume in 0.5 sec to FVC (FEV0.5/FVC: Z-score = 1.15) with no significant change following an inhaled bronchodilator. There was also a marked reduction in DLCO /VA (Z-score = -4.74) compared to age-matched controls. Follow-up demonstrated progressive clinical improvement as well as an increase in Z-FVC and normalization of DLCO /VA . Our in vivo physiological findings are consistent with previous reports that symptom resolution correlated with histological thinning of the alveolar septae upon repeat lung biopsy. The restrictive lung disease we observed is consistent with expected reduced compliance of an alveolar interstitial lung process like PIG, whereas the absence of a reduction in FEV0.5/FVC confirms the absence of obstructive airway disease.