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Pediatric pulmonologists have been involved in the care of adult COVID-19 patients in a variety of ways, particularly in areas with a high concentration of cases. This invited commentary is a series of questions to Dr Mikhail Kazachkov, a pediatric pulmonologist at New York University, about his experiences to date in a major COVID-19 "hotspot" and his thoughts about how other pediatric pulmonologists facing this situation can best support their colleagues., (© 2020 Wiley Periodicals LLC.)

Pediatric Pulmonology publishes original research, case reports, and review articles on topics related to a wide range of children's respiratory disorders. In this article, we highlight the past year's publications in the topic areas of rare lung diseases, respiratory complications of neuromuscular disorders, and diagnostic testing, as well as selected literature in these areas from other journals., (© 2019 Wiley Periodicals, Inc.)

Pediatric Pulmonology publishes original research, reviews, and case reports related to a wide range of children's respiratory disorders. In our "Year in Review" series, we summarize publications in our major topic areas from 2018, in the context of selected literature in these areas from other journals relevant to our discipline. This review covers selected articles on asthma, physiology/lung function testing, and respiratory infections., (© 2019 Wiley Periodicals, Inc.)

Pediatric Pulmonology publishes original research, case reports and review articles on topics related to a wide range of children's respiratory disorders. In this article (Part 1 of a series), we summarize the past year's publications in our major topic areas, as well as selected literature in these areas from other journals. In Part 1, we review selected articles on diagnostic testing/endoscopy, respiratory complications of neuromuscular disorders, and rare lung diseases., (© 2018 Wiley Periodicals, Inc.)

Pediatric Pulmonology publishes original research, reviews, and case reports related to a wide range of children's respiratory disorders. We here summarize the past year's publications in our major topic areas, in the context of selected literature in these areas from other journals relevant to our discipline. This review (Part 3 of a 5-part series) covers selected articles on asthma, physiology/lung function testing, and respiratory infections., (© 2018 Wiley Periodicals, Inc.)

Pediatric Pulmonology publishes original research, case reports and review articles on topics related to a wide range of children's respiratory disorders. In this article (Part 4 of a 5-part series), we summarize the past year's publications in sleep medicine, in the context of selected literature in this area from other journals. Articles are highlighted on topics including diagnosis and treatment of OSAS, sleep duration and position, and sleep disorders in chronic disease., (© 2018 Wiley Periodicals, Inc.)

Pediatric Pulmonology continues to publish research and clinical topics related to the entire range of children's respiratory disorders. As we have done annually in recent years, we here summarize the past year's publications in our major topic areas, as well as selected literature in these areas from other core journals relevant to our discipline. This review (Part 1) covers selected articles on sleep, diagnostic testing/endoscopy, respiratory complications of neuromuscular disorders, and rare lung diseases., (© 2017 Wiley Periodicals, Inc.)

Pediatric Pulmonology continues to publish research and clinical topics related to the entire range of children's respiratory disorders. As we have done annually in recent years, we here summarize some of the past year's publications in our major topic areas, as well as selected literature in these areas from other core journals relevant to our discipline. This review (Part 2) covers selected articles on neonatology, asthma, physiology and lung function testing, and infectious diseases., (© 2017 Wiley Periodicals, Inc.)

This review seeks to re-introduce cystic fibrosis (CF) clinicians to the pharmacology of drug-drug interactions among medications commonly used in CF and provide a framework for understanding these interactions among medications outside the scope of this discussion. We here focus on drugs impacted by the cytochrome P-450 (CYP450) enzyme system and on interactions involving antimicrobials, psychotropic medications, and cystic fibrosis transmembrane conductance regulator (CFTR) modulators. Particular attention is needed when prescribing rifampin, azole antifungals and the CFTR modulators, ivacaftor, and lumacaftor/ivacaftor, in combination with other medications. The complexities of these interactions provide a strong rationale for case management by pharmacists and pharmacologists as a routine part of CF care. Pediatr Pulmonol. 2016;51:S61-S70. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Our journal covers a broad range of research and scholarly topics related to children's respiratory disorders. For updated perspectives on the rapidly expanding knowledge in our field, we will summarize the past year's publications in our major topic areas, as well as selected publications in these areas from the core clinical journal literature outside our own pages. The current review (Part 3) covers articles on asthma, diagnostic testing/endoscopy, respiratory complications of neuromuscular disorders, and rare lung diseases. Pediatr Pulmonol. 2016;51:747-753. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Our journal covers a broad range of research and scholarly topics related to children's respiratory disorders. For updated perspectives on the rapidly expanding knowledge in our field, we will summarize the past year's publications in our major topic areas, as well as selected publications in these areas from the core clinical journal literature outside our own pages. The current review covers articles on neonatal lung disease, pulmonary physiology, and respiratory infection. Pediatr Pulmonol. 2016;51:733-739. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

To better meet the needs of our readership for updated perspectives on the rapidly expanding knowledge in our field, we here summarize the past year's publications in our major topic areas, as well as selected publications in these areas from the core clinical journal literature outside our own pages. This is Part 2 of a series and covers articles on neonatal lung disease, pulmonary physiology, and respiratory infection., (© 2015 Wiley Periodicals, Inc.)

Our discipline and our journal cover an extremely broad range of research and scholarly topics related to children's respiratory disorders. To better meet the needs of our readership for updated perspectives on the rapidly expanding knowledge in our field, we here summarize the past year's publications in our major topic areas, as well as selected publications in these areas from the core clinical journal literature outside our own pages., (© 2015 Wiley Periodicals, Inc.)

Introduction: Transforming growth factor beta-1 (TGF-β₁) is an important genetic modifier of lung disease severity in cystic fibrosis (CF), yet the mechanism behind this disease association remains unknown. Initial steps in the investigation of the relationship between TGF-β₁ and CF lung disease include determining the most appropriate available biospecimen for TGF-β₁ protein measurement., Hypothesis: In hospitalized pediatric CF patients, plasma TGF-β₁ is increased in association with clinical parameters of lung disease severity., Methods: Serum and plasma were obtained pre- and post-intravenous antibiotic therapy in pediatric CF patients hospitalized for a pulmonary exacerbation. Total TGF-β₁ , measured via ELISA, was compared with markers of lung disease, including airway microbiology, lung function, and response to therapy., Results: Forty CF children were studied, 15 of whom underwent bronchoalveolar lavage (BAL) at the time of admission. Plasma TGF-β₁ positively correlated with BAL fluid (BALF) TGF-β₁ (r=0.59, P<0.05). Admission plasma TGF-β₁ was increased in subjects positive for Pseudomonas aeruginosa (P=0.014) and was inversely associated with diminished lung function (P<0.038) after therapy. Treatment with antibiotics significantly decreased plasma TGF-β(1) (P<0.001). Serum TGF-β₁ was not associated with plasma TGF-β(1) , BALF TGF-β₁, or these clinical parameters of lung disease., Conclusion: In pediatric CF, plasma (but not serum) TGF-β₁ is increased in association with Pseudomonas infection and lung disease, and is reduced in response to therapy. These findings emphasize the importance of optimizing biospecimen selection for future studies investigating the role of TGF-β(1) in CF lung disease., (Copyright © 2011 Wiley-Liss, Inc.)

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.

Rationale: Transforming factor beta(1) (TGF-beta(1)) genetic polymorphisms have been identified as a modifier of cystic fibrosis (CF) lung disease severity. However, few data link TGF-beta(1) protein levels and clinical markers of CF lung disease severity., Objectives: To determine the association between protein levels of TGF-beta(1) in pediatric CF bronchoalveolar lavage fluid (BALF) and clinical parameters of CF lung disease severity., Methods: Total TGF-beta(1) was measured in BALF from 30 pediatric CF patients and 12 non-CF disease controls undergoing clinically indicated flexible bronchoscopy, and compared to four indicators of clinical disease: infection, inflammation, pulmonary function, and recent/recurrent hospitalization., Results: TGF-beta(1) was elevated in CF BALF compared to non-CF controls (135 +/- 15 pg/ml vs. 57 +/- 10 pg/ml, P < 0.01). In CF BALF, increased TGF-beta(1) was associated with elevated BALF PMN % (r = 0.67, P < 0.01). BALF TGF-beta(1) was increased in CF subjects whose FEV(1) after the completion of antibiotic therapy remained below CF age-normative median values (205.9 +/- 20.5 pg/ml vs. 106.4 +/- 24.0, P = 0.01). BALF TGF-beta(1) was increased in CF children hospitalized in the previous year compared to those not recently hospitalized (169.9 +/- 21.6 pg/ml vs. 107.5 +/- 17.5 pg/ml, P = 0.04). Neither the presence of a bacterial pathogen nor bacterial quantity was associated with BALF TGF-beta(1)., Conclusions: In CF, BALF TGF-beta(1) is elevated compared to non-CF controls. Increased BALF TGF-beta(1) is associated with neutrophilic inflammation, diminished lung function and recent hospitalization. Further investigation is needed to address mechanisms behind these associations.

Background: The relationship between lower airway markers of inflammation and infection with physiologic findings is poorly understood in young children with cystic fibrosis (CF). The goal of this study was to evaluate the association of bronchoalveolar lavage fluid (BALF) markers of infection and inflammation, including mediators linked to airway remodeling, to infant lung function values in young children with CF undergoing clinically indicated bronchoscopy., Methods: Plethysmography and the raised volume rapid thoracoabdominal compression (RVRTC) technique were performed in 16 sedated infants and young children with CF prior to bronchoscopy. BALF was collected and analyzed for pathogen density, cell count, % neutrophils, transforming growth factor beta 1 (TGF-beta(1)), matrix metalloproteinases (MMP), and interleukin-8 (IL-8)., Results: There was a significant direct correlation between functional residual capacity (FRC), the ratio of residual volume to total lung capacity (RV/TLC) and FRC/TLC with % neutrophils (P < 0.05). Forced expiratory flows were inversely correlated to % neutrophils (P < 0.01). Lung function parameters did not differentiate those with and without lower airway infection; however, pathogen density directly correlated with FRC and inversely correlated with flows (P < 0.05). In a subset of the population, MMP-2 directly correlated with RV/TLC and inversely correlated with flows (P < 0.05) and TGF-beta(1) directly correlated with FRC (P < 0.05)., Conclusions: Results from this study suggest that lower airway inflammation as well as mediators linked to airway remodeling play an active role in pulmonary deterioration in CF infants and young children undergoing clinically indicated bronchoscopy., ((c) 2009 Wiley-Liss, Inc.)

Assessing airway disease in young children with wheeze and/or cough is challenging. We conducted a prospective, descriptive study of lung function in children <3 years old with recurrent wheeze and/or cough, who had failed empiric antiasthma and/or antireflux therapy and subsequently underwent flexible bronchoscopy. Our goals were to describe radiographic, anatomical, microbiological, and physiological findings in these children, and generate hypotheses about their respiratory physiology. Plethysmography and raised-volume rapid thoracoabdominal compression (RVRTC) techniques were performed prior to bronchoscopy. Mean Z-scores (n = 19) were -1.34 for forced expiratory volume at 0.5 sec (FEV(0.5)), -2.28 for forced expiratory flows at 75% of forced vital capacity (FVC) (FEF(75)), -2.25 for forced expiratory flows between 25-75% of FVC (FEF(25-75)), 2.53 for functional residual capacity (FRC), and 2.23 for residual volume divided by total lung capacity (RV/TLC). Younger, shorter children had markedly depressed FEF(75) and FEF(25-75) Z-scores (P = 0.002 and P = <0.001, respectively). As expected, lower airway anatomical abnormalities, infection, and inflammation were common. Elevated FRC was associated with anatomical lower airway abnormalities (P = 0.03). FVC was higher in subjects with neutrophilic inflammation (P = 0.03). There was no association between other physiologic variables and bronchoscopic/bronchoalveolar lavage fluid findings. Half of those with elevated RV/TLC ratios (Z-score >2) had no evidence of chest radiograph hyperinflation. We conclude that in this population, plethysmography and RVRTC techniques are useful in identifying severity of hyperinflation and airflow obstruction, and we hypothesize that younger children may have relatively small airways caliber, significantly limiting airflow, and thus impairing secretion clearance and predisposing to lower airway infection.

Bronchoalveolar lavage fluid (BALF) in cystic fibrosis (CF) shows increased inflammation, which could be due to abnormal cytokine regulation. Bronchial epithelial cells and migratory inflammatory cells produce these cytokines, but few quantitative in vivo data are available comparing young CF patients with controls. We hypothesized that IL-8 mRNA abundance was higher in young CF vs. non-CF disease control patients in lung epithelium and inflammatory cells. Bronchial epithelial cells (BEC) were obtained by brush biopsy, and airway inflammatory cells (BALFC) by bronchoalveolar lavage, in 17 CF and 21 non-CF patients <5 years old undergoing clinically indicated bronchoscopy. Cellular mRNA expression was quantified by real-time PCR and normalized to glyceraldehyde-3-phosphate dehydrogenase (GAPDH). Abundance of IL-8/GAPDH in BEC was significantly higher in CF (14.8 +/- 3.3) than non-CF (4.2 +/- 0.6) samples, and this difference was also significant when patients were stratified according to infection. In BALFC, the difference in IL-8 expression did not reach statistical significance: CF (17.1 +/- 6.5) vs. non-CF (6.8 +/- 1.9), but BALF cell number/ml was significantly higher in CF. IL-10 mRNA was very low in all samples, without showing a decrease in CF vs. non-CF patients. We conclude that early in the disease, IL-8 mRNA expression in BEC is increased in CF in vivo. Although IL-8 mRNA in migratory cells was not significantly higher in CF, these cells may still contribute to elevated IL-8 in airway secretions, secondary to increased cell density in BALF., (Copyright 2004 Wiely-Liss, Inc.)