Pediatric Pulmonology (Pediatr Pulmonol)

Publisher: Wiley

Journal description

Pediatric Pulmonology publishes reports on laboratory research and clinical investigations or observations concerning the respiratory system during its entire development from the fetal stage throughout childhood and adolescence.

Current impact factor: 2.70

Impact Factor Rankings

2016 Impact Factor Available summer 2017
2014 / 2015 Impact Factor 2.704
2013 Impact Factor 2.297
2012 Impact Factor 2.375
2011 Impact Factor 2.533
2010 Impact Factor 2.239
2009 Impact Factor 1.816
2008 Impact Factor 1.883
2007 Impact Factor 2.267
2006 Impact Factor 1.965
2005 Impact Factor 1.589
2004 Impact Factor 1.662
2003 Impact Factor 1.917
2002 Impact Factor 1.739
2001 Impact Factor 1.742
2000 Impact Factor 1.545
1999 Impact Factor 1.192
1998 Impact Factor 0.978
1997 Impact Factor 0.945
1996 Impact Factor 1.018
1995 Impact Factor 1.483
1994 Impact Factor 1.184
1993 Impact Factor 0.997
1992 Impact Factor 1.395

Impact factor over time

Impact factor

Additional details

5-year impact 2.60
Cited half-life 7.50
Immediacy index 0.59
Eigenfactor 0.01
Article influence 0.86
Website Pediatric Pulmonology website
Other titles Pediatric pulmonology (Online), Pediatric pulmonology
ISSN 1099-0496
OCLC 39030100
Material type Document, Periodical, Internet resource
Document type Internet Resource, Computer File, Journal / Magazine / Newspaper

Publisher details


  • Pre-print
    • Author can archive a pre-print version
  • Post-print
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  • Restrictions
    • 12 months embargo
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    • On author's personal website, institutional repositories, arXiv, AgEcon, PhilPapers, PubMed Central, RePEc or Social Science Research Network
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    • Author's pre-print must acknowledge acceptance for publication
    • Non-Commercial
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    • Publisher source must be acknowledged with citation
    • Must link to publisher version with set statement (see policy)
    • If OnlineOpen is available, BBSRC, EPSRC, MRC, NERC and STFC authors, may self-archive after 12 months
    • If OnlineOpen is available, AHRC and ESRC authors, may self-archive after 24 months
    • Publisher last contacted on 07/08/2014
    • This policy is an exception to the default policies of 'Wiley'
  • Classification

Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: Bronchopulmonary dysplasia (BPD) is characterized by developmental arrest of the alveolar tissue. Oxidative stress is causally associated with development of BPD. The effects of hydrogen have been reported in a wide range of disease models and human diseases especially caused by oxidative stress. We made a rat model of BPD by injecting lipopolysaccharide (LPS) into the amniotic fluid at E16.5. The mother started drinking hydrogen-rich water from E9.5 and also while feeding milk. Hydrogen normalized LPS-induced abnormal enlargement of alveoli at P7 and P14. LPS increased staining for nitrotyrosine and 8-OHdG of the lungs, and hydrogen attenuated the staining. At P1, LPS treatment decreased expressions of genes for FGFR4, VEGFR2, and HO-1 in the lungs, and hydrogen increased expressions of these genes. In contrast, LPS treatment and hydrogen treatment had no essential effect on the expression of SOD1. Inflammatory marker proteins of TNFα and IL-6 were increased by LPS treatment, and hydrogen suppressed them. Treatment of A549 human lung adenocarcinoma epithelial cells with 10% hydrogen gas for 24 hr decreased production of reactive oxygen species in both LPS-treated and untreated cells. Lack of any known adverse effects of hydrogen makes hydrogen a promising therapeutic modality for BPD. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
    No preview · Article · Feb 2016 · Pediatric Pulmonology
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    ABSTRACT: Bronchiectasis is described classically as a chronic pulmonary disorder characterized by a persistent productive cough and irreversible dilatation of one or more bronchi. However, in children unable to expectorate, cough may instead be wet and intermittent and bronchial dilatation reversible in the early stages. Although still considered an orphan disease, it is being recognized increasingly as causing significant morbidity and mortality in children and adults in both affluent and developing countries. While bronchiectasis has multiple etiologies, the final common pathway involves a complex interplay between the host, respiratory pathogens and environmental factors. These interactions lead to a vicious cycle of repeated infections, airway inflammation and tissue remodelling resulting in impaired airway clearance, destruction of structural elements within the bronchial wall causing them to become dilated and small airway obstruction. In this review, the current knowledge of the epidemiology, pathobiology, clinical features, and management of bronchiectasis in children are summarized. Recent evidence has emerged to improve our understanding of this heterogeneous disease including the role of viruses, and how antibiotics, novel drugs, antiviral agents, and vaccines might be used. Importantly, the management is no longer dependent upon extrapolating from the cystic fibrosis experience. Nevertheless, substantial information gaps remain in determining the underlying disease mechanisms that initiate and sustain the pathophysiological pathways leading to bronchiectasis. National and international collaborations, standardizing definitions of clinical and research end points, and exploring novel primary prevention strategies are needed if further progress is to be made in understanding, treating and even preventing this often life-limiting disease. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
    No preview · Article · Feb 2016 · Pediatric Pulmonology
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    ABSTRACT: Fractional exhaled nitric oxide (FeNO) is a non-invasive biomarker of eosinophilic inflammation which may be used to guide the management of asthma in childhood. To synthesise the available evidence on the efficacy of FeNO-guided management of childhood asthma. Databases including MEDLINE and the Cochrane Library were searched, and randomised controlled trials (RCTs) comparing FeNO-guided management with any other monitoring strategy were included. Study quality was assessed using the Cochrane risk of bias tool for RCTs, and a number of outcomes were examined, including: exacerbations, medication use, quality of life, adverse events, and other markers of asthma control. Meta-analyses were planned if multiple studies with suitable heterogeneity were available. However, due to wide variations in study characteristics, meta-analysis was not possible. Seven RCTs were identified. There was some evidence that FeNO-guided monitoring results in improved asthma control during the first year of management, although few results attained statistical significance. The impact on severe exacerbations was unclear. Similarly, the impact on use of anti-asthmatic drugs was unclear, and appears to depend on the step up/down protocols, and the clinical characteristics of patients. The potential benefit of FeNO monitoring is equivocal. Trends toward reduced exacerbation and increased medication use were seen, but typically failed to reach statistical significance. There are a number of issues that complicate data interpretation, including differences in the likely severity of included cohorts and variations in treatment algorithms. Further work is needed to systematically explore the impact of these parameters. Pediatr Pulmonol.
    No preview · Article · Feb 2016 · Pediatric Pulmonology
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    ABSTRACT: Introduction: In contrast to adults, coccidioidomycosis is a rare disease in infants and the mechanisms of disease acquisition are not well described in infants. The purpose of this study was to describe the clinical presentation, treatment, and outcome of pulmonary coccidioidomycosis in infants in an endemic area. Methods: We performed a retrospective observational study of all patients less than 12 months of age admitted to a tertiary free standing children's hospital from 2003-2012 diagnosed with coccidioidomycosis. Results: Thirteen infants were hospitalized during the study period. The majority of the patients presented with upper and/or lower respiratory tract infection. The most common presenting symptoms included fever (77%), cough (61%), and respiratory distress (38%). Disseminated disease, included pericardial effusion, neck abscess, and lesions in the cerebellum, basal ganglia and left temporoparietal skull. Fluconazole was the initial, antifungal agent used. Amphotericin B was reserved for significant lung disease and disseminated cases. Failed response to fluconazole and amphotericin B were treated with a combination of voriconazole and caspofungin. Average length of treatment was 4 years. All patients survived to hospital discharge. The majority of the patients had resolution of chest radiograph and coccidiodal complement fixing antibody titers. Discussion: Infant coccidioidomycosis has a non-specific presentation and can mimic common infant respiratory illnesses. In endemic areas, coccidioidomycosis should be considered in the differential diagnosis of infants with pulmonary symptoms unresponsive to conventional treatment. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
    No preview · Article · Feb 2016 · Pediatric Pulmonology
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    ABSTRACT: RSV causes considerable morbidity and mortality in children. In cystic fibrosis (CF) viral infections are associated with worsening respiratory symptoms and bacterial colonization. Palivizumab is effective in reducing RSV hospitalization in high risk patient groups. Evidence regarding its effectiveness and safety in CF is inconclusive. CF screening in N. Ireland enabled timely palivizumab prophylaxis, becoming routine in 2002. To determine the effect of palivizumab on RSV-related hospitalization and compare lung function and bacterial colonization at age 6 years for those born pre- and post-introduction of palivizumab prophylaxis. A retrospective audit was conducted for all patients diagnosed with CF during the period from 1997 to 2007 inclusive. RSV-related hospitalization, time to Pseudomonas aeruginosa (PA) 1st isolate, lung function and growth parameters were recorded. Comparisons were made for outcomes pre- and post-introduction of routine palivizumab administration in 2002. A cost evaluation was also performed. Ninety-two children were included; 47 pre- and 45 post-palivizumab introduction. The overall RSV-positive hospitalization rate was 13%. The relative risk of RSV infection in palivizumab non-recipients versus recipients was 4.78 (95%CI: 1.1–20.7), P = 0.027. Notably, PA 1st isolate was significantly earlier in the palivizumab recipient cohort versus non-recipient cohort (median 57 vs. 96 months, P < 0.025) with a relative risk of 2.5. Chronic PA infection at 6 years remained low in both groups, with similar lung function and growth parameters. Total costs were calculated at £96,127 ($151,880) for the non-recipient cohort versus £137,954 ($217,967) for the recipient cohort. Palivizumab was effective in reducing RSV-related hospitalization infection in CF patients. Surprisingly, we found a significantly earlier time to 1st isolate of PA in palivizumab recipients which we could not explain by altered or improved diagnostic tests. Pediatr Pulmonol.
    No preview · Article · Jan 2016 · Pediatric Pulmonology
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    ABSTRACT: Rationale: Maternal prepregnancy obesity has been associated with early wheeze and childhood asthma in their offspring. Some of these studies have been in minority, urban, and disadvantaged populations using parental recall and questionnaires. The association of maternal prepregnancy obesity with bronchodilator dispensing to their offspring, in a primarily insured, non-urban, White population in the United States is unknown. Objectives and methods: We conducted a retrospective cohort study using pharmacy dispensing data from the electronic medical records of a large United States health maintenance organization to examine the relationship between maternal prepregnancy body mass index (BMI) and inhaled bronchodilator dispensing in the offspring to 4 years of age. We included infants ≥37 weeks' gestation with birth weight ≥2.5 kg which yielded 6,194 mother-baby pairs. Maternal prepregnancy BMI was categorized as underweight (<18.5 kg/m(2) ), normal (18.5-24.9 kg/m(2) ), overweight (25-29.9 kg/m(2) ), or obese (≥30 kg/m(2) ). Results: In the entire cohort, 27.6% of the offspring received a bronchodilator dispensing. This ranged from 19.2% in the offspring of underweight mothers to 31.3% of those born to obese mothers. In the fully adjusted model using normal BMI as the referent, children of obese mothers had a 22% higher rate of bronchodilator dispensing (adjusted OR = 1.22; 95%CI 1.05-1.41; P = 0.008). Conclusions: In this insured, non-urban, White population, maternal prepregnancy obesity was associated with bronchodilator dispensing in the offspring in early life. These results extend previous data and reaffirm the potential widespread public health impact that prepregnancy obesity may have on subsequent childhood respiratory health. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
    No preview · Article · Jan 2016 · Pediatric Pulmonology
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    ABSTRACT: A 12-year-old boy developed severe acute respiratory distress during a school break requiring resuscitative measures. The episode started shortly after a short choking episode with a cookie. History, physical examination, laboratory results, chest X-ray, and clinical course supported the diagnosis of negative pressure pulmonary edema (NPPE). NPPE occurring outside a hospital setting, especially following a short episode of choking on a cookie, is rarely reported in children. Understanding the pathophysiological mechanisms contributing to pulmonary edema can help in distinguishing NPPE from other causes of fulminant respiratory distress, and especially from other causes of noncardiogenic pulmonary edema. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
    No preview · Article · Jan 2016 · Pediatric Pulmonology
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    ABSTRACT: ABCA3 is highly expressed in alveolar epithelial type 2 cells and is associated with surfactant homeostasis. Patients with ABCA3 mutations develop various respiratory complications, such as fatal respiratory distress syndrome or interstitial lung disease. We describe a patient with pulmonary fibrosis and emphysema with pulmonary hypertension, associated with compound heterozygous mutations of the ABCA3 gene. This is the first report showing that mutations in the ABCA3 gene lead to pulmonary fibrosis and emphysema, including combined pulmonary fibrosis and emphysema, in childhood. Treatment with prostacyclin analogue, warfarin, and inhaled oxygen was effective to improve patient's hemodynamic condition as well as pulmonary fibrosis and emphysema. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
    No preview · Article · Jan 2016 · Pediatric Pulmonology

  • No preview · Article · Jan 2016 · Pediatric Pulmonology
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    ABSTRACT: Introduction: The association between obesity and asthma control/quality of life commonly relies on body mass index (BMI) as the anthropomorphic measure. Due to limitations of BMI and the existence of alternative measures, such as neck circumference (NC), we examined the association between NC and asthma control/quality of life, with particular attention to male-female differences. Materials and methods: The AsthMaP-2 Project is an observational study of youth with physician-diagnosed asthma. NC was stratified according to age- and sex-specific cutoffs and associated with asthma control (via Asthma Control Test [ACT]) and quality of life (via Integrated Therapeutics Group [ITG]-Asthma Short Form). Results: The mean ± SD age was 11.9 ± 3.6 years, and 53% were male (N = 116). The mean BMI percentile was at the 71 ± 28 percentile. Thirty-one participants (27%) met criteria for high NC. Males with high NC had significantly worse asthma control (P = 0.02) and lower quality of life than those with low NC. No similar association was found for females and the proportion of variability in ACT and ITG was best explained by BMI percentile. Conversely, for males, the proportion of variability in these scores explained by NC was larger than BMI percentile alone (Cohen's f(2) = 0.04-0.09, a small to medium effect size). Discussion: Among male youth with asthma, combined use of NC and BMI percentile explained asthma control and quality of life better than BMI alone. Future studies of asthma should include measurement of NC and other anthropogenic measures of regional adiposity to clarify sex differences in asthma. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
    No preview · Article · Jan 2016 · Pediatric Pulmonology
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    ABSTRACT: Background and objective: Despite the growing evidence of a possible link between asthma and food allergy (FA), so far, the involvement of food in inducing respiratory symptoms has not been fully evaluated. The objective of this study was to evaluate the impact of food allergens on respiratory symptoms and bronchial reactivity (BHR) in schoolchildren with asthma. Methods: The initial study group consisted of 362 children with asthma. In the end, 22 children with concomitant FA, and 18 without FA, were selected to participate in the study. Spirometry and Methacholine Inhalation Challenge (MIC) were conducted prior to and after the completion of a double blind placebo control food challenge (DBPCFC). Results: The food-induced asthmatic reactions were observed in nine (2.5%) out of all 362 children with asthma. Mean FEV1 prior to and after allergen or placebo challenge did not differ between the groups studied. Increase of BHR after DBPCFC was seen in 17 (4.7%) children with asthma. The mean PC20 value in children with FA was 1.41 ± 1.12 mg/ml prior to the allergen challenge and 0.86 ± 0.71 mg/ml (P = 0.002) after the test, whereas these values were 1.93 ± 1.68 mg/ml and 2.02 ± 1.75 mg/ml, respectively, in children without FA (P > 0.05). Significant differences were noted after the allergen provocation in children with FA as compared to children without FA (P = 0.007). Conclusions: Although food allergens are a rare trigger of food-induced asthmatic reactions in schoolchildren with asthma, they could enhance BHR, despite a lack of evident clinical respiratory signs and decreased in FEV1 values after food challenge. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
    No preview · Article · Jan 2016 · Pediatric Pulmonology
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    ABSTRACT: Descending necrotizing mediastinitis (DNM) is an uncommon disease that can cause significant morbidity and mortality. Herein, we report a 17-month-old girl of DNM with an initial normal chest radiograph followed by rapid clinical deterioration during a period of 4 days, from deep neck infection to mediastinitis and then complicated empyema. She was managed successfully with intravenous antibiotics and surgical debridement by video-assisted thoracic surgery (VATS) and cervical drainage. Early recognition and meticulous intensive care of patients with DNM are important to minimize morbidity and mortality. VATS is an effective and minimally invasive therapeutic strategy in children with DNM. Pediatr Pulmonol. © 2015 Wiley Periodicals, Inc.
    No preview · Article · Dec 2015 · Pediatric Pulmonology
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    ABSTRACT: Pediatric eosinophilic pneumonias (EPs) are characterized by a significant infiltration of the alveolar spaces and lung interstitium by eosinophils, with conservation of the lung structure. In developed countries, EPs constitute exceptional entities in pediatric care. Clinical symptoms may be transient (Löffler syndrome), acute (<1 month and mostly <7 days), or chronic (>1 month). Diagnosis relies on demonstration of alveolar eosinophilia on bronchoalveolar lavage, whether or not associated with blood eosinophilia. EPs are a heterogeneous group of disorders divided into: (i) secondary forms (seen mainly in parasitic infections, allergic bronchopulmonary aspergillosis, and drug reactions); and (ii) primary forms (eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome, idiopathic chronic eosinophilic pneumonia, and idiopathic acute eosinophilic pneumonia). Despite their rarity, the etiological approach to EP must be well-defined as some causes can be rapidly life-threatening without initiation of the proper treatment. This approach (i) eliminates secondary forms, with comprehensive history taking and minimal biological assessment, (ii) is oriented in primary forms by the acute or chronic setting, and the existence of extrapulmonary symptoms. Treatment of primary forms has traditionally relied on corticosteroids, usually with a dramatic response. Specific treatments or the adjunction of corticosteroid-sparing treatment or immunosuppressors are currently being evaluated in order to improve the prognosis and the side effects associated with corticosteroid treatment in a pediatric setting. Pediatr Pulmonol. © 2015 Wiley Periodicals, Inc.
    No preview · Article · Dec 2015 · Pediatric Pulmonology
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    ABSTRACT: Background: Asthma is associated with poorer outcomes in sickle cell disease (SCD). Whether AHR can exist in SCD as a distinct entity, separate and independent of asthma, is unknown. Aims: Our goal was to elucidate the prevalence of AHR, as measured by a methacholine challenge test (MCT), in children with SCD who did not have concomitant asthma or any recent history of acute chest syndrome (ACS). To determine if AHR was associated with asthma-like symptoms, we compared the results of the MCT to a validated asthma questionnaire. We also examined if a correlation between AHR and inflammatory markers exists. Methods: AHR was identified with a positive MCT defined as a provocation concentration (PC20 ) < 4 mg/ml. The children and/or their parents completed the ISAAC (International Study of Asthma and Allergies in Children) questionnaire. We obtained blood, urine, and exhaled breath condensate samples. We measured cysteinyl leukotriene levels in urine and exhaled breath condensate via enzyme immunoassay. Results: Twenty-nine of forty children (72.5%) had a positive MCT. Nine (31.0%) also reported asthma-like symptoms on questionnaire. Inflammatory markers did not correlate with AHR. Among MCT positive subjects, those on hydroxyurea had significantly less severe AHR as quantified by PC20 (P = 0.014). Conclusions: In children with SCD, there is a high prevalence of AHR that is not associated with asthma-like symptoms. AHR may be a distinct entity in children with SCD, existing in the absence of concomitant asthma. Hydroxyurea therapy might lessen the severity of AHR in affected individuals. Pediatr Pulmonol. 2015;9999:1-9. © 2015 Wiley Periodicals, Inc.
    No preview · Article · Dec 2015 · Pediatric Pulmonology
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    ABSTRACT: Objective: To determine if fetal growth restriction (FGR) in the setting of sterile intra-uterine milieu would be associated with a decrease in respiratory distress syndrome (RDS) of preterm-neonates. Methods: The relationship between FGR and neonatal RDS was examined in 92 singleton preterm-neonates (gestational age [GA]: 24.5-33.4 weeks) born to mothers with sterile intra-uterine milieu, which consisted of both sterile amniotic fluid (AF), and inflammation-free placenta. Sterile AF was defined in the absence of both infection and inflammation in AF. AF inflammation was defined in the presence of AF MMP-8 ≥23 ng/ml. FGR was defined in the presence of birth-weight (BW) below the 5th percentile for GA. Results: FGR was present in 32% and RDS was found in 46% of patients. RDS was less common (24% vs. 56%; P < 0.01) and umbilical arterial pH at birth ≤7.15 was more common (33% vs. 13%; P < 0.05) in preterm-neonates with FGR than in those without FGR. There is a stepwise increase in RDS with increasing BW (i.e., below the 5th percentile, the 5th ∼10th percentile, and above the 10th percentile for GA) (P < 0.01, linear by linear association). Logistic regression analysis demonstrated that FGR was a better independent predictor of a decrease in RDS (OR = 0.049, 95%CI 0.009-0.259, P < 0.0005) than advanced GA at delivery (OR = 0.584, 95%CI 0.423-0.806, P < 0.005) after adjusting for other confounding variables. Conclusion: FGR in the setting of sterile intra-uterine milieu is associated with a decrease in RDS of preterm-neonates. This observation suggests that chronic intra-uterine hypoxic stress related to FGR may be beneficial to fetal lung maturation. Pediatr Pulmonol. © 2015 Wiley Periodicals, Inc.
    No preview · Article · Dec 2015 · Pediatric Pulmonology
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    ABSTRACT: Background: We hypothesized airway inflammation can be detected non-invasively by induced sputum (IS) or peripheral blood eosinophilia, and IS can detect bacterial and viral infection in preschool children with airway disease, with results comparable to broncho-alveolar lavage (BAL). Methods: Preschool children with cystic fibrosis, recurrent wheeze, or wet cough underwent IS with nebulized hypertonic saline, chest physiotherapy, and oropharyngeal suction. Samples were analyzed for inflammation by cytology and bacterial culture, viral detection by PCR. Results were compared to BAL and blood in a sub-group undergoing clinically indicated bronchoscopy. Results: 64 children (median age 33 [7-76] months) underwent IS without adverse events. IS was obtained from 61/64. Twenty out of sixty-four underwent BAL and IS, no IS was obtained in 2/23. Thirteen out of twenty-one (62%) had matching bacteria and viruses, 4/21 had positive BAL bacterial growth with negative IS, and 3/21 had negative BAL growth with positive IS. 67% of sputum samples were processed for cytology, 46% had <80% squamous cells; the proportion of squamous cells reduced with increasing age (r = -0.55, P < 0.01). IS was significantly more neutrophilic and less eosinophilic than BAL; 2/21 IS samples contained eosinophils compared to 17/23 BAL. There was a positive correlation between blood and BAL eosinophilia (r = 0.75, P < 0.01). Conclusion: IS from preschool children can be used to assess infection. BAL and IS culture concurred in approximately two-thirds. However, inflammation was measureable in only one-third of IS samples and the cell differential was predominantly neutrophilic compared to BAL. Blood eosinophils may provide a better reflection of lower airway eosinophilia in this age group. Pediatr Pulmonol. © 2015 WileyPeriodicals, Inc.
    No preview · Article · Dec 2015 · Pediatric Pulmonology
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    ABSTRACT: Background: Hypersensitivity pneumonitis (HP) is an immune-mediated diffuse lung disease. Significant improvement in lung function and diffusing capacity after treatment was previously demonstrated, while long-term data focusing specifically on peripheral airway impairment and peak oxygen uptake (fitness) are lacking. Hence, the aim of this study was to conduct a comprehensive study to determine the stability of pulmonary function and fitness in patients previously diagnosed with HP. Methods: We performed a cross-sectional follow-up study with inclusion of longitudinal data if available in patients previously diagnosed with biopsy and high-resolution computed tomography-verified HP during childhood. We performed multiple breath wash-out (LCI2.5 ), spirometry (FEV1 ), bronchiodilator responsiveness test, diffusing capacity (DLCO and DLCO /VA ), body-plethysmography (TLC), and peak oxygen uptake (VO2peak ). St. George Respiratory Questionnaire was used as a measure of respiratory quality of life. Results: Twenty two patients were assessed. LCI2.5 was abnormal in 47.4% compared to abnormal FEV1 in only 9.1% and without significant bronchiodilator responsiveness. DLCO and TLC were abnormal in 40.9 and 13.6%, respectively, while DLCO /VA was within normal range. Only 11.1% demonstrated abnormal VO2peak . All longitudinally assessed outcomes remained unchanged between end of treatment and time of follow up. Conclusions: A large proportion of patients previously diagnosed with HP had abnormal LCI2.5 in contrast to normal spirometry. Spirometric outcomes, TLC, and diffusing capacity were persistently slightly reduced, but stable, and VO2peak was excellent at time of follow-up. Long-term prognosis in children with HP appears favorable although persistent peripheral airway involvement of unknown clinical significance was demonstrated in almost half of the patients. Pediatr Pulmonol. © 2015 Wiley Periodicals, Inc.
    No preview · Article · Dec 2015 · Pediatric Pulmonology

  • No preview · Article · Dec 2015 · Pediatric Pulmonology