Pulmonary Exacerbations In Children Research Articles

Acute respiratory failure due to pulmonary exacerbation in children with cystic fibrosis admitted in a pediatric intensive care unit: outcomes and factors associated with mortality

BackgroundChildren with advanced pulmonary disease due to cystic fibrosis (CF) are at risk of acute respiratory failure due to pulmonary exacerbations leading to their admission to pediatric intensive care units (PICU). The objectives of this study were to determine short and medium-term outcomes of children with CF admitted to PICU for acute respiratory failure due to pulmonary exacerbation and to identify prognosis factors.MethodsThis retrospective monocentric study included patients less than 18 years old admitted to the PICU of a French university hospital between 2000 and 2020. Cox proportional hazard regression methods were used to determine prognosis factors of mortality or lung transplant.ResultsPrior to PICU admission, the 29 patients included (median age 13.5 years) had a severe lung disease (median Forced Expiratory Volume in 1 s percentage predicted at 29%). Mortality rates were respectively 17%, 31%, 34%, 41% at discharge and at 3, 12 and 36 months post-discharge. Survival rates free of lung transplant were 34%, 32%, 24% and 17% respectively. Risk factors associated with mortality or lung transplant using the univariate analysis were female sex and higher pCO2 and chloride levels at PICU admission, and following pre admission characteristics: home respiratory and nutritional support, registration on lung transplant list and Stenotrophomonas Maltophilia bronchial colonization.ConclusionChildren with CF admitted to PICU for acute respiratory failure secondary to pulmonary exacerbations are at high risk of death, both in the short and medium terms. Lung transplant is their main chance of survival and should be considered early.

Effect of Azithromycin on Lung Function and Pulmonary Exacerbations in Children with Post-infectious Bronchiolitis Obliterans. A Double-blind, Placebo-controlled Trial

Introduction: Acute lower respiratory infection (ALRI) of viral etiology is a frequent consultation in pediatrics. Post-infectious bronchiolitis obliterans (PIBO) is a rare and potentially severe disorder following ALRI, characterized by partial or complete obstruction of the small airways by inflammatory tissue. There is evidence that macrolides reduce morbidity and mortality in diffuse panbronchiolitis, which may have similar inflammatory and obstructive components. We hypothesized that the effect of azithromycin (AZ) may improve lung function and reduce pulmonary exacerbations in PIBO. Methods Study design: A double-blind, randomized, placebo-controlled trial. Patients: We enrolled patients with PIBO followed-up at the Pulmonology department between 5 years to 18 years. Treatment regimen: The patients were randomized to receive active drug or placebo three times a week. Clinical evaluation: Clinical evaluation, pulse oximetry, lung function, and 6-min walk test were performed before and after study initiation and at 1, 3, and 6 months. CT scan and a quality of life questionnaire were performed at the beginning and the end of the Study. Results: 29 patients, 15 in G1 (10 males) and 14 in G2 (7 males) were included. There were no significant differences in FVC, FEV1, TLC, RV, or sGaw between the treatment group and controls. In addition, no significant differences were observed in exacerbations, quality of life questionnaire, or HRCT scan scores. Conclusion: No differences were observed between the groups. Further studies are necessary to allow us to find a better treatment, as azithromycin does not seem to be efficacious.

SPLUNC1 as a biomarker of pulmonary exacerbations in children with cystic fibrosis

BackgroundShort palate, lung, and nasal epithelium clone 1 (SPLUNC1) is an innate defence protein that acts as an anti-microbial agent and regulates airway surface liquid volume through inhibition of the epithelial sodium channel (ENaC). SPLUNC1 levels were found to be reduced in airway secretions of adults with cystic fibrosis (CF). The potential of SPLUNC1 as a biomarker in children with CF is unknown. MethodsWe quantified SPLUNC1, interleukin-8 (IL-8) and neutrophil elastase (NE) in sputum of CF children treated with either intravenous antibiotics or oral antibiotics for a pulmonary exacerbation (PEx)s, and in participants of a prospective cohort of CF children with preserved lung function on spirometry, followed over a period of two years. ResultsSputum SPLUNC1 levels were significantly lower before compared to after intravenous and oral antibiotic therapy for PEx. In the longitudinal cohort, SPLUNC1 levels were found to be decreased at PEx visits compared to both previous and subsequent stable visits. Higher SPLUNC1 levels at stable visits were associated with longer PEx-free time (hazard ratio 0.85, p = 0.04). SPLUNC1 at PEx visits did not correlate with IL-8 or NE levels in sputum or forced expiratory volume in one second (FEV1) but did correlate with the lung clearance index (LCI) (r=-0.53, p < 0.001). ConclusionSPLUNC1 demonstrates promising clinometric properties as a biomarker of PEx in children with CF.

Factors influencing treatment response of pulmonary exacerbation in children with cystic fibrosis.

Pulmonary exacerbations in cystic fibrosis (CF) significantly impact morbidity and mortality. This study aimed to assess treatment response rates and identify contributing factors towards treatment response. In this single-center, retrospective, longitudinal study spanning four years, we analyzed all pulmonary exacerbation admissions. We compared lung function at baseline, admission, end of treatment, and 6-week follow-up. Treatment response was defined as ≥95% recovery of baseline FEV<inf>1</inf>%. There were 78 children who required a total of 184 admissions. The mean duration of treatment was 14.9±2.9 days. FEV<inf>1</inf>% returned to 95% of baseline in 59% following treatment. The magnitude of the decline in lung function on admission in children who did not respond to treatment was 21.7±15.2% while the decline in children who responded to treatment was 8.3±9.4%, P<0.001. Children who experienced a decline in FEV<inf>1</inf>% greater than 40% exhibited an 80% reduced likelihood of returning to their baseline values (OR -0.8, 95% CI -0.988; -0.612). Similarly, those with FEV<inf>1</inf>% reductions in the ranges of 30-39% (OR -0.63, 95% CI -0.821; -0.439), 20-29% (OR -0.52, 95% CI -0.657; -0.383), and 10-19% (OR -0.239, 95% CI -0.33; -0.148) showed progressively lower odds of returning to baseline. Fourty-eight children required readmission within 7.7±5.4 months, children who responded to treatment had a longer time taken to readmission (8.9±6.4 months) versus children who did not respond to treatment (6.4±3.5 months), (OR: -0.20, 95% CI -0.355; -0.048). A greater decline in lung function on admission and readmission within 6 months of the initial admission predicts non-response to treatment. This highlights the importance of re-evaluating follow-up strategies following discharge.

Association of the three-minute step test with the occurrence of pulmonary exacerbations in children with cystic fibrosis: a cross-sectional study.

Cystic fibrosis (CF) patients experience pulmonary exacerbations and the three-minute step test (3-min step test) may be a simple and easy-to-perform functional test to help identify such episodes. The present study aimed to evaluate the association of the 3-min step test with the occurrence of pulmonary exacerbations in children with CF. Cross-sectional study of CF patients aged 6 years and older. Pulmonary exacerbations were assessed using both the Fuchs criteria and the Kanga Score. The 3-min step test was performed using a 15 cm-high step and heart rate (HR), oxygen saturation (SpO<inf>2</inf>), and dyspnea were measured before and after the test. Correlations between the test and the scores, as well as comparisons between patients experiencing or not an exacerbation, were performed. Sixty-two patients (11.1±4.3 years) were included. Both the Fuchs criteria and the Kanga score correlated significantly with age, forced expiratory volume in the first second (FEV<inf>1</inf>), final SpO<inf>2</inf>, and 1-min recovery SpO<inf>2</inf>. A fall greater than 4% in the final SpO<inf>2</inf> was significantly associated with the presence of a pulmonary exacerbation, considering both Fuchs and Kanga criteria. Age, resting HR, and HR after 1-min recovery were significantly higher, while FEV<inf>1</inf>, SpO<inf>2</inf> at rest, final SpO<inf>2</inf>, and SpO<inf>2</inf> after 1-min recovery were significantly lower in patients classified as exacerbated. Physiological responses to the 3-min step test are associated with the occurrence of pulmonary exacerbation in children with CF. Desaturation at the end of the test or during 1-min recovery may be the best variable to monitor.

How Does the Corrected Exhalyzer Software Change the Predictive Value of LCI in Pulmonary Exacerbations in Children with Cystic Fibrosis?

Aim: Recently, the most commonly used for multiple breath washout device, the Exhalyzer D, has been shown to overestimate lung clearance index (LCI) results due to a software error. Our study aimed to compare the predictive values of LCI in the CF pulmonary exacerbations (PE) calculated with the updated (3.3.1) and the previous (3.2.1) version of the Spiroware software. Materials and Methods: The measurements were performed during 259 visits in CF pediatric patients. We used 39ΔPE pairs (PE preceded by stable visit) and 138ΔS pairs (stable visit preceded by stable visit) to compare the LCI changes during PE. The areas under the receiver operating curves (AUCROC) and odds ratios were calculated based on the differences between ΔPEs and ΔSs. The exacerbation risk was estimated using a logistic regression model with generalized estimating equations (GEE). Results: There were statistically significant differences in LCI 2.5% median values measured using the two versions of the software in the stable condition but not during PE. The AUCROC for changes between the two consecutive visits for LCI did not change significantly using the updated Spiroware software. Conclusions: Despite the lower median values, using the recalculated LCI values does not influence the diagnostic accuracy of this parameter in CF PE.

Impact of viral respiratory infections on pulmonary exacerbations in children with cystic fibrosis.

Viral respiratory infections trigger pulmonary exacerbations (PEs) in children with cystic fibrosis (CF), but their clinical impact is not well understood. A retrospective review of pediatric patients with CF who underwent nasopharyngeal respiratory viral paneltesting during hospitalization for a PE between 2011 and 2018 was conducted. Patients were dichotomized into viral-positive and viral-negative groups. The results of spirometry, respiratory cultures, duration of hospitalization, and risk for subsequent PEs were analyzed. Ninety-five patients had 210 hospitalizations for PE (viral-positive = 71/210, 34%) during the study period. Rhinovirus/enterovirus was the most common virus (52/71, 73%) identified. Viral-positive patients were younger (p < 0.001), had higher baseline forced expiratory volume in 1 s(FEV1) (p = 0.037), continued to maintain higher FEV1 at 3 and 6 months following PE (p = 0.003 and 0.002, respectively), and had a shorter duration of hospitalization (p = 0.006) compared to the viral-negative group. There was no difference between the two groups in the rate of recovery of FEV1 at 3 and 6 months following PE (p = 0.71 and 0.405, respectively), time to the next PE (hazard ratio = 1.34, p = 0.157), number of subsequent PEs in 6 months (p = 0.99), or Pseudomonas aeruginosa(PA) acquisition (p = 0.707). In this single pediatric CF center cohort, one-third of PEs requiring hospitalization were associated with a viral infection, with rhinovirus/enterovirus being the most common. Viral-positive PEs were not associated with a greater decline or delayed recovery of lung function, increased risk for PA acquisition, shortened duration to next PE, longer hospital stay, or an increase in the frequency of subsequent PEs in 6 months compared to viral-negative PEs.

Antibiotics and outcomes of CF pulmonary exacerbations in children infected with MRSA and Pseudomonas aeruginosa

Limited data exist to inform antibiotic selection among people with cystic fibrosis (CF) with airway infection by multiple CF-related microorganisms. This study aimed to determine among children with CF co-infected with methicillin-resistant Staphylococcus aureus (MRSA) and Pseudomonas aeruginosa (Pa) if the addition of anti-MRSA antibiotics to antipseudomonal antibiotic treatment for pulmonary exacerbations (PEx) would be associated with improved clinical outcomes compared with antipseudomonal antibiotics alone. Retrospective cohort study using data from the CF Foundation Patient Registry-Pediatric Health Information System linked dataset. The odds of returning to baseline lung function and having a subsequent PEx requiring intravenous antibiotics were compared between PEx treated with anti-MRSA and antipseudomonal antibiotics and those treated with antipseudomonal antibiotics alone, adjusting for confounding by indication using inverse probability of treatment weighting. 943 children with CF co-infected with MRSA and Pa contributed 2,989 PEx for analysis. Of these, 2,331 (78%) PEx were treated with both anti-MRSA and antipseudomonal antibiotics and 658 (22%) PEx were treated with antipseudomonal antibiotics alone. Compared with PEx treated with antipseudomonal antibiotics alone, the addition of anti-MRSA antibiotics to antipseudomonal antibiotic therapy was not associated with a higher odds of returning to ≥90% or ≥100% of baseline lung function or a lower odds of future PEx requiring intravenous antibiotics. Children with CF co-infected with MRSA and Pa may not benefit from the addition of anti-MRSA antibiotics for PEx treatment. Prospective studies evaluating optimal antibiotic selection strategies for PEx treatment are needed to optimize clinical outcomes following PEx treatment.

Volatile Organic Compounds in Exhaled Breath as Biomarkers of Pulmonary Exacerbations in Children with Cystic Fibrosis

Background/Objective: Cystic Fibrosis (CF) is the most common lethal genetic disease in Caucasians, but CF affects all races and ethnicities. A characteristic manifestation of CF is the pulmonary exacerbation (PEx), which involves an increase in pulmonary symptoms, decrease in pulmonary function, energy loss, weight loss, and changes to clinical findings, and correlates with lung disease progression and lower quality of life. However, in children with CF, PExs have a subtle presentation, similar to that of the cold or flu, and there is a need for a test that can identify PExs. For this project, our objective was to establish a proof of concept for the use of volatile organic compounds (VOCs) in exhaled breath as PEx biomarkers.&#x0D; &#x0D; Methods: This project enrolled 19 participants with a diagnosis of CF between the ages of 8 and 18, at Riley Cystic Fibrosis Center in Indianapolis. From each participant, exhaled breath was collected in a Tedlar Bag, in addition to lung function data and PEx status. Exhaled breath was analyzed for VOCs using Solid Phase Microextraction coupled to Gas Chromatography-Mass Spectrometry.&#x0D; &#x0D; Results: There were 13 VOCs that exhibited statistically significant differences in concentration between exhaled breath at baseline and during a PEx. These VOCs could be used in a Principal Component Analysis to differentiate baseline and PEx conditions. Finally, using Linear Regression, this project identified 22 VOCs with a significant correlation to lung function, an important potential confounder. Limitations include: (1) comparisons of multiple VOCs increase the likelihood of a false positive result and (2) small sample size.&#x0D; &#x0D; Conclusions: VOCs in exhaled breath are promising candidates as biomarkers to diagnose the presence and severity of PExs. In future experiments, we aim to expand the number of participants and track more clinical details to provide further evidence and applications for this proof of concept.

Evaluating FEV1 decline in diagnosis and management of pulmonary exacerbations in children with cystic fibrosis.

RationaleForced expiratory volume in 1 s (FEV1) decline (ΔFEV1) is associated with pulmonary exacerbation (PEx) diagnosis in cystic fibrosis (CF). Spirometry may not be available during telehealth visits and could impair clinician ability to diagnose PEx. This study aims to (1) identify the associations between degrees of ΔFEV1 (decrease of <5% predicted vs. 5%−9% predicted vs. ≥10% predicted from baseline), clinical symptoms, and clinician‐diagnosed PEx and (2) evaluate the correlation between respiratory symptoms, ΔFEV1, and antibiotic treatment.MethodsRetrospective, descriptive study of PEx diagnosis and management in 628 outpatient clinical encounters with spirometry in 178 patients with CF ages 6−17 years at Riley Hospital for Children during 2019. Odds ratios (OR) of symptoms associated with clinician‐defined PEx diagnosis and antibiotic management stratified by ΔFEV1 decline were determined.ResultsClinician‐diagnosed PEx occurred at 199 (31.7%) visits; increased cough (77.4%) and sputum/wet cough (57.8%) were the most frequently reported symptoms. Compared to no ΔFEV1, the odds of a clinician‐diagnosed PEx were increased when ΔFEV15%−9% and ΔFEV1≥10% was present with increased cough (OR 1.56, 95% confidence interval [CI] 1.25−1.94 and OR 1.82, 95% CI 1.52−2.19, respectively), increased sputum (OR 1.59, 95% CI 1.20−2.12 and OR 1.78, 95% CI 1.37−2.32, respectively), and increased cough and sputum together (OR 1.51, 95% CI 1.08−2.13 and OR 1.68, 95% CI 1.22−2.31, respectively).ConclusionsΔFEV1 is associated with increased likelihood that cough and sputum are diagnosed as a PEx. Spirometry is essential for PEx diagnosis and treatment and is a necessary component of all clinical encounters.

Use of systemic steroids during pulmonary exacerbations in children and adolescent with Cystic fibrosis.

To determine the role of systemic steroids in cystic fibrosis patients and its effects on pulmonary exacerbation in children and adolescents. The retrospective cohort study was conducted at the Aga Khan University Hospital, Karachi, and comprised data from January 2015 to December 2019 of cystic fibrosis patients aged 3-18 years hospitalised with pulmonary exacerbations. The patients were divided into systemic steroid group A and non-systemic steroid group B. Patients in group A received parenteral steroids during acute exacerbation of cystic fibrosis in the first two weeks of admission, while those in group B did not receive systemic steroids. Length of hospital stay and number of days on oxygen support were compared between the groups. Data was analysed using SPSS 22. Of the 124 patient charts evaluated, 84(67.7%) were included; 40(47.6%) in group A and 44(52.4%) in group B. There were no significant differences between the groups related to age, age at diagnosis, weight, height, and pulmonary exacerbations (p>0.05). Group A had significantly fewer days on oxygen support compared to group B (p<0.001), but there was inter-group difference in mean length of hospital stay (p=0.53). Systemic steroid usage during hospitalisation for acute exacerbation of cystic fibrosis was associated with decreased duration of oxygen requirement with standard treatment.

Impact of azithromycin on serum inflammatory markers in children with cystic fibrosis and new Pseudomonas

Chronic azithromycin improves outcomes in cystic fibrosis (CF), but its mechanism of action is unclear. The OPTIMIZE trial demonstrated improvement in time to first pulmonary exacerbation in children with new Pseudomonas treated with azithromycin. Azithromycin effect on systemic markers of inflammation over 18 months was assessed by change from baseline for high-sensitivity C-reactive protein, myeloperoxidase, calprotectin and absolute neutrophil count in the OPTIMIZE population. Subjects treated with chronic azithromycin or placebo had samples collected at baseline, 39 and 78 weeks of treatment. In 129 subjects, a significant decrease in high-sensitivity C-reactive protein was present at 39 weeks in the azithromycin group compared to placebo, but no significant difference between the groups at 78 weeks. No differences in change from baseline in myeloperoxidase, calprotectin or absolute neutrophil count were present at either time point. This supports the concept of a transient immunomodulatory effect for chronic azithromycin therapy in children with CF.

Measuring the impact of an empiric antibiotic algorithm for pulmonary exacerbation in children and young adults with cystic fibrosis.

BackgroundAntimicrobial stewardship is a systematic effort to change prescribing attitudes that can provide benefit in the provision of care to persons with cystic fibrosis (CF). Our objective was to decrease the unwarranted use of broad‐spectrum antibiotics and assess the impact of an empiric antibiotic algorithm using quality improvement methodology.MethodsWe assembled a multidisciplinary team with expertise in CF. We assessed baseline antibiotic use for treatment of pulmonary exacerbation (PEx) and developed an algorithm to guide empiric antibiotic therapy. We included persons with CF admitted to Children's National Hospital for treatment of PEx between January 2017 and March 2020. Our primary outcome measure was reducing unnecessary broad‐spectrum antibiotic use, measured by use consistent with the empiric antibiotic algorithm. The primary intervention was the initiation of the algorithm. Secondary outcomes included documentation of justification for broad‐spectrum antibiotic use and use of infectious disease (ID) consult.ResultsData were collected from 56 persons with CF who had a total of 226 PEx events. The mean age at first PEx was 12 (SD 6.7) years; 55% were female, 80% were white, and 29% were Hispanic. After initiation of the algorithm, the proportion of PEx with antibiotic use consistent with the algorithm increased from 46.2% to 79.5%. Documentation of justification for broad‐spectrum antibiotics increased from 56% to 85%. Use of ID consults increased from 17% to 54%.ConclusionAntimicrobial stewardship initiatives are beneficial in standardizing care and fostering positive working relationships between CF pulmonologists, ID physicians, and pharmacists.

A new path for CF clinical trials through the use of historical controls

BackgroundGiven future challenges in conducting large randomized, placebo controlled trials for future CF therapeutics development, we evaluated the potential for using external historical controls to either enrich or replace traditional concurrent placebo groups in CF trials. MethodsThe study included data from sequentially completed, randomized, controlled clinical trials, EPIC and OPTIMIZE respectively, evaluating optimal antibiotic therapy to reduce the risk of pulmonary exacerbation in children with early Pseudomonas aeruginosa infection. The primary treatment effect in OPTIMIZE, the risk of pulmonary exacerbation associated with azithromycin, was re-estimated in alternative designs incorporating varying numbers of participants from the earlier trial (EPIC) as historical controls. Bias and precision of these estimates were characterized. Propensity scores were derived to adjust for baseline differences across study populations, and both Poisson and Cox regression were used to estimate treatment efficacy. ResultsReplacing 86 OPTIMIZE placebo participants with 304 controls from EPIC to mimic a fully historically controlled trial resulted an 8% reduction in risk of pulmonary exacerbations (Hazard ratio (HR):0.92 95% CI 0.61, 1.34) when not adjusting for key baseline differences between study populations. After adjustment, a 37% decrease in risk of exacerbation (HR:0.63, 95% CI 0.50, 0.80) was estimated, comparable to the estimate from the original trial comparing the 86 placebo participants to 77 azithromycin participants on azithromycin (45%, HR:0.55, 95% CI: 0.34, 0.86). Other adjusted approaches provided similar estimates for the efficacy of azithromycin in reducing exacerbation risk: pooling all controls from both studies provided a HR of 0.60 (95% x`CI 0.46, 0.77) and augmenting half the OPTIMIZE placebo participants with EPIC controls gave a HR 0.63 (95% CI 0.48, 0.82). ConclusionsThe potential exists for future CF trials to utilize historical control data. Careful consideration of both the comparability of controls and of optimal methods can reduce the potential for biased estimation of treatment effects.

176: Evaluating use of FEV1 decline in diagnosis and management of pulmonary exacerbations in children with cystic fibrosis

176: Evaluating use of FEV1 decline in diagnosis and management of pulmonary exacerbations in children with cystic fibrosis

TOPIC SESSIONS

TOPIC SESSIONS

Exhaled volatile organic compounds detect pulmonary exacerbations early in children with cystic fibrosis: results of a 1 year observational pilot study

In patients with cystic fibrosis (CF), pulmonary exacerbations (PEx) have an important influence on well-being, quality of life, and lung function decline. Early detection combined with early treatment may prevent severe PEx. To determine whether early detection of PEx is possible by non-invasive markers (volatile organic compounds) in exhaled breath. In a 1 year prospective observational pilot study, 49 children with CF were studied. At clinical visits with an interval of 2 months, lung function, volatile organic compounds (VOCs) in exhaled breath by means of gas chromatography-time-of-flight-mass spectrometry, and medication use were assessed. PEx were recorded. Random forest (RF) classification modelling was used to select discriminatory VOCs, followed by building of receiver operating characteristic curves. An inverse relation between the predictive power of a set of VOCs and time between exhaled breath sampling and the onset of PEx was found. When this time period was within 7 d, the RF model with the nine most discriminatory VOCs was able to correctly predict 79% of the children with an upcoming PEx or remaining stable (sensitivity 79% and specificity 78%). This result was validated by means of bootstrapping within the RF classification model. PEx in children with CF can be detected at an early stage by means of exhaled VOCs. The highest predictive value was reached if time between sampling and the onset of an exacerbation was no longer than 7 d.

Vitamin D status and pulmonary exacerbations in children and adolescents with cystic fibrosis: Experience from a tertiary care center.

Background:The function of Vitamin D in preventing inflammation and infection has been studied previously for different pathologies in different populations globally. Relationships between serum Vitamin D levels and its effect on pulmonary exacerbations in the cystic fibrosis (CF) population are not well studied in our part of the world. Therefore, we aimed to ascertain the Vitamin D status in pediatric and adolescent CF patients and its association with pulmonary exacerbations.Materials and Methods:A retrospective study was conducted at The Aga Khan University Hospital from 2015 to 2018. Patients of CF with sweat chloride value >60 mmol/l and who had at least one measurement of 25 hydroxy Vitamin D (25 OHD) were included in the study. Annual serum Vitamin D levels were documented for enrolled patients and their past 1-year data were analyzed for pulmonary exacerbations, average length of stay, and tracheal/airway colonization with organisms.Results:69 patients were included in the study. 28 patients (40.57%) were found to be Vitamin D deficient, 22 patients (31.88%) were Vitamin D insufficient and 19 patients (27.53%) were labeled as Vitamin D insufficient. The average number of exacerbations per year was significantly high in Vitamin D deficient group (3.71 ± 0.96) in comparison with insufficient (3.18 ± 1.09) and sufficient groups (2.26 ± 0.93) (P < 0.001).Conclusion:Vitamin D deficiency is related to an increased number of annual pulmonary exacerbations and pseudomonas infections.

Telephone surveillance during the COVID-19 pandemic: Is it a helpful diagnostic tool for detecting acute pulmonary exacerbations in children with chronic lung disease?

Introduction The global burden of the 2019 novel coronavirus disease pandemic on the healthcare system, as well as the high transmission risk of coronavirus disease has led to the use of alternative strategies for evaluation of children with chronic conditions. In this context, telemedicine has become the effective and affordable choice. In this study we aimed to evaluate the efficiency of telephone visits to determine pulmonary exacerbations and hospitalization rates of children with cystic fibrosis and interstitial lung disease. Methods A total of 119 children with cystic fibrosis or interstitial lung disease were enrolled and provided cases in which telephone visits were applied during the peak time of the coronavirus disease pandemic in our country. The recordings of respiratory, gastrointestinal and other symptoms, nutrition status, rate of acute pulmonary exacerbation, treatments initiated by telephone visits, referral to hospital and hospitalization were established from the electronic health reports of the patients. Results Thirteen patients (10.9%) were symptomatic, 12 of them (10%) were diagnosed with acute pulmonary exacerbation. One patient was diagnosed with peripheral facial paralysis. Nine patients were recalled to the hospital and seven patients (5.8%) were hospitalised. Discussion Using telemedicine the health status of patients can be defined, and patients can be guided on proper healthcare that they need, especially during the times of pandemics which we are facing. Communication with patients while minimising the risk of exposure to coronavirus disease is an important advantage of telemedicine. Telemedicine will have to be implemented on our daily medical practice in the near future.

BAL Inflammatory Markers Can Predict Pulmonary Exacerbations in Children With Cystic Fibrosis

Pulmonary exacerbations in cystic fibrosis are characterized by airway inflammation and may cause irreversible lung damage. Early identification of such exacerbations may facilitate early initiation of treatment, thereby potentially reducing long-term morbidity. Is it possible to predict pulmonary exacerbations in children with cystic fibrosis, using inflammatory markers obtained from BAL fluid? A longitudinal analysis was conducted of children aged 0 to 7 years included in the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) study between 2005 and 2015. The association between inflammatory markers from annual BAL fluid and time to pulmonary exacerbation requiring hospital admission in the 6-month period post-BAL was analyzed using Kaplan-Meier curves and Weibull regression, adjusting for annually repeated measurements. Admissions for Pseudomonas eradication were excluded in the main analysis, because of the standard policy in participating centers to treat Pseudomonas in-hospital. Nine hundred seventy-six BAL samples from 308 children were analyzed. After exclusion of admissions for Pseudomonas eradication (n= 43), there were 145 pulmonary exacerbations recorded within 6months of BAL; median time to exacerbation was 31days (interquartile range, 9-100). In univariate analyses, high IL-8 (hazard ratio [HR], 2.25 for 75th vs 25th percentile; 95%CI, 1.87-2.72), neutrophil elastase (HR, 3.00; 95%CI, 2.03-4.42), and high neutrophil percentage (HR, 1.80 for 75th vs25th percentile; 95%CI, 1.56-2.04) were all significantly associated with risk for a pulmonary exacerbation (P< .001). The inflammatory markers remained significant predictors after adjustment for clinical predictive variables. Inflammatory markers in BAL fluid are significant predictors of pulmonary exacerbations in young children with cystic fibrosis. The development of noninvasive measures of lung inflammation may facilitate routine surveillance of cystic fibrosis.