The role of procalcitonin as a biomarker for acute pulmonary exacerbation in subjects with cystic fibrosis and non-cystic fibrosis bronchiectasis

<b>Objective:</b> This study was designed to assess obstructive sleep apnea (OSA) in adult patients with cystic fibrosis (CF) and non-CF bronchiectasis (BE) and to relate it with clinical characters. <b>Methods:</b> 35 CF (27yrs) and 35 non-CF (24yrs) BE patients were included. Demographic characteristics, medications, comorbidities, BMI, dyspnea scales, pulmonary functions, sputum cultures, exacerbations, and hospitalizations were recorded. Epworth Sleepiness Scale (ESS) questionnaire was filled and polysomnography was performed for each patient. <b>Results:</b> ESS scores did not show any significant difference between CF and non-CF BE patients. 37 (53%) of all patients had OSA. There was no significant difference for OSA risk between CF and non-CF BE patients (54% vs 51%, respectively). Male gender was found to be a risk factor for OSA (68% of males vs 41% of females, respectively, p:0.026). Total sleep time, sleep efficiency, sleep latency, time spent awake after falling asleep, ODI, AHI, AHI in supine position and REM phase did not show any significant difference between CF and non-CF patients. CF patients had significantly lower mean oxygen saturation (p:0.001) and lowest oxygen saturation (p:0.0024) levels and higher heart rate (p:0.02) compared to non-CF BE patients. Multiple logistic regression analysis of all patients revealed male gender and disease duration as risk factors for OSA (p:0.023 and p:0.041 respectively). <b>Conclusion:</b> It is remarkable that more than half of the patients in both CF and non-CF bronchiectasis groups had OSA. Male gender and disease duration were found as risk factors for OSA.

Introduction and ObjectivesBronchiectasis is a dilation of the peripheral airways with subsequent mucus hypersecretion. Bronchiectasis can be either genetic, that is cystic fibrosis (CF) or described as non-CF bronchiectasis (eg,...

Background. Recurrent bacterial infections play a key role in the pathogenesis of bronchiectasis, but conventional microbiologic methods may fail to identify pathogens in many cases. We characterized and compared the pulmonary bacterial communities of cystic fibrosis (CF) and non-CF bronchiectasis patients using a culture-independent molecular approach. Methods. Bacterial 16S rRNA gene libraries were constructed from lung tissue of 10 non-CF bronchiectasis and 21 CF patients, followed by DNA sequencing of isolates from each library. Community characteristics were analyzed and compared between the two groups. Results. A wide range of bacterial diversity was detected in both groups, with between 1 and 21 bacterial taxa found in each patient. Pseudomonas was the most common genus in both groups, comprising 49% of sequences detected and dominating numerically in 13 patients. Although Pseudomonas appeared to be dominant more often in CF patients than in non-CF patients, analysis of entire bacterial communities did not identify significant differences between these two groups. Conclusions. Our data indicate significant diversity in the pulmonary bacterial community of both CF and non-CF bronchiectasis patients and suggest that this community is similar in surgically resected lungs of CF and non-CF bronchiectasis patients.

BackgroundVitamin D deficiency occurs frequently in cystic fibrosis (CF) and non-CF bronchiectasis patients. Yet, few studies have assessed the impact of vitamin D status on the clinical outcomes in pediatric bronchiectasis. This study is designed to assess vitamin D level and determine its effect on exacerbations, bacterial colonization, and lung function in pediatric patients with CF and non-CF bronchiectasis.ResultsThis cross-sectional case-control study assessing vitamin D level was performed in a total of sixty cases under the age of 18 years—forty cases with CF and non-CF bronchiectasis and twenty age- and sex-matched healthy controls. Associations between serum vitamin D and clinical and laboratory parameters were assessed in the patient groups. Vitamin D deficiency was more prevalent among CF and non-CF bronchiectasis patients (75%, 45%) compared to controls (10%) (P < 0.001). In addition, vitamin D deficiency was associated with more frequent and severe pulmonary exacerbations (66.7%, 46.7%) (P=0.033, < 0.001), chronic Pseudomonas infection (80%) (P=0.060) among CF patients, and with lower FEV1 (66%) (P= 0.071) among non-CF bronchiectasis. Moreover, a cutoff value of vitamin D level equal or less than 22.5 ng/ml was accurate in differentiating moderate from mild pulmonary exacerbations in both patients’ groups (AUC=0.809) (p=0.004).ConclusionsVitamin D deficiency is not uncommon in both CF and non-CF bronchiectasis. In this population, vitamin D deficiency is associated with more frequent pulmonary exacerbations, chronic Pseudomonas infection, and worse lung function.

Objective: To determine and compare the effect of vitamin D3 supplementation on modifying the disease severity in cystic fibrosis (CF) and non-CF bronchiectasis pediatric patients. Methods: A randomized clinical trial evaluating the role of oral vitamin D3 supplementation for six months, was performed in forty patients with CF and non-CF bronchiectasis under the age of 18 years with vitamin D deficiency or insufficiency. The primary outcome was to reach the sufficient Vitamin D level, the secondary outcome was to reevaluate bronchiectasis severity by following up the frequency, severity of pulmonary exacerbations and lung function after vitamin D3 supplementation. Results: Forty patients completed the trial. The percentage of improvement of vitamin D level after vitamin D3 supplementation for six months was significantly higher in CF (88.3%) than non-CF bronchiectasis patients (59.82%) (P = 0.03). Additionally, moderate to severe pulmonary exacerbations significantly decreased by more than 60%, 45% (P = 0.001, 0.005) and frequent exacerbations decreased by 15%, 10% (P = 0.327, 0.490), while the forced expiratory volume in 1 (FEV1) significantly increased by 17% and 15% in non CF bronchiectasis and CF patients respectively (p 0.001). Conclusions: Vitamin D3 therapy was effective in decreasing the frequency and severity of pulmonary exacerbations and preserving lung function. Thereby, improving the disease severity even more in non-CF bronchiectasis than CF patients.

<b>OBJECTIVE:</b> Bronchiectasis (BE) is a chronic suppurative lung disease that significantly impacts the patients's mood. The potential impact of poor sleep on depression in BE patients remains largely unstudied. The aim of this study is to evaluate the role of obstructive sleep apnea (OSA) on depression in Cystic Fibrosis (CF) and non-CF adult BE patients. <b>Methods:</b> In total, 35 CF (27 yrs) and 35 non-CF(24 yrs) BE patients were enrolled in this study. Demographics, dyspnea score, BMI, lung function, sputum cultures, exacerbations, and hospitalisations were recorded. All of the participants underwent Epworth Sleepiness Scale (ESS) assessment and Polysomnography (PSG). Depression was examined by using Zung depression scale. <b>Results:</b> In all study participants, OSA was diagnosed in 37 (53%), (19 with CF and 18 with non-CF) and depression was diagnosed in 40 (57%), (20 with CF and 20 with non-CF) of patients. Female patients had higher risk for depression than male patients (67% vs 33 %, respectively, p:0.022). Depression risk was not different between patients with OSA and those without OSA (54% vs 61%, respectively, p:0.580). However, patients with depression had greater ESS when compared with those without depression (5.2±3.8 vs 3.1 ±2.8, p:0.009), and Zung depression scores correlated with ESS (r=0.361, p:0.002) in all patients. There was no statistically significant difference between Zung scales of CF and non-CF BE patients (40.5±12.3 vs 42.9±10.0, respectively, p:0.369). <b>Conclusion:</b> These results emphasize the importance of identifying excessive daytime sleepiness as a significant determinant of depression in CF and non-CF BE patients.

There is currently less experience with inhaled tobramycin in non-cystic fibrosis bronchiectasis than in cystic fibrosis (CF). Intravenous formulation and solution for inhalation (TSI) have been studied in non-CF bronchiectasis patients with chronic P. aeruginosa bronchial infection. An improvement in clinical parameters and a reduction in bacterial density have been shown with both inhaled solutions in these patients. However, further trials are needed to determine the most effective dose and administration protocol in these patients. Based on the current evidence, recommendations cannot be made regarding the use of TSI to treat exacerbations. Although no systemic toxicity has been reported in studies specifically investigating this treatment, patients with known kidney disease or ear disorders should be treated with caution. Adverse respiratory effects are reported to be more common in non-CF patients than in CF patients, who tend to be non-smokers and younger. Research is being conducted into the possibility of combining tobramycin with other antibiotics to increase its antibacterial activity. In this review we will present and discuss the published evidence regarding the use of inhaled tobramycin in non–CF bronchiectasis.

In patients with cystic fibrosis (CF) and non-CF bronchiectasis, Pseudomonas aeruginosa is the most important respiratory pathogen. It is able to synthesise hydrogen cyanide, a potent inhibitor of cellular respiration. The present study investigated whether cyanide is present in the sputum of CF and non-CF bronchiectasis patients infected with P. aeruginosa, and whether the detection of cyanide affected lung function. Cyanide was measured in sputum using a cyanide ion selective electrode. Cyanide was detected in sputum from 15 out of 25 CF and non-CF bronchiectasis patients with current P. aeruginosa infection; however, it was not detected in any of the 10 patients without this organism. Maximum levels were 130 microM (mean+/-SE 72+/-6.6 microM). Concurrent lung function data were available on all 21 P. aeruginosa-infected CF patients; the group with measurable sputum cyanide (n = 11) was not different from those without (n = 10) on the basis of age or sex. However, those with detectable cyanide had significantly poorer lung function than those without (forced expiratory volume in one second (% predicted) 26.8+/-3.8 versus 46.0+/-6.7%; forced vital capacity (% pred) 44.4+/-4.9 versus 60.1+/-7.7%). Cyanide is detectable in sputum from cystic fibrosis and non-cystic fibrosis bronchiectasis patients infected with Pseudomonas aeruginosa, and is also associated with impaired lung function.

Objective To assess the value of sputum neutrophil elastase as a biomarker of severity in cases of non-cystic fibrosis bronchectasis &amp; cystic fibrosis. Methods This cross-sectional study was conducted on 50 bronchiectasis patients aged from 1 to 13 years presented to pediatric chest clinic and department of Ain shams university hospital., there were classified into 2 groups; Group A cystic fibrosis patients(30) and group B non-cystic fibrosis bronchiectasis patients(20). Inclusion criteria :children aged from 1 to 16 years, patients were diagnosed with bronchiectasis clinically and with HRCT chest and cystic Fibrosis patients were diagnosed by positive sweat chloride test and genetic study. Exclusion Criteria includes no other known chronic lung diseases. All patients were evaluated by full medical history and full clinical examination, the following lab investigations were done :sweat chloride test, CFTR mutation study, sputum sampling and culture. Quantitative sputum neutrophil elastase assay was done, and the severity of disease was assessed using clinical scores as SK score ,FACED score and bronchiectasis severity index and by using radiological scores as Bhalla and modified Reiff score. Results Steatorrhea was more prevalent among cystic fibrosis patients (80%) than non-CF bronchiectasis patients (0%).(P = 0.00). Clubbing was more prevalent among non -cystic fibrosis bronchectasis (10) (50 %), than cystic fibrosis (2 patients) (6.7 %).(P = 0.00). In cystic fibrosis, neutrophil elastase was significantly higher among severe group (126 ng/mL) than among the moderate (30 ng/mL) and mild (16 ng/mL) groups.(P = 0.00). While in non-CF bronchiectasis patients, neutrophil elastase was significantly higher among severe group (67 ng/mL) than among the moderate (24 ng/mL) and mild (11 ng/mL) groups.(P = 0.00) Conclusion There was a significant correlation between sputum neutrophil elastase and disease severity in bronchiectasis (both cystic fibrosis and non-CF).

Bactéries émergentes dans la mucoviscidose et les dilatations des bronches hors mucoviscidose. Le point de vue du microbiologiste

To evaluate the diagnostic and prognostic value of the serum procalcitonin (PCT) level in the non-acquired immune deficiency syndrome (AIDS) immunocompromised critically ill patients suspected to have infection. A retrospective study was conducted in the non-AIDS immunocompromised patients who were admitted to Department of Critical Care Medicine of Xiangya Hospital, Central South University during January 2011 to December 2014. Demographic characteristics, underlying disease, acute physiology and chronic health evaluation II (APACHEII) score at admission, and clinical records including baseline and peak levels of temperature, white blood count (WBC), PCT, and survival rate within 28 days, infection focus, infectious agents (bacterial, fungi or mixed infection), and the severity of infection (sepsis, severe sepsis, or septic shock) were recorded. Receiver operating characteristic (ROC) curve was plotted, and the diagnostic and protective value of above parameters was evaluated. A total of 98 patients (43 male and 55 female) were enrolled in the study with a median age of 44 (28, 52) years old and a median APACHEII score of 17 (11, 20); 47 with malignant hematological tumor, 45 with autoimmune diseases, and 6 post solid organ transplantation. Among them 53 patients (54.1%) died within 28 days. Twenty-seven patients were diagnosed as systemic inflammatory response syndrome (SIRS) without infection. Among 71 patients with infection, 45 were diagnosed as bacterial infection, 10 with fungal infection, and 16 with mixed infection. Sepsis was diagnosed in 7 patients, severe sepsis in 32 patients, and septic shock in 32 patients. (1) There was no statistical significance in the baseline and peak levels of PCT and WBC, or baseline level of temperature between the groups of SIRS patients without infection and infected patients. The peak level of temperature was significantly higher in the patients with infection as compared with that of the SIRS without infection patients [centigrade: 39.4 (38.9, 40.0) vs. 38.8 (37.8, 39.2), Z=-3.268, P=0.001]. It was showed by subgroup analysis that in patients with hematological malignant disease or autoimmune diseases, higher level of body temperature was found in infection group compared with non-infection SIRS group [centigrade: 39.5 (39.0, 40.0) vs. 39.0 (38.4, 39.4), Z=-2.349, P=0.019; 39.0 (38.4, 39.5) vs. 38.2 (37.0, 38.9), Z=-2.221, P=0.026]. (2) The baseline level of PCT (μg/L) were 0.54 (0.20, 4.19), 2.78 (0.50, 9.54), 1.00 (0.45, 6.89), and 0.22 (0.07, 1.86) in non-infection SIRS patients or the patients with bacterial, fungal, and mixed infection, respectively. The peak level of PCT (μg/L) were 4.19 (1.95, 13.42), 12.37 (3.82, 45.89), 1.82 (0.49, 17.86), and 5.14 (2.66, 12.62), respectively, in each subgroup. When the comparison was conducted among the patients with different infectious agent, the baseline level of PCT in patients with bacterial infection was significantly higher than that in SIRS patients without infection (P=0.026) and mixed infection patients (P=0.001), and the peak level of PCT was significantly higher than that in the SIRS patients without infection (P=0.009) and the patients with fungal infection (P=0.016). ROC curve showed that the higher value was found in the baseline and peak levels of PCT for diagnosis of septic shock in all patients [ area under ROC curve (AUC) of baseline level=0.681±0.054, P=0.001; AUC of peak level=0.690±0.054, P=0.002], and the same value was also found in the baseline and peak levels of PCT for diagnosis of bacterial infection in the patients with malignant hematological tumor (AUC of baseline level=0.687±0.080, P=0.008; AUC of peak level=0.697±0.079, P=0.021). (3) The peak level of PCT (μg/L) were 4.05 (0.53, 31.22), 5.78 (2.14, 16.68), and 11.64 (2.94, 58.14) in subgroup of patients with sepsis, severe sepsis and septic shock, respectively, and they showed no statistical significance among subgroups (P>0.05). A high serum level of peak PCT strongly indicated the presence of septic shock (AUC=0.646±0.060, P=0.019), especially in the subgroup of patients with systemic autoimmune disease (AUC=0.689±0.081, P=0.035). (4) The peak level of PCT (μg/L) in the APACHEII>18 group (38 cases) was significantly higher than that of APACHEII≤18 group [ 60 cases, PCT (μg/L): 11.64 (3.36, 39.39) vs. 4.42 (1.32, 14.70), P=0.016]; there was a certain correlation between the peak level of PCT and the severity of the disease. (5) The peak level of PCT in death group was significantly higher than that of the survival group [μg/L: 9.07 (3.05, 33.09) vs. 4.19 (1.26, 14.61), P=0.043]. ROC curve showed that the peak level of PCT might be valuable in predicting the prognosis in immunocompromised patients (AUC=0.619±0.057, P=0.043). The serum level of PCT is found to be a reliable marker for the diagnosis of bacterial infection in immunocompromised critical patients, especially in those with hematologic malignancy. Additionally, PCT provides a useful tool for evaluating the severity of infection and the prognosis of critically ill patients.

BackgroundBoth cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis are characterized by permanent bronchial dilation, impaired mucociliary clearance, and development of chronic colonization and infection. Although the major airway microbiota in both CF and non-CF bronchiectasis may be similar, there are some differences in clinical and microbiologic features. There may also be differences in antibiotic susceptibility patterns between the CF and non-CF populations. Therefore, analysis and comparison of the microbiota and antibiotic susceptibility pattern in CF bronchiectasis versus non-CF bronchiectasis would help to improve the management of both conditions.MethodsTwo authors will independently search the electronic databases PubMed, EMBASE, the Cochrane Library, and LIVIVO, for studies reporting bacterial colonization of the respiratory tract in adults and children diagnosed with bronchiectasis in either CF or non-CF. We will include studies examining any respiratory tract specimen, using conventional bacterial culture or other specialized techniques such as molecular methods. We will also examine the antimicrobial susceptibility patterns in people with CF bronchiectasis versus non-CF bronchiectasis. The authors will independently assess the risk of bias in each included study using the Newcastle Ottawa Scale (NOS). We will present the data with descriptive statistics and provide pooled estimates of outcomes, wherever it is feasible to perform meta-analysis. Heterogeneity in studies will be explored by visual inspection of forest plots as well as using the Higgins and Thompson I2 method. We will contact the corresponding authors of studies where data is/are missing and try to obtain the missing data. We will undertake sensitivity analysis to explore the impact of study quality and subgroup analysis based on pre-set criteria. We will prepare a summary of findings’ table and assess the confidence in the evidence using the GRADE methodology.DiscussionTo date, there are no locally applicable evidence-based guidelines for antimicrobial treatment of non-CF bronchiectasis patients. In general, treatment is based on extrapolation of evidence in people with CF bronchiectasis. An insight into the microbiota and antimicrobial susceptibility patterns in the two conditions would facilitate appropriate rather than empiric antimicrobial therapy and hopefully reduce the burden of antimicrobial resistance created by rampant usage of antibiotics.Systematic review registrationThe protocol has been registered in PROSPERO on July 26, 2020 (PROSPERO registration number: CRD42020193859).

To the Editor: In both cystic fibrosis (CF) and non-CF bronchiectasis (NCFBr) chronic Pseudomonas aeruginosa infection is adversely prognostic [1, 2]. In CF, epidemic infections with specific clones of P. aeruginosa are associated with further adverse outcomes [3, 4]. This cross-infection risk has led to segregation of patients [5]. There are few data on P. aeruginosa cross-infection in NCFBr. As a result, segregation in NCFBr has not been addressed in guidelines [6]. Our aim was to undertake a cross-infection study in NCFBr. This was undertaken in an adult bronchiectasis service in the north-east of England (UK) that is separated from the regional CF unit (sited 2 miles (3 km) away). The service was initiated in 2007 with a weekly specialist clinic without a Pseudomonas -specific clinic. When NCFBr patients are hospitalised, there is a preference for cubicle-based (single-patient room) management, but when cubicles are unavailable, patients are managed in six-bed bays. All patients had computed tomographic confirmation and had predominantly idiopathic or post-infectious bronchiectasis with CF excluded following current guidelines [6]. The study had ethical permission and Caldicott approval (Newcastle and North Tyneside National Research Ethics Service Committee). 56 isolates were selected for analysis. Six were chosen from CF patients as laboratory controls. 50 were NCFBr isolates collected between 2008 and 2011 from …

Bronchiectasis is usually classified as cystic fibrosis (CF) related or CF unrelated (non-CF); the latter is not considered an orphan disease any more, even in developed countries. Irrespective of the underlying etiology, bronchiectasis is the result of interaction between host, pathogens, and environment. Vitamin D is known to be involved in a wide spectrum of significant immunomodulatory effects such as down-regulation of pro-inflammatory cytokines and chemokines. Respiratory epithelial cells constitutively express 1α-hydroxylase leading to the local transformation of the inactive 25(OH)-vitamin D to the active 1,25(OH)2-vitamin D. The latter through its autocrine and paracrine functions up-regulates vitamin D dependent genes with important consequences in the local immunity of lungs. Despite the scarcity of direct evidence on the involvement of vitamin D deficiency states in the development of bronchiectasis in either CF or non-CF patients, it is reasonable to postulate that vitamin D may play some role in the pathogenesis of lung diseases and especially bronchiectasis. The potential contribution of vitamin D deficiency in the process of bronchiectasis is of particular clinical importance, taking into consideration the increasing prevalence of vitamin D deficiency worldwide and the significant morbidity of bronchiectasis. Given the well-established association of vitamin D deficiency with increased inflammation, and the indicative evidence for harmful consequences in lungs, it is intriguing to speculate that the administration of vitamin D supplementation could be a reasonable and cost effective supplementary therapeutic approach for children with non-CF bronchiectasis. Regarding CF patients, maybe in the future as more data become available, we have to re-evaluate our policy on the most appropriate dosage scheme for vitamin D.

Background and aimsPseudomonas aeruginosa (PsA) is associated with considerable morbidity and mortality in Non-Cystic Fibrosis bronchiectasis (NCFB) and Cystic Fibrosis (CF) patients. Ticarcillin, a carboxypenicillin, is occasionally used in NCFB...