Diagnosis Of Non-cystic Fibrosis Bronchiectasis Research Articles

Inhaled Medications for Maintenance Therapy in Pediatric Noncystic Fibrosis Bronchiectasis.

This review focuses on evaluating literature for the use of inhaled mucolytics (hypertonic saline, mannitol, and dornase alfa), inhaled antibiotics (tobramycin, aztreonam, colistin, and amikacin), and inhaled corticosteroids in pediatric noncystic fibrosis bronchiectasis. A literature search via PubMed was conducted using the search terms "non-cystic fibrosis bronchiectasis," "primary ciliary dyskinesia," and "bronchiectasis" in combination with each inhaled agent of interest. Studies were included if they were specific to patients with a clinical diagnosis of noncystic fibrosis bronchiectasis published from 1998 to July 2024. Several inhaled medications can be considered as maintenance therapies for pediatric patients with noncystic fibrosis bronchiectasis. Hypertonic saline could be considered for its potential airway clearance benefits and low risk of causing harm. Inhaled antipseudomonal antibiotics should be considered in patients who are colonized with Pseudomonas aeruginosa. Inhaled corticosteroid therapy should be reserved for patients with concomitant asthma. Dornase alfa has shown worse outcomes in adults with noncystic fibrosis bronchiectasis and should be used with caution. Risks and benefits should be carefully considered when evaluating these therapies for use in noncystic fibrosis bronchiectasis, and patient-specific treatment regimens should be developed. Chronic management of pediatric noncystic fibrosis bronchiectasis remains challenging due to paucity of applicable literature. Risks and benefits of different agents are discussed in this article with recommendations for application to clinical practice based on studies performed in both adult and pediatric patients with noncystic fibrosis bronchiectasis. Several inhaled medications could be considered as maintenance therapies for pediatric patients with noncystic fibrosis bronchiectasis, with more robust evidence to support use of inhaled antipseudomonal antibiotics and hypertonic saline compared with other available agents. Further investigation is needed to identify a clear place in therapy for inhaled therapies in pediatric noncystic fibrosis bronchiectasis.

Impact of Pseudomonas aeruginosa on resource utilization and costs in patients with exacerbated non-cystic fibrosis bronchiectasis

Aims Non‐cystic fibrosis bronchiectasis (NCFB) is a chronic progressive respiratory disorder occurring at a rate ranging from 4.2 to 278.1 cases per 100,000 persons, depending on age, in the United States. For many patients with NCFB, the presence of Pseudomonas aeruginosa (PA) makes treatment more complicated and typically has worse outcomes. Management of NCFB can be challenging, warranting a better understanding of the burden of illness for NCFB, treatments applied, healthcare resources used, and subsequent treatment costs. Comparing patients diagnosed with exacerbated NCFB, with or without PA on antibiotic utilization, treatments, and healthcare resources utilization and costs was the purpose of this study. Materials and methods This was a retrospective cohort study of commercial claims from IQVIA’s PharMetrics Plus database (January 1,2006–December 31, 2020). Study patients with a diagnosis of NCFB were stratified into two groups based on the presence or absence of PA, then followed to identify demographic characteristics, comorbid conditions, antibiotic treatment regimen prescribed, healthcare resources utilized, and costs of care. Results The results showed that patients with exacerbated NCFB who were PA+ had significantly more oral antibiotic fills per patient per year, more inpatient admissions with a longer length of stay, and more outpatient encounters than those who were PA−. For costs, PA+ patients also had significantly greater total healthcare costs per patient when compared to those who were PA−. Conclusion Exacerbated NCFB with PA+ was associated with increased antibiotic usage, greater resource utilization, and increased costs. The major contributor to the cost differences was the use of inpatient services. Treatment strategies aimed at reducing the need for inpatient treatment could lessen the disparities observed in patients with NCFB.

Measuring accuracy of International Classification of Diseases codes in identification of patients with non-cystic fibrosis bronchiectasis.

Non-cystic fibrosis bronchiectasis is a disease which is increasing in incidence and prevalence worldwide. The incidence of the disease is frequently estimated using databases that rely on International Classification of Diseases, ninth and tenth revisions, clinical modification (ICD-9-CM/ICD-10-CM) discharge diagnoses. Code accuracy has proved to be a major issue for other diagnoses using ICD codes. This study aims to investigate the accuracy of the ICD codes for the diagnosis of non-cystic fibrosis bronchiectasis. This is a retrospective diagnostic accuracy study which compares the radiologist's diagnosis of bronchiectasis with the ICD code reflection of that diagnosis at discharge. Sensitivities were 34% (same for both ICD-9-CM and ICD-10-CM windows) and specificities ranged from 69% for the ICD-9-CM window to 81% for ICD-10-CM window. We observed that ICD codes are an insufficient method to identify patients with a radiologist diagnosis of bronchiectasis.

Self-management for bronchiectasis.

Bronchiectasis is a long term respiratory condition with an increasing rate of diagnosis. It is associated with persistent symptoms, repeated infective exacerbations, and reduced quality of life, imposing a burden on individuals and healthcare systems. The main aims of therapeutic management are to reduce exacerbations and improve quality of life. Self-management interventions are potentially important for empowering people with bronchiectasis to manage their condition more effectively and to seek care in a timely manner. Self-management interventions are beneficial in the management of other airways diseases such as asthma and COPD (chronic obstructive pulmonary disease) and have been identified as a research priority for bronchiectasis. To assess the efficacy, cost-effectiveness and adverse effects of self-management interventions for adults and children with non-cystic fibrosis bronchiectasis. We searched the Cochrane Airways Specialised Register of trials, clinical trials registers, reference lists of included studies and review articles, and relevant manufacturers' websites up to 13 December 2017. We included all randomised controlled trials of any duration that included adults or children with a diagnosis of non-cystic fibrosis bronchiectasis assessing self-management interventions delivered in any form. Self-management interventions included at least two of the following elements: patient education, airway clearance techniques, adherence to medication, exercise (including pulmonary rehabilitation) and action plans. Two review authors independently screened searches, extracted study characteristics and outcome data and assessed risk of bias for each included study. Primary outcomes were, health-related quality of life, exacerbation frequency and serious adverse events. Secondary outcomes were the number of participants admitted to hospital on at least one occasion, lung function, symptoms, self-efficacy and economic costs. We used a random effects model for analyses and standard Cochrane methods throughout. Two studies with a total of 84 participants were included: a 12-month RCT of early rehabilitation in adults of mean age 72 years conducted in two centres in England (UK) and a six-month proof-of-concept RCT of an expert patient programme (EPP) in adults of mean age 60 years in a single regional respiratory centre in Northern Ireland (UK). The EPP was delivered in group format once a week for eight weeks using standardised EPP materials plus disease-specific education including airway clearance techniques, dealing with symptoms, exacerbations, health promotion and available support. We did not find any studies that included children. Data aggregation was not possible and findings are reported narratively in the review.For the primary outcomes, both studies reported health-related quality of life, as measured by the St George's Respiratory Questionnaire (SGRQ), but there was no clear evidence of benefit. In one study, the mean SGRQ total scores were not significantly different at 6 weeks', 3 months' and 12 months' follow-up (12 months mean difference (MD) -10.27, 95% confidence interval (CI) -45.15 to 24.61). In the second study there were no significant differences in SGRQ. Total scores were not significantly different between groups (six months, MD 3.20, 95% CI -6.64 to 13.04). We judged the evidence for this outcome as low or very low. Neither of the included studies reported data on exacerbations requiring antibiotics. For serious adverse events, one study reported more deaths in the intervention group compared to the control group, (intervention: 4 of 8, control: 2 of 12), though interpretation is limited by the low event rate and the small number of participants in each group.For our secondary outcomes, there was no evidence of benefit in terms of frequency of hospital admissions or FEV1 L, based on very low-quality evidence. One study reported self-efficacy using the Chronic Disease Self-Efficacy scale, which comprises 10 components. All scales showed significant benefit from the intervention but effects were only sustained to study endpoint on the Managing Depression scale. Further details are reported in the main review. Based on overall study quality, we judged this evidence as low quality. Neither study reported data on respiratory symptoms, economic costs or adverse events. There is insufficient evidence to determine whether self-management interventions benefit people with bronchiectasis. In the absence of high-quality evidence it is advisable that practitioners adhere to current international guidelines that advocate self-management for people with bronchiectasis.Future studies should aim to clearly define and justify the specific nature of self-management, measure clinically important outcomes and include children as well as adults.

Evaluation of obstructive sleep apnea in non-cystic fibrosis bronchiectasis: A cross-sectional study.

The relationship between sleep disorders and bronchiectasis has not been well described. We hypothesize that, due to the irreversible dilatation of the bronchi, the presence of secretions, and airflow obstruction, patients with non-cystic fibrosis bronchiectasis may be predisposed to hypoxemia during sleep, or to symptoms that may lead to arousal. A cross-sectional observational study was performed involving 49 patients with a clinical diagnosis of non-cystic fibrosis bronchiectasis (NCFB). All patients underwent clinical evaluation, spirometry, and polysomnography, and were evaluated for the presence of excessive daytime sleepiness (EDS) and risk of obstructive sleep apnea (OSA). The mean age of the participants was 50.3 ± 13.6 years; 51.1% of patients were male and had a mean body mass index of 23.8 ± 3.4 kg/m2. The mean total sleep time (TST) was 325.15 ± 64.22 min with a slight reduction in sleep efficiency (84.01 ± 29.2%). Regarding sleep stages, stage 1 sleep and REM sleep were abnormal. OSA was present in 40.82% of the patients. The mean arousal index was 5.6 ± 2.9/h and snoring was observed in 71.43% of the patients. The oxygen desaturation index (ODI) was 14.35 ± 15.36/h, mean minimum oxygen saturation (SpO2 nadir) was 83.29 ± 7.99%, and mean TST with an SpO2 less than 90% was 30.21 ± 60.48 min. EDS was exhibited by 53.06% of the patients and 55.1% were at high risk of developing OSA. The patients infected by Pseudomonas aeruginosa had higher apnea-hypopnea indices, ODI, and TST with SpO2 < 90%, and lower values of SpO2 nadir. Adult patients with clinically stable NCFB, especially those infected by Pseudomonas aeruginosa, display EDS and a high prevalence of OSA, associated with considerable oxygen desaturation during sleep.

Observational study of sleep, respiratory mechanics and quality of life in patients with non-cystic fibrosis bronchiectasis: a protocol study

IntroductionBronchiectasis is a chronic disorder characterised by permanent and irreversible abnormal dilation of the bronchi and bronchioles, primarily caused by repeated cycles of pulmonary infections and inflammation, which lead to...

Mortality in non-cystic fibrosis bronchiectasis: A prospective cohort analysis

There is limited data on mortality and associated morbidity in non-cystic fibrosis bronchiectasis (NCFB). Our aim was to analyze the overall mortality for all newly diagnosed patients from June 2006 onwards and to evaluate risk factors for mortality in this cohort. 245 patients who had a new diagnosis of NCFB between June 2006 and October 2012 at the University Hospital of Leuven, Belgium, were included in the analysis. Death was analyzed until end of November 2013. All patients had chest HRCT scan confirming the presence of bronchiectatic lesions and had symptoms of chronic productive cough. Univariate and multivariate Cox proportional hazard survival regression analysis was used to estimate hazard ratios (HR) and their 95% confidence intervals (CI) of variables possibly predicting mortality. Overall mortality in NCFB patients who had a median follow-up of 5.18 years was 20.4%. Patients with NCFB and associated chronic obstructive pulmonary disease (COPD) had a mortality of 55% in that period. Univariate analysis showed higher mortality according to age, gender, smoking history, Pseudomonas aeruginosa status, spirometry, radiological extent, total number of sputum bacteria and underlying etiology. Multivariate analysis showed significant higher mortality with increasing age (HR=1.045; p=0.004), with increasing number of lobes affected (HR=1.53; p=0.009) and when patients had COPD associated NCFB (HR=2.12; p=0.038). The majority of the 50 deaths were respiratory related (n=29; 58%). NCFB patients with associated COPD disease had the highest mortality rates compared to the other NCFB patients. Additional risk factors for lower survival were increasing age and number of lobes affected.

Risk factors for morbidity and death in non-cystic fibrosis bronchiectasis: a retrospective cross-sectional analysis of CT diagnosed bronchiectatic patients

IntroductionThere is a relative lack of information about the death rate and morbidity of non-cystic fibrosis bronchiectasis and most studies are limited due to referral bias. We wanted to assess death rate and morbidity in those patients at our hospital.MethodsAdult patients seen at our department between June 2006 and November 2009 were recruited if the key string "bronchiect-" was mentioned in electronic clinical records and if chest CT imaging was available. Clinical records of all patients with confirmed radiologic diagnosis of bronchiectasis were reviewed and clinical characteristics were analyzed.Results539 patients with a radiographic diagnosis of non-cystic fibrosis bronchiectasis were identified in a retrospective cross-sectional analysis giving a prevalence of 2.6% in our hospital population. A wide range of etiologies was found with idiopathic bronchiectasis in 26%. In the 41 months interval, 57 patients (10.6%) died. We found a median exacerbation rate of 1.94 per year. Bacterial colonization status was associated with more deaths, exacerbation rate, symptoms and reduced pulmonary function. Pulmonary hypertension was found in 48% of our patients.ConclusionsWe evaluated a large non-cystic fibrosis bronchiectasis population, and provided new epidemiological data on associations between clinical characteristics and deaths and morbidity in these patients.

Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis

Sputum retention is a distressing feature of non-cystic fibrosis bronchiectasis and has been shown to contribute to the vicious cycle of infection seen in this disease. In a previous study we demonstrated that nebulised 7% hypertonic saline was both safe and effective in this patient population. Patients with a clinical diagnosis of non-cystic fibrosis bronchiectasis, confirmed by HRCT, were entered into a randomised single blind cross-over study to evaluate 0.9% sodium chloride (IS) and 7% hypertonic saline (HS). Following a 4 week run in patients received a random order active HS or IS daily for 3 months. A 4 week wash-out phase was included between phases. We report lung function, quality of life, and health care utilisation responses. 32 patients mean age 56.6 years (SD 14.6), 16 male, were recruited of which 28 were randomised and completed the study. Lung function (%change from baseline) improved in HS vs. IS (FEV(1): 15.1, 1.8 p<0.01; FVC: 11.2, 0.7 p<0.01. SGRQ improved significantly from baseline (HS 6.0, IS 1.2; p<0.05). There were reductions in annualised antibiotic usage (HS 2.4, IS 5.4 courses per patient per year), annualised emergency health care utilisation visits were reduced (HS 2.1, IS 4.9 events per patient per year). There were also improvements in sputum viscosity and ease of expectoration (visual analogue scale). Regular use of 7% hypertonic saline improves lung function, quality of life and health care utilisation in non-cystic fibrosis bronchiectasis patients.

A neglected problem of developing countries: Noncystic fibrosis bronchiectasis

BACKGROUND:Bronchiectasis has been defined as the abnormal and permanent dilation of the bronchi. It is still an important problem in many developing countries.AIM:The aim of this study was to identify the chacteristics and underlying etiology of children followed with the diagnosis of noncystic fibrosis bronchiectasis.MATERIALS AND METHODS:Children with bronchiectasis confirmed with high-resolution computed tomography were enrolled into the study. The data of the patients, including symptoms of the disease, age at the onset of symptoms, findings of physical examination, labrotory investigations performed in order to identify the etiology of bronchiectasis, etiology of bronchiectasis if found, radiologic findings and treatment modalities were noted.RESULTS:Sixty-six children between 1 and 17 years were included in the study retrospectively. Forty-four of them were males (66.7%) and 22 (33.3%) were females. The most common presenting symptoms were cough (100%) and sputum expectoration (50%). An underlying etiology was identified in 44 (66.7%) of the study subjects. The four most common underlying causes were found as infections (21.2%), asthma (16.7%), aspiration syndromes and/or gastroesophageal reflux disease (9.1%) and immunodeficiency syndromes (7.6%), respectively.CONCLUSION:Identifying an underlying etiology will have a significant effect on the management of noncystic fibrosis bronchiectasis. Defining the cause of bronchiectasis may also decrease its incidence, progression and complications.