Microbiol Res. 2022 Jul 30;263:127152. doi: 10.1016/j.micres.2022.127152. Online ahead of print.

ABSTRACT

Antibiotic resistance is a serious health and social problem that will have a substantial impact in the coming years on the world health and economy. Thus, the increasing demand for innovative antibiotics, has prompted many researchers in the medical, microbiological, and biochemical fields to exploit the properties of antimicrobial peptides (AMPs). When properly used, designed, and conveyed, AMPs can really represent a valid alternative to conventional drugs especially in situations that are particularly difficult to treat such as chronic infections found in Cystic Fibrosis (CF) patients. In this review we focused on the applications of AMPs in the specific field of CF, illustrating different types of peptides from natural, naturally modified, synthetic as well as the different strategies used to overcome the barriers, and the physiological conditions in which AMPs must operate.

PMID:35944357 | DOI:10.1016/j.micres.2022.127152

PLoS One. 2022 Aug 9;17(8):e0272091. doi: 10.1371/journal.pone.0272091. eCollection 2022.

ABSTRACT

INTRODUCTION: Cystic fibrosis (CF) is a hereditary autosomal recessive disorder caused by a range of mutations in the CF Transmembrane Conductance Regulator (CFTR) gene. This gene encodes the CFTR protein, which acts as a chloride channel activated by cyclic AMP (cAMP). This meta-analysis aimed to compare the responsiveness of induced pluripotent stem cells (iPSCs) to cAMP analogues to that of commonly used animal models.

METHODS: Databases searched included PubMed, Scopus, and Medline from inception to January 2020. A total of 8 and 3 studies, respectively, for animal models and iPSCs, were analyzed. Studies were extracted for investigating cAMP-stimulated anion transport by measuring the short circuit current (Isc) of chloride channels in different animal models and iPSC systems We utilized an inverse variance heterogeneity model for synthesis.

RESULTS: Our analysis showed considerable heterogeneity in the mean Isc value in both animal models and iPSCs studies (compared to their WT counterparts), and both suffer from variable responsiveness based on the nature of the underlying model. There was no clear advantage of one over the other.

CONCLUSIONS: Studies on both animal and iPSCs models generated considerable heterogeneity. Given the potential of iPSC-derived models to study different diseases, we recommend paying more attention to developing reproducible models of iPSC as it has potential if adequately developed.

PMID:35944004 | DOI:10.1371/journal.pone.0272091

Cochrane Database Syst Rev. 2022 Aug 9;8:CD002768. doi: 10.1002/14651858.CD002768.pub5.

ABSTRACT

BACKGROUND: Physical activity (including exercise) may form an important part of regular care for people with cystic fibrosis (CF). This is an update of a previously published review.

OBJECTIVES: To assess the effects of physical activity interventions on exercise capacity by peak oxygen uptake, lung function by forced expiratory volume in one second (FEV1), health-related quality of life (HRQoL) and further important patient-relevant outcomes in people with cystic fibrosis (CF).

SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. The most recent search was on 3 March 2022. We also searched two ongoing trials registers: clinicaltrials.gov, most recently on 4 March 2022; and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP), most recently on 16 March 2022. SELECTION CRITERIA: We included all randomised controlled trials (RCTs) and quasi-RCTs comparing physical activity interventions of any type and a minimum intervention duration of two weeks with conventional care (no physical activity intervention) in people with CF.

DATA COLLECTION AND ANALYSIS: Two review authors independently selected RCTs for inclusion, assessed methodological quality and extracted data. We assessed the certainty of the evidence using GRADE. MAIN RESULTS: We included 24 parallel RCTs (875 participants). The number of participants in the studies ranged from nine to 117, with a wide range of disease severity. The studies' age demographics varied: in two studies, all participants were adults; in 13 studies, participants were 18 years and younger; in one study, participants were 15 years and older; in one study, participants were 12 years and older; and seven studies included all age ranges. The active training programme lasted up to and including six months in 14 studies, and longer than six months in the remaining 10 studies. Of the 24 included studies, seven implemented a follow-up period (when supervision was withdrawn, but participants were still allowed to exercise) ranging from one to 12 months. Studies employed differing levels of supervision: in 12 studies, training was supervised; in 11 studies, it was partially supervised; and in one study, training was unsupervised. The quality of the included studies varied widely. This Cochrane Review shows that, in studies with an active training programme lasting over six months in people with CF, physical activity probably has a positive effect on exercise capacity when compared to no physical activity (usual care) (mean difference (MD) 1.60, 95% confidence interval (CI) 0.16 to 3.05; 6 RCTs, 348 participants; moderate-certainty evidence). The magnitude of improvement in exercise capacity is interpreted as small, although study results were heterogeneous. Physical activity interventions may have no effect on lung function (forced expiratory volume in one second (FEV1) % predicted) (MD 2.41, 95% CI ‒0.49 to 5.31; 6 RCTs, 367 participants), HRQoL physical functioning (MD 2.19, 95% CI ‒3.42 to 7.80; 4 RCTs, 247 participants) and HRQoL respiratory domain (MD ‒0.05, 95% CI ‒3.61 to 3.51; 4 RCTs, 251 participants) at six months and longer (low-certainty evidence). One study (117 participants) reported no differences between the physical activity and control groups in the number of participants experiencing a pulmonary exacerbation by six months (incidence rate ratio 1.28, 95% CI 0.85 to 1.94) or in the time to first exacerbation over 12 months (hazard ratio 1.34, 95% CI 0.65 to 2.80) (both high-certainty evidence); and no effects of physical activity on diabetic control (after 1 hour: MD ‒0.04 mmol/L, 95% CI ‒1.11 to 1.03; 67 participants; after 2 hours: MD ‒0.44 mmol/L, 95% CI ‒1.43 to 0.55; 81 participants; moderate-certainty evidence). We found no difference between groups in the number of adverse events over six months (odds ratio 6.22, 95% CI 0.72 to 53.40; 2 RCTs, 156 participants; low-certainty evidence). For other time points (up to and including six months and during a follow-up period with no active intervention), the effects of physical activity versus control were similar to those reported for the outcomes above. However, only three out of seven studies adding a follow-up period with no active intervention (ranging between one and 12 months) reported on the primary outcomes of changes in exercise capacity and lung function, and one on HRQoL. These data must be interpreted with caution. Altogether, given the heterogeneity of effects across studies, the wide variation in study quality and lack of information on clinically meaningful changes for several outcome measures, we consider the overall certainty of evidence on the effects of physical activity interventions on exercise capacity, lung function and HRQoL to be low to moderate.

AUTHORS' CONCLUSIONS: Physical activity interventions for six months and longer likely improve exercise capacity when compared to no training (moderate-certainty evidence). Current evidence shows little or no effect on lung function and HRQoL (low-certainty evidence). Over recent decades, physical activity has gained increasing interest and is already part of multidisciplinary care offered to most people with CF. Adverse effects of physical activity appear rare and there is no reason to actively discourage regular physical activity and exercise. The benefits of including physical activity in an individual's regular care may be influenced by the type and duration of the activity programme as well as individual preferences for and barriers to physical activity. Further high-quality and sufficiently-sized studies are needed to comprehensively assess the benefits of physical activity and exercise in people with CF, particularly in the new era of CF medicine.

PMID:35943025 | DOI:10.1002/14651858.CD002768.pub5

Microbiol Spectr. 2022 Aug 8:e0171422. doi: 10.1128/spectrum.01714-22. Online ahead of print.

ABSTRACT

Infections due to Mycobacterium abscessus are a major cause of mortality and morbidity in cystic fibrosis (CF) patients. Furthermore, M. abscessus has been suspected to be involved in person-to-person transmissions. In 2016, dominant global clonal complexes (DCCs) that occur worldwide among CF patients have been described. To elucidate the epidemiological situation of M. abscessus among CF patients in Germany and to put these data into a global context, we performed whole-genome sequencing of a set of 154 M. abscessus isolates from 123 German patients treated in 14 CF centers. We used MTBseq pipeline to identify clusters of closely related isolates and correlate those with global findings. Genotypic drug susceptibility for macrolides and aminoglycosides was assessed by characterization of the erm(41), rrl, and rrs genes. By this approach, we could identify representatives of all major DCCs (Absc 1, Absc 2, and Mass 1) in our cohort. Intrapersonal isolates showed higher genetic relatedness than interpersonal isolates (median 3 SNPs versus 16 SNPs; P < 0.001). We further identified four clusters with German patients from same centers clustering with less than 25 SNPs distance (range 3 to 18 SNPs) but did not find any hint for in-hospital person-to-person transmission. This is the largest study investigating phylogenetic relations of M. abscessus isolates in Germany. We identified representatives of all reported DCCs but evidence for nosocomial transmission remained inconclusive. Thus, the occurrence of genetically closely related isolates of M. abscessus has to be interpreted with care, as a direct interhuman transmission cannot be directly deduced. IMPORTANCE Mycobacterium abscessus is a major respiratory pathogen in cystic fibrosis (CF) patients. Recently it has been shown that dominant global clonal complexes (DCCs) have spread worldwide among CF patients. This study investigated the epidemiological situation of M. abscessus among CF patients in Germany by performing whole-genome sequencing (WGS) of a set of 154 M. abscessus from 123 German patients treated in 14 CF centers. This is the largest study investigating the phylogenetic relationship of M. abscessus CF isolates in Germany.

PMID:35938728 | DOI:10.1128/spectrum.01714-22

Front Cell Infect Microbiol. 2022 Jul 22;12:943346. doi: 10.3389/fcimb.2022.943346. eCollection 2022.

ABSTRACT

Chronic respiratory infection (CRI) with Pseudomonas aeruginosa (Pa) presents many unique challenges that complicate treatment. One notable challenge is the hypermutator phenotype which is present in up to 60% of sampled CRI patient isolates. Hypermutation can be caused by deactivating mutations in DNA mismatch repair (MMR) genes including mutS, mutL, and uvrD. In vitro and in vivo studies have demonstrated hypermutator strains to be less virulent than wild-type Pa. However, patients colonized with hypermutators display poorer lung function and a higher incidence of treatment failure. Hypermutation and MMR-deficiency create increased genetic diversity and population heterogeneity due to elevated mutation rates. MMR-deficient strains demonstrate higher rates of mucoidy, a hallmark virulence determinant of Pa during CRI in cystic fibrosis patients. The mucoid phenotype results from simple sequence repeat mutations in the mucA gene made in the absence of functional MMR. Mutations in Pa are further increased in the absence of MMR, leading to microcolony biofilm formation, further lineage diversification, and population heterogeneity which enhance bacterial persistence and host immune evasion. Hypermutation facilitates the adaptation to the lung microenvironment, enabling survival among nutritional complexity and microaerobic or anaerobic conditions. Mutations in key acute-to-chronic virulence "switch" genes, such as retS, bfmS, and ampR, are also catalyzed by hypermutation. Consequently, strong positive selection for many loss-of-function pathoadaptive mutations is seen in hypermutators and enriched in genes such as lasR. This results in the characteristic loss of Pa acute infection virulence factors, including quorum sensing, flagellar motility, and type III secretion. Further study of the role of hypermutation on Pa chronic infection is needed to better inform treatment regimens against CRI with hypermutator strains.

PMID:35937684 | PMC:PMC9355025 | DOI:10.3389/fcimb.2022.943346

Front Pediatr. 2022 Jul 22;10:909925. doi: 10.3389/fped.2022.909925. eCollection 2022.

ABSTRACT

Congenital exocrine pancreatic insufficiency is a rare condition. In a vast majority of patients, exocrine dysfunction occurs as part of a multisystemic disease, the most prevalent being cystic fibrosis and Shwachman-Bodian-Diamond syndrome. Recent fundamental studies have increased our understanding of the pathophysiology of these diseases. Exocrine pancreatic dysfunction should be considered in children with failure to thrive and fatty stools. Treatment is mainly supportive and consists of pancreatic enzyme replacement and liposoluble vitamins supplementation.

PMID:35935370 | PMC:PMC9354839 | DOI:10.3389/fped.2022.909925

J Cyst Fibros. 2022 Aug 4:S1569-1993(22)00636-1. doi: 10.1016/j.jcf.2022.07.016. Online ahead of print.

NO ABSTRACT

PMID:35934642 | DOI:10.1016/j.jcf.2022.07.016

J Cyst Fibros. 2022 Aug 4:S1569-1993(22)00637-3. doi: 10.1016/j.jcf.2022.07.015. Online ahead of print.

ABSTRACT

RATIONAL: People with cystic fibrosis carrying residual function (RF) mutations are considered to have a mild disease course. This may influence caregivers and patients on how intensive the treatments should be.

OBJECTIVES: Characterize disease severity of patients carrying RF mutations, using the European CF Society Patient Registry (ECFSPR) data.

METHODS: Demographic, clinical characteristics, lung function and death probability of patients carrying at least one RF mutation were analyzed and compared to patients homozygous to minimal function mutations (MF).

MAIN RESULTS: Of the 44,594 eligible patients (median age 19.5 years, IQR 10-29.8), 6,636 (14.6%) carried RF mutations, and 37,958 (85.1%) MF mutations. Patients carrying RF mutations were older, diagnosed at a later age, had lower sweat chloride at diagnosis and better FEV1pp at each age group. However, their FEV1pp declined with age and rates of chronic Pseudomonas aeruginosa increased with age. A significant number of patients with RF had FEV1pp similar to patients with MF at each age group. 4.5% of RF patients were treated with oxygen and 2.61% had a lung transplant. With increasing age, 26.6% of RF patients were treated with pancreatic enzymes associated with a more severe lung disease. RF patients had shortened life spans, with mortality starting around the age of 20 years.

CONCLUSIONS: Patients carrying an RF mutations experience a decline of pulmonary function with age, leading to life-shortening. Standard of care therapies and augmenting CFTR function may improve their survival and quality of life.

PMID:35934641 | DOI:10.1016/j.jcf.2022.07.015

Pediatr Neurol. 2022 Jul 5;135:1-3. doi: 10.1016/j.pediatrneurol.2022.06.021. Online ahead of print.

ABSTRACT

BACKGROUND: Children presenting with complex febrile seizures (FS) have an increased risk of developing epilepsy. This study aimed to investigate the occurrence of complex seizures in children presenting with FS and those with both convulsions associated with mild gastroenteritis (CwG) and fever.

METHODS: Children admitted to our Pediatric Emergency Department between January 2017 and April 2019 with seizures were enrolled in this cross-sectional study. Patients were grouped according to the etiology as FS and febrile CwG. FS classification criteria of simple FS and complex FS was applied to both groups to allow a comparison between them. Prevalence ratios (PRs) of complex seizures, estimated through a log binomial model, were used to compare the occurrence of complex seizures between the two groups, using the FS group as reference category.

RESULTS: A total of 294 patients were enrolled: 231 with FS and 63 with febrile CwG. Complex seizures occurred in 31 patients with FS (13.4%) and 21 patients (33.3%) with febrile CwG. The PR of complex seizures was 2.48 (95% confidence interval, 1.54 to 4.01).

CONCLUSIONS: Children with febrile CwG showed a higher rate of complex seizures when compared with those with FS.

PMID:35933805 | DOI:10.1016/j.pediatrneurol.2022.06.021

Pediatr Pulmonol. 2022 Aug 7. doi: 10.1002/ppul.26103. Online ahead of print.

ABSTRACT

Carriers of the cystic fibrosis transmembrane conductance regulator (CFTR) gene ("carriers") have been found to have an increased risk of persistent asthma. However, it is unclear at what level of CFTR function this risk exists and whether it is modified by asthmogens, such as air pollution. We conducted a retrospective cohort study of children born in California between July 2007 and December 2013, linking CFTR genotype data from the California newborn screening program to Medicaid claims records through March 17, 2020 to identify asthma cases, and to air pollution data from CalEnviroScreen 3.0 to identify levels of particulate matter with diameter 2.5 microns or smaller (PM2.5 ). Log-binomial regression models for asthma risk were fitted, adjusting for race/ethnicity and sex. Compared to population controls, carriers had higher risk of asthma (adjusted risk ratio (aRR)=1.29, 95% confidence interval (CI): 0.98, 1.69; p<0.1). Other non-CF-causing variants on the second allele did not appear to further increase risk. Genotypes with the greatest asthma risk were F508del with an intron 10 T7 or (TG)11T5 in trans (aRR=1.52, 95% CI: 1.10, 2.12). This association was higher among children living in areas at or above (aRR=1.80) versus below (aRR=1.37) the current national air quality standard for PM2.5 , though this difference was not statistically significant (pinteraction >0.2). These results suggest carriers with CFTR functional levels between 25% and 45% of wildtype are at increased risk of asthma. Knowledge of CFTR genotype in asthmatics may be important to open new CFTR-related treatment options for these patients. This article is protected by copyright. All rights reserved.

PMID:35933722 | DOI:10.1002/ppul.26103

Cell Death Dis. 2022 Aug 6;13(8):684. doi: 10.1038/s41419-022-05124-w.

ABSTRACT

Pattern recognition receptors (PRRs) and interferons (IFNs) serve as essential antiviral defense against SARS-CoV-2, the causative agent of the COVID-19 pandemic. Type III IFNs (IFN-λ) exhibit cell-type specific and long-lasting functions in auto-inflammation, tumorigenesis, and antiviral defense. Here, we identify the deubiquitinating enzyme USP22 as central regulator of basal IFN-λ secretion and SARS-CoV-2 infections in human intestinal epithelial cells (hIECs). USP22-deficient hIECs strongly upregulate genes involved in IFN signaling and viral defense, including numerous IFN-stimulated genes (ISGs), with increased secretion of IFN-λ and enhanced STAT1 signaling, even in the absence of exogenous IFNs or viral infection. Interestingly, USP22 controls basal and 2'3'-cGAMP-induced STING activation and loss of STING reversed STAT activation and ISG and IFN-λ expression. Intriguingly, USP22-deficient hIECs are protected against SARS-CoV-2 infection, viral replication, and the formation of de novo infectious particles, in a STING-dependent manner. These findings reveal USP22 as central host regulator of STING and type III IFN signaling, with important implications for SARS-CoV-2 infection and antiviral defense.

PMID:35933402 | DOI:10.1038/s41419-022-05124-w

Chem Biol Interact. 2022 Aug 3:110048. doi: 10.1016/j.cbi.2022.110048. Online ahead of print.

ABSTRACT

Mucus gel constitutes of heavily cross-linked mucin fibers forming a viscoelastic, dense porous network that coats all the exposed epithelia not covered with the skin. The layer provides protection to the underlying gastrointestinal, respiratory, and female reproductive tracts, in addition to the organs such as the surface of eye by trapping the pathogens, irritants, environmental fine particles, and potentially hazardous foreign matter. However, this property of mucus gel poses a substantial challenge for realizing the localized and sustained drug delivery across the mucosal surfaces. The mucus permeating particles that spare the protective properties of mucus gel improve the therapeutic potency of the drugs aimed at the management of diseases, including sexually transmitted infections, lung cancer, irritable bowel disease, degenerative eye diseases and infections, and cystic fibrosis. As such, the mucoadhesive materials conjugated with drug molecules display a prolonged retention time in the mucosal gel that imparts a sustained release of the deliberated drug molecules across the mucosa. The contemporarily developed mucus penetrating materials for drug delivery applications comprise of a finer size, appreciable hydrophilicity, and a neutral surface to escape the entrapment within the cross-inked mucus fibers. Pertaining to the mucus secretion as a first line of defence in respiratory tract in response to the invading physical, chemical, and biological pathogens, the development of mucus penetrating materials hold promise as a stalwart approach for revolutionizing the respiratory drug delivery paradigm. The present review provides an epigrammatic collation of the mucus penetrating/mucoadhesive materials for achieving a controlled/sustained release of the cargo pharmaceutics and drug molecules across the respiratory mucus barrier.

PMID:35932910 | DOI:10.1016/j.cbi.2022.110048

Int J Pediatr Otorhinolaryngol. 2022 Jul 30;161:111249. doi: 10.1016/j.ijporl.2022.111249. Online ahead of print.

ABSTRACT

OBJECTIVE: This analysis investigates any potential differences in pulmonary function test (PFT) outcomes among pediatric patients with cystic fibrosis (CF) receiving both medical management (MM) and functional endoscopic sinus surgery (FESS) versus MM alone for CF exacerbation.

STUDY DESIGN: Prospective cohort.

SETTING: Pediatric tertiary care facility.

METHODS: The data was prospectively collected from July 2011 to March 2020. Diagnosis of CF and age ≤ to 18 were required. All patients were hospitalized and treated for CF exacerbations with both FESS with MM and MM alone at variable time intervals, although the order of initial treatment received differed. Two-way ANOVA with repeated measures were used to determine the effect of receiving FESS with MM versus MM alone on PFT outcomes over time (during admission, at discharge, at 3 months, at 6 months, and at 12 months).

RESULTS: 13 pediatric patients, 7 of which had FESS with MM initially and 6 who had MM alone initially, and 20 events of both FESS and MM were included for analysis. For PFT outcomes, there was no statistically significant two-way interaction between treatment type and time following treatment, p = 0.492. The main effect of treatment did not show a statistically significant difference in FEV1 between treatment types, p = 0.737. There was no statistically significant association between treatment type and time between hospital readmission in months, p = 0.111.

CONCLUSION: There was no significant difference between PFT outcomes in pediatric patients hospitalized for CF exacerbation treated with MM with or without FESS at any time interval.

PMID:35932623 | DOI:10.1016/j.ijporl.2022.111249

J Coll Physicians Surg Pak. 2022 Aug;32(8):1042-1046. doi: 10.29271/jcpsp.2022.08.1042.

ABSTRACT

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This study aims to determine the genotypic and phenotypic spectrum of the CFTR gene mutations reported in the literature for Pakistani-origin CF patients. Databases were searched for such studies from 1947-2019 for sample size, method of diagnosis, and CFTR gene mutations. The authors identified 12 studies reporting 33 CFTR gene mutations, both intronic as well as exonic in Pakistani origin patients. The most widely tested mutation was D508 with a frequency of 17%-60%. No hotspot zone was identified and not all reported mutations were causing disease. There is a need to identify common mutations in the Pakistani population to develop population-specific CFTR mutations panel. This will enable the researchers to perform phenotype-genotype correlation studies to improve the CF detection rate. Key Words: Cystic fibrosis, Pakistan, Mutations, CFTR.

PMID:35932130 | DOI:10.29271/jcpsp.2022.08.1042

Folia Microbiol (Praha). 2022 Aug 5. doi: 10.1007/s12223-022-00990-5. Online ahead of print.

ABSTRACT

Pseudomonas aeruginosa (PA) is considered the first causal agent of morbidity and mortality in people with cystic fibrosis (CF) disease. Multi-resistant strains have emerged due to prolonged treatment with specific antibiotics, so new alternatives have been sought for their control. In this context, there is a renewed interest in therapies based on bacteriophages (phages) supported by several studies suggesting that therapy based on lytic phages and biofilm degraders may be promising for the treatment of lung infections in CF patients. However, there is little clinical data about phage studies in CF and the effectiveness and safety in patients with this disease has not been clear. Therefore, studies regarding on phage characterization, selection, and evaluation in vitro and in vivo models will provide reliable information for designing effective cocktails, either using mixed phages or in combination with antibiotics, making a great progress in clinical research. Hence, this review focuses on the most relevant and recent findings on the activity of lytic phages against PA strains isolated from CF patients and hospital environments, and discusses perspectives on the use of phage therapy on the treatment of PA in CF patients.

PMID:35931928 | DOI:10.1007/s12223-022-00990-5

Pediatr Pulmonol. 2022 Aug 5. doi: 10.1002/ppul.26101. Online ahead of print.

ABSTRACT

BACKGROUND: A cystic fibrosis-specific cognitive-behavioral therapy intervention (CF-CBT) was developed in partnership with the CF community to advance preventive mental health care. Multidisciplinary providers across 3 centers were trained to deliver CF-CBT for this pilot assessing feasibility/acceptability and preliminary effectiveness of an integrated model of care.

METHODS: The 8-session CF-CBT was delivered to 14 adults with mild depression and/or anxiety symptoms in-person and via audio telehealth. Assessment of attrition, engagement, homework completion, treatment satisfaction, and treatment fidelity informed feasibility/acceptability assessment. Mental health outcomes included depression, anxiety, quality of life (CFQ-R), perceived stress and coping. Preliminary effectiveness was evaluated with Cohen's d metric of effect sizes (ES) of pre-post mean change scores.

RESULTS: A total of 108 sessions were conducted; 13 adults completed the intervention; 1 discontinued early. Engagement, homework completion, and treatment acceptability were highly rated (mean=30; SD=2, range 27-32 on a 32-point scale). Fidelity scores ranged from 85.7% to 93.6%. Large ES changes reflected improvements in depressive symptoms (-0.83), CFQ-R (Vitality scale; 1.11), and Relaxation Skills (0.93); moderate ES for CFQ-R Role Functioning (0.63), Awareness of Tension (0.62), Coping Confidence (0.70) and CF-Specific Coping (0.55); and small ES for anxiety symptoms (-0.22), perceived stress (-0.25), Behavioral Activation (0.29), and several CFQ-R domains, including Emotional Functioning (0.29). Two CFQ-R subscales decreased (Body Image, Eating Concerns).

CONCLUSIONS: Results indicated feasibility and acceptability of CF-CBT and its integration into team-based CF care with promising effectiveness, especially for depression. A multi-center RCT of CF-CBT will further examine effectiveness of a CF-specific integrated care model. This article is protected by copyright. All rights reserved.

PMID:35931665 | DOI:10.1002/ppul.26101

Pediatr Pulmonol. 2022 Aug 5. doi: 10.1002/ppul.26102. Online ahead of print.

ABSTRACT

INTRODUCTION: There is evidence for increased risk of eating disorders in individuals with diet-treated chronic illnesses, however, data in patients with Cystic Fibrosis (CF) is less clear. No studies have evaluated avoidant/restrictive food intake disorder (ARFID) in the CF population. We investigated the prevalence of eating disorders, including ARFID, in adolescents and young adults with CF.

METHODS: Patients with CF aged 14-35 years were recruited to complete three validated surveys: (1)Eating Disorder Examination Questionnaire (EDE-Q), (2)Nine-Item Avoidant/Restrictive Food Intake Disorder Scale (NIAS), and (3)Cystic Fibrosis Questionnaire-Revised (CFQ-R). Univariate linear regression analysis identified baseline risk factors associated with these survey scores. Variables with univariate p<0.20 were considered for inclusion in a multivariable linear regression model. Backwards stepwise linear regression was used to identify the final model.

RESULTS: A total of 52 patients enrolled. The prevalence of a positive screen on the EDE-Q was 9.6%, and on the NIAS was 13.5%. The CFQ-R eating and weight subscales were associated with scores on the EDE-Q, and CFQ-R eating subscale and being dF508 homozygous were correlated with the NIAS total score.

DISCUSSION: A clinically significant number of participants screened positive for eating disorders on the EDE-Q and NIAS. Scores on the eating and weight scales of the CFQ-R were associated with the scores on these surveys. Further work is needed to better understand the optimal way to use such tools to screen and treat for eating disorders in individuals with CF. This article is protected by copyright. All rights reserved.

PMID:35931664 | DOI:10.1002/ppul.26102

Med Clin (Barc). 2022 Aug 2:S0025-7753(22)00316-5. doi: 10.1016/j.medcli.2022.04.017. Online ahead of print.

ABSTRACT

INTRODUCTION: Nebulized hypertonic saline (HS) improves quality of life and reduces exacerbations in patients with cystic fibrosis. It is unknown if it would offer the same benefits in other hypersecretory pathologies.

METHODS: Retrospective observational study. Patients who passed the tolerance test and started HS 5.8% with one year of follow-up were included. Clinical and healthcare parameters were quantified in the year before and after the start of treatment.

RESULTS: 101 patients, 60.4% women, 65years (95%CI: 62.4-67.9): 82 (81.2%) bronchiectasis, 6 (5.9%) COPD, 2 (2%) asthma, 1 (1%) ILD, and 10 (9.9%) other causes. There was a reduction in bronchorrhea (91.1% vs 75.2%), recurrent infections (57.4% vs 22.8%) and cycles of antibiotic therapy (1.54 vs 0.55), as well as an increase in FEV1 (1881ml vs. 1942ml) and a decrease in visits to primary care (2.94 vs. 1.1), emergencies (0.36 vs. 0.17) and hospitalizations (0.17 vs. 0.17). 06). 73 patients (72.3%) presented an adequate tolerance.

CONCLUSION: Nebulization of HS 5.8% in patients with bronchial hypersecretion is safe and has a remarkable clinical and healthcare impact.

PMID:35931569 | DOI:10.1016/j.medcli.2022.04.017

Microbiol Res. 2022 Jul 22;263:127140. doi: 10.1016/j.micres.2022.127140. Online ahead of print.

ABSTRACT

Bacteria belonging to the genus Achromobacter are widely distributed in natural environments and have been recognized as emerging pathogens for their contribution to a wide range of human infections. In particular, patients with cystic fibrosis (CF) are the subjects most frequently colonized by Achromobacter spp., which can cause persistent infections in their respiratory tract. Although many clinical aspects and pathogenic mechanisms still remain to be elucidated, Achromobacter spp. have been a source of expanding interest in recent years. This review examines the current literature regarding Achromobacter spp. role in CF, focusing on taxonomy, prevalence in CF lung infections, genomic characteristics, and adaptation strategies including modifications of metabolism and virulence, acquisition of antibiotic resistance, exchange of mobile genetic elements and development of hypermutation.

PMID:35931003 | DOI:10.1016/j.micres.2022.127140

ACS Med Chem Lett. 2022 Jun 30;13(7):1014-1015. doi: 10.1021/acsmedchemlett.2c00288. eCollection 2022 Jul 14.

NO ABSTRACT

PMID:35928853 | PMC:PMC9345374 | DOI:10.1021/acsmedchemlett.2c00288