BMJ Paediatr Open. 2023 Dec 20;7(1):e002251. doi: 10.1136/bmjpo-2023-002251.

ABSTRACT

INTRODUCTION: Following lung transplantation, it is unknown how children/adolescents self-assess their performance in everyday activities, importance of these activities, and whether resumption of everyday activities influences self-reported quality of life. The aim was to examine the effect of bilateral lung transplantation on children's/adolescent's perception of engagement in everyday activities over the first 18 months post-transplant.

METHODS: A multiple-single-case-study pre-post test design was conducted at a National Paediatric Lung Transplant Service. Participants were aged under 18 years and were 3 months post first bilateral lung transplant at the time of recruitment. Outcomes were self-reported quality of life (Paediatric Quality of Life Inventory (PedsQL), Transplant Module and engagement in meaningful life roles (Child Occupational Self-Assessment (COSA) Scale) measured at 3 months and 18 months post-transplant from participants, and their parent(s) (PedsQL). Analysis included paired between-time differences and descriptive analysis.

RESULTS: Seven participants were recruited to the trial with a mean age of 13 (SD 4) years and a diagnosis of cystic fibrosis. The total mean scores on PedsQL remained stable between 3 months and 18 months for both participants and parents. The PedsQL transplant module total mean scores of participants significantly increased from 3 months (M=66.58, SD=11.83) to 18 months (M=80.25, SD=11.56), t(5) = 2.91, p=0.03 whereas parents' scores remained stable. COSA responses reported 'big problems' with self-care tasks, family engagement and coping with worries at 3 months. At 18 months, the 'big problem' activities shifted to community engagement and independence.

CONCLUSION: Time influences self-reported quality of life and engagement in meaningful life roles for children/adolescents and their parents 3 months to 18 months post-lung transplant. Our findings highlight the importance of focusing on occupational roles and occupational performance of children and adolescents when designing post-transplant interventions, suggesting a key role for occupational therapy after transplantation.

PMID:38128948 | DOI:10.1136/bmjpo-2023-002251

Am J Med. 2023 Dec 19:S0002-9343(23)00766-0. doi: 10.1016/j.amjmed.2023.12.011. Online ahead of print.

ABSTRACT

OBJECTIVE: Determine whether sleep characteristics are associated with incidence of treated diabetes in postmenopausal individuals.

METHODS: Postmenopausal participants ages 50-79 reported sleep duration, sleep-disordered breathing, and/or insomnia at baseline and again in a subsample 3 years later. The primary outcome was self-reported new diagnosis of diabetes treated with oral drugs or insulin at any time after baseline. Multivariable Cox proportional hazards models were used.

RESULTS: In 135,964 participants followed for 18.1 (±6.3) years, there was a non-linear association between sleep duration and risk of treated diabetes. Participants sleeping ≤5 hours at baseline had a 21% increased risk of diabetes compared to those sleeping 7 hours (adjusted hazard ratio [aHR] 1.21; 95% confidence interval [CI] 1.00,1.47). Those who slept for ≥9 hours had a nonsignificant 6% increased risk of diabetes compared to those sleeping 7 hours (aHR 1.06; 0.97, 1.16). Participants whose sleep duration had declined at 3 years had a 9% [aHR 1.09; 1.02, 1.16] higher risk of diabetes than participants with unchanged sleep duration. Participants who reported increased sleep duration at 3 years had a similar risk of diabetes [HR 1.01; 0.95, 1.08] to those with no sleep duration change. Participants at high risk of sleep-disordered breathing at baseline had a 31% higher risk of diabetes than those without [aHR 1.31; 1.26, 1.37]. No association was found between self-reported insomnia score and diabetes risk.

CONCLUSIONS: Sleep-disordered breathing and short or long sleep duration were associated with higher diabetes risk in a postmenopausal population.

PMID:38128859 | DOI:10.1016/j.amjmed.2023.12.011

J Cyst Fibros. 2023 Dec 20:S1569-1993(23)01733-2. doi: 10.1016/j.jcf.2023.12.009. Online ahead of print.

NO ABSTRACT

PMID:38129256 | DOI:10.1016/j.jcf.2023.12.009

J Cyst Fibros. 2023 Dec 20:S1569-1993(23)01727-7. doi: 10.1016/j.jcf.2023.12.002. Online ahead of print.

ABSTRACT

This is the second in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on establishing and maintaining health. The guidance is produced using an evidence-based framework and with wide stakeholder engagement, including people from the CF community. Authors provided a narrative description of their topic and statements, which were more directive. These statements were reviewed by a Delphi exercise, achieving good levels of agreement from a wide group for all statements. This guidance reinforces the importance of a multi-disciplinary CF team, but also describes developing models of care including virtual consultations. The framework for health is reinforced, including the need for a physically active lifestyle and the strict avoidance of all recreational inhalations, including e-cigarettes. Progress with cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy is reviewed, including emerging adverse events and advice for dose reduction and interruption. This paper contains guidance that is pertinent to all people with CF regardless of age and eligibility for and access to modulator therapy.

PMID:38129255 | DOI:10.1016/j.jcf.2023.12.002

Evolution. 2023 Dec 21:qpad225. doi: 10.1093/evolut/qpad225. Online ahead of print.

ABSTRACT

How does genetic background influence a population's evolutionary response to an environmental change? Which traits are selected for and how quickly does the population adapt? Filipow et al. (2023) address these questions by exploiting the natural genetic variation of Pseudomonas aeruginosa, a bacterium often found in the lungs of cystic fibrosis patients. They find that, while genetic background influences the rate of phenotypic evolution, it does not alter the evolutionary outcome. Their findings contribute to a growing body of work that connects genetic background to future evolvability.

PMID:38126968 | DOI:10.1093/evolut/qpad225

Oncoimmunology. 2023 Nov 9;12(1):2272352. doi: 10.1080/2162402X.2023.2272352. eCollection 2023.

ABSTRACT

Recent clinical trials have compared the use of different chemotherapeutic regimens as "immune induction therapies" to sensitize cancers to immune checkpoint inhibitors (ICI). Cytotoxic drugs reputed to be inducers of immunogenic cell death (ICD) appeared to be particularly efficient for this purpose. A trial published in Nature Medicine by Thibaudin et al. reveals the capacity of oxaliplatin-based chemotherapy to sensitize RAS-mutant unresectable metastatic colorectal cancer to ICIs blocking CTLA-4 and PD-L1.

PMID:38126035 | PMC:PMC10732660 | DOI:10.1080/2162402X.2023.2272352

Breathe (Sheff). 2023 Dec;19(4):230141. doi: 10.1183/20734735.0141-2023. Epub 2023 Dec 19.

ABSTRACT

E-cigarettes are products delivering nicotine via inhalation and are devised to mimic tobacco smoking. While they were initially introduced as a device putatively to aid with smoking cessation, their use is now far broader than that. Use by children is significantly increasing. There is growing evidence of the potential harms of vaping. E-liquids used for e-cigarettes contain a wide range of harmful substances, and the clinical consequences of this are now being increasingly demonstrated, such as the rise in cases of e-cigarette- or vaping-associated lung injury. In addition, early use may result in long-term nicotine addiction. Vaping companies utilise marketing methods that distinctly target young people, and weak legislation in the UK allows them free rein to expose children to vaping. In this review we demonstrate why children must be protected from vaping. We must have stringent legislation to prevent easy access to e-cigarettes, including banning the convenience and affordability disposable vapes provide, and prevent marketing that does not warn about the potential health effects. The Australia approach of prescription or pharmacy only access for smoking cessation should be considered to limit exposure of children and minimise use by nonsmokers.

PMID:38125806 | PMC:PMC10729810 | DOI:10.1183/20734735.0141-2023

Breathe (Sheff). 2023 Dec;19(4):230123. doi: 10.1183/20734735.0123-2023. Epub 2023 Dec 19.

ABSTRACT

The role of the pharmacist has evolved significantly, not least over the last 20 years. It delivers a skilled profession with a vital role in medicines optimisation and the management of patients with a respiratory or sleep disorder. While pharmacists are capable of acting as independent practitioners delivering direct patient care, this article explores their contribution to multidisciplinary teams within asthma, COPD, cystic fibrosis, tuberculosis, interstitial lung disease and sleep medicine. Having identified patient cohorts needing specialist medicines support, notably those with poor medicines adherence or specific medicines-related needs (for example during adolescence, or women who are pregnant or breastfeeding), these pharmacists work within primary, secondary and specialist tertiary care. The aim of this review is to share and inspire innovative models of working to include more pharmacists in respiratory and sleep medicine.

PMID:38125801 | PMC:PMC10729827 | DOI:10.1183/20734735.0123-2023

Biomacromolecules. 2023 Dec 20. doi: 10.1021/acs.biomac.3c01043. Online ahead of print.

ABSTRACT

Airway mucus works as a protective barrier in the human body, as it entraps pathogens that will be later cleared from the airways by ciliary transport or by coughing, thus featuring the rheological properties of a highly stretchable gel. Nonetheless, the study of these physical barrier as well as transport properties remains limited due to the restricted and invasive access to lungs and bronchi to retrieve mucus and to the poor repeatability inherent to native mucus samples. To overcome these limits, we report on a biobased synthetic mucus prepared from snail slime and multibranched thiol cross-linker, which are able to establish disulfide bonds, in analogy with the disulfide bonding of mucins, and therefore build viscoelastoplastic hydrogels. The gel macroscopic properties are tuned by modifying the cross-linker and slime concentrations and can quantitatively match those of native sputum from donors with cystic fibrosis (CF) or non-cystic fibrosis bronchiectasis (NCFB) both in the small- and large-deformation regimes. Heterogeneous regimes were locally found in the mucus model by passive microrheology, in which both diffusive and non-diffusive motion are present, similar to what is observed in sputa. The biobased synthetic approach proposed in the present study thus allows to produce, with commercially available components, a promising model to native respiratory mucus regarding both mechanical and, to a lesser extent, physicochemical aspects.

PMID:38124283 | DOI:10.1021/acs.biomac.3c01043

Arch Dis Child. 2023 Dec 18:archdischild-2023-326221. doi: 10.1136/archdischild-2023-326221. Online ahead of print.

ABSTRACT

OBJECTIVE: Intestinal inflammation with contradictory data on faecal calprotectin (fCP) levels is documented in patients with cystic fibrosis (CF). The aim of this study was to longitudinally evaluate fCP in a cohort of children with CF and their relationship with clinical variables.

DESIGN: Prospective observational study to assess evolution of fCP levels, primary aimed at improving fat absorption. Along 1.5 years of follow-up (November 2016-May 2018) with four study visits pertaining to a pilot study (two of four) and to a clinical trial (two of four), the study outcomes were measured.

SETTING: Six European CF centres in the context of MyCyFAPP Project.

SUBJECTS: Children with CF and pancreatic insufficiency (2-18 years old).

MAIN OUTCOME MEASUREMENTS: fCP levels, pulmonary function (percentage of forced expiratory volume in 1 s (FEV1%)) and coefficient of fat absorption (CFA). Additionally, in the last two visits, gastrointestinal (GI) symptoms were evaluated through the PedsQL-GI Questionnaire. Linear mixed regression models were applied to assess association between fCP and FEV1, CFA and GI symptoms.

RESULTS: Twenty-nine children with CF and pancreatic insufficiency were included. fCP levels were inversely associated with total modified specific PedsQL-GI score (p=0.04) and positively associated with diarrhoea (p=0.03), but not with CFA. Along the four study visits, fCP significantly increased (from 62 to 256 µg/g) and pulmonary function decreased (from 97% to 87%), with a significant inverse association between the two study outcomes (p<0.001).

CONCLUSIONS: In children with CF, fCP levels are inversely associated with pulmonary function and thus the specificity of fCP as a marker of intestinal inflammation in paediatric patients with CF warrants further investigation.

PMID:38123920 | DOI:10.1136/archdischild-2023-326221

Sci Rep. 2023 Dec 20;13(1):22692. doi: 10.1038/s41598-023-50121-4.

ABSTRACT

Cystic fibrosis (CF) is an autosomal recessive disorder characterized by respiratory failure due to a vicious cycle of defective Cystic Fibrosis Transmembrane conductance Regulator (CFTR) function, chronic inflammation and recurrent bacterial and fungal infections. Although the recent introduction of CFTR correctors/potentiators has revolutionized the clinical management of CF patients, resurgence of inflammation and persistence of pathogens still posit a major concern and should be targeted contextually. On the background of a network-based selectivity that allows to target the same enzyme in the host and microbes with different outcomes, we focused on sphingosine-1-phosphate (S1P) lyase (SPL) of the sphingolipid metabolism as a potential candidate to uniquely induce anti-inflammatory and antifungal activities in CF. As a feasibility study, herein we show that interfering with S1P metabolism improved the immune response in a murine model of CF with aspergillosis while preventing germination of Aspergillus fumigatus conidia. In addition, in an early drug discovery process, we purified human and A. fumigatus SPL, characterized their biochemical and structural properties, and performed an in silico screening to identify potential dual species SPL inhibitors. We identified two hits behaving as competitive inhibitors of pathogen and host SPL, thus paving the way for hit-to-lead and translational studies for the development of drug candidates capable of restraining fungal growth and increasing antifungal resistance.

PMID:38123809 | DOI:10.1038/s41598-023-50121-4

J Cyst Fibros. 2023 Dec 19:S1569-1993(23)01730-7. doi: 10.1016/j.jcf.2023.12.005. Online ahead of print.

ABSTRACT

BACKGROUND: Our understanding of the epidemiology of sleep breathing disorders among adults with cystic fibrosis (CF) is limited. Our purpose was to describe the frequency, risk factors and treatment of sleep breathing disorders among adults with CF.

METHODS: This was a retrospective analysis of linked data from laboratory-based diagnostic polysomnography (PSG) undertaken at St. Michael's Hospital (Toronto, Canada) and the Canadian CF Registry. Adults (≥19 years old) with CF that underwent a diagnostic PSG at St. Michael's Hospital between 2002 and 2021 were included. Sleep breathing disorder frequency, risk factors, and treatment were described, using descriptive statistics and logistic regression.

RESULTS: There were 42 patients included (33.3 % women and median age at diagnostic PSG was 34.7 years). Obstructive sleep apnea [OSA] was the most commonly observed sleep breathing disorder (found in 64.3 %), followed by sustained nocturnal hypoxemia (16.7 %), and sleep hypoventilation (9.5 %). Only 41 % of individuals with an elevated total apnea-hypopnea index were receiving positive airway pressure [PAP] therapy. Corticosteroid use (either oral or inhaled) was the only factor with a significant positive association with presence of any sleep breathing disorder (odds ratio 5.00, 95 % confidence interval 1.28-22.78).

CONCLUSIONS: Among adults with CF, OSA occurs more commonly than previously appreciated and the majority of sleep breathing disorders were not being treated with PAP or supplemental oxygen. Management of sleep breathing disorders among adults with CF reflects a potentially important care gap, but further research is needed to determine the health impacts of treating sleep breathing disorders in CF.

PMID:38123381 | DOI:10.1016/j.jcf.2023.12.005

J Allergy Clin Immunol Pract. 2023 Dec 18:S2213-2198(23)01371-5. doi: 10.1016/j.jaip.2023.11.048. Online ahead of print.

ABSTRACT

Severity of eczema at patch application site during epicutaneous immunotherapy could be an independent predictor of subsequent response to oral immunotherapy. The delay between epicutaneous immunotherapy discontinuation and initiation of oral immunotherapy does not appear to affect response to the latter.

PMID:38122864 | DOI:10.1016/j.jaip.2023.11.048

PLoS One. 2023 Dec 20;18(12):e0291910. doi: 10.1371/journal.pone.0291910. eCollection 2023.

ABSTRACT

BACKGROUND: Healthcare-associated acquisition and transmission of nontuberculous mycobacteria (NTM) among people with cystic fibrosis (pwCF) has been described, and remains a concern for both patients and providers. This report describes the design of a prospective observational study utilizing the standardized epidemiologic investigation toolkit for healthcare-associated links in transmission of NTM among pwCF.

METHODS: This is a parallel multi-site study of pwCF who have infections with respiratory NTM isolates and receive healthcare within a common CF Care Center. Participants have a history of one or more NTM positive airway cultures and have been identified as having NTM infections suggestive of a possible outbreak within a single Center, based on NTM isolate genomic analysis. Participants are enrolled in the study over a 3-year period. Primary endpoints are identification of shared healthcare-associated source(s) among pwCF in a Center, identification of healthcare environmental dust and water biofilm NTM isolates that are genetically highly-related to respiratory isolates, and identification of common home of residence watersheds among pwCF infected with clustered isolates. Secondary endpoints include characterization of healthcare-associated transmission and/or acquisition modes and settings as well as description of incidence and prevalence of healthcare-associated environmental NTM species/subspecies by geographical region.

DISCUSSION: We hypothesize that genetically highly-related isolates of NTM among pwCF cared for at the same Center may arise from healthcare sources including patient-to-patient transmission and/or acquisition from health-care environmental dust and/or water biofilms. This study design utilizes a published, standardized, evidence-based epidemiologic toolkit to facilitate confidential, independent healthcare-associated NTM outbreak investigations within CF Care Centers. This study will facilitate real-time, rapid detection and mitigation of healthcare-associated NTM outbreaks to reduce NTM risk, inform infection prevention and control guidelines, and characterize the prevalence and origin of NTM outbreaks from healthcare-associated patient-to-patient transmission and/or environmental acquisition. This study will systematically characterize human disease causing NTM isolates from serial collection of healthcare environmental dust and water biofilms and define the most common healthcare environmental sources harboring NTM biofilms.

TRIAL REGISTRATION: ClinicalTrials.gov NCT05686837.

PMID:38117792 | DOI:10.1371/journal.pone.0291910

Microbiology (Reading). 2023 Dec;169(12). doi: 10.1099/mic.0.001422.

ABSTRACT

Intravenous gallium nitrate therapy is a novel therapeutic strategy deployed to combat chronic Pseudomonas aeruginosa biofilm infections in the lungs of cystic fibrosis (CF) patients by interfering with iron (Fe3+) uptake. The therapy is a source of Ga3+, which competes with Fe3+ for siderophore binding, subsequently disrupting iron metabolism and inhibiting biofilm proliferation in vivo. It was recently demonstrated that the Pseudomonas quinolone signal (PQS) can chelate Fe3+ to assist in bacterial iron uptake. However, it is unknown whether exogenous gallium also targets [Fe(PQS)3] uptake, which, in turn, would extend the mechanism of gallium therapy beyond siderophore competition, potentially supporting use of the therapy against P. aeruginosa mutants deficient in siderophore uptake proteins. To that end, the thermodynamic feasibility of iron-for-gallium cation exchange into [Fe(PQS)3] was evaluated using quantum chemical density functional theory (DFT) modelling and verified experimentally using 1H nuclear magnetic resonance (NMR). We demonstrate here that Ga3+ can strongly bind to three PQS molecules and, furthermore, displace and substitute Fe3+ from the native chelate pocket within PQS complexes, through a Trojan horse mechanism, retaining the key structural features present within the native ferric complex. As such, [Fe(PQS)3] complexes, in addition to ferric-siderophore complexes, represent another target for gallium therapy.

PMID:38117289 | DOI:10.1099/mic.0.001422

Pediatr Pulmonol. 2023 Dec 20. doi: 10.1002/ppul.26797. Online ahead of print.

NO ABSTRACT

PMID:38116856 | DOI:10.1002/ppul.26797

J Assist Reprod Genet. 2023 Dec 20. doi: 10.1007/s10815-023-03004-6. Online ahead of print.

ABSTRACT

PURPOSE: The cystic fibrosis transmembrane conductance regulator (CFTR) is the most common causative gene attributed to congenital obstructive azoospermia (OA). The aim of this study was to conduct an epidemiological survey of congenital OA patients, to screen for CFTR mutations, and to follow their pregnancy outcomes in assisted reproductive technology (ART).

METHODS: This cohort study enrolled congenital OA patients undergoing ART and whole-exome sequencing from January 2018 to September 2023. Semen parameters, sex hormones, and seminal plasma biochemistry were evaluated. CFTR mutations identified in OA patients were analyzed. In addition, the laboratory outcomes, clinical outcomes, and neonatal outcomes were compared between OA patients carrying two CFTR mutations and the others after surgical sperm extraction-intracytoplasmic sperm injection (ICSI) treatment.

RESULTS: A total of 76 patients with congenital OA were enrolled. CFTR mutations were identified in 35 (46.1%) congenital OA patients. A total of 60 CFTR mutation sites of 27 types were identified, and 10 of them were novel. The average frequency was 1.71 (60/35) per person. The most common mutation was c.1210-11T > G (25%, 15/60). After ICSI treatment, there were no statistically significant differences in laboratory outcomes, clinical outcomes, and neonatal outcomes between OA patients carrying two CFTR mutations (n = 25) and other OA patients (n = 51).

CONCLUSION: Apart from the IVS9-5T mutation, the genetic mutation pattern of CFTR in Chinese OA patients is heterogeneous, which is significantly different from that of Caucasians. Although carrying two CFTR mutations or not had no effect on the pregnancy outcomes in OA patients after ICSI, genetic counseling is still recommended for such patients.

PMID:38114870 | DOI:10.1007/s10815-023-03004-6

Am J Vet Res. 2023 Oct 16:1-9. doi: 10.2460/ajvr.23.08.0182. Online ahead of print.

ABSTRACT

OBJECTIVE: Right dorsal colitis causes chronic colic associated with long-term treatment with nonsteroidal antiinflammatory drugs (NSAIDs). This study was designed to determine if NSAIDs could inhibit anion transporters that protect against intestinal mucosal injury in other species.

ANIMALS: 20 healthy horses.

METHODS: The effects of indomethacin (INDO) and firocoxib (FIR), on short-circuit current (Isc) in mucosa from the right dorsal colon (RDC) and right ventral colon (RVC) were measured in Ussing chambers by standard electrophysiological techniques. Immunohistochemical methods were used to detect apoptosis (caspase-3) with these NSAIDs and phenylbutazone (PBZ) and to locate the NKCC1 transporter.

RESULTS: The Isc in RDC and RVC incubated with INDO or FIR was increased almost 3-fold (P < .0001) by prostaglandin E2 (PGE2) through a system inhibited by loop diuretics (P < .0001). Although these findings and anion replacement studies were consistent with anion secretion, the RDC also displayed an Isc response suggestive of a unique transporter apparently absent in RVC or NSAID-free solutions. In RDC, FIR, INDO, and PBZ induced apoptosis in the lower half of crypts. However, significant differences in apoptotic index were recorded in the RDC between NSAID-treated and control tissues (no NSAID).

CLINICAL RELEVANCE: The effects of NSAIDs on Isc were consistent with reduced anion secretion, which could represent the pharmacological equivalent of the transport failure responsible for Cystic Fibrosis (CF) in other species. Failure of anion secretion could interfere with buffering acid from intraluminal fermentation, which could suggest a treatment target for right dorsal colitis.

PMID:38113643 | DOI:10.2460/ajvr.23.08.0182

Am J Respir Crit Care Med. 2023 Dec 19. doi: 10.1164/rccm.202311-2159ED. Online ahead of print.

NO ABSTRACT

PMID:38113403 | DOI:10.1164/rccm.202311-2159ED

Front Pediatr. 2023 Dec 4;11:1322360. doi: 10.3389/fped.2023.1322360. eCollection 2023.

ABSTRACT

BACKGROUND: Currently, there are no guidelines or consensus statements about the usage of inhaled mucoactive drugs in pediatric respiratory disease conditions from an Indian perspective.

OBJECTIVE: To develop a practical consensus document to help pediatricians in clinical decision-making when choosing an appropriate mucoactive drug for the management of specific respiratory disease conditions.

METHODS: A committee of nine experts with significant experience in pediatric respiratory disease conditions and a microbiological expert constituted the panel. An electronic search of the PubMed/MEDLINE, Cochrane Library, Scopus, and Embase databases was undertaken to identify relevant articles. Various combinations of keywords such as inhaled, nebulized, mucoactive, mucolytic, mucokinetic, expectorants, mucoregulators, mucociliary clearance, respiratory disorders, pediatric, cystic fibrosis (CF), non-CF bronchiectasis, acute wheezing, asthma, primary ciliary dyskinesia (PCD), critically ill, mechanical ventilation, tracheomalacia, tracheobronchomalacia, esophageal atresia (EA), tracheoesophageal fistula (TEF), acute bronchiolitis, sputum induction, guideline, and management were used. Twelve questions were drafted for discussion. A roundtable meeting of experts was conducted to arrive at a consensus. The level of evidence and class of recommendation were weighed and graded.

CONCLUSIONS: Inhaled mucoactive drugs (hypertonic saline, dry powder mannitol, and dornase alfa) can enhance mucociliary clearance in children with CF. Experts opined that hypertonic saline could be beneficial in non-CF bronchiectasis, acute bronchiolitis, and PCD. The current state of evidence is inadequate to support the use of inhaled mucoactive drugs in asthma, acute wheezing, tracheomalacia, tracheobronchomalacia, and EA with TEF.

PMID:38111626 | PMC:PMC10725989 | DOI:10.3389/fped.2023.1322360