Solid State Nucl Magn Reson. 2024 Oct 25;134:101975. doi: 10.1016/j.ssnmr.2024.101975. Online ahead of print.

ABSTRACT

People with the genetic disease cystic fibrosis (CF) often have chronic airway infections and produce airway secretions called sputum. A better understanding of sputum composition is desired in order to track changes in response to CF therapeutics and to improve laboratory models for the study of CF airway infections. The glycosylated protein mucin is a primary component. Along with extracellular DNA, mucin gives rise to the high viscoelasticity of sputum, which inhibits airway clearance and is thought to promote chronic airway infections in people with CF. Past studies of sputum composition identified additional biomolecular components of sputum including other proteins, both glycosylated and not glycosylated, free amino acids, and lipids. Typically, studies of sputum, as well as other complex biological materials, have focused on soluble or isolated components. Solid-state NMR is not limited to the study of soluble components. Instead, it can provide molecular-level information about insoluble biological samples. Additionally, solid-state NMR can provide information about sample composition without requiring any processing of the sample, eliminating the possibility of misestimating certain components due to insolubility or potential sample loss in isolation steps. In this study, we used both 13C and 31P CPMAS to investigate the total composition of sputum samples obtained from six people with CF. We compared these spectra to those of commercially available mucin, DNA, and phospholipid samples. Lastly, we performed complementary biochemical analyses to identify specific proteins present in the sputum samples. Overall, our findings provide insight into the composition of unprocessed sputum samples from people with CF, which can be used as a benchmark for future investigations of CF and infections in the airways of people with CF. Further, this study provides opportunities to expand the solid-state NMR approach to include dynamic nuclear polarization (DNP) to obtain high-resolution information of sputum and similar biological samples that are not feasible to isotopically enrich.

PMID:39489104 | DOI:10.1016/j.ssnmr.2024.101975

Rev Mal Respir. 2024 Nov 1:S0761-8425(24)00298-5. doi: 10.1016/j.rmr.2024.10.002. Online ahead of print.

ABSTRACT

Chronic obstructive pulmonary disease (COPD), commonly defined as irreversible airflow limitation, is associated with specific morphological changes involving all three parts of the lung, namely the bronchi, parenchyma and pulmonary vessels. In vivo imaging, with its ability to describe the different types of lung alterations and their regional distribution, helps to elucidate the relationship between lung structure and respiratory function. High-resolution computed tomography (CT) of the lung is the imaging modality best suited to assessing the pathological changes associated with airflow obstruction occurring in COPD. Over the last few decades, numerous studies have demonstrated the role of CT as a morphological and functional method conducive to the phenotyping of COPD patients. This review proposes to examine the data on CT imaging of COPD with a critical approach to recent data, and to determine the extent to which CT could be integrated into care or clinical research on patients with this/these disease(s).

PMID:39488460 | DOI:10.1016/j.rmr.2024.10.002

J Cyst Fibros. 2024 Oct 31:S1569-1993(24)01804-6. doi: 10.1016/j.jcf.2024.10.011. Online ahead of print.

ABSTRACT

BACKGROUND: The cystic fibrosis (CF) regimen is time-consuming and burdensome leading to barriers to self-management. This mixed-methods study developed the Daily Care Check-in (DCC) that is specific to the barriers faced by people with CF (PWCF) and evaluated its validity.

METHODS: Qualitative methods were used to identify barriers to self-management and develop items, with "think aloud" cognitive interviews conducted to refine the items. A multisite, cross-sectional study was conducted to test the internal consistency, test-retest reliability, and validity of the DCC scores, comparing them to objective medication adherence (composite medication possession ratio (cMPR)) and psychosocial measures (self-efficacy, medication beliefs, executive functioning, depressive and anxiety symptoms, treatment burden, and treatment complexity).

RESULTS: The DCC (18 items) includes two scales: Occurrence (score range 0-18) and Interference (score range 0-90). 405 participants completed the DCC, 344 (85 %) completed the survey, and 365 (90 %) had a cMPR calculated. On average, 6.8 barriers were reported (SD = 4.2 Occurrence Scale), and the Interference Scale had a mean score of 18.4 (SD = 14.0). Reliability was acceptable to good. cMPR was negatively correlated with the DCC (rho=-0.26, Occurrence and rho = -0.31, Interference, p-values<0.0001). A priori hypotheses between the DCC and the other measures were supported and demonstrated construct validity.

CONCLUSIONS: This study provides evidence supporting the validity of the DCC for assessing the presence and impact of barriers to CF self-management, including medication adherence. Formal screening of self-management barriers (e.g., using the DCC) should be considered to facilitate conversations with the care team and identify tailored interventions to support CF self-management.

PMID:39487080 | DOI:10.1016/j.jcf.2024.10.011

Stem Cells Transl Med. 2024 Nov 1:szae084. doi: 10.1093/stcltm/szae084. Online ahead of print.

ABSTRACT

Cystic fibrosis transmembrane conductance regulator (CFTR) gene editing and transplantation of CFTR-gene corrected airway basal cells has the potential to cure CF lung disease. Although mouse studies established that cell transplantation was feasible, the engraftment rate was typically low and frequently less than the estimated therapeutic threshold. The purpose of this study was to identify genes and culture conditions that regulate the therapeutic potential of human bronchial basal cells. Factor 3 (F3, Tissue Factor 1) is a component of the extrinsic coagulation pathway and activates a cascade of proteases that convert fibrinogen to fibrin. Based on reports that F3 was necessary for human basal cell survival and adhesion in vitro, the present study evaluated F3 as a potential determinant of therapeutic fitness. The gene expression profile of F3 mRNA-positive human bronchial basal cells was evaluated by scRNAseq and the impact of the lung environment on F3 expression was modeled by varying in vitro culture conditions. F3 necessity for adhesion, proliferation, and differentiation was determined by CRISPR/Cas9 knockout (KO) of the F3 gene. Finally, the impact of F3 manipulation on engraftment was determined by orthotropic co-transplantation of wild-type and F3-KO cells into the airways of immunocompromised mice. In contrast with the hypothesis that F3 increases the therapeutic fitness of basal cells, F3 expression decreased engraftment. These studies guide the ongoing development of cellular therapies by showing that in vitro assessments may not predict therapeutic potential and that the lung milieu influences the functional properties of transplanted bronchial basal cells.

PMID:39485996 | DOI:10.1093/stcltm/szae084

Epigenetics. 2024 Dec;19(1):2421631. doi: 10.1080/15592294.2024.2421631. Epub 2024 Nov 1.

ABSTRACT

To investigate the clinical value of methylation levels of peripheral blood DDR1 and CtBP genes in evaluating the severity of acute pancreatitis (AP). Collect 90 blood samples from AP patients and healthy volunteers, and test methylation levels of SPINK1, STAT3, KIT, CFTR, DDR1, CtBP1, CtBP2 genes by bisulfite amplicon sequencing (BSAS). The gene methylation and clinical predictors of SAP early prediction were determined by univariate and multifactorial analysis, respectively. (1) The methylation level of CtBP1 gene and MCTSI score were independent predictors of SAP, with AUC values of 0.723 and 0.8895, respectively. (2) The methylation levels of DDR1, CtBP2, CFTR and SPINK1 genes were statistically significant in HC group vs AP group, HC group vs MAP group, and HC group vs SAP group. (3) The combined detection of CtBP1 gene methylation level and MCTSI score predicted the sensitivity, specificity, AUC, and 95%CI of SAP were 0.750, 0.957, 0.902, and 0.816-0.989, respectively. (1) The methylation level of CtBP1 gene in peripheral blood is an independent risk factor for predicting SAP and is a potentially good predictor of SAP, and the combined testing with the MCTSI score does not further significantly improve the early predictive value for SAP. (2) The methylation levels of DDR1, SPINK1, CtBP2, and CFTR genes were potential indicators for recognizing AP.

PMID:39485950 | DOI:10.1080/15592294.2024.2421631

Ann Am Thorac Soc. 2024 Nov;21(11):1483-1484. doi: 10.1513/AnnalsATS.202407-694ED.

NO ABSTRACT

PMID:39485168 | DOI:10.1513/AnnalsATS.202407-694ED

bioRxiv [Preprint]. 2024 Oct 21:2024.10.21.619475. doi: 10.1101/2024.10.21.619475.

ABSTRACT

Cystic fibrosis (CF) is a multi-organ genetic disorder that affects more than 100,000 individuals worldwide. Chronic respiratory infections are among the hallmark complications associated with CF lung disease, and these infections are often due to polymicrobial communities that colonize the airways of persons with CF (pwCF). Such infections are a significant cause of morbidity and mortality, with studies indicating that pwCF who are co-infected with more than one organism experience more frequent pulmonary exacerbations, leading to a faster decline in lung function. Previous work established an in vitro CF-relevant polymicrobial community model composed of P. aeruginosa , S. aureus , S. sanguinis , and P. melaninogenica . P. melaninogenica cannot survive in monoculture in this model. In this study, we leverage this model to investigate the interactions between P. aeruginosa and P. melaninogenica , allowing us to understand the mechanisms by which the two microbes interact to support the growth of P. melaninogenica specifically in the context of the polymicrobial community. We demonstrate a cross-feeding mechanism whereby P. melaninogenica metabolizes mucin into short-chain fatty acids that are in turn utilized by P. aeruginosa and converted into metabolites (succinate, acetate) that are cross-fed to P. melaninogenica , supporting the survival of this anaerobe in the CF lung-relevant model.

IMPORTANCE: Polymicrobial interactions impact disease outcomes in pwCF who suffer from chronic respiratory infections. Previous work established a CF-relevant polymicrobial community model that allows experimental probing of these microbial interactions to achieve a better understanding of the factors that govern the mechanisms by which CF lung microbes influence each other. In this study, we investigate the interaction between P. aeruginosa and P. melaninogenica , which are two highly prevalent and abundant CF lung microbes. We uncover a cross-feeding mechanism that requires the metabolism of mucin by P. melaninogenica to generate short-chain fatty acids that are cross-fed to P. aeruginosa , and into metabolized into metabolites which are then cross-fed back to P. melaninogenica to support the growth of this anaerobe.

PMID:39484496 | PMC:PMC11527032 | DOI:10.1101/2024.10.21.619475

New Microbes New Infect. 2024 Oct 10;62:101504. doi: 10.1016/j.nmni.2024.101504. eCollection 2024 Dec.

NO ABSTRACT

PMID:39483702 | PMC:PMC11525161 | DOI:10.1016/j.nmni.2024.101504

J Cyst Fibros. 2024 Oct 31:S1569-1993(24)01799-5. doi: 10.1016/j.jcf.2024.10.007. Online ahead of print.

ABSTRACT

This case report presents a 14-month-old boy with a history of cystic fibrosis (CF) carrier status, diagnosed following a positive newborn screening for CF (CF-NBS), who developed symptoms suggestive of Pseudo-Bartter syndrome (PBS). Despite initial evaluations not meeting CF diagnostic criteria, subsequent investigations revealed an intermediate sweat chloride concentration, a second CFTR mutation, and CFTR dysfunction through rectal organoid morphology analysis (ROMA) consistent with CFTR-related disorder (CFTR-RD). This case raises important considerations regarding the diagnosis and management of CFTR-RD. PBS can be considered as a rare presentation of CFTR-RD and can occur in children with sweat chloride below the CF range. Functional testing of CFTR by ROMA enabled a more accurate diagnosis. Despite the negative work-up after CF-NBS, this infant developed CFTR-RD, but this should not be considered as a screen failure. Follow-up of children with CFTR-RD at a CF centre is preferred, because of the risk of developing CF.

PMID:39482189 | DOI:10.1016/j.jcf.2024.10.007

Lancet Respir Med. 2024 Nov;12(11):e65. doi: 10.1016/S2213-2600(24)00306-0.

NO ABSTRACT

PMID:39481918 | DOI:10.1016/S2213-2600(24)00306-0

Respir Med. 2024 Oct 29:107840. doi: 10.1016/j.rmed.2024.107840. Online ahead of print.

ABSTRACT

BACKGROUND: People with cystic fibrosis (pwCF) have barriers to physical activity including exercise intolerance and fatigue. The advent of small molecule cystic fibrosis transmembrane conductance regulator (CFTR) modulators have shown great clinical improvements in pwCF; however, the effect of CFTR modulators on exercise perception and participation is unknown. The purpose of this study was to investigate whether the administration of CFTR modulators changed the perception and participation in sport and exercise in pwCF.

METHODS: A survey-based, retrospective cohort study was conducted including individuals aged 18 and above.

RESULTS: Eighty-three participants were recruited with a mean age of 30.0 ± 10.5 years. The majority (82%) of participants were taking CFTR modulators. Participants in the modulator group rated enjoyment of exercise and importance of exercise higher than the non-modulator group and were more likely to exercise as a means to socialize. Participants in the modulator group reported lack of time as the most frequent barrier to sport and exercise whereas fatigue was most reported in the non-modulator group. Twenty-eight percent of the modulator group participated in team/structured sports, whereas no participant engaged in team and structured sports in the non-modulator group.

CONCLUSIONS: Adults with CF who used CFTR modulators rated exercise more favorably and engaged in more team sport activities compared to pwCF who did not use CFTR modulators. The most frequent barrier to exercise in pwCF who used modulators might no longer be CF-related. More research is needed to confirm whether CFTR modulator administration resulted in increased overall physical activity.

PMID:39481659 | DOI:10.1016/j.rmed.2024.107840

Physiol Meas. 2024 Oct 31. doi: 10.1088/1361-6579/ad8da4. Online ahead of print.

ABSTRACT

Objective.Sulfur hexafluoride (SF6) multiple-breath washout (MBW) assesses ventilation inhomogeneity, as an early marker of obstructive respiratory diseases. Primary outcomes are customarily washout-derived, and it is unclear whether the preceding SF6-washin can provide similar estimates. We aimed to assess comparability of primary SF6-MBW outcomes between washin and washout phases of infant SF6-MBW data measured with the WBreath (ndd Medizintechnik AG, Zurich, Switzerland) and Spiroware (Eco Medics AG, Duernten, Switzerland) MBW-setups, respectively. &#xD;Approach.We assessed mean relative differences in lung clearance index (LCI) and functional residual capacity (FRC) between the washin and washout of existing SF6-MBW data from healthy infants and infants with cystic fibrosis (CF). We assessed whether these differences exceeded the mean relative within-test between-trial differences of washout-derived outcomes, which can be attributed to natural variability. We also explored non-physiological factors using a pediatric lung simulator. &#xD;Main results.LCI and FRC from washin and washout were not comparable, for both setups. The mean difference (SD) in LCI between washin and washout was 2.3(10.8)% for WBreath and -9.7(8.0)% for Spiroware, while in FRC it was -4.7(7.8)% for WBreath and -2.3(9.7)% for Spiroware. These differences exceeded the within-test between-trial differences in washout-derived outcomes. Outcomes from washin and washout were also not comparable in a pediatric lung simulator.&#xD;Significance.Outcomes of the washin and washout were not comparable due to an interplay of physiological and non-physiological factors, and cannot be used interchangeably.

PMID:39481237 | DOI:10.1088/1361-6579/ad8da4

Radiography (Lond). 2024 Oct 30;31(1):6-11. doi: 10.1016/j.radi.2024.10.016. Online ahead of print.

ABSTRACT

INTRODUCTION: Children and adolescents have the right to participate in decisions about their health, including during radiological examinations. This study explores their participation experiences in this context.

METHODS: This qualitative field study examines the importance of active participation from a Child-Centered Care perspective. Fostering active participation requires supportive structures that recognize each child as a unique social actor. Data was collected through observations and semi-structured interviews with 10 children and adolescents diagnosed with cystic fibrosis undergoing High Resolution Computed Tomography (CT) scans. Thematic analysis was performed on the transcribed data to identify central themes and patterns.

RESULTS: Parental presence and humor during CT scans helped reduce anxiety among participants. Key factors influencing participation included examination duration and pain, with many expressing a desire for greater involvement, especially during longer, more painful procedures. Few children reported experiencing active participation in hospital settings, particularly during CT scans. Younger and more expressive participants tended to have more opportunities for involvement. While most desired active participation during hospital visits and CT scans, they showed less interest in making treatment decisions.

CONCLUSION: The radiographer's affirming and humorous approach is essential, as are considerations of children and adolescents' preferences regarding parental presence, examination duration, and pain management. Participation levels vary, and limited opportunities can undermine their rights. Children and adolescents express a strong desire for active participation in hospital and radiological settings but often feel insecure about making treatment decisions.

IMPLICATIONS FOR PRACTICE: This study highlights critical issues related to children and adolescents' participation in radiological examinations, offering valuable insights for healthcare professionals to enhance participation, which is a fundamental right and crucial aspect of their care.

PMID:39481182 | DOI:10.1016/j.radi.2024.10.016

Dokl Biochem Biophys. 2024 Oct 31. doi: 10.1134/S1607672924701151. Online ahead of print.

ABSTRACT

The article presents the results of studies of the drug Tigerase® (inhalation solution manufactured by JSC GENERIUM, Russia), conducted to obtain evidence of its similarity (comparability) to the reference drug Pulmozyme® (inhalation solution, manufactured by Hoffmann-La Roche Ltd., Switzerland). Both drugs contain human recombinant deoxyribonuclease I (dornase alfa) as an active substance and are intended for the treatment of cystic fibrosis with pulmonary manifestations (mucoviscidosis). The enzymatic activity of dornase alfa, contained in the studied drugs, was investigated in vitro and ex vivo on samples of purulent sputum of patients. The pharmacokinetic parameters of the drugs in the blood serum, bronchi, and lungs, as well as the main physiological parameters (body weight and temperature, the state of the cardiovascular, respiratory, excretory systems, hematological and biochemical blood parameters, pathomorphological changes in internal organs (including the state of the cornea), and mortality rates) were investigated in comparative studies of subchronic toxicity in juvenile and mature rats with 28-day inhalation at doses of 0.2 mg/kg for mature animals and 0.26 mg/kg for juvenile animals (the dose was 6 times higher than the dose recommended for clinical use). The results of the studies allow us to conclude that the drugs are comparable in enzymatic, mucolytic (secretolytic) DNase activity, safety profile and main pharmacokinetic parameters.

PMID:39480637 | DOI:10.1134/S1607672924701151

Ann Am Thorac Soc. 2024 Oct 31. doi: 10.1513/AnnalsATS.202405-451RL. Online ahead of print.

NO ABSTRACT

PMID:39480171 | DOI:10.1513/AnnalsATS.202405-451RL

Infect Immun. 2024 Oct 31:e0031624. doi: 10.1128/iai.00316-24. Online ahead of print.

ABSTRACT

The Burkholderia cepacia complex contains opportunistic pathogens that cause chronic infections and inflammation in the lungs of people with cystic fibrosis. Two closely related species within this complex are Burkholderia cenocepacia and the recently classified Burkholderia orbicola. B. cenocepacia and B. orbicola encode a type VI secretion system and the effector TecA, which is detected by the pyrin/caspase-1 inflammasome, and triggers macrophage inflammatory death. We previously showed that the pyrin inflammasome was dispensable for lung inflammation in mice infected with B. orbicola AU1054, indicating this species activates an alternative pathway of macrophage inflammatory death. Notably, B. cenocepacia strains J2315 and K56-2 can damage macrophage phagosomes, and K56-2 triggers activation of the caspase-11 inflammasome, which detects cytosolic lipopolysaccharide. Here, we investigated inflammatory cell death in pyrin- (Mefv-/-) or caspase-1/caspase-11- (Casp1/11-/-) deficient mouse macrophages infected with B. cenocepacia J2315 or K56-2 or B. orbicola AU1054 or PC184. Macrophage inflammatory death was measured by cleavage of gasdermin D protein, the release of cytokines IL-1α and IL-1β, and plasma membrane rupture. We found that J2315 and K56-2 are detected by the caspase-11 inflammasome in Mefv-/- macrophages, resulting in IL-1β release. By contrast, inflammasome activation was not detected in Mefv-/- macrophages infected with AU1054 or PC184. Instead, AU1054 triggered an alternative macrophage inflammatory death pathway that required TecA and resulted in plasma membrane rupture and IL-1α release. Structural modeling of TecA orthologs in B. cenocepacia and B. orbicola suggested that amino acid changes in the latter may underlie its ability to trigger a non-inflammasome macrophage death pathway.

PMID:39480100 | DOI:10.1128/iai.00316-24

Orphanet J Rare Dis. 2024 Oct 30;19(1):405. doi: 10.1186/s13023-024-03392-7.

ABSTRACT

BACKGROUND: The Brazilian Policy for Comprehensive Care for People with Rare Diseases was implemented in 2014; however, national epidemiological data on rare diseases (RDs) are scarce and mainly focused on specific disorders. To address this gap, University Hospitals, Reference Services for Neonatal Screening, and Reference Services for Rare Diseases, all of which are public health institutions, established the Brazilian Rare Diseases Network (RARAS) in 2020. The objective of this study was to perform a comprehensive nationwide epidemiological investigation of individuals with RDs in Brazil. This retrospective survey collected data from patients receiving care in 34 healthcare facilities affiliated with RARAS in 2018 and 2019.

RESULTS: The survey included 12,530 participants with a median age of 15.0 years, with women representing 50.5% of the cohort. Classification according to skin color demonstrated that 5044 (47.4%) participants were admixed. Most had a confirmed diagnosis (63.2%), with a predominance of phenylketonuria (PKU), cystic fibrosis (CF), and acromegaly. Common clinical manifestations included global developmental delay and seizures. The average duration of the diagnostic odyssey was 5.4 years (± 7.9 years). Among the confirmed diagnoses, 52.2% were etiological (biochemical: 42.5%; molecular: 30.9%), while 47.8% were clinical. Prenatal diagnoses accounted for 1.2%. Familial recurrence and consanguinity rates were 21.6% and 6.4%, respectively. Mainstay treatments included drug therapy (55.0%) and rehabilitation (15.6%). The Public Health System funded most diagnoses (84.2%) and treatments (86.7%). Hospitalizations were reported in 44.5% of cases, and the mortality rate was 1.5%, primarily due to motor neuron disease and CF.

CONCLUSION: This study marks a pioneering national-level data collection effort for rare diseases in Brazil, offering novel insights to advance the understanding, management, and resource allocation for RDs. It unveils an average diagnostic odyssey of 5.4 years and a higher prevalence of PKU and CF, possibly associated with the specialized services network, which included newborn screening services.

PMID:39478612 | DOI:10.1186/s13023-024-03392-7

BMC Pulm Med. 2024 Oct 30;24(1):543. doi: 10.1186/s12890-024-03149-9.

ABSTRACT

BACKGROUND: The rare monogenic syndrome Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED) leads to multisystemic autoimmunity with possible lung involvement. Autoimmune pneumonitis is a rare manifestation, with bronchiectasis being the most frequent radiologic pattern, and may lead to fatal outcome. The Sardinian population in Italy has a high incidence of APECED, although no case of lung manifestation has been reported yet in this cohort. This is the case of a Sardinian APECED patient referred to a bronchiectasis clinic. Our aim is to raise awareness and screen these patients earlier for pulmonary involvement and to initiate multidisciplinary treatment for better outcome.

CASE PRESENTATION: A 49-year-old female native of Sardinia from consanguineous parents was diagnosed with APECED in childhood and was referred to our bronchiectasis clinic in March 2023. In addition to typical APECED features, she reported recurrent respiratory infections since childhood, chronic purulent sputum and a hospitalization for pneumonia. She came to our attention with a recent isolation of P. aeruginosa on sputum culture and diffuse cylindrical and varicoid bronchiectasis on her first CT scan. She underwent aetiologic screening for bronchiectasis with no evidence of another cause of disease. Lung treatment was optimized according to bronchiectasis guidelines, and during follow-up the patient developed methicillin-resistant Staphylococcus aureus (MRSA) infection and M. intracellulare pulmonary disease. The patient was offered P. aeruginosa eradication treatment with intravenous antibiotics and initiation of antimycobacterial therapy.

CONCLUSION: This is the first documented lung involvement case of APECED in a Sardinian patient, and the first patient reported to enter a bronchiectasis program. The patient was prescribed lung imaging late in time when bronchiectasis complications were already present. Our case report highlights the need for early pulmonary screening and multidisciplinary management in patients with APECED.

PMID:39478519 | DOI:10.1186/s12890-024-03149-9

Dig Liver Dis. 2024 Oct 29:S1590-8658(24)01046-6. doi: 10.1016/j.dld.2024.10.002. Online ahead of print.

ABSTRACT

BACKGROUND AND AIMS: Foreign body ingestion (FBI) in children is a critical health concern. This study aimed to describe the epidemiology of FBI in children in Italy.

METHODS: We retrospectively enrolled children <18 years admitted for FBI from January 2015 to December 2020. Data were collected across 21 hospitals with dedicated pediatric endoscopy services and normalized by the population of the corresponding municipalities.

RESULTS: A total of 5,771 FBI cases were analyzed. FBI incidents showed consistent time trends across age groups, with most events occurring at home and being witnessed (94.7 %). Children <6 years accounted for 74.3 % of cases. Comorbidities were present in 5.3 % of cases, primarily neurologic/psychiatric disorders in older children (6-17 years). Blunt objects accounted for 65.5 % of ingestions. Young males commonly ingested button batteries, while females showed higher rates of ingesting hair products and jewelry. Most children were discharged (60 %) or observed briefly (75 % of total admissions), with endoscopic removal performed in 24 % of cases.

CONCLUSIONS: Rates of FBI have remained stable over the years, including during the COVID-19 pandemic. FBI predominantly occurs in domestic settings among healthy young children, particularly those ≤5 years old. These findings emphasize the need for preventive measures to reduce the impact of FBI among children.

PMID:39477708 | DOI:10.1016/j.dld.2024.10.002

Eur Respir Rev. 2024 Oct 30;33(174):240087. doi: 10.1183/16000617.0087-2024. Print 2024 Oct.

ABSTRACT

Bronchiectasis is a chronic lung condition which is characterised by recurrent chest infections, chronic sputum production and cough, and limited exercise tolerance. While bronchiectasis may be caused by various aetiologies, these features are shared by most patients with bronchiectasis regardless of the cause. This review consolidates the existing evidence on patient-managed interventions for adults with bronchiectasis, while also outlining areas for future research. Airway clearance techniques and hyperosmolar agents are key components of the bronchiectasis management and consistently recommended for clinical implementation. Questions around their prescription, such as optimal sequence of delivery, are still to be answered. Pulmonary rehabilitation and exercise are also recommended for patients with bronchiectasis. Relatively strong evidence underpins this recommendation during a clinically stable stage of the disease, although the role of pulmonary rehabilitation following an exacerbation is still unclear. Additionally, self-management programmes feature prominently in bronchiectasis treatment, yet the lack of consensus regarding their definition and outcomes presents hurdles to establishing a cohesive evidence base. Moreover, cough, a cardinal symptom of bronchiectasis, warrants closer examination. Although managing cough in bronchiectasis may initially appear risky, further research is necessary to ascertain whether strategies employed in other respiratory conditions can be safely and effectively adapted to bronchiectasis, particularly through identifying patient responder populations and criteria where cough may not enhance airway clearance efficacy and its control is needed. Overall, there is a growing recognition of the importance of patient-managed interventions in the bronchiectasis management. Efforts to improve research methodologies and increase research funding are needed to further advance our understanding of these interventions, and their role in optimising patient care and outcomes.

PMID:39477356 | DOI:10.1183/16000617.0087-2024