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1.
Respiration ; 101(4): 367-375, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-34875659

RESUMO

BACKGROUND: Nontuberculous mycobacteria (NTM) are increasingly identified in industrialized countries, and their role as pathogens is more frequently recognized. The relative prevalence of NTM strains shows an important geographical variability. Thus, establishing the local relative prevalence of NTM strains is relevant and useful for clinicians. METHODS: Retrospective analysis (2015-2020) of a comprehensive database was conducted including all results of cultures for mycobacteria in a University Hospital (Geneva, Switzerland), covering a population of approximately 500,000 inhabitants. All NTM culture-positive patients were included in the analyses. Patients' characteristics, NTM strains, and time to culture positivity were reported. RESULTS: Among 38,065 samples analyzed during the study period, 411 were culture-positive for NTM, representing 236 strains, and 231 episodes of care which occurred in 222 patients. Patients in whom NTM were identified were predominantly female (55%), with a median age of 62 years, and a low BMI (median: 22.6 kg/m2). The Mycobacterium avium complex (MAC) was the most frequently identified group (37% of strains) followed by Mycobacterium gordonae (25%) and Mycobacterium xenopi (12%) among the slowly growing mycobacteria (SGM), while the Mycobacterium chelonae/abscessus group (11%) were the most frequently identified rapidly growing mycobacteria (RGM). Only 19% of all patients were treated, mostly for pulmonary infections: the MAC was the most frequently treated NTM (n = 19, 43% of cases in patients treated) followed by RGM (n = 15, 34%) and M. xenopi (n = 6, 14%). Among those treated, 23% were immunosuppressed, 12% had pulmonary comorbidities, and 5% systemic comorbidities. Cultures became positive after a median of 41 days (IQR: 23; 68) for SGM and 28 days (14; 35) for RGM. CONCLUSIONS: In Western Switzerland, M. avium and M. gordonae were the most prevalent NTM identified. Positive cultures for NTM led to a specific treatment in 19% of subjects. Patients with a positive culture for NTM were mostly female, with a median age of 62 years, a low BMI, and a low prevalence of immunosuppression or associated severe comorbidities.


Assuntos
Infecções por Mycobacterium não Tuberculosas , Mycobacterium xenopi , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Infecções por Mycobacterium não Tuberculosas/epidemiologia , Infecções por Mycobacterium não Tuberculosas/microbiologia , Complexo Mycobacterium avium , Micobactérias não Tuberculosas , Estudos Retrospectivos
2.
Rev Med Suisse ; 18(764-5): 64-68, 2022 Jan 19.
Artigo em Francês | MEDLINE | ID: mdl-35048583

RESUMO

Among the new therapeutic developments in pulmonology during 2021, we have identified three topics of interest. A new biotherapy is now available for the management of severe uncontrolled non-Th2 asthma. In the field of pulmonary arterial hypertension, upfront triple therapy at the time of diagnosis is associated with a survival benefit in high-risk patients. Riociguat is a therapeutic option for patients that remain at intermediate risk despite treatment with iPDE5. Sotatercept, a promising new class of drug for treatment of group 1 PAH will soon be available. Finally, the use of transbronchial cryobiopsies as a valid alternative to surgical lung biopsy for the diagnosis of diffuse interstitial lung diseases will also be discussed in this review.


Parmi les nouveautés thérapeutiques en pneumologie au cours de l'année 2021, nous aborderons trois sujets. Une nouvelle biothérapie est désormais disponible pour la prise en charge de l'asthme sévère non contrôlé non T-Helper 2. Concernant l'hypertension artérielle pulmonaire (HTAP), une triple thérapie d'emblée au moment du diagnostic est associée à un bénéfice sur la survie chez les patients à haut risque. Le riociguat est une option thérapeutique lors d'HTAP restant à risque intermédiaire malgré un traitement par inhibiteur de la phosphodiestérase de type 5, et le sotatercept vise une nouvelle cible thérapeutique prometteuse pour l'HTAP du groupe 1. Enfin, la place des cryobiopsies transbronchiques comme alternative valable à la biopsie chirurgicale pour le diagnostic des pneumopathies interstitielles diffuses selon des recommandations récentes se précise.


Assuntos
Doenças Pulmonares Intersticiais , Pneumologia , Biópsia , Humanos , Pulmão
3.
Respiration ; 100(9): 909-917, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34130277

RESUMO

BACKGROUND: During the first wave of the SARS-CoV-2 pandemic in Switzerland, confinement was imposed to limit transmission and protect vulnerable persons. These measures may have had a negative impact on perceived quality of care and symptoms in patients with chronic disorders. OBJECTIVES: To determine whether patients under long-term home noninvasive ventilation (LTHNIV) for chronic respiratory failure (CRF) were negatively affected by the 56-day confinement (March-April 2020). METHODS: A questionnaire-based survey exploring mood disturbances (HAD), symptom scores related to NIV (S3-NIV), and perception of health-care providers during confinement was sent to all patients under LTHNIV followed up by our center. Symptom scores and data obtained by ventilator software were compared between confinement and the 56 days prior to confinement. RESULTS: Of a total of 100 eligible patients, 66 were included (median age: 66 years [IQR: 53-74]): 35 (53%) with restrictive lung disorders, 20 (30%) with OHS or SRBD, and 11 (17%) with COPD or overlap syndrome. Prevalence of anxiety (n = 7; 11%) and depressive (n = 2; 3%) disorders was remarkably low. Symptom scores were slightly higher during confinement although this difference was not clinically relevant. Technical data regarding ventilation, including compliance, did not change. Patients complained of isolation and lack of social contact. They felt supported by their relatives and caregivers but complained of the lack of regular contact and information by health-care professionals. CONCLUSIONS: Patients under LTHNIV for CRF showed a remarkable resilience during the SARS-CoV-2 confinement period. Comments provided may be helpful for managing similar future health-care crises.


Assuntos
COVID-19 , Controle de Doenças Transmissíveis , Serviços de Assistência Domiciliar/normas , Ventilação não Invasiva , Insuficiência Respiratória , Idoso , COVID-19/epidemiologia , COVID-19/prevenção & controle , COVID-19/psicologia , Doença Crônica , Controle de Doenças Transmissíveis/métodos , Controle de Doenças Transmissíveis/estatística & dados numéricos , Feminino , Necessidades e Demandas de Serviços de Saúde , Humanos , Assistência de Longa Duração/métodos , Masculino , Transtornos do Humor/epidemiologia , Transtornos do Humor/fisiopatologia , Ventilação não Invasiva/métodos , Ventilação não Invasiva/estatística & dados numéricos , Pesquisa Qualitativa , Qualidade da Assistência à Saúde/estatística & dados numéricos , Insuficiência Respiratória/epidemiologia , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/psicologia , Insuficiência Respiratória/terapia , SARS-CoV-2 , Apoio Social , Suíça/epidemiologia , Avaliação de Sintomas/métodos , Avaliação de Sintomas/estatística & dados numéricos
4.
Rev Med Suisse ; 17(759): 1962-1966, 2021 Nov 17.
Artigo em Francês | MEDLINE | ID: mdl-34787969

RESUMO

Ultrasound of the diaphragm is an emerging technique that is performed at the bedside and allows assessment of diaphragm function in a variety of settings. Ultrasound is widely available, can be repeated if necessary, and is non-irradiating. First developed in intensive care, mainly for weaning from mechanical ventilation, its use is now extending to pulmonology. Different measurements are described such as diaphragmatic excursion, diaphragmatic thickness and diaphragmatic thickening fraction. The latter helps to diagnose dysfunction and is correlated with pulmonary hyperinflation. The main use in pulmonology is for the respiratory evaluation of patients with neuromuscular diseases, for the search of isolated diaphragmatic impairment and for patients with COPD. Numerous studies are in progress to better determine the role of diaphragmatic ultrasound.


La diffusion de l'accès à l'échographie a permis le développement de cette technique en pneumologie avec, plus récemment, l'évaluation du diaphragme. Plusieurs mesures sont décrites comme l'excursion, l'épaisseur et la fraction d'épaississement du diaphragme. Cela permet d'identifier la présence d'une dysfonction diaphragmatique, d'estimer l'activité et la capacité de réserve diaphragmatique. Cet outil non invasif, non irradiant et dynamique s'intègre de plus en plus à la prise en charge du patient. Des études ont permis de montrer l'existence d'une bonne corrélation entre l'échographie et les épreuves fonctionnelles respiratoires. D'autres sont en cours pour déterminer plus précisément l'impact de l'échographie diaphragmatique dans différentes prises en charge.


Assuntos
Diafragma , Respiração Artificial , Cuidados Críticos , Diafragma/diagnóstico por imagem , Humanos , Estudos Prospectivos , Ultrassonografia
5.
Rev Med Suisse ; 17(723): 209-213, 2021 Jan 27.
Artigo em Francês | MEDLINE | ID: mdl-33507663

RESUMO

In this review of the recent medical literature, we have identified 4 topics of interest for the readers of Revue Médicale Suisse. Use of antifibrotic drugs in interstitial lung diseases will soon be extended to a phenotype labeled « progressive fibrosing interstitial lung disease ¼ (PF-ILD). While awaiting for evidence-based treatment, consensual recommendations for a treatment algorithm in pulmonary sarcoidosis has been published. New guidance for non-invasive ventilation in COPD and obesity-hypoventilation syndrome are available in Switzerland and are in line with international recommendations. New treatments targeting CFTR protein activity have become available and could become a therapeutic option for up to 85% of cystic fibrosis patients in Switzerland.


Dans cette revue de la littérature médicale récente, nous avons identifié quatre sujets d'intérêts pour les lecteurs de la Revue Médicale Suisse. L'utilisation de médicaments antifibrotiques dans les maladies pulmonaires interstitielles sera bientôt étendue à un phénotype étiqueté « maladie pulmonaire interstitielle fibrosante progressive ¼. Des recommandations consensuelles pour un algorithme de traitement dans la sarcoïdose pulmonaire ont été publiées. De nouvelles directives pour la ventilation non invasive dans la BPCO et le syndrome d'obésité-hypoventilation sont disponibles en Suisse et sont conformes aux recommandations internationales. De nouveaux traitements ciblant l'activité protéique CFTR (Cystic Fibrosic Transmembrane Conductance Regulator) sont disponibles et pourraient devenir une option thérapeutique pour 85 % des patients atteints de mucoviscidose en Suisse.


Assuntos
Doenças Pulmonares Intersticiais , Fibrose Pulmonar , Progressão da Doença , Humanos , Fenótipo , Suíça
6.
Rev Med Suisse ; 16(698): 1229-1235, 2020 Jun 17.
Artigo em Francês | MEDLINE | ID: mdl-32558451

RESUMO

Cystic Fibrosis is a genetic disorder resulting in the absence or dysfunction of the CFTR protein, a chloride channel present on the surface of epithelia, particularly respiratory. Until recently, treatments only concerned the consequences of the disease. But a new type of molecules called «â€…modulators ¼, is already available to some patients and targets the origin of the disease. «â€…Modulators ¼ are divided into «â€…potentiators ¼, which improve the transport of chloride by the CFTR protein, and «â€…correctors ¼, increasing the amount of CFTR proteins. An oral triple therapy combining a potentiator and two correctors has just been approved in the USA and will treat 85 % of patients. The clinical benefit of «â€…modulators ¼ is remarkable, and these drugs are revolutionizing the treatment of Cystic Fibrosis.


La mucoviscidose est une maladie génétique entraînant une absence ou des dysfonctions de la protéine Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), un canal chlore présent à la surface des épithélia, notamment respiratoire. Jusqu'à récemment, les traitements ne concernaient que les conséquences de la maladie. Mais un nouveau type de molécules appelées «â€…modulateurs ¼ est déjà à la disposition de certains patients et cible l'origine de la maladie. Les «â€…modulateurs ¼ sont divisés en «â€…potentiateurs ¼, permettant d'améliorer le transport du chlore par la protéine CFTR, et en «â€…correcteurs ¼, augmentant la quantité de protéines CFTR. Une trithérapie orale combinant un potentiateur et deux correcteurs vient d'être approuvée aux États-Unis et permettra de traiter 85 % des patients. Le bénéfice clinique des «â€…modulateurs ¼ est remarquable et ces médicaments bouleversent le traitement de la mucoviscidose.


Assuntos
Fibrose Cística/terapia , Fibrose Cística/metabolismo , Regulador de Condutância Transmembrana em Fibrose Cística/genética , Regulador de Condutância Transmembrana em Fibrose Cística/metabolismo , Humanos , Mutação
7.
Rev Med Suisse ; 15(N° 632-633): 96-100, 2019 Jan 09.
Artigo em Francês | MEDLINE | ID: mdl-30629379

RESUMO

In this review of novel therapies in pulmonary disorders in 2018, we cover 3 different entities. In GINA stage 1 and 2 asthma, new strategies allow a more individualized treatment. In more severe asthma, there is an increasing interest in biotherapies, with dupilumab, an anti-IL-4 receptor, completing the already available panel which includes anti-IgE, anti-IL-5 and anti-IL-5 receptor antibodies. In cystic fibrosis, a better understanding of the pathogenesis and the genetics of the disease is leading to new treatments acting directly on the function of the CFTR (Cystic fibrosis transmembrane conductance regulator), which, when used in combination, show very promising results. Finally, nocturnal positive pressure treatment (either CPAP or non invasive ventilation : NIV) is used more and more frequently. Recent studies have clarified therapeutic algorithms for the most frequent indications for NIV : COPD and obesity-hypoventilation.


Trois thématiques seront abordées dans cette revue annuelle 2018. Dans l'asthme léger, une approche plus personnalisée des patients en paliers 1 et 2 GINA est préconisée, alors que dans l'asthme plus sévère, la place des biothérapies progresse avec un antirécepteur de l'IL-4 (dupilumab) qui vient compléter la gamme des biothérapies disponibles en Suisse (anti-IgE, anti-IL-5 et antirécepteur de l'IL-5). Une compréhension plus approfondie de la pathogénie et de la génétique de la mucoviscidose ouvre la voie à de nouveaux traitements agissant directement sur le fonctionnement du CFTR qui, en combinaison, montrent des résultats très prometteurs pour certains génotypes. Enfin, l'assistance respiratoire nocturne (pression positive continue ou ventilation non invasive) est de plus en plus largement utilisée. Les algorithmes de prise en charge dans la BPCO et dans le syndrome obésité hypoventilation alvéolaire, indications les plus fréquentes, se clarifient.


Assuntos
Asma , Fibrose Cística , Ventilação não Invasiva , Pneumologia , Asma/terapia , Pressão Positiva Contínua nas Vias Aéreas , Fibrose Cística/terapia , Humanos , Pneumologia/tendências
8.
Artigo em Inglês | MEDLINE | ID: mdl-29437612

RESUMO

Aspergillus fumigatus is a ubiquitous opportunistic pathogen. This fungus can acquire resistance to azole antifungals due to mutations in the azole target (cyp51A). Recently, cyp51A mutations typical for environmental azole resistance acquisition (for example, TR34/L98H) have been reported. These mutations can also be found in isolates recovered from patients. Environmental azole resistance acquisition has been reported on several continents. Here we describe, for the first time, the occurrence of azole-resistant A. fumigatus isolates of environmental origin in Switzerland with cyp51A mutations, and we show that these isolates can also be recovered from a few patients. While the TR34/L98H mutation was dominant, a single azole-resistant isolate exhibited a cyp51A mutation (G54R) that was reported only for clinical isolates. In conclusion, our study demonstrates that azole resistance with an environmental signature is present in environments and patients of Swiss origin and that mutations believed to be unique to clinical settings are now also observed in the environment.


Assuntos
Antifúngicos/farmacologia , Aspergillus fumigatus/efeitos dos fármacos , Aspergillus fumigatus/genética , Azóis/farmacologia , Sistema Enzimático do Citocromo P-450/genética , Farmacorresistência Fúngica/genética , Proteínas Fúngicas/genética , Testes de Sensibilidade Microbiana , Suíça
9.
Rev Med Suisse ; 14(627): 2079-2083, 2018 Nov 14.
Artigo em Francês | MEDLINE | ID: mdl-30427602

RESUMO

The diagnosis of interstitial lung disease (ILD) is challenging and relies on a multidisciplinary discussion involving clinical, radiological and sometimes histological features. Bronchoscopic lung cryobiopsies have emerged as a new minimally invasive method of lung sampling and an alternative to surgical lung biopsies. A good diagnostic performance and excellent safety profile make it an interesting and worthful procedure which could decrease the number of patients without proper diagnosis and treatment. There is a need for further studies to standardize the technique in expert centers and to establish its role in the diagnostic work-up of ILD.


Le diagnostic des pneumopathies interstitielles (PI) est complexe et repose sur l'analyse d'éléments cliniques, radiologiques et parfois histologiques dans le cadre d'une discussion multidisciplinaire. Pour l'obtention de biopsies pulmonaires, les cryobiopsies transbronchiques constituent une nouvelle méthode minimalement invasive, alternative aux biopsies chirurgicales. Leur très bonne performance diagnostique et leur profil de sécurité favorable expliquent l'intérêt grandissant pour cette technique qui pourrait permettre de diminuer le nombre de patients n'ayant pas de diagnostic définitif établi et de traitement adapté. Des études restent encore nécessaires, au sein de centres experts, afin de standardiser les modalités pratiques de cette technique et de déterminer sa place dans l'algorithme de prise en charge des PI.


Assuntos
Doenças Pulmonares Intersticiais , Biópsia , Broncoscopia , Humanos , Doenças Pulmonares Intersticiais/diagnóstico
10.
Rev Med Suisse ; 13(583): 2001-2004, 2017 Nov 15.
Artigo em Francês | MEDLINE | ID: mdl-29143505

RESUMO

Bronchiectasis is irreversible bronchial dilatation associated with chronic respiratory symptoms. Management is aimed at reducing symptoms and slowing the progression of the disease by interrupting the vicious circle: bronchial infection, inflammation, altered mucociliary clearance, lung destruction. Unlike the literature on inhaled antibiotics in cystic fibrosis, literature data are limited and of low quality for bronchiectasis of other causes. However, new recommendations from the European Respiratory Society propose the conditional use of inhaled antibiotics to prevent repeated infectious exacerbations and to eradicate Pseudomonas aeruginosa colonization.


Les bronchiectasies sont des dilatations irréversibles des bronches associées à une symptomatologie respiratoire chronique. La prise en charge vise à réduire les symptômes et ralentir la progression de la maladie en interrompant le cercle vicieux : infection bronchique, inflammation, clairance mucociliaire altérée, destruction pulmonaire. Contrairement à la littérature concernant les antibiotiques inhalés dans la mucoviscidose, les données de la littérature sont peu nombreuses et de faible qualité en ce qui concerne les bronchiectasies d'autres causes. Toutefois, de nouvelles recommandations de l'European Respiratory Society proposent l'utilisation conditionnelle des antibiotiques inhalés pour prévenir les exacerbations infectieuses à répétition et pour l'éradication d'une nouvelle colonisation par Pseudomonas aeruginosa.


Assuntos
Antibacterianos , Bronquiectasia , Fibrose Cística , Administração por Inalação , Antibacterianos/administração & dosagem , Bronquiectasia/tratamento farmacológico , Bronquiectasia/etiologia , Fibrose Cística/complicações , Progressão da Doença , Humanos
11.
Respiration ; 92(6): 404-413, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27820928

RESUMO

BACKGROUND: Endoscopic lung volume reduction by coils (LVRC) is a recent treatment approach for severe emphysema. Furthermore, dual-energy computed tomography (DECT) now offers a combined assessment of lung morphology and pulmonary perfusion. OBJECTIVES: The aim of our study was to assess the impact of LVRC on pulmonary perfusion with DECT. METHODS: Seventeen patients (64.8 ± 6.7 years) underwent LVRC. DECT was performed prior to and after LVRC. For each patient, lung volumes and emphysema quantification were automatically calculated. Then, 6 regions of interest (ROIs) on the iodine perfusion map were drawn in the anterior, mid, and posterior right and left lungs at 4 defined levels. The ROI values were averaged to obtain lung perfusion as assessed by the lung's iodine concentration (CLung, µg·cm-3). The CLung values were normalized using the left atrial iodine concentration (CLA) to take into account differences between successive DECT scans. RESULTS: The 6-min walk distance (6MWD) improved significantly after the procedure (p = 0.0002). No lung volume changes were observed between successive DECT scans for any of the patients (p = 0.32), attesting the same suspended inspiration. After LVRC, the emphysema index was significantly reduced in the treated lung (p = 0.0014). Lung perfusion increased significantly adjacent to the treated areas (CLung/CLA from 3.4 ± 1.7 to 5.6 ± 2.2, p < 0.001) and in the ipsilateral untreated areas (from 4.1 ± 1.4 to 6.6 ± 1.7, p < 0.001), corresponding to a mean 65 and 61% increase in perfusion, respectively. No significant difference was observed in the contralateral upper and lower areas (from 4.4 ± 1.9 to 4.8 ± 2.1, p = 0.273, and from 4.9 ± 2.0 to 5.2 ± 1.7, p = 0.412, respectively). A significant correlation between increased 6MWD and increased perfusion was found (p = 0.0027, R2 = 0.3850). CONCLUSIONS: Quantitative analysis based on DECT acquisition revealed that LVRC results in a significant increase in perfusion in the coil-free areas adjacent to the treated ones, as well as in the ipsilateral untreated areas. This suggests a possible role for LVRC in the improvement of the ventilation/perfusion relationship.


Assuntos
Broncoscopia/métodos , Pulmão/irrigação sanguínea , Pneumonectomia/métodos , Enfisema Pulmonar/cirurgia , Idoso , Feminino , Humanos , Pulmão/diagnóstico por imagem , Pulmão/cirurgia , Masculino , Pessoa de Meia-Idade , Imagem de Perfusão , Enfisema Pulmonar/fisiopatologia , Tomografia Computadorizada por Raios X , Teste de Caminhada
12.
Rev Med Suisse ; 12(500): 80-2, 84-5, 2016 Jan 13.
Artigo em Francês | MEDLINE | ID: mdl-26946711

RESUMO

During the past year, among the many novelties in the field of pulmonary medicine, the authors chose to focus on 4 items: the positive contribution of systemic steroids on clinical improvement and length of stay in patients hospitalized for community-acquired pneumonia; the interesting results obtained with high flow oxygen, heated and humidified, in acute care and in normocapnic respiratory failure, a device which warrants further clinical testing in other indications; the now documented benefits of lung volume reduction procedures by bronchoscopy using coils in severe emphysema with hyperinflation; and the publication of new recommendations regarding pulmonary hypertension, with an emphasis on new molecules and their efficacy, on an early use of combination treatments, and on the importance of expert centres in managing these patients.


Assuntos
Pneumopatias/terapia , Pneumologia/tendências , Broncoscopia/métodos , Hospitalização , Humanos , Pneumopatias/fisiopatologia
14.
Case Rep Oncol ; 17(1): 549-555, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38618277

RESUMO

Introduction: Lung cancer is the second most common cancer; however, synchronous lung cancer is rare and challenging to treat. Case Presentation: We report the case of an 80-year-old female patient who presented with two lung lesions with primary tumor characteristics, which revealed squamous cell carcinoma and synchronous adenocarcinoma after histological sampling. Next-generation sequencing (NGS) analysis revealed a MET Exon 14 skipping mutation in squamous cell carcinoma and an epidermal growth factor receptor mutation in adenocarcinoma. Capmatinib and stereotactic radiotherapy were initiated for the adenocarcinoma with a good clinical response. Capmatinib treatment had to be discontinued because of stage 3 edema of the lower limbs, after which a left lobectomy was performed. Currently, the patient is considered to be in remission. Conclusion: This case highlights the need for histological analysis of every lung lesion with primary tumor characteristics, as well as for NGS analysis in search of specific mutations enabling the introduction of targeted therapies. mesenchymal-epithelial transition.

15.
Rev Med Suisse ; 14(591): 227-228, 2018 Jan 24.
Artigo em Francês | MEDLINE | ID: mdl-29380987
16.
J Cyst Fibros ; 2023 Nov 22.
Artigo em Inglês | MEDLINE | ID: mdl-37996316

RESUMO

BACKGROUND: Good data quality is essential when rare disease registries are used as a data source for pharmacovigilance studies. This study investigated data quality of the Swiss cystic fibrosis (CF) registry in the frame of a European Cystic Fibrosis Society Patient Registry (ECFSPR) project aiming to implement measures to increase data reliability for registry-based research. METHODS: All 20 pediatric and adult Swiss CF centers participated in a data quality audit between 2018 and 2020, and in a re-audit in 2022. Accuracy, consistency and completeness of variables and definitions were evaluated, and missing source data and informed consents (ICs) were assessed. RESULTS: The first audit included 601 out of 997 Swiss people with CF (60.3 %). Data quality, as defined by data correctness ≥95 %, was high for most of the variables. Inconsistencies of specific variables were observed because of an incorrect application of the variable definition. The proportion of missing data was low with <5 % for almost all variables. A considerable number of missing source data occurred for CFTR variants. Availability of ICs varied largely between centers (10 centers had >5 % of missing documents). After providing feedback to the centers, availability of genetic source data and ICs improved. CONCLUSIONS: Data audits demonstrated an overall good data quality in the Swiss CF registry. Specific measures such as support of the participating sites, training of data managers and centralized data collection should be implemented in rare disease registries to optimize data quality and provide robust data for registry-based scientific research.

17.
ERJ Open Res ; 7(1)2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33718487

RESUMO

RATIONALE AND OBJECTIVES: Prone positioning as a complement to oxygen therapy to treat hypoxaemia in coronavirus disease 2019 (COVID-19) pneumonia in spontaneously breathing patients has been widely adopted, despite a lack of evidence for its benefit. We tested the hypothesis that a simple incentive to self-prone for a maximum of 12 h per day would decrease oxygen needs in patients admitted to the ward for COVID-19 pneumonia on low-flow oxygen therapy. METHODS: 27 patients with confirmed COVID-19 pneumonia admitted to Geneva University Hospitals were included in the study. 10 patients were randomised to self-prone positioning and 17 to usual care. MEASUREMENTS AND MAIN RESULTS: Oxygen needs assessed by oxygen flow on nasal cannula at inclusion were similar between groups. 24 h after starting the intervention, the median (interquartile range (IQR)) oxygen flow was 1.0 (0.1-2.9) L·min-1 in the prone position group and 2.0 (0.5-3.0) L·min-1 in the control group (p=0.507). Median (IQR) oxygen saturation/fraction of inspired oxygen ratio was 390 (300-432) in the prone position group and 336 (294-422) in the control group (p=0.633). One patient from the intervention group who did not self-prone was transferred to the high-dependency unit. Self-prone positioning was easy to implement. The intervention was well tolerated and only mild side-effects were reported. CONCLUSIONS: Self-prone positioning in patients with COVID-19 pneumonia requiring low-flow oxygen therapy resulted in a clinically meaningful reduction of oxygen flow, but without reaching statistical significance.

18.
BMJ Case Rep ; 13(7)2020 Jul 07.
Artigo em Inglês | MEDLINE | ID: mdl-32641438

RESUMO

Systemic lupus erythematosus is a multisystem autoimmune disease with wide-ranging pleuropulmonary manifestations. Acute lupus pneumonitis is one of its uncommon complications. We report a 36-year-old woman with acute lupus pneumonitis as the initial presentation of systemic lupus erythematosus. Clinical, biological, radiological and functional improvements were noticed with the administration of steroids, hydroxychloroquine and immunoglobulin.


Assuntos
Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Adulto , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Doenças Pulmonares Intersticiais/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Esteroides/uso terapêutico , Resultado do Tratamento
19.
BMJ Case Rep ; 12(2)2019 Feb 26.
Artigo em Inglês | MEDLINE | ID: mdl-30814101

RESUMO

Certain interventional pulmonology procedures such as the placement of a tracheal stent or resection of stenosing tracheal tumours require rigid bronchoscopy under general anaesthesia. Unlike an endotracheal tube with a cuff, the rigid bronchoscope only partially protects the airway from bronchoaspiration. For this reason, this procedure is performed on an elective basis in fasted patients. We describe the case of a 60-year-old man with acute respiratory distress requiring emergent rigid bronchoscopy following distal migration of a tracheal stent. One hour before the procedure, the patient had eaten a full meal. Gastric emptying was accelerated by perfusion of intravenous erythromycin and verified by endoscopy with a small diameter gastric endoscope under local anaesthesia. This 1 min procedure was very well tolerated by the patient and allowed to verify with certainty that the stomach was empty. The urgent rigid bronchoscopy for stent retrieval could then be performed safely without any risk of bronchoaspiration.


Assuntos
Broncoscopia/métodos , Remoção de Dispositivo/métodos , Eritromicina/administração & dosagem , Esvaziamento Gástrico/efeitos dos fármacos , Falha de Prótese/efeitos adversos , Insuficiência Respiratória/etiologia , Endoscopia , Fármacos Gastrointestinais/administração & dosagem , Humanos , Injeções Intravenosas , Masculino , Pessoa de Meia-Idade , Insuficiência Respiratória/terapia , Stents , Vigília
20.
ERJ Open Res ; 5(3)2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31528637

RESUMO

Fibrinolysis can be used to improve fluid drainage in pleural infection. Treatment with either urokinase or tissue plasminogen activator (t-PA) in association with DNAse via a chest tube has been effective at reducing the need for surgery. This study is the first to compare the efficacy of these two treatments. We performed a single-centre, controlled, prospective cohort study. All individuals with pleural infection admitted to our hospital between January 2014 and December 2017 who were treated with antibiotics, a chest tube and fibrinolysis were included in this study. The rate of additional procedure requirements (additional chest tube or surgery) after initial fibrinolysis, complications, costs, and radiological and biological outcomes were analysed. Among the 93 patients included in this study, 34% required additional procedures after an initial fibrinolysis, including 21% who received an additional chest tube and 13% who underwent thoracoscopy. The need for additional procedures arose due to presence of multiple pleural collections (p=0.01) and was associated with the use of large-bore drain (p=0.01). The success rate of fibrinolysis was not significantly different between urokinase and t-PA/DNAse (p=0.35). The differences in drainage duration and in length of hospital stay were not significant either (p=0.05 and p=0.12, respectively). Treatment with t-PA/DNAse was cheaper (p=0.04) but was associated with a higher rate of haemothorax (p=0.002). In conclusion, treatment with urokinase is safer and equally effective when compared with treatment with t-PA/DNAse.

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