Karger Kompass最新文献_第8页

Spektrum Ophthalmologie – wissenswert, kompakt, anregend Spektrum 眼科 - 值得了解、紧凑、刺激

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000533823

引用次数: 0

El papel de los antibióticos inhalados en la era de los moduladores de CFTR de alta eficacia 吸入抗生素在高效CFTR调节剂时代的作用

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000533457

J. Stuart Elborn, Francesco Blasi, Pierre-Régis Burgel, Daniel Peckham

{"title":"El papel de los antibióticos inhalados en la era de los moduladores de CFTR de alta eficacia","authors":"J. Stuart Elborn, Francesco Blasi, Pierre-Régis Burgel, Daniel Peckham","doi":"10.1159/000533457","DOIUrl":"https://doi.org/10.1159/000533457","url":null,"abstract":"Las infecciones bacterianas recurrentes y crónicas son frecuentes en pacientes con fibrosis quística (FQ), y contribuyen al deterioro de la función pulmonar. Los antibióticos constituyen la base del tratamiento de las exacerbaciones e infecciones bacterianas crónicas en la FQ. Los antibióticos inhalados son eficaces para tratar infecciones bacterianas respiratorias crónicas y erradicar a Pseudomonas aeruginosa de las vías respiratorias, con efectos adversos sistémicos limitados. En la última década, los moduladores del regulador de la conductancia transmembrana de la fibrosis quística (CFTR) de alta eficacia se han convertido en una nueva opción terapéutica que corrige/reabre parcialmente el transporte de cloruro en pacientes con mutaciones específicas del CFTR, restaurando la hidratación del moco y mejorando el aclaramiento mucociliar. La reciente combinación triple de moduladores CFTR está aprobada para ∼80–90% de la población con FQ, y reduce significativamente las exacerbaciones pulmonares y mejora los síntomas respiratorios y la función pulmonar. Los moduladores de CFTR han puesto en el centro de atención del tratamiento sintomático a la medicina personalizada/de precisión, al dirigirse a defectos de CFTR específicos del genotipo. Aunque son muy eficaces, no normalizan por completo la fisiología pulmonar, no detienen la inflamación ni resuelven el daño pulmonar crónico, como las bronquiectasias. Es probable que el impacto de estos nuevos fármacos en la salud pulmonar cambie el tratamiento futuro de las infecciones pulmonares crónicas en pacientes con FQ. Este artículo revisa el papel de los antibióticos inhalados en la era de los moduladores de CFTR.","PeriodicalId":477056,"journal":{"name":"Karger Kompass","volume":"46 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136257266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Niños con fibrosis quística: Impacto en la vida real de la terapia moduladora de CFTR altamente efectiva 囊性纤维化儿童:高效CFTR调节疗法对现实生活的影响

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000533336

Maria René Álvarez-Arroyo

{"title":"Niños con fibrosis quística: Impacto en la vida real de la terapia moduladora de CFTR altamente efectiva","authors":"Maria René Álvarez-Arroyo","doi":"10.1159/000533336","DOIUrl":"https://doi.org/10.1159/000533336","url":null,"abstract":"Introduction: Recently, cystic fibrosis transmembrane regulator modulator therapy with elexacaftor/tezacaftor/ivacaftor has become available for children with cystic fibrosis (CF) carrying at least one F508del mutation. Objective: To assess the intermediate term effects of elexacaftor/tezacaftor/ivacaftor in children with cystic fibrosis in a real-world setting. Methods: We performed a retrospective analysis of records of children with cystic fibrosis, who started elexacaftor/tezacaftor/ivacaftor between 8/2020 and 10/2022. Pulmonary function tests, nutritional status, sweat chloride and laboratory data were assessed before, 3 and 6 months after the start of elexacaftor/tezacaftor/ivacaftor respectively. Results: Elexacaftor/tezacaftor/ivacaftor was started in 22 children 6–11 years and in 24 children 12–17 years. Twenty-seven (59%) patients were homozygous for F508del (F/F) and 23 (50%) patients were transitioned from ivacaftor/lumacaftor (IVA/LUM) or tezacaftor/ivacaftor (TEZ/IVA) to elexacaftor/tezacaftor/ivacaftor. Overall, mean sweat chloride concentration decreased by 59.3 mmol/L (95% confidence interval: -65.0 to -53.7 mmol/L, p &#x3c; 0.0001) under elexacaftor/tezacaftor/ivacaftor. Sweat chloride concentration also decreased significantly after transition from IVA/LUM or TEZ/IVA to elexacaftor/tezacaftor/ivacaftor (-47.8 mmol/l; 95% confidence interval: -57.6 to -37.8 mmol/l, n = 14, p &#x3c; 0.0001). Sweat chloride reduction was more marked in children with the F/F than in those with the F/MF genotype (69.4 vs 45.9 mmol/L, p &#x3c; 0.0001). At 3 months follow-up, body-mass-index-z-score increased by 0.31 (95% CI, 0.2–0.42, p &#x3c; 0.0001) with no further increase at 6 months. BMI-for-age-z-score was more markedly improved in the older group. Overall pulmonary function (percent predicted FEV1) at 3 months follow-up increased by 11.4% (95% CI: 8.0–14.9, p &#x3c; 0.0001) with no further significant change after 6 months. No significant differences were noted between the age groups. Children with the F/MF genotype had a greater benefit regarding nutritional status and pulmonary function tests than those with the F/F genotype. Adverse events led to elexacaftor/tezacaftor/ivacaftor dose reduction in three cases and a temporary interruption of therapy in four cases. Conclusion: In a real-world setting, elexacaftor/tezacaftor/ivacaftor therapy had beneficial clinical effects and a good safety profile in eligible children with cystic fibrosis comparable to previously published data from controlled clinical trials. The positive impact on pulmonary function tests and nutritional status seen after 3 months of elexacaftor/tezacaftor/ivacaftor therapy was sustained at 6 months follow-up.","PeriodicalId":477056,"journal":{"name":"Karger Kompass","volume":"10 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136257267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

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Veranstaltungskalender 酒店

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000534871

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Veranstaltungskalender 酒店

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000534843

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Dupilumab: Zulassung als erste Systemtherapie bei PN 切换网路疗程

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000534684

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OphthalmoCampus OphthalmoCampus

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000534925

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Risikobehaftet? Transbronchiale Mikrowellenablation beim nicht metastasierten Lungenkarzinom Risikobehaftet ?未转移的肺结核传导微波炉

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000533174

Lars Hagmeyer

{"title":"Risikobehaftet? Transbronchiale Mikrowellenablation beim nicht metastasierten Lungenkarzinom","authors":"Lars Hagmeyer","doi":"10.1159/000533174","DOIUrl":"https://doi.org/10.1159/000533174","url":null,"abstract":"Background: Image-guided percutaneous thermal ablation is an established treatment option for early-stage lung cancer in medically inoperable patients but carries a high risk of pleura-related complications, particularly pneumothorax. Objective: This study aimed to determine if image-guided transbronchial microwave ablation (tMWA) is a feasible approach to treat peripheral stage 1 lung cancer. Method: A prospective, single-arm, multicenter study sought to enroll 40 adults who were medically inoperable or declined surgery for peripheral stage 1 lung tumors (≤20 mm). Ablation was performed using navigational bronchoscopy and a flexible MWA probe, guided by cone-beam CT with augmented fluoroscopy. Follow-up at 1, 6, and 12 months included CT imaging of the ablation zone and possible tumor recurrence, adverse events (AEs), pulmonary function, and quality of life. Results: Across 2 sites, 11 tumors (10 NSCLC, 1 carcinoid) were treated in 10 enrolled patients. Median tumor diameter was 13 × 14 mm (7–19 mm) and median minimum ablative margin was 11 mm (5–19 mm). Technical success and technique efficacy were achieved in all patients. No tumor recurrence was seen during 12-month follow-up. No pneumothorax, pleural effusion, or bronchopleural fistula were noted. Minor AEs included scant hemoptysis, pain, cough, and dyspnea. Two serious AEs occurred ≤30 days of ablation and included a COPD exacerbation (day 9) and a death of unknown cause (day 15). The death led the sponsor to halt enrollment. Pulmonary function and quality-of-life indices remained stable.","PeriodicalId":477056,"journal":{"name":"Karger Kompass","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136029374","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

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Mukoviszidose: Wegbereiter für die Diagnostik und Therapie anderer seltener Lungenerkrankungen 栓塞症:切断其他罕见肺癌的诊断和治疗

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000533723

Ernst Rietschel

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Fibrosis quística: Facilitar urgentemente el acceso al diagnóstico temprano también en los países emergentes 囊性纤维化:紧急促进早期诊断，包括在新兴国家

Karger Kompass Pub Date : 2023-01-01 DOI: 10.1159/000533890

Esperanza Figueroa-Hurtado, Diana Lizbeth Ortiz-Farías, Arturo Cortés-Telles

{"title":"Fibrosis quística: Facilitar urgentemente el acceso al diagnóstico temprano también en los países emergentes","authors":"Esperanza Figueroa-Hurtado, Diana Lizbeth Ortiz-Farías, Arturo Cortés-Telles","doi":"10.1159/000533890","DOIUrl":"https://doi.org/10.1159/000533890","url":null,"abstract":"Background: The Cystic Fibrosis Foundation Patient Registry (CFFPR) reports a high prevalence of asthma (34.6%) in people with Cystic Fibrosis (PwCF). While our current understanding of this relationship is limited, a type 2 inflammatory (T2) phenotype has often been identified in CF patients. Research question: This study aimed to evaluate the relationship between the eosinophilic CF T2 inflammatory phenotype and CF-related pulmonary outcomes and microbiological data. Study design: and methods: We conducted a retrospective chart review of adult patients with CF (18 and older; n = 93) receiving their care at University of Virginia Medical Center adult program from January, 2013 through December, 2018. Data collected included demographic data, CFTR (CF transmembrane conductance regulator) mutation, CF comorbidities, medications, Absolute Eosinophil Counts (AEC) in cells/µL and Immunoglobulin E (IgE) levels in IU/mL. Results: Of 93 patients screened for study eligibility, 74 were included in the final analysis; 19 patients were excluded due to lack of longitudinal data across the study timeline. Lung function decline correlated with increased AEC (p &#x3c; 0.001) and IgE (p &#x3c; 0.001) even when adjusting for covariates: age, gender, presence of Pseudomonas spp., MRSA, other bacterial spp., Aspergillus spp., and other fungi (p &#x3c; 0.001). Univariate analysis demonstrated that people with CF who experienced more than 2 exacerbations requiring hospitalizations and/or intravenous antibiotics a year were more likely to have high AEC (p = 0.018). Logistic regression showed that as AEC increases, the probability that the measurement was taken during a CF exacerbation increases (p = 0.0039). A linear mixed model showed that each additional annual exacerbation event increased on average the log IgE by 0.04. (p = 0.015). This finding remained stable in a multivariate model (p = 0.0145). When adjusted for atopy, log IgE increases as the number of exacerbation events increases (p = 0.022). There was no association between AEC and IgE and microbiological colonization.","PeriodicalId":477056,"journal":{"name":"Karger Kompass","volume":"82 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136209832","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0