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Spektrum Ophthalmologie – wissenswert, kompakt, anregend Spektrum 眼科 - 值得了解、紧凑、刺激
Pub Date : 2023-01-01 DOI: 10.1159/000533823
Der Melaningehalt der Iris kann sich auf die Augengesundheit auswirken. Denn so wie in der Haut schützt das Melanin auch in der Iris vor dem Einfluss des Sonnenlichts. Es filtert sowohl den sichtbaren Teil des Lichtspektrums – Menschen mit sehr hellen Augen reagieren daher besonders empfindlich auf starken Lichteinfall – als auch dessen UV-Anteil. Bei niedrigerem Melaningehalt steigt deshalb auch das Risiko, an einem uvealen Melanom zu erkranken. «Dieser Krebstyp ist zwar sehr selten, er findet sich jedoch bei Menschen europäischer Abstammung 20bis 30-mal häufiger als bei Menschen asiatischer oder afrikanischer Abstammung», erläutert Prof. Nikolaos Bechrakis (Essen). Mit einem geringeren Schutz vor den schädlichen Auswirkungen des Sonnenlichts lässt sich vermutlich auch die Beobachtung erklären, dass Menschen mit hellen Augen eher eine AMD entwickeln. «Eine umfangreiche Metaanalyse mit fast 130 000 Teilnehmenden konnte belegen, dass zumindest die Kompass Ophthalmol 2023;9:128–131 DOI: 10.1159/000533823
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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
Pub Date : 2023-01-01 DOI: 10.1159/000533457
J. Stuart Elborn, Francesco Blasi, Pierre-Régis Burgel, Daniel Peckham
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.
慢性和复发性细菌感染在囊性纤维化(cf)患者中很常见,并导致肺功能恶化。抗生素是治疗慢性炎症和细菌感染的基础。吸入式抗生素对治疗慢性呼吸道细菌感染和根除铜绿假单胞菌有效。呼吸道,对全身的不良影响有限。在过去的10年中,调制器电导transmembrana调节阀的囊性纤维化(CFTR)高效已成为一种新的治疗选择部分更正/ reabre运输氯CFTR的特定突变,恢复患者的水化粘液并改善体内的纤毛。最近CFTR调控的三重组合被批准用于80 - 90%的cf患者,并显著减少肺部恶化,改善呼吸道症状和肺功能。CFTR调节剂通过解决基因型特异性CFTR缺陷,使对症治疗成为个性化/精准药物的焦点。虽然它们非常有效,但它们不能完全使肺生理正常化,不能阻止炎症或解决慢性肺损伤,如支气管扩张。这些新药物对肺部健康的影响可能会改变未来对cf患者慢性肺部感染的治疗。这篇文章回顾了吸入抗生素在CFTR调节剂时代的作用。
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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
Pub Date : 2023-01-01 DOI: 10.1159/000533336
Maria René Álvarez-Arroyo
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.
& lt; b>介绍:& lt; / b>最近,囊性纤维化跨膜调节剂治疗(elexaftor /tezacaftor/ivacaftor)可用于携带至少一种F508del< /i>突变。& lt; b>目的:& lt; / b>在现实世界中评估elexaftor /tezacaftor/ivacaftor治疗囊性纤维化儿童的中期效果。& lt; b>方法:& lt; / b>我们对囊性纤维化儿童的记录进行了回顾性分析,这些儿童在2020年8月至2022年10月期间开始使用elexaftor /tezacaftor/ivacaftor。分别在开始使用elexaftor /tezacaftor/ivacaftor前、3个月和6个月评估肺功能、营养状况、汗液氯化物和实验室数据。& lt; b>结果:& lt; / b>22名6-11岁儿童和24名12-17岁儿童开始使用Elexacaftor/tezacaftor/ivacaftor。27例(59%)患者为<i>F508del</i>(F/F), 23例(50%)患者从IVA/LUM (IVA/LUM)或TEZ/IVA (TEZ/IVA)过渡到elexaftor /tezacaftor/ivacaftor。总体而言,平均汗液氯化物浓度下降了59.3 mmol/L(95%置信区间:-65.0至-53.7 mmol/L, <i> </i>, # x3c;0.0001)在elexacaftor/tezacaftor/ivacaftor下。从IVA/LUM或TEZ/IVA过渡到萃取物/萃取物/萃取物后,汗液氯化物浓度也显著降低(-47.8 mmol/l;95%置信区间:-57.6 ~ -37.8 mmol/l, n = 14, <i> </i>, # x3c;0.0001)。与F/MF基因型儿童相比,F/F儿童的汗液氯化物减少更为明显(69.4 vs 45.9 mmol/L, p <0.0001)。随访3个月时,身体质量指数-z得分增加0.31 (95% CI, 0.2-0.42, <i> </i>, # x3c;0.0001), 6个月后没有进一步增加。年龄-z- bmi评分在老年组中有更显著的改善。随访3个月时,总体肺功能(预测FEV< sub</sub>百分比)增加11.4% (95% CI: 8.0-14.9, < p</i>, # x3c;0.0001), 6个月后无进一步显著变化。年龄组之间没有明显差异。与F/F基因型相比,F/MF基因型儿童在营养状况和肺功能测试方面有更大的益处。不良事件导致3例elexaftor /tezacaftor/ivacaftor剂量减少,4例暂时中断治疗。& lt; b>结论:& lt; / b>在现实环境中,elexaftor /tezacaftor/ivacaftor治疗在符合条件的囊性纤维化儿童中具有有益的临床效果和良好的安全性,与先前发表的对照临床试验数据相当。elexaftor /tezacaftor/ivacaftor治疗3个月后对肺功能测试和营养状况的积极影响持续到6个月的随访。
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引用次数: 0
Veranstaltungskalender 酒店
Pub Date : 2023-01-01 DOI: 10.1159/000534871
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引用次数: 0
Veranstaltungskalender 酒店
Pub Date : 2023-01-01 DOI: 10.1159/000534843
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引用次数: 0
Dupilumab: Zulassung als erste Systemtherapie bei PN 切换网路疗程
Pub Date : 2023-01-01 DOI: 10.1159/000534684
{"title":"Dupilumab: Zulassung als erste Systemtherapie bei PN","authors":"","doi":"10.1159/000534684","DOIUrl":"https://doi.org/10.1159/000534684","url":null,"abstract":"","PeriodicalId":477056,"journal":{"name":"Karger Kompass","volume":"27 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":"135561432","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
OphthalmoCampus OphthalmoCampus
Pub Date : 2023-01-01 DOI: 10.1159/000534925
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引用次数: 0
Risikobehaftet? Transbronchiale Mikrowellenablation beim nicht metastasierten Lungenkarzinom Risikobehaftet ?未转移的肺结核传导微波炉
Pub Date : 2023-01-01 DOI: 10.1159/000533174
Lars Hagmeyer
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.
背景:图像引导下经皮热消融术是早期肺癌医学上不能手术的患者的一种成熟的治疗选择,但它具有胸膜相关并发症的高风险,特别是气胸。目的:本研究旨在确定图像引导下经支气管微波消融(tMWA)是否是治疗周围期1期肺癌的可行方法。方法:一项前瞻性、单臂、多中心研究,旨在招募40名因周围期1期肺肿瘤(≤20 mm)无法手术或拒绝手术的成年人。使用导航支气管镜和柔性MWA探针进行消融,锥形束CT引导增强透视。随访1、6和12个月,包括消融区CT成像、肿瘤复发可能性、不良事件(ae)、肺功能和生活质量。结果:在10名入组患者中,在2个部位治疗了11个肿瘤(10个非小细胞肺癌,1个类癌)。中位肿瘤直径为13 × 14 mm (7 ~ 19 mm),中位最小消融缘为11 mm (5 ~ 19 mm)。所有患者均获得技术成功和技术效果。随访12个月未见肿瘤复发。无气胸、胸腔积液、支气管胸膜瘘。轻微的不良反应包括少量咯血、疼痛、咳嗽和呼吸困难。消融≤30天发生2例严重ae,包括COPD加重(第9天)和不明原因死亡(第15天)。死亡导致赞助商停止招收。肺功能和生活质量指标保持稳定。
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引用次数: 0
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
Pub Date : 2023-01-01 DOI: 10.1159/000533723
Ernst Rietschel
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引用次数: 0
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
Pub Date : 2023-01-01 DOI: 10.1159/000533890
Esperanza Figueroa-Hurtado, Diana Lizbeth Ortiz-Farías, Arturo Cortés-Telles
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.
& lt; b>背景:& lt; / b>囊性纤维化基金会患者登记处(cfpr)报告称,囊性纤维化(PwCF)患者的哮喘患病率很高(34.6%)。虽然我们目前对这种关系的理解有限,但在CF患者中经常发现2型炎症(T2)表型。& lt; b>研究问题:& lt; / b>本研究旨在评估嗜酸性CF T2炎症表型与CF相关肺结局和微生物学数据之间的关系。& lt; b>研究设计:& lt; / b>方法:我们对成年CF患者(18岁及以上;n = 93),从2013年1月到2018年12月在弗吉尼亚大学医学中心成人项目接受治疗。收集的数据包括人口统计学数据、CFTR (CF跨膜传导调节因子)突变、CF合并症、药物、细胞绝对嗜酸性粒细胞计数(AEC) /µL和免疫球蛋白E (IgE)水平(IU/mL)。& lt; b>结果:& lt; / b>在筛选研究资格的93例患者中,74例被纳入最终分析;19例患者因缺乏贯穿研究时间线的纵向数据而被排除。肺功能下降与AEC升高相关(p <0.001)和IgE (p <0.001),即使校正了协变量:年龄、性别、假单胞菌、MRSA、其他细菌、曲霉菌和其他真菌(p <0.001)。单变量分析表明,一年有2次以上病情加重需要住院和/或静脉注射抗生素的CF患者更有可能有高AEC (p = 0.018)。Logistic回归显示,随着AEC的增加,在CF加重期间进行测量的概率增加(p = 0.0039)。线性混合模型显示,年加重事件每增加一次,log IgE平均增加0.04。(p = 0.015)。这一发现在多变量模型中保持稳定(p = 0.0145)。当针对特应性进行调整时,log IgE随着加重事件数量的增加而增加(p = 0.022)。AEC和IgE与微生物定植没有相关性。
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引用次数: 0
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Karger Kompass
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