Journal of Cystic Fibrosis最新文献_第9页

The day after. Rethinking the Cystic Fibrosis model of care and structure of the CF team in the era of triple combination therapy 后天。在三联疗法时代，重新思考囊性纤维化的治疗模式和囊性纤维化团队的结构。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.09.016

Philippe Reix

引用次数: 0

News article

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.10.013

引用次数: 0

Role of viable but non culturable cells in patients with cystic fibrosis in the era of highly effective modulator therapy 高效调节剂疗法时代囊性纤维化患者体内可存活但不可培养的细胞的作用。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.02.013

Natalia Cirilli , Valentina Schiavoni , Valentina Tagliabracci , Rosaria Gesuita , Luca Tiano , Benedetta Fabrizzi , Anastasia D'Antuono , Arianna Peruzzi , Nicholas Cedraro , Flavia Carle , Marco Moretti , Luigi Ferrante , Carla Vignaroli , Francesca Biavasco , Gianmarco Mangiaterra

Background

Lung infections antibiotic treatment in Cystic Fibrosis patients (pwCF) is often complicated by bacterial persisters, including the so-called Viable but Non Culturable (VBNC) forms, live cells undetected by the routine cultural microbiological methods. This study investigated the occurrence of VBNC cells of five CF bacterial pathogens in 94 pwCF over one year and the possible associations with the patients’ clinical features.

Methods

Sputum samples, recovered at routine visits and during exacerbation episodes, were analyzed for the presence of the five pathogens by both routine culture-based assays and species-specific qPCR. VBNC cells were estimated as the difference between molecular and cultural counts and their presence was matched with the clinical data in particular the therapeutic regimens.

Results

All but ten pwCF showed the presence of VBNC cells at least once during the study. Pseudomonas aeruginosa and methicillin-susceptible Staphylococcus aureus were the species most frequently found in the VBNC state. Only the former showed a significant association between chronic infection and VBNC cells presence; VBNC-MSSA positive patients significantly increased overtime. The presence of non culturable bacteria was generally concurrent with poor lung functionality and more frequent pulmonary exacerbations. No significant association with modulator treatment was evidenced.

Conclusions

The obtained data demonstrated the overwhelming occurrence of bacterial VBNC cells in CF lung infections, warranting a constant monitoring of pwCF and underlining the need of implementing the routine culture-based assays with culture-independent techniques. This is pivotal to understand the CF bacterial population dynamics and to efficiently contrast the lung infection progression and worsening.

背景：囊性纤维化患者（pwCF）的肺部感染抗生素治疗通常会因细菌持久存在而变得复杂，包括所谓的 "可存活但不可培养（VBNC）"形式，即常规微生物培养方法无法检测到的活细胞。本研究调查了 94 名儿童肺结核患者一年内五种 CF 细菌病原体 VBNC 细胞的发生情况，以及与患者临床特征的可能关联：方法：通过常规培养法和物种特异性 qPCR 分析在常规就诊和病情加重期间采集的痰液样本中是否存在五种病原体。VBNC 细胞是根据分子计数和培养计数的差值估算的，它们的存在与临床数据特别是治疗方案相匹配：结果：除 10 例 pwCF 外，其他所有 pwCF 在研究期间至少出现过一次 VBNC 细胞。铜绿假单胞菌和对甲氧西林敏感的金黄色葡萄球菌是在 VBNC 状态下最常发现的菌种。只有前者显示出慢性感染与 VBNC 细胞存在之间的显著关联；VBNC-MSSA 阳性患者随时间推移显著增加。不可培养细菌的存在通常与肺功能低下和更频繁的肺部恶化同时存在。结论：获得的数据表明，细菌 VBNC 细胞在 CF 肺部感染中的发生率极高，因此有必要对 pwCF 进行持续监测，并强调有必要使用独立于培养的技术进行常规培养检测。这对于了解 CF 细菌种群动态、有效对比肺部感染进展和恶化至关重要。

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引用次数: 0

Cystic fibrosis foundation position paper: Redefining the CF care model 囊性纤维化基金会立场文件：重新定义 CF 护理模式。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.08.007

D.M. Goetz , R.F. Brown , S.S. Filigno , S.L. Bichl , A.L. Nelson , C.A. Merlo , R. Juel , P. Lomas , S.E. Hempstead , Q. Tran , A.W. Brown , P.A. Flume , CFF Care Model Committee

Specialized care is provided to people with cystic fibrosis (pwCF) by interdisciplinary teams nested within the CF Foundation's accredited care center network. This network allows for standardization of the care model, implementation of clinical care guidelines, efficient communication, and outcomes reporting. Recent developments have impacted this care model. Increased access to CFTR modulator therapies has improved overall health for many, although not all pwCF. The COVID-19 pandemic resulted in a rapid adoption of telemedicine and remote monitoring to ensure continuity of CF care. A collaboration of care providers, pwCF, and parent caregivers reevaluated key aspects of the current care model and considered potential modifications based on a widening range of needs. Available evidence was used to evaluate components of routine clinical practice and identify potential adaptations to care. The review included identification of patient characteristics warranting intensive monitoring, while embracing patient-centric care, and emphasizing the integration of telemedicine and at-home health technologies. Despite the changing landscape, the importance of the relationship between pwCF, their support system, and the care team was confirmed as a timeless and foundational aspect of the care model. Shared decision making, partnership, and coproduced care plans between pwCF and their CF care teams guide the best adaptations of the care model to support individual priorities and wellbeing. As health care advances and pwCF age, further research is needed to understand the impact of the care model on long-term health outcomes and to identify best practices that support pwCF to live longer healthier lives.

囊性纤维化患者（pwCF）由囊性纤维化基金会认可的护理中心网络内的跨学科团队提供专业护理。该网络实现了护理模式的标准化、临床护理指南的实施、高效沟通和结果报告。最近的发展对这种护理模式产生了影响。CFTR 调节器疗法的普及改善了许多患者的总体健康状况，但并非所有肺结核患者都能获得这种治疗。COVID-19 大流行导致远程医疗和远程监控的迅速普及，以确保 CF 护理的连续性。护理提供者、儿童肺结核患者和家长护理人员合作，重新评估了当前护理模式的关键方面，并根据不断扩大的需求范围考虑了可能的修改。现有证据被用来评估常规临床实践的组成部分，并确定对护理进行调整的可能性。审查包括确定需要加强监测的患者特征，同时接受以患者为中心的护理，并强调远程医疗和居家保健技术的整合。尽管情况在不断变化，但患者、其支持系统和护理团队之间关系的重要性得到了确认，这是护理模式永恒的基础。护理模式中的共同决策、合作关系以及护理团队共同制定的护理计划为护理模式的最佳调整提供了指导，以支持个人的优先事项和福祉。随着医疗保健事业的发展和贫困儿童年龄的增长，需要进一步开展研究，以了解护理模式对长期健康结果的影响，并确定支持贫困儿童健康长寿的最佳实践。

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引用次数: 0

Tezacaftor is a direct inhibitor of sphingolipid delta-4 desaturase enzyme (DEGS) Tezacaftor 是鞘脂 delta-4 去饱和酶（DEGS）的直接抑制剂。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.05.004

Dinu Zinovie Ciobanu , Nara Liessi , Valeria Tomati , Valeria Capurro , Sine Mandrup Bertozzi , Maria Summa , Rosalia Bertorelli , Nicoletta Loberto , Dorina Dobi , Massimo Aureli , Lucilla Nobbio , Tiziano Bandiera , Nicoletta Pedemonte , Rosaria Bassi , Andrea Armirotti

Background

We recently demonstrated that 48 h exposure of primary human bronchial epithelial (hBE) cells, obtained from both CF (F508del homozygous) and non-CF subjects, to the triple drug combination Elexacaftor/Tezacaftor/Ivacaftor (ETI) results in a CFTR genotype-independent modulation of the de novo synthethic pathway of sphingolipids, with an accumulation of dihydroceramides (dHCer). Since dHCer are converted into ceramides (Cer) by the action of a delta-4 sphingolipid desaturase (DEGS) enzyme, we aimed to better understand this off-target effect of ETI (i.e., not related to CFTR rescue)

Methods

hBE cells, both F508del and wild-type, were cultured to create fully differentiated bronchial epithelia. We analyzed Cer and dHCer using an LC-MS based method previously developed by our lab. DEGS expression levels in differentiated hBE cells lysates were quantified by western blot analysis.

Results

We demonstrated that 1) dHCer accumulate in hBE with time following prolonged ETI exposure, that 2) similar inhibition occurs in wild-type primary human hepatocytes and that 3) this does not result in an alteration of DEGS expression. We then proved that 4) ETI is a direct inhibitor of DEGS, that 5) Tezacaftor is the molecule responsible for this effect, that 6) the inhibition is concentration dependent. Finally, after repeated oral administration of ETI to naïve, non-CF, mice, we observed a slight accumulation of dHCer in the brain.

Conclusions

We believe that further investigations on Tezacaftor should be envisaged, particularly for the use of ETI during pregnancy, breastfeeding and in the early stages of development. DEGS dysfunction and dHCer accumulation causes impairment in the development of the nervous system, due to a derangement in myelin formation and maintenance.

背景：我们最近证明，将来自 CF（F508del 同源）和非 CF 受试者的原代人类支气管上皮细胞（hBE）暴露于 Elexacaftor/Tezacaftor/Ivacaftor 三联药物组合（ETI）48 小时后，会导致 CFTR 基因型无关的鞘脂从头合成途径的调节，并导致二氢甘油三酯（dHCer）的积累。由于 dHCer 是在δ-4 脂肪鞘脂去饱和酶（DEGS）的作用下转化为神经酰胺（Cer）的，因此我们旨在更好地了解 ETI 的这种脱靶效应（即与 CFTR 挽救无关）方法：培养 F508del 和野生型 hBE 细胞，以创建完全分化的支气管上皮。我们使用实验室之前开发的一种基于 LC-MS 的方法分析了 Cer 和 dHCer。分化的 hBE 细胞裂解物中 DEGS 的表达水平通过 Western 印迹分析进行量化：结果：我们证明了：1）长期暴露于 ETI 后，dHCer 在 hBE 中会随着时间的推移而积累；2）野生型原代人类肝细胞中也存在类似的抑制作用；3）这不会导致 DEGS 表达的改变。我们随后证明：4）ETI 是 DEGS 的直接抑制剂；5）Tezacaftor 是产生这种效应的分子；6）抑制作用与浓度有关。最后，在给天真、非慢性阻塞性肺病小鼠反复口服 ETI 后，我们观察到 dHCer 在大脑中有轻微积累：我们认为，应该对 Tezacaftor 进行进一步研究，尤其是在孕期、哺乳期和发育早期使用 ETI。DEGS功能障碍和dHCer蓄积会导致神经系统发育受损，这是髓鞘形成和维持失调所致。

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引用次数: 0

Cardiac Structure and Function in People with Cystic Fibrosis 囊性纤维化患者的心脏结构和功能。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.09.012

Lisa Steen Duus , Maria Dons , Rebekka Faber Thudium , Susanne Dam Nielsen , Mette F Olsen , Tavs Qvist , Mats C Højbjerg Lassen , Kristoffer Grundtvig Skaarup , Niklas Dyrby Johansen , Thomas Mørk-Strøm Bluhme , Terese L Katzenstein , Tacjana Pressler , Daniel Faurholt-Jepsen , Tor Biering-Sørensen

Background

The extent of cardiac involvement in cystic fibrosis (CF) remains to be determined. The remarkable therapeutic advancements with new highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator treatment and subsequent increase in life expectancy substantiates further research. We aimed to explore the prevalence of cardiac alterations in people with CF (pwCF) compared to matched controls and investigate potential cardiovascular risk factors.

Methods

In this cross-sectional study, 104 pwCF underwent clinical and echocardiographic assessment. All participants were matched 1:1 with controls from the general population.

Results

Of 104 pwCF, 44 % were female, mean age was 34 years, and 93 % received CFTR modulator treatment. The prevalence of abnormal cardiac function in pwCF was 44 %, more than double the prevalence in controls. PwCF were found to have smaller left ventricular (LV) dimensions, worse LV diastolic function, and reduced right ventricle (RV) as well as LV systolic function. After multivariable adjustment, LV diastolic function as well as LV and RV systolic function remained poorer in pwCF as compared to controls. Male sex and decreasing FEV1/FVC ratio remained independently associated with abnormal cardiac function in pwCF (male sex: OR 3.94 (1.56; 9.95), p = 0.004 and FEV1/FVC ratio: OR 2.05 per 0.1 unit decrease (1.21; 3.52), p = 0.008, respectively).

Conclusions

Both left- and right-sided cardiac alterations were found in pwCF. After adjustments for risk factors, both RV and LV systolic measures remained altered in pwCF, compared to controls. Male sex and decreasing pulmonary function evaluated by FEV1/FVC-ratio were associated with abnormal cardiac function in pwCF.

背景：囊性纤维化（CF）的心脏受累程度仍有待确定。新型高效囊性纤维化跨膜传导调节剂（CFTR）调节剂的治疗取得了显著进展，预期寿命也随之延长，这为进一步研究提供了依据。我们的目的是探讨与匹配对照组相比，囊性纤维化患者（pwCF）心脏改变的发生率，并调查潜在的心血管风险因素：在这项横断面研究中，104 名 CF 患者接受了临床和超声心动图评估。所有参与者均与普通人群中的对照组进行了 1:1 匹配：结果：在 104 名儿童心脏病患者中，44% 为女性，平均年龄为 34 岁，93% 接受过 CFTR 调节剂治疗。患儿心脏功能异常的比例为 44%，是对照组的两倍多。研究发现，pwCF 的左心室（LV）尺寸较小，左心室舒张功能较差，右心室（RV）和左心室收缩功能降低。经多变量调整后，与对照组相比，肥胖症患者的左心室舒张功能以及左心室和右心室收缩功能仍然较差。男性性别和 FEV1/FVC 比率的降低仍与 pwCF 的心功能异常独立相关（男性性别：OR 3.94（1.56））：OR 3.94 (1.56; 9.95)，p = 0.004，FEV1/FVC 比率：结论：结论：在肥胖症患者中发现了左侧和右侧心脏的改变。在对风险因素进行调整后，与对照组相比，慢性阻塞性肺病患者的左心室和左心室收缩功能仍有改变。男性和通过 FEV1/FVC 比值评估的肺功能下降与慢性阻塞性肺病患者的心功能异常有关。

{"title":"Cardiac Structure and Function in People with Cystic Fibrosis","authors":"Lisa Steen Duus , Maria Dons , Rebekka Faber Thudium , Susanne Dam Nielsen , Mette F Olsen , Tavs Qvist , Mats C Højbjerg Lassen , Kristoffer Grundtvig Skaarup , Niklas Dyrby Johansen , Thomas Mørk-Strøm Bluhme , Terese L Katzenstein , Tacjana Pressler , Daniel Faurholt-Jepsen , Tor Biering-Sørensen","doi":"10.1016/j.jcf.2024.09.012","DOIUrl":"10.1016/j.jcf.2024.09.012","url":null,"abstract":"<div><h3>Background</h3><div>The extent of cardiac involvement in cystic fibrosis (CF) remains to be determined. The remarkable therapeutic advancements with new highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator treatment and subsequent increase in life expectancy substantiates further research. We aimed to explore the prevalence of cardiac alterations in people with CF (pwCF) compared to matched controls and investigate potential cardiovascular risk factors.</div></div><div><h3>Methods</h3><div>In this cross-sectional study, 104 pwCF underwent clinical and echocardiographic assessment. All participants were matched 1:1 with controls from the general population.</div></div><div><h3>Results</h3><div>Of 104 pwCF, 44 % were female, mean age was 34 years, and 93 % received CFTR modulator treatment. The prevalence of abnormal cardiac function in pwCF was 44 %, more than double the prevalence in controls. PwCF were found to have smaller left ventricular (LV) dimensions, worse LV diastolic function, and reduced right ventricle (RV) as well as LV systolic function. After multivariable adjustment, LV diastolic function as well as LV and RV systolic function remained poorer in pwCF as compared to controls. Male sex and decreasing FEV1/FVC ratio remained independently associated with abnormal cardiac function in pwCF (male sex: OR 3.94 (1.56; 9.95), <em>p</em> = 0.004 and FEV1/FVC ratio: OR 2.05 per 0.1 unit decrease (1.21; 3.52), <em>p</em> = 0.008, respectively).</div></div><div><h3>Conclusions</h3><div>Both left- and right-sided cardiac alterations were found in pwCF. After adjustments for risk factors, both RV and LV systolic measures remained altered in pwCF, compared to controls. Male sex and decreasing pulmonary function evaluated by FEV1/FVC-ratio were associated with abnormal cardiac function in pwCF.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"23 6","pages":"Pages 1138-1145"},"PeriodicalIF":5.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142347589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Room to breathe: The promise of a more flexible cystic fibrosis care model 呼吸的空间：更灵活的囊性纤维化护理模式的前景。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.09.024

Katherine Bruening Wajda , Alex H. Gifford

引用次数: 0

Creation of a CF-specific antibiotic spectrum index (ASI) as an antimicrobial stewardship initiative 创建 CF 特异性抗生素谱指数 (ASI)，作为抗菌药物管理举措。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.03.004

Jonathan D. Cogen , Sonya L. Heltshe , Adam W. Brothers , Donald R. VanDevanter , Jeffrey S. Gerber , Matthew P. Kronman , Ranjani Somayaji

Antibiotics are frequently utilized for cystic fibrosis (CF)-related pulmonary exacerbation treatment. The antibiotic spectrum index (ASI) is an antimicrobial stewardship tool developed to compare the relative breadth of individual antibiotics. This study aimed to create two expanded CF-specific ASI scoring indices for use in antimicrobial stewardship research and clinical care. The first scoring index expanded the original ASI to include bacterial microorganisms common to CF airway infections (CF-ASI). The second scoring system only included scores for bacterial microorganisms classically identified in CF airway infections (CF-sASI). Sixty-two antibiotics were evaluated and included in the updated ASIs. When multiple antibiotics are prescribed, we proposed using an additive ASI approach whereby the sum of the individual prescribed antibiotic scores represents the total ASI score. The application of CF-focused ASIs into CF research and stewardship programs can help to optimize antibiotic benefits, minimize harms and allow for increased sustainability of antibiotic use in CF.

抗生素经常用于治疗与囊性纤维化（CF）相关的肺部恶化。抗生素谱指数（ASI）是一种抗菌药物管理工具，用于比较各种抗生素的相对广谱性。本研究旨在创建两个针对 CF 的扩展 ASI 评分指数，用于抗菌药物管理研究和临床护理。第一个评分指数扩展了原有的 ASI，纳入了 CF 气道感染常见的细菌微生物（CF-ASI）。第二种评分系统只包括在 CF 气道感染中常见的细菌微生物（CF-sASI）。经评估，有 62 种抗生素被纳入更新的 ASI。当处方中使用多种抗生素时，我们建议使用加法 ASI 方法，即处方中单种抗生素得分之和代表 ASI 总分。将以CF为重点的ASI应用于CF研究和监管计划中，有助于优化抗生素的益处、减少危害并提高抗生素在CF中使用的可持续性。

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引用次数: 0

Olfactory loss in people with cystic fibrosis: Community perceptions and impact 囊性纤维化患者嗅觉丧失:社区认知和影响。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2023.11.006

Jessa E. Miller , Christine M. Liu , Edith T. Zemanick , Jason C. Woods , Christopher H. Goss , Jennifer L. Taylor-Cousar , Daniel M. Beswick

Background

Olfactory dysfunction (OD) is prevalent in people with cystic fibrosis (PwCF) and can negatively impact quality-of-life (QOL). This study evaluated perceptions of OD, investigated how OD impacts QOL, and assessed willingness to participate in OD research among the CF community.

Methods

A 21-question survey was distributed through the CF Foundation's Community Voice program in 2023. The survey included questions on olfaction and interest in research. The Brief Questionnaire of Olfactory Disorders (BQOD), a validated person-reported outcome measure to assess QOL, was included.

Results

Seventy-six responses were received. Overall, 91% (69/76) reported olfactory problems. Mean BQOD score was 5.0 (standard deviation=4.8), indicating olfactory QOL impairment was present. Ninety-five percent (72/76) reported research on OD is worthwhile and were willing to participate in research.

Conclusion

Among PwCF, OD and olfactory-specific QOL impairments are prevalent. There is strong interest and willingness to participate in OD research among the CF community.

背景:嗅觉功能障碍(OD)在囊性纤维化(PwCF)患者中普遍存在，并可能对生活质量(QOL)产生负面影响。本研究评估了CF群体对OD的感知，调查了OD如何影响生活质量，并评估了参与OD研究的意愿。方法:在2023年通过CF基金会的社区之声项目进行了一项包含21个问题的调查。调查的问题包括嗅觉和研究兴趣。嗅觉障碍简要问卷(BQOD)是一种经过验证的评估生活质量的个人报告结果测量方法。结果:共收到76份回复。总体而言，91%(69/76)报告嗅觉问题。平均BQOD评分为5.0(标准差为4.8)，表明存在嗅觉生活质量下降。95%(72/76)的人表示对吸毒过量的研究是值得的，并且愿意参与研究。结论:在PwCF患者中，OD和嗅觉特异性生活质量损害较为普遍。CF社区对参与OD研究有强烈的兴趣和意愿。

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引用次数: 0

Clinical outcomes after liver transplant in people with cystic fibrosis: A systematic review and meta-analysis 囊性纤维化患者肝移植后的临床疗效：系统回顾和荟萃分析。

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis

Pub Date : 2024-11-01 DOI: 10.1016/j.jcf.2024.06.012

Faisal A. Albaiz , Julie Semenchuk , Xiayi Ma , David Lightfoot , Tanja Gonska , Elizabeth Tullis , Anne L. Stephenson

Background

Data on the impact of liver transplantation (LT) in cystic fibrosis (CF) on lung function and exacerbations are limited. The objective of this study was to summarize the literature on lung function, nutritional status, survival, and complications following LT in people with CF.

Methods

Three databases were searched until September 2023, to identify the impact of LT in CF. Lung transplant prior to LT and simultaneous liver-lung transplant were excluded. Pooled hazard ratios were calculated using random-effects models.

Results

Thirty studies were included in this review, with 3 and 9 studies included in meta-analyses for nutritional status and lung function, respectively. Eighty-three percent of the studies used data that was more than a decade old. There was a significant increase in percent-predicted forced expiratory volume with mean change of 7.16 % (2.13, 12.19; p = 0.005) one year post-LT. Pulmonary exacerbations decreased in the short-term, however there was no significant change in body mass index (BMI). One-year survival post-LT ranged between 75 and 100 %, while five-year survival was lower at 64–89 %.

Conclusion

Existing data suggest that LT improves lung function in the short term and does not increase the likelihood of pulmonary exacerbations, despite ongoing immunosuppression in the setting of chronic lung infection.

背景：有关囊性纤维化（CF）患者肝移植（LT）对肺功能和病情恶化影响的数据十分有限。本研究旨在总结有关CF患者肝移植后肺功能、营养状况、存活率和并发症的文献：方法：检索了三个数据库，以确定LT对CF患者的影响。不包括LT之前的肺移植和同时进行的肝肺移植。采用随机效应模型计算汇总危险比：本综述纳入了 30 项研究，其中 3 项和 9 项研究分别纳入了营养状况和肺功能的荟萃分析。83%的研究使用了十多年前的数据。LT 一年后，预测用力呼气容积百分比明显增加，平均变化率为 7.16% (2.13, 12.19; p = 0.005)。肺部恶化在短期内有所减少，但体重指数（BMI）没有明显变化。LT术后一年的存活率在75%至100%之间，而五年存活率较低，为64%至89%：现有数据表明，尽管在慢性肺部感染的情况下免疫抑制仍在持续，但LT在短期内可改善肺功能，并且不会增加肺部恶化的可能性。

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引用次数: 0