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开拓视野:非特发性肺纤维化间质性肺疾病的肺移植。

Expanding horizons: lung transplantation for non-IPF interstitial lung diseases.

机构信息

Department of Thoracic Surgery, Kartal Kosuyolu High Specialization Education & Research Hospital, Istanbul, Turkey.

Department of Pulmonology, Kartal Kosuyolu High Specialization Education & Research Hospital, Istanbul, Turkey.

出版信息

BMC Pulm Med. 2024 Oct 2;24(1):482. doi: 10.1186/s12890-024-03291-4.

Abstract

OBJECTIVE

Interstitial lung diseases (ILDs) are diverse pulmonary disorders marked by diffuse lung inflammation and fibrosis. The variability in characteristics and treatment approaches complicates diagnosis and management. In advanced cases requiring transplantation, determining indications and selecting suitable candidates presents additional challenges.

METHODS

Of all patients with non-IPF ILD between December 2016 to December 2022 were analyzed retrospectively. Patients were categorized into two groups: transplanted patients and deceased patients on the waiting list. Clinical data and survival outcomes were compared between groups.

RESULTS

Of the 43 patients, 20 underwent lung transplantation while 23 died awaiting transplantation. Waiting list mortality was 53.4%, with median waiting times similar between groups (3 months for transplant patients and 6 months for those on the waiting list). There were no significant differences between groups in age, gender, height, BMI, 6-minute walk test (6MWT), or forced vital capacity (FVC). The prevalence of pulmonary hypertension (PH) was 76.7% in right heart catheterizations, similar in both groups. One single and 19 bilateral lung transplants were performed. Overall, 13 of the 20 patients survived to discharge from the hospital. One-year mortality was 7/20 (35%). The median follow-up was 34 months, with a 1-year conditional survival of 90.9% at 3 years and 70.7% at 5 years.

CONCLUSIONS

This study underscores the importance of further research into non-IPF ILDs. Lung transplantation remains a viable option that can significantly enhance both the quality and longevity of life for patients with advanced ILD.

摘要

目的

间质性肺疾病(ILDs)是多种肺部疾病的统称,其特征为弥漫性肺炎症和纤维化。由于这些疾病的特征和治疗方法存在差异,因此诊断和管理变得复杂。在需要移植的晚期病例中,确定适应证和选择合适的患者会带来更多挑战。

方法

回顾性分析了 2016 年 12 月至 2022 年 12 月期间所有非特发性肺纤维化(IPF)ILD 患者。将患者分为两组:接受肺移植的患者和等待移植名单上死亡的患者。比较两组间的临床数据和生存结果。

结果

43 例患者中,20 例行肺移植,23 例在等待移植期间死亡。等待名单上的死亡率为 53.4%,两组间的中位等待时间相似(移植患者为 3 个月,等待名单上的患者为 6 个月)。两组间在年龄、性别、身高、BMI、6 分钟步行试验(6MWT)或用力肺活量(FVC)方面无显著差异。在右心导管检查中,肺动脉高压(PH)的患病率为 76.7%,两组间相似。共进行了 1 次单肺和 19 次双肺移植。总体而言,20 例患者中有 13 例出院后存活。1 年死亡率为 7/20(35%)。中位随访时间为 34 个月,1 年条件生存率为 90.9%(3 年)和 70.7%(5 年)。

结论

本研究强调了进一步研究非特发性肺纤维化ILD 的重要性。肺移植仍然是一种可行的选择,可以显著提高晚期ILD 患者的生活质量和寿命。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/94d3/11448434/6841f2931998/12890_2024_3291_Fig1_HTML.jpg

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