遗传性结缔组织疾病所致气胸 844 例的治疗及转归。

Treatment and Outcomes of 844 Cases of Pneumothorax in Heritable Connective Tissue Disorders.

机构信息

Department of Pediatric Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan; Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan.

Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan.

出版信息

Ann Thorac Surg. 2024 Dec;118(6):1187-1195. doi: 10.1016/j.athoracsur.2024.05.037. Epub 2024 Jun 13.

DOI:10.1016/j.athoracsur.2024.05.037

PMID:38878950

Abstract

BACKGROUND

Characteristics, practice patterns, and clinical outcomes of secondary spontaneous pneumothorax (SSP) with heritable connective tissue disorders (Marfan, Ehlers-Danlos, and Birt-Hogg-Dubé syndromes) are unclear.

METHODS

A nationwide Japanese inpatient database that included data of 524 patients with SSP (884 hospitalizations) and 137,821 with primary spontaneous pneumothorax between July 2010 and March 2020 was used in this study. Hospitalization for SSP (n = 884) was categorized into surgical (n = 459) and nonsurgical (n = 425) groups, and the patient characteristics, treatment, and outcomes were compared between the groups. Multivariable analyses were performed to evaluate risk factors for pneumothorax recurrence. We also compared the characteristics of patients with different underlying heritable connective tissue disorders.

RESULTS

Compared with the nonsurgical group, the surgical group had less frequent readmission for pneumothorax (26% vs 44%; hazard ratio, 0.47; 95% CI, 0.38-0.58). Young patients (2.46; 1.83-3.32) or those with Birt-Hogg-Dubé syndrome (2.53; 1.77-3.63) had a high risk of recurrence. Pneumothorax occurred frequently in teenagers with Marfan syndrome, in those aged 20 to 39 years with Ehlers-Danlos syndrome, and in those aged ≥40 years with Birt-Hogg-Dubé syndrome.

CONCLUSIONS

Detailed information on the characteristics and clinical course of SSP in heritable connective tissue disorders will aid in the clinical decision-making process.

摘要

背景

遗传性结缔组织疾病（马凡氏综合征、埃勒斯-当洛斯综合征和 Birt-Hogg-Dubé 综合征）相关的继发性自发性气胸（SSP）的特征、治疗模式和临床结局尚不清楚。

方法

本研究使用了一个全国性的日本住院患者数据库，该数据库包含了 2010 年 7 月至 2020 年 3 月期间 524 例 SSP 患者（884 例住院）和 137821 例原发性自发性气胸患者的数据。将 SSP 的住院治疗（n=884）分为手术（n=459）和非手术（n=425）两组，比较两组患者的特征、治疗和结局。采用多变量分析评估气胸复发的危险因素。我们还比较了不同潜在遗传性结缔组织疾病患者的特征。

结果

与非手术组相比，手术组气胸复发的再入院率较低（26% vs 44%；风险比，0.47；95%置信区间，0.38-0.58）。年轻患者（2.46；1.83-3.32）或 Birt-Hogg-Dubé 综合征患者（2.53；1.77-3.63）复发风险较高。马凡氏综合征的青少年、埃勒斯-当洛斯综合征的 20 至 39 岁患者和 Birt-Hogg-Dubé 综合征的≥40 岁患者气胸发生率较高。