Risk of spontaneous pneumothorax due to air travel and diving in patients with Birt-Hogg-Dubé syndrome.
Risk of spontaneous pneumothorax due to air travel and diving in patients with Birt-Hogg-Dubé syndrome.
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DOI：
10.1186/s40064-016-3009-4
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发表时间：
2016
期刊：
影响因子：
--
通讯作者：
Postmus PE
中科院分区：
文献类型：
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作者：
Johannesma PC;van de Beek I;van der Wel JW;Paul MA;Houweling AC;Jonker MA;van Waesberghe JH;Reinhard R;Starink TM;van Moorselaar RJ;Menko FH;Postmus PE
birt - hogg - dub<s:1>综合征是一种常染色体显性遗传病，以种系卵泡蛋白（FLCN）突变引起的皮肤纤维滤泡瘤、肺囊肿、自发性气胸和肾细胞癌为特征（Menko等人在Lancet Oncol 10(12): 1199-1206）。本研究的目的是评估BHD患者在航空旅行和潜水期间或之后不久发生自发性气胸的发生率。对190例BHD患者进行问卷调查，并对这些患者的医疗档案进行评估。所有患者的FLCN突变分析证实了BHD的诊断。我们评估了在航空旅行或潜水后1个月内发生的自发性气胸（SP）的数量。158例（83.2%）患者填写了问卷。共有145名患者有航空旅行史。其中有SP病史61例（42.1%），平均2.48次（范围1 ~ 10）。24例（35.8%）患者有双侧气胸病史。13例（9.0%）患者在航空旅行后出现SP < 1个月，2例（3.7%）患者在潜水后出现SP < 1个月。我们发现，在这些BHD患者中，每次飞行的气胸风险为0.63%，每次潜水的气胸风险为0.33%。30例（20.7%）患者在航空旅行后出现可能与SP有关的症状，10例（18.5%）患者在潜水后出现可能与SP有关的症状。根据这项回顾性研究的结果，BHD患者暴露于与飞行和潜水相关的相当大的大气压变化可能与发生症状性气胸的风险增加有关。飞行和潜水期间或之后不久报告的症状可能与气胸的早期阶段有关。应考虑到患者的偏好和需求，给出个性化的建议。
Birt–Hogg–Dubé syndrome is an autosomal dominant disorder characterized by skin fibrofolliculomas, lung cysts, spontaneous pneumothorax and renal cell cancer due to germline folliculin (FLCN) mutations (Menko et al. in Lancet Oncol 10(12):1199–1206,). The aim of this study was to evaluate the incidence of spontaneous pneumothorax in patients with BHD during or shortly after air travel and diving. A questionnaire was sent to a cohort of 190 BHD patients and the medical files of these patients were evaluated. The diagnosis of BHD was confirmed by FLCN mutations analysis in all patients. We assessed how many spontaneous pneumothoraces (SP) occurred within 1 month after air travel or diving. In total 158 (83.2 %) patients returned the completed questionnaire. A total of 145 patients had a history of air travel. Sixty-one of them had a history of SP (42.1 %), with a mean of 2.48 episodes (range 1–10). Twenty-four (35.8 %) patients had a history of pneumothorax on both sides. Thirteen patients developed SP < 1 month after air travel (9.0 %) and two patients developed a SP < 1 month after diving (3.7 %). We found in this population of BHD patients a pneumothorax risk of 0.63 % per flight and a risk of 0.33 % per episode of diving. Symptoms possible related to SP were perceived in 30 patients (20.7 %) after air travel, respectively in ten patients (18.5 %) after diving. Based on the results presented in this retrospective study, exposure of BHD patients to considerable changes in atmospheric pressure associated with flying and diving may be related to an increased risk for developing a symptomatic pneumothorax. Symptoms reported during or shortly after flying and diving might be related to the early phase of pneumothorax. An individualized advice should be given, taking also into account patients’ preferences and needs.