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肺动静脉畸形:诊断和治疗特征。

Pulmonary arteriovenous malformations: diagnostic and treatment characteristics.

机构信息

. Divisão de Pneumologia, Instituto do Coração - InCor - Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo (SP) Brasil.

. Serviço de Radiologia Intervencionista, Instituto do Coração - InCor - Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo (SP) Brasil.

出版信息

J Bras Pneumol. 2019 Jun 19;45(4):e20180137. doi: 10.1590/1806-3713/e20180137.

Abstract

OBJECTIVE

To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered.

METHODS

This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM).

RESULTS

A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection.

CONCLUSIONS

In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.

摘要

目的

介绍一组肺动静脉畸形(PAVM)病例,描述主要临床发现、肺血管异常的数量/位置、临床并发症和治疗措施。

方法

这是一项回顾性观察性研究,评估了分为遗传性出血性毛细血管扩张症(HHT)和特发性 PAVM(iPAVM)两组的 PAVM 患者。

结果

共选择了 41 例患者纳入研究,但仅有 33 例患有 PAVM。经过临床评估,分别诊断为 HHT 和 iPAVM 的患者有 27 例和 6 例。在 HHT 组中,平均年龄为 49.6 岁,88.9%为女性。在该组中,有 4 例患者的 SpO2<90%,最常见的临床发现是鼻出血。在 iPAVM 组中,平均年龄为 48.1 岁,83.3%为女性。在该组中,有 3 例患者的 SpO2<90%。计算机断层肺动脉造影显示,大多数 PAVM 位于下叶:HHT 组为 56.4%,iPAVM 组为 85.7%。对 23 例患者(两组均有)进行了栓塞治疗。在撰写本文时,有 10 例患者计划接受该手术。接受栓塞治疗的患者中有 1 例随后转至肺切除术。

结论

在两组 PAVM 患者中,女性和下叶瘘均占主导地位。少数患者有呼吸系统症状,大多数患者的 SpO2>90%。所有患者选择的治疗方法均为经皮经导管栓塞。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/550a/6733713/659d5cc2a303/1806-3713-jbpneu-45-04-e20180137-gf1.jpg

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