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门肺高压:联合药物治疗取得成功

Portopulmonary hypertension: Success with combined medical treatment.

作者信息

Hernández María, Esteban Violeta, Ruiz Paloma, Boira Ignacio, Wickmann Phillip, Sancho-Chust José N, Gil Joan

机构信息

Department of Respiratory Medicine Hospital Universitario de San Juan Alicante Spain.

Department of Respiratory Medicine Hospital General Universitario de Alicante Alicante Spain.

出版信息

Respirol Case Rep. 2023 Mar 16;11(4):e01114. doi: 10.1002/rcr2.1114. eCollection 2023 Apr.

Abstract

Portopulmonary hypertension is an uncommon disease associated with high morbidity and mortality, so its early diagnosis and treatment are essential. We report here the case of a 57-year-old man with portopulmonary hypertension caused by chronic hepatosplenic schistosomiasis and also liver cirrhosis due to hepatitis C and alcoholism. As well as treating both diseases, portopulmonary hypertension was successfully managed with tadalafil and macitentan as maintenance therapy. This case reminds clinicians that pulmonary hypertension can be multifactorial, a good diagnosis and a multidisciplinary treatment can lead to improved prognosis.

摘要

门肺高压是一种罕见疾病,发病率和死亡率高,因此早期诊断和治疗至关重要。我们在此报告一例57岁男性患者,其门肺高压由慢性肝脾血吸虫病引起,同时伴有丙型肝炎和酒精性肝硬化。除了对这两种疾病进行治疗外,使用他达拉非和马西替坦作为维持治疗成功控制了门肺高压。该病例提醒临床医生,肺动脉高压可能是多因素导致的,良好的诊断和多学科治疗可改善预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4beb/10020675/f7adcb690241/RCR2-11-e01114-g004.jpg

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