CAJ | 학술논문

目的初步探讨遗传性出血性毛细血管扩张症相关性肺动脉高压的临床特征.方法回顾性分析6例临床诊断为遗传性出血性毛细血管扩张症相关性肺动脉高压的临床表现.结果 6例患者年龄8～67岁,平均34岁,均有反复鼻出血病史,4例诊断为重度肺动脉高压,2例诊断为中度肺动脉高压.6例患者首次入院诊断均未明确肺动脉高压原因.除2例合并弥漫肝动静脉瘘患者外,其余4例经内科治疗后症状改善明显.结论遗传性出血性毛细血管扩张症合并肺动脉高压易误诊,需对其相应的病症采用及时有效的治疗.
목적 초보탐토유전성출혈성모세혈관확장증상관성폐동맥고압적림상특정.방법 회고성분석6례림상진단위유전성출혈성모세혈관확장증상관성폐동맥고압적림상표현.결과 6례환자년령8～67세,평균34세,균유반복비출혈병사,4례진단위중도폐동맥고압,2례진단위중도폐동맥고압.6례환자수차입원진단균미명학폐동맥고압원인.제2례합병미만간동정맥루환자외,기여4례경내과치료후증상개선명현.결론 유전성출혈성모세혈관확장증합병폐동맥고압역오진,수대기상응적병증채용급시유효적치료.
Objective To investigate the clinical manifestations of patients with pulmonary artery hypertension (PAH) associated with hereditary hemorrhagic telangiectasia (HHT). Methods This retrospective analysis summarized the clinical features of 6 patients with PAH associated with HHT hospitalized at department of cardiology in Cardiovascular Institute and Fuwai Hospital between January 2006 and May 2009. Results The mean age of the 6 patients (3 male) was 34 years (8 -67years). Recurrent epistaxis were present in all patients, there were 4 patients with severe PAH and 2 patients with moderate PAH. All of the six patients with PAH associated with HHT were misdiagnosed at the first hospital visit.Clinical symptoms were significantly improved in 4 patients and remained unchanged in 2 patients combined hepatic venous malformation post medical therapy. Conclusions Misdiagnosis for patients with PAH associated with HHT is a common phenomenon in daily clinical practice. Patients could benefit from the corresponding medical therapy after the establishment of the correct diagnosis.