Pulmonary Arterial Hypertension and Intraductal Papillary Mucinous Neoplasms of the Pancreas: A Novel Association?
Pulmonary Arterial Hypertension and Intraductal Papillary Mucinous Neoplasms of the Pancreas: A Novel Association?
复制标题
肺动脉高压与胰腺导管内乳头状粘液性肿瘤：一种新的关联？
DOI：
10.1016/j.chest.2021.06.078
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发表时间：
2021
期刊：
影响因子：
9.6
通讯作者：
Bull,ToddM
中科院分区：
文献类型：
--
作者：
Lee,MichaelH;Doran,Jennifer;Bang,TamiJ;Hohsfield,Robin;Hountras,Peter;Boddie,Genevieve;Wagh,MihirS;Badesch,David;Bull,ToddM
方法我们强调了来自科罗拉多大学肺动脉高压诊所的6例患者的关键特征，所有患者均接受了≥ 10年的PAH治疗，随后根据影像学特征诊断为IPMN。我们还讨论了独特的方面的临床管理，并提出潜在的统一机制，这两种diseases.ResultsWe确定了6例PAH患者在大学的科罗拉多医院在1996年和2021年之间，四个IPAH，门脉高压症，和一个PAH引起的未分化的全身性风湿性疾病。我们的诊所每年约有3,000人次就诊;其中，每年约有65名新转诊的患者被诊断为PAH。这6例患者患有重度肺动脉高压，需要多种药物治疗，包括静脉前列环素（PGI 2）类似物治疗多年（14±4.9年）（表1）。最常见的症状包括腹痛、腹泻、恶心、呕吐、体重减轻、厌食和疲劳。在CT或磁共振胰胆管造影（MRCP）上可以看到IPMN的证据（图1）。
MethodsWe highlight key characteristics of six patients from the pulmonary hypertension clinic at the University of Colorado, all managed for≥ 10 years with PAH, who were subsequently diagnosed with IPMN based on radiographic features. We also discuss unique aspects of clinical management and propose potential unifying mechanisms underlying both diseases.ResultsWe identified six PAH patients treated at the University of Colorado Hospital between 1996 and 2021, four with IPAH, one with portopulmonary hypertension, and one with PAH caused by undifferentiated systemic rheumatic disease. Our clinic sees approximately 3,000 visits annually; of these, approximately 65 newly referred patients are diagnosed with PAH each year. These six patients had severe pulmonary hypertension requiring multiple medications, including IV prostacyclin (PGI 2) analog therapy for many (14±4.9 years (Table 1). The most common symptoms included abdominal pain, diarrhea, nausea, vomiting, weight loss, anorexia, and fatigue. Evidence of IPMN was seen on CT or magnetic resonance cholangiopancreatography (MRCP)(Fig 1).