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題 名 | Severe Portopulmonary Hypertension in Congenital Hepatic Fibrosis=嚴重門脈肺動脈高血壓併發於一先天性肝纖維化之病例 |
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作 者 | 許振銘; 邱正堂; 連昭明; 吳桂芳; | 書刊名 | 長庚醫學 |
卷 期 | 26:3 2003.03[民92.03] |
頁 次 | 頁193-198 |
分類號 | 415.384 |
關鍵詞 | 先天性肝纖維化; 肺動脈高血壓; 門脈高血壓; 網狀肺動脈病變; Congenital hepatic fibrosis; Pulmonary hypertension; Portal hypertension; Plexogenic pulmonary arteriopathy; |
語 文 | 英文(English) |
中文摘要 | 門脈肺動脈高血壓(portopulmonary hypertension)為門脈高壓一不常見之併發症。雖然一些藥物如epoprostenol灌注,一氧化氮吸入,isosorbide-5-mononitrate,以及鈣離子阻斷劑,可?低門脈肺動脈高血壓患者之肺動脈壓力,有症狀之患者其預後仍然很差。我們報告一罕見之先天性肝纖維化(congenital hepatic fibrosis)併發門脈肺動脈高血壓病例。一位42歲之先天性肝纖維化男性患者因昏倒而到本院求治。病患於此之前有呼吸困難的症狀二週。他於28歲就已知有門脈高血壓所造成的骨靜脈瘤。病患於一周後因重度右心室衰竭死亡。死亡解剖發現大體上右心房與其右心室擴大,顯微鏡下呈現網狀肺動脈病變(plexogenic pulmonary arteriopathy)。對於有喘症狀之門脈高血壓患者,要診斷出門脈肺動脈高血壓需要察覺到此一疾病與臨床上存在高度懷疑。 |
英文摘要 | Portopulmonary hypertension is a rare complication of portal hypertension. Although epoprostenol infusion, nitric oxide inhalation, isosorbide-5-mononitrate, nitroglycerin, and calcium channel blockers may reduce pulmonary artery pressure in patients with portopulmonary hypertension, the prognosis remains poor. We present a case of congenital hepatic fibrosis associated with pulmonary hypertension. A 42-year-old man with congenital hepatic fiberosis visited our hospital with syncope. The man had suffered from breathlessness on exertion for 2 weeks before the episode of syncope. He also had a history of portal hypertension with documented gastric cardiac varices at the age of 28 years. Despite undergoing intensive care, the patient died 1 week after admission owing to severe right-sided heart failure. Autopsy revealed dilatation of the right atrium and right ventricle grossly and plexogenic pulmonary arteriopathy microscopically. Accurate diagnosis of portopulmonary hypertension requires awareness of the disease and a high index of suspicion when examining patients with portal hypertension and dyspnea. |
本系統中英文摘要資訊取自各篇刊載內容。