頁籤選單縮合
題名 | A Case of 17α-Hydroxylase Deficiency in Genetic Female with Normal Plasma Aldosterone Level and Rudimentary Uterus=一例17α-羥化酶缺乏的女性其血漿醛酮濃度正常且子宮發育不全 |
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作者 | 顏韶宏; 陳宏德; | 書刊名 | 內科學誌 |
卷期 | 13:3 2002.06[民91.06] |
頁次 | 頁141-146 |
分類號 | 415.939 |
關鍵詞 | 17α-羥化酶缺乏; 血漿醛酮濃度; 子宮發育不全; 17α-hydroxylase deficiency; Aldosterone; Rudimentary uterus; Secondary hypertension; Hypogonadism; |
語文 | 英文(English) |
中文摘要 | Behet's疾病的病理表現為血管炎,主要是以反覆發生口腔潰爛、生殖器潰瘡及皮膚病變為主要表現,但亦會侵犯身上其他各種系統,一旦此疾病影響到中樞神經系統、心臟血管系統或腸胃系統,通常預後不好。本文報告一位46歲男性病患,四年前被診斷有Behet's疾病,主訴最近三個月發生運動時呼吸困難、喘、胸口不適等症狀,入院後一系列檢查發現病患為一 Behet's疾病侵犯心血管,造成主動脈竇動脈瘤,以及嚴重瓣膜性心臟病(主動脈脫垂及嚴重逆流、二尖瓣、三尖瓣中度逆流)併發心臟衰竭。在加強免疫療法之下,建議病患進一步接受手術治療。由於病患的猶豫、延遲,在未接受手術前,即在院外發生摔死。本文從文獻回顧來瞭解Behet's疾病對心臟血管系統各方面的影響及預後,心臟方面包括心包炎、心肌炎、心內膜炎、傳導系統阻滯、心房顫動、心室心搏過速及顫動、冠狀動脈疾病、急性心肌梗塞、瓣膜性疾病。血管方面包括靜脈栓塞、動脈栓塞及動脈瘤。如能提高警覺、早期診斷、早期積極治療,必要時佐以外科治療、並密切追蹤檢查,期能降低病人的死亡率、延長生命。 |
英文摘要 | Behet's disease, a multisystem disorder, is characterized by recurrent oral and genital aphthous ulceration, eye lesions, and skin changes, whose pathologic feature is vasculitis. Prognosis in Behet's disease depends on the presence of CNS, cardiovascular or intestinal involvement. This report describes a patient who had been diagnosed as Behet's disease for four years, and had complained of dyspnea, short of breath on exertion and chest discomfort since three months prior to admission. A series of examinations demonstrated that the patient had Behet's disease complicating with aneurysm of aortic sinus of Valsalva and severe valvular heart disease, which resulted in congestive heart failure. Although being suggested to receive intensive immunologic therapy and surgical intervention as soon as possible, this patient still succumbed to sudden death, most likely caused by disastrous rupture of the aneurysm, during his waiting for operation. According to the literature review, we discussed the influence and prognosis of cariodvasculo-Behet's disease. The cardiac manifestations include pericarditis, myocarditis, endocarditis, conduction system disturbances, atrial fibrillation, ventricular tachycardia and fibrillation, coronary artery disease, acute myocardial infarction and valvular heart disease. Three major manifestations of vasculo-Behet's disease are venous occlusion, arterial occlusion and aneurysm formation. Literature reveals that high alertness, early diagnosis and prompt treatment, including surgical treatment if necessary, and prudent patient follow up would lead to improvement of mortality and prolongation of life. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。