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題 名 | 以主動脈竇動脈瘤及瓣膜性病變為心臟表現的Behcet's疾病=Behcet's Disease Complicating with Aneurysm of Aortic Sinus of Valsava and Valvular Heart Disease |
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作 者 | 陳正熙; 吳懿哲; 侯嘉殷; 周友三; 蔡正河; | 書刊名 | 內科學誌 |
卷 期 | 13:3 2002.06[民91.06] |
頁 次 | 頁128-132 |
分類號 | 415.649 |
關鍵詞 | Behcet's疾病; 主動脈竇動脈瘤; 瓣膜性心臟病; Behcet's disease; Aneurysm of aortic sinus of valsalva; Valvular heart disease; |
語 文 | 中文(Chinese) |
中文摘要 | 17α-羥化酉每缺乏是先天性腎上腺增生及內分泌高血壓中少見的原因 , 文獻中 只有約 124 例。一位 35 歲臺灣人女性有嚴重高血壓 (220/130mmHg) 、缺乏第 二性徵及原發性無月經。染色體分析顯示 46XX, 實驗室數據 : 低血鉀、血漿腎 素活性抑制、性類固醇低而性促素高、早晨血中皮質醇濃度 :1.8 μ g/dl 而腎上 腺皮促素濃度:235pg/ml 。其血漿中醛酮濃度正常但偏高。我們診斷為 17 α -羥化酉每缺乏。之前大部分的病例血漿中醛酮濃度都受抑制 , 然而亦有為數不少者 為正常或高。我們發現她的子宮發育不全 , 在這之前的女性 (46XX) 病患僅有一 例類似的報告。結論 : 我們報告了一例 17 α -羥化酉每缺乏的女性 , 其血漿醛酮濃度正常且子宮 發育不全。 |
英文摘要 | Background: 17α-hydroxylase deficiency is a rare cause of congenital adrenal hyperplasia and endocrine hypertension. Only around 124 cases were reported in literature. Case: A 35-year-old Taiwanese female presented with severe hypertension (220/130 mmHg), absence of secondary sexual characteristics and primary amenorrhea. Chromosome study revealed 46XX karyotye. The laboratory data revealed hypokalemia, suppressed plasma renin activity, low level of sex steroids with high gonadotropin, morning cortisol level: 1.8μg/dl and ACTH: 235 pg/ml. Her plasma level of aldosterone (sitting position) was at high level of normal (23.6 ng/dl). We made the diagnosis of 17α-hydroxylase deficiency. Plasma aldosterone level was suppressed in most of the reported ca-ses while normal or elevated levels were also described in a considerable number. Her uterus was found to be rudimentary, which was reported in only one genetic female case before. Conclusion : We report a case of 17α-hydroxylase deficiency in genetic female with normal le-vel of plasma aldosterone and rudimentary uterus. |
本系統中英文摘要資訊取自各篇刊載內容。