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題 名 | Familial Bartter's Syndrome--A Study of Free Water Clearance=家族氏巴特氏症候群--水廓清率的研究 |
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作 者 | 李宗德; | 書刊名 | 中華民國小兒科醫學會雜誌 |
卷 期 | 32:6 民80.11-12 |
頁 次 | 頁365-371 |
分類號 | 417.5934 |
關鍵詞 | 家族氏巴特氏症候群; 水廓清率; |
語 文 | 英文(English) |
中文摘要 | 有一家族的三個兄弟姊妹因身材矮小而入院接受檢查,理學檢查並無特殊發現,血壓也正常,血液檢查發現有低血鉀,代謝性鹼血症,高腎素血症,哥哥的血液檢查並有低血鈉及高皮賓醛酮血症,所有實驗室檢查結果符合巴特氏症候羣。為了瞭解巴特氏症候羣的致病機轉,我們做了水廓清率(free water clearance)的研究,結果顯示遠端腎小管對氯離子重吸收(distal fractional chloride reabsorption)降低;哥哥的近端腎小管對鈉的重吸收(proximal sodium reabosrption)是正常的,而弟弟妹妹的近端腎小管對鈉的重吸收有代償性增強,從以上的數據顯示巴特氏症候羣的致病機轉主要是在於亨利氏環(Henle’s loop)對氯離子的重吸收障礙,而近端腎小管對鈉離子重吸收的代償性增強,對於維持血液中鈉濃度的正常是非常重要的。 |
英文摘要 | Three siblings in one family were found to have Banter's syndrome. Ale of them had persistent hypokalemia, metabolic alkalosis, hyperreninemia and normal blood pressure. Patient 1 also had hyponatremia and hyperaldosteronism. Free water clearance studies daring a maximal water diuresis showed that all of them had mild decrease in distal fractional chloride reabsorption. Patient I had normal proximal sodium reabsorption, however, patient 2 and patient 3 had enhanced proximal sodium reabsorption. These data suggest that the defect in chloride reabsorption at the Henle's loop may contribute to the pathogenesis of Bartter's syndrome, and compensatory proximal sodium reabsorption is very important in maintaining normal plasma sodium in Banter's syndrome. |
本系統中英文摘要資訊取自各篇刊載內容。