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- Clinical Aspects of Bartter's Syndrome in Taiwanese
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題名 | Clinical Aspects of Bartter's Syndrome in Taiwanese=巴特氏症候群在臺灣的臨床表現 |
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作者 | 蘇育禎; 林清淵; Su, Yu-chen; Lin, Ching-yuang; |
期刊 | 臺灣腎臟醫學會雜誌 |
出版日期 | 20010600 |
卷期 | 15:2 2001.06[民90.06] |
頁次 | 頁42-48+87 |
分類號 | 415.934 |
語文 | eng |
關鍵詞 | 巴特氏症候群; 生長遲緩; 代謝性鹼中毒; Batter's syndrome; Clinical aspects; Growth failure; |
中文摘要 | 巴特氏症候群為一十分罕見疾病,臨床上的表現主要為生長遲緩、低血鉀、代謝性鹹中毒、高醛類脂醇血症及正常血壓,迄今,台灣尚未見到此病的臨床追蹤報告。我們將過去12年間經歷過的5位病人做回顧性的整理及研究,其中,有2位男性,3位女性,所有病人均為個別病例,並無家族病史。所有病人都呈現典型的巴特氏症候群的理學及生化檢驗特微,包括生長遲緩、多尿、肌肉無力、低血鉀、代謝性鹹中毒、血清腎素活性及血清醛類脂醇濃度明顯升高,以及鈉排泄率及24小時尿中PGE2濃度升高。3位病人曾有脫水現象,2位合併低血清鎂,所有病人在治療後生長狀況均有改善。 |
英文摘要 | Bartter’s syndrome is a rare disease with growth retardation, hypokalemia, metabolic alkalosis, hyperaldosteronism, and normal blood pressure. Until now there is no clinical follow-up data of Bartter’s syndrome in Taiwan. We retrospectively studied 5 patients (2 males and 3 females) of Bartter’s syndrome in our hospital in the past 12 years to delineate the clinical aspects of this disease. All five patients appeared to be sporadic cases with no family history. All five patients had growth retardation, polyuria, polydipsia, muscular weakness, hypokalemia, metabolic alkalosis, significantly elevated plasma rennin activity and plasma aldosterone level, normal serum sodium, chloride and calcium levels, elevated FENa and increased urinary PGE2. Three of five patients had dehydration. Two of five patients had hypomagnesemia. Three of five patients had decreased 24-hours CCr. 4 patients had sufficient follow-up growth data. After treatment, all patients had improvement over body height and weight. |
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