文章摘要
刘晓云,许馨予,王知笑,任海滨,杨 涛,孙 敏,张 梅,段 宁,周红文.Gitelman综合征14例临床特征分析[J].南京医科大学学报,2011,(7):1045~1049
Gitelman综合征14例临床特征分析
Clinical analysis of 14 cases of Gitelman syndrome
投稿时间:2011-01-17  
DOI:10.7655
中文关键词: Gitelman综合征  低钾血症  低镁血症  血浆肾素活性
英文关键词: Gitelman syndrome  hypokalemia  hypomagnesaemia  plasma renin activity
基金项目:国家自然科学基金(30671010,30971405)
作者单位
刘晓云 南京医科大学第一附属医院内分泌科,江苏 南京 210029 
许馨予 南京医科大学第一附属医院内分泌科,江苏 南京 210029 
王知笑 南京医科大学第一附属医院内分泌科,江苏 南京 210029 
任海滨 南京医科大学第一附属医院肾科,江苏 南京 210029 
杨 涛 南京医科大学第一附属医院内分泌科,江苏 南京 210029 
孙 敏 南京医科大学第一附属医院内分泌科,江苏 南京 210029 
张 梅 南京医科大学第一附属医院内分泌科,江苏 南京 210029 
段 宁 南京医科大学第一附属医院内分泌科,江苏 南京 210029 
周红文 南京医科大学第一附属医院内分泌科,江苏 南京 210029 
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中文摘要:
      目的:分析Gitelman综合征(GS)的临床特征,以提高该病的诊治水平?方法: 回顾性分析南京医科大学第一附属医院内分泌科近5年住院诊治的14例GS病例资料?结果:患者发病年龄13~54(32.9 ± 12.0)岁,男5例,女9例?病程最短为1个月,最长为15年?体质指数(BMI):(21.0 ± 2.9) kg/m2,收缩压:(109.6 ± 10.8) mmHg,舒张压(69.6 ± 6.7) mmHg?临床上以双下肢无力?发作性四肢软瘫?多饮?多尿?夜尿增加?手足抽搐等为主要表现?实验室检查均表现为低血钾?低血镁?低尿钙?高尿钾?代谢性碱中毒?卧立位试验结果显示:卧位及立位血浆肾素活性(PRA)?血管紧张素Ⅱ和醛固酮有不同程度升高?肾脏穿刺病理提示球旁器增生(3/4)?补钾补镁?联合安体舒通等药物治疗后症状缓解,但血钾(11/14)?血镁(10/14)未升至正常水平?结论: GS的临床特征包括:低血钾?低血镁?低尿钙?正常偏低血压以及高PRA?高血管紧张素Ⅱ?高醛固酮和高血气pH值,可以总结为“四低四高”?本病治疗以补钾补镁为主,可以联合应用醛固酮拮抗剂,一般预后良好?
英文摘要:
      Objective:To analyze the clinical features of Gitelman syndrome(GS) in order to improve our understanding of this syndrome. Methods:Retrospective study was conducted on 14 cases of GS in past 5 years in Department of Endocrinology, the First Affiliated Hospital of Nanjing Medical University. Results:The age ranged from 13~54(32.9±12.0) years old with 5 males and 9 females. The history ranged from one month to 15 years. Average body mass index (BMI) was (21.0 ± 2.9) kg/m2 while average systolic blood pressure was (109.6 ± 10.8) mmHg and diastolic blood pressure was (69.6 ± 6.7) mmHg. Clinical symptoms included lower extremities weakness, recurrent paralysis, polydipsia, polyuria, nocturia and tetany. Laboratory findings included hypokalemia, hypomagnesaemia, excessive urine potassium, hypocalciuria, metabolic alkalosis. Recumbent-upright tests showed: supine and upright plasma renin activity (PRA) augmentation, angiotensin-Ⅱ (ATⅡ) elevation and hyperaldosteronism. Renal biopsies revealed juxtaglomerular apparatus hyperplasia (3/4). All symptoms were relieved after potassium and magnesium supplementation or with combined spironolactone. However serum potassium(11/14) and magnesium(10/14) levels were still below normal range. Conclusion:The clinical features of GS includes hypokalemia, hypomagnesaemia, hypocalciuria, hypotension and hyperreninemia, high level of ATⅡ, hyperaldosteronism and high pH in arterial blood gas analysis which can be shorten as “four hypos and four hypers”. The treatment of this syndrome includes potassium and magnesium supplementation, or combination with anti-aldosterone medications. Generally, these patients have good prognosis.
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