Yang Guo-qing, Zhao Lei, Xi Wen-qi, Mu Yi-ming, Dou Jing-tao, Lu Ju-ming
Department of Endocrinology, Chinese PLA General Hospital, Beijing 100853, China.
Zhonghua Nei Ke Za Zhi. 2006 Aug;45(8):650-3.
To analyze the clinical characteristics of Gitelman syndrome.
Clinical data of 9 cases of Gitelman syndrome seen in the past 25 years in Chinese PLA General Hospital were analyzed retrospectively.
The age of onset of Gitelman syndrome was 14 - 33 years. Main symptoms included weakness, tetany, polydipsia, polyuria, nocturia and paralysis. All patients had normal blood pressure. The biochemical tests showed hypokalemic alkalosis (9/9), hypocalciuria and hypomagnesaemia (9/9), low urine Ca/Cr ratio (<0.2, 5/5) and hyperreninemia (9/9). Renal pathological examination showed juxtaglomerular apparatus hyperplasia (2/2). All the patients' symptoms were relieved after treatment with potassium and magnesium supplementation or with combined spironolactone and indomethacin. However, serum potassium and magnesium levels were still lower than normal range (8/9); only one patient's serum potassium recovered to normal level.
When clinical features such as weakness, hypokalemic alkalosis with normotension were encountered, Gitelman syndrome should be suspected. Serum magnesium as well as urine magnesium and calcium should be measured for confirmation of diagnosis. The treatment of choice included potassium and magnesium supplementation, or combination with anti-aldosterone medications, prostaglandin inhibitors and angiotensin-converting enzyme inhibitor. Generally, these patients have good prognosis.
分析吉特曼综合征的临床特征。
回顾性分析中国人民解放军总医院过去25年中收治的9例吉特曼综合征患者的临床资料。
吉特曼综合征发病年龄为14～33岁。主要症状包括乏力、手足抽搐、烦渴、多尿、夜尿和麻痹。所有患者血压正常。生化检查显示低钾性碱中毒（9/9）、低钙尿症和低镁血症（9/9）、低尿钙/肌酐比值（<0.2，5/5）和高肾素血症（9/9）。肾脏病理检查显示肾小球旁器增生（2/2）。所有患者经补钾、补镁或联合螺内酯和吲哚美辛治疗后症状缓解。然而，血清钾和镁水平仍低于正常范围（8/9）；仅1例患者血清钾恢复至正常水平。
当遇到乏力、血压正常的低钾性碱中毒等临床特征时，应怀疑吉特曼综合征。应检测血清镁以及尿镁和钙以确诊。治疗选择包括补钾、补镁，或联合抗醛固酮药物、前列腺素抑制剂和血管紧张素转换酶抑制剂。一般来说，这些患者预后良好。
