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老年男性低钾性麻痹的罕见病因:干燥综合征。

Unusual cause of hypokalemic paralysis in aged men: Sjögren syndrome.

作者信息

Cheng Chih-Jen, Chiu Jainn-Shiun, Chen Chun-Chi, Lin Shih-Hua

机构信息

Division of Nephrology, Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.

出版信息

South Med J. 2005 Dec;98(12):1212-5. doi: 10.1097/01.smj.0000189906.32780.0c.

Abstract

Hypokalemic paralysis is a less recognized but reversible disorder in elderly patients. This report describes two elderly Chinese males (age 74 and 78 years) who had progressive muscle weakness and eventually paralysis. Physical examination showed symmetrical flaccid paralysis of extremities. Both had the major biochemical abnormality of profound hypokalemia (1.4 and 1.8 mmol/L) accompanied by high urine K+ excretion and hyperchloremic metabolic acidosis. A positive urine anion gap and alkaline urine pointed to the diagnosis of distal renal tubular acidosis. Large doses of potassium chloride supplementation were required to restore muscle strength. Pertinent investigations, including elevated titers of antinuclear antibody and rheumatoid factor, positive anti-Ro antibody, low serum C3 and C4 levels, and delayed saliva excretion on salivary scintigraphy suggested Sjögren syndrome. Despite the lack of sicca syndrome at the initial presentation, both had development of typical sicca syndrome and positive Schirmer test at the 5-month and 1-year follow-up, respectively. Potassium citrate supplement and prednisolone therapy completely corrected the hypokalemia and metabolic acidosis. Extraglandular involvement with distal renal tubular acidosis preceding the typical sicca syndrome may induce hypokalemic paralysis and unveil Sjögren syndrome in elderly males.

摘要

低钾性麻痹在老年患者中是一种较不为人所知但可逆转的疾病。本报告描述了两名中国老年男性(年龄分别为74岁和78岁),他们出现进行性肌无力并最终发展为麻痹。体格检查显示四肢对称性弛缓性麻痹。两人均有严重低钾血症(1.4和1.8 mmol/L)这一主要生化异常,伴有高尿钾排泄和高氯性代谢性酸中毒。尿阴离子间隙阳性和碱性尿提示诊断为远端肾小管酸中毒。需要补充大剂量氯化钾以恢复肌肉力量。相关检查,包括抗核抗体和类风湿因子滴度升高、抗Ro抗体阳性、血清C3和C4水平降低以及唾液闪烁显像显示唾液排泄延迟,提示干燥综合征。尽管最初表现时缺乏干燥综合征,但两人分别在5个月和1年随访时出现了典型的干燥综合征且Schirmer试验阳性。补充枸橼酸钾和泼尼松龙治疗完全纠正了低钾血症和代谢性酸中毒。在典型干燥综合征之前出现远端肾小管酸中毒的腺外受累可能诱发老年男性低钾性麻痹并揭示干燥综合征。

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