Selvaganesh M, Murali A, Mookambik R V, Jayachandran K
J Assoc Physicians India. 2013 Nov;61(11):818-20.
38 year old woman was admitted with acute onset of quadriplegia. Biochemical investigation revealed severe hypokalaemia with hyperchloraemic metabolic acidosis, alkaline urine, and positive urinary anion gap which are the hallmark of distal tubular acidosis. In addition she also had hypophosphataemia, normoglycaemic glycosuria, aminoaciduria, and hyperphosphaturia suggestive of proximal tubular dysfunction. Further evaluation confirmed the diagnosis of Sjogren's syndrome. Interestingly our patient also had carpopedal spasm despite normal calcium and magnesium level. Quadriplegia and carpopedal spasm improved with correction of hypokalaemia and acidosis. Proximal tubular abnormalities (except albuminuria) were normalised at the time of discharge. Distal tubular acidosis is a well known renal manifestation of Sjogren's syndrome. But this type of transient proximal tubular dysfunction with distal tubular acidosis in Sjogren's syndrome is very rare and hypokalaemic tetany also deserves mention.
一名38岁女性因急性四肢瘫痪入院。生化检查显示严重低钾血症伴高氯性代谢性酸中毒、碱性尿及尿阴离子间隙阳性，这些都是远端肾小管酸中毒的特征。此外，她还存在低磷血症、血糖正常性糖尿、氨基酸尿和高磷尿，提示近端肾小管功能障碍。进一步评估确诊为干燥综合征。有趣的是，尽管患者钙和镁水平正常，但仍出现了手足搐搦。低钾血症和酸中毒纠正后，四肢瘫痪和手足搐搦有所改善。出院时近端肾小管异常（除蛋白尿外）恢复正常。远端肾小管酸中毒是干燥综合征常见的肾脏表现。但干燥综合征中这种伴有远端肾小管酸中毒的短暂性近端肾小管功能障碍非常罕见，低钾性手足搐搦也值得一提。
