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复发性低钾性周期性麻痹揭示无干燥症状的干燥综合征

Recurrent Hypokalemic Periodic Paralysis Unmasks Sjogren Syndrome without Sicca Symptoms.

作者信息

Hung Yao-Min, Huang Neng-Chyan, Wann Shue-Ren, Chang Yun-Te, Wang Jyh-Seng

机构信息

Department of Emergency Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan. / School of Medicine, National Yang-Ming University, Taipei, Taiwan.

Department of Emergency Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan. / School of Medicine, National Yang-Ming University, Taipei, Taiwan. / Department of Environmental and Occupational Health, National Cheng Kung University, Tainan, Taiwan.

出版信息

J Coll Physicians Surg Pak. 2015 Apr;25 Suppl 1:S36-8.

Abstract

Hypokalemic Periodic Paralysis (HPP) may occur as a rare complication of Sjogren Syndrome (SS) and Renal Tubular Acidosis (RTA). A 64-year male patient came with HPP, and was later diagnosed with distal RTA. The patient, who had no xerostomia and xerophthalmia, was diagnosed with primary SS from serologic and histologic findings of minor salivary gland biopsy. The patient recovered after potassium replacement therapy. Renal biopsy was also performed and revealed evidence of tubulointerstitial nephritis. Corticosteroids were administered and there was no recurrence of HPP during a 4-year follow-up period. The case highlights the significance of acute hypokalemia management in emergency department as it can unmask SS even if the SS is not associated with sicca symptoms. Hypokalemic paralysis associated with normal anion gap metabolic acidosis should prompt toward the diagnosis of SS.

摘要

低钾性周期性麻痹(HPP)可能是干燥综合征(SS)和肾小管酸中毒(RTA)的一种罕见并发症。一名64岁男性患者因HPP前来就诊,后来被诊断为远端RTA。该患者无口干和眼干症状,根据小唾液腺活检的血清学和组织学结果被诊断为原发性SS。患者经补钾治疗后康复。还进行了肾活检,显示有肾小管间质性肾炎的证据。给予皮质类固醇治疗,在4年的随访期内HPP未复发。该病例强调了急诊科急性低钾血症管理的重要性,因为即使SS与干燥症状无关,它也可能揭示SS。与正常阴离子间隙代谢性酸中毒相关的低钾性麻痹应促使对SS进行诊断。

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