远端肾小管酸中毒伴低钾血症性麻痹为干燥综合征无干燥症状的主要表现:一个不寻常的病例表现

Umesh T. Varyani, Pankaj R. Shah, Vivek B. Kute, Aruna V. Vanikar, H.L. Trivedi
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引用次数: 1

摘要

干燥综合征是一种系统性自身免疫性疾病,以外分泌腺的慢性炎症为特征,具有腺外表现。18-67%的病例累及肾脏,慢性肾小管间质性肾炎是最常见的病理,可导致远端肾小管酸中毒,其特征是正常阴离子间隙酸中毒伴低钾血症和碱性尿ph。低钾血症性周期性麻痹可由多种原因引起,原发性或继发于缺钾。原发性干燥综合征是一种罕见的原因,可导致肾脏受累产生远端肾小管酸中毒和低钾性麻痹。
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Distal renal tubular acidosis with hypokalemic paralysis as primary presentation of Sjogren's syndrome without sicca symptoms: An unusual case presentation

Sjogren's syndrome is a systemic autoimmune disorder characterized by chronic inflammation of the exocrine glands with extra-glandular manifestations. Renal involvement occurs in 18–67% of cases, with chronic tubulo-interstitial nephritis being the most frequent pathology which can lead to distal renal tubular acidosis characterized by normal anion gap acidosis with hypokalemia and alkaline urinary pH. Hypokalemic periodic paralysis can be primary or secondary to potassium deficiency which can arise from several causes. Primary Sjogren's syndrome is a rare cause which can lead to renal involvement producing distal renal tubular acidosis with hypokalemic paralysis.

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