罕见的1型肾小管性酸中毒伴膜性肾病,表现为低钾性麻痹。

Case reports in nephrology and urology Pub Date : 2013-07-01 Print Date: 2013-06-01 DOI:10.1159/000353768
Sham Sunder, Satyanand Sathi, K Venkataramanan, Himanshu Verma, Minakshi Bhardwaj, J Rajesh, Himanshu Mahapatra
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引用次数: 3

摘要

1型肾小管酸中毒(RTA)或远端肾小管酸中毒(dRTA)是一种肾小管酸化疾病,通常无症状,但很少表现为低钾血症性麻痹。在此,我们报告一个年轻男性的病例,他表现为四肢突然无力和2个月的腿部肿胀史。调查显示低钾血症、代谢性酸中毒和肾病综合征。其他分析显示正常阴离子间隙代谢性酸中毒伴尿阴离子间隙和dRTA阳性。肾活检显示膜性肾病(MN)。病人的虚弱在补充钾后得到改善。血清钾水平恢复正常,蛋白尿消失,并补充ACE抑制剂。本病例为罕见的dRTA合并MN合并低钾血症性麻痹和髓质性肾钙质沉着症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

A Rare Case of Type I RenalTubular Acidosis with Membranous Nephropathy Presenting as Hypokalemic Paralysis.

A Rare Case of Type I RenalTubular Acidosis with Membranous Nephropathy Presenting as Hypokalemic Paralysis.

A Rare Case of Type I RenalTubular Acidosis with Membranous Nephropathy Presenting as Hypokalemic Paralysis.

A Rare Case of Type I RenalTubular Acidosis with Membranous Nephropathy Presenting as Hypokalemic Paralysis.

Type 1 renal tubular acidosis (RTA), or distal RTA (dRTA), is a disorder of renal tubular acidification, which is generally asymptomatic but may rarely present as hypokalemic paralysis. Here, we report the case of a young male who presented with sudden onset weakness of all 4 limbs and a 2-month history of swelling of the legs. An investigation revealed hypokalemia, metabolic acidosis, and nephrotic syndrome. Additional analyses revealed normal anion gap metabolic acidosis with a positive urine anion gap and dRTA. Renal biopsy showed evidence of membranous nephropathy (MN). The patient's weakness improved with potassium supplements. Normalization of the serum potassium level and disappearance of proteinuria were established with an ACE inhibitor and potassium supplementation. This case is an unusual combination of dRTA with MN coupled with the rare presenting symptoms of hypokalemic paralysis and medullary nephrocalcinosis.

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