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自身免疫性疾病患者伴肾小管酸中毒的低钾性周期性麻痹

Hypokalemic Periodic Paralysis with Renal Tubular Acidosis in a Patient with Autoimmune Disorder.

作者信息

Dutta Dipankar, Sein Simran, Gupta Manish, Mehrotra Akash, Ahmad Danish

机构信息

Consultant, Department of Critical Care Medicine, Max Super Speciality Hospital, Delhi; India;, Corresponding Author, Orcid: https://orcid.org/0009-0001-0842-7717.

Senior Resident; Department of Critical Care Medicine, Max Super Speciality Hospital, Ghaziabad, Uttar Pradesh, India.

出版信息

J Assoc Physicians India. 2025 May;73(5):e41-e42. doi: 10.59556/japi.73.0935.

Abstract

BACKGROUND

Hypokalemic periodic paralysis (HPP) is characterized by transient episodes of skeletal muscle weakness due to hypokalemia. It can arise from primary or secondary causes, with renal tubular acidosis (RTA) being a notable secondary etiology. Here, we present a case of HPP associated with RTA in a patient with an underlying autoimmune disorder, Sjögren syndrome.

CASE DESCRIPTION

A 29-year-old female presented with dry eyes and progressive weakness in both upper and lower limbs, leading to immobilization. Clinical examination revealed hypokalemia, normal anion gap metabolic acidosis, and alkaline urinary pH consistent with RTA type I. Immunological tests indicated markers suggestive of Sjögren syndrome. Treatment with potassium chloride resulted in motor function improvement.

DISCUSSION

The coexistence of HPP and RTA underscores the complex interplay between electrolyte disturbances and autoimmune pathology. Sjögren syndrome, an autoimmune exocrinopathy, can manifest with extraglandular involvement, including RTA. The diagnosis was supported by clinical, biochemical, and immunological findings.

CONCLUSION

This case highlights the clinical significance of recognizing HPP as a potential differential diagnosis in patients presenting with limb weakness, particularly in the context of autoimmune disorders such as Sjögren syndrome. Early diagnosis and appropriate management are crucial for preventing life-threatening complications associated with HPP and optimizing patient outcomes.

摘要

背景

低钾性周期性麻痹(HPP)的特征是由于低钾血症导致骨骼肌无力的短暂发作。它可由原发性或继发性原因引起,肾小管酸中毒(RTA)是一个显著的继发性病因。在此,我们报告一例患有潜在自身免疫性疾病——干燥综合征的患者发生的与RTA相关的HPP病例。

病例描述

一名29岁女性出现眼睛干涩以及双上肢和双下肢进行性无力,最终导致肢体不能活动。临床检查发现低钾血症、正常阴离子间隙代谢性酸中毒以及与I型RTA相符的碱性尿pH值。免疫学检查显示有提示干燥综合征的标志物。氯化钾治疗使运动功能得到改善。

讨论

HPP和RTA的共存突显了电解质紊乱与自身免疫病理之间复杂的相互作用。干燥综合征是一种自身免疫性外分泌病,可表现为包括RTA在内的腺体外受累。临床、生化和免疫学检查结果支持该诊断。

结论

本病例强调了在出现肢体无力的患者中,尤其是在干燥综合征等自身免疫性疾病背景下,将HPP作为潜在鉴别诊断的临床意义。早期诊断和适当管理对于预防与HPP相关的危及生命的并发症以及优化患者预后至关重要。

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