儿童慢性炎症性脱髓鞘性多根神经病变与Sjögren综合征相关。

IF 0.8 4区 医学 Q4 PEDIATRICS
Nurşah Yeniay Süt, Ayşe Tuğba Kartal, Şeyma Ertem, Fatma Aydın, Miraç Yıldırım, Ömer Bektaş, Zeynep Birsin Özçakar, Serap Teber
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引用次数: 0

摘要

背景:慢性炎症性脱髓鞘性多神经根神经病变(CIDP)是一种与多重自身免疫相关的周围神经系统疾病。病例:我们报告了一名13岁的健康男孩,他被转介到我们的门诊,步态障碍和下肢远端无力已经增加了6个月。患者上肢深肌腱反射减弱,下肢无肌腱反射,下肢远端和近端肌力减弱,肌肉萎缩,下垂足,针刺感正常。根据临床表现和电生理检查,该患者被诊断为CIDP。研究了自身免疫性疾病和感染因子在触发CIDP方面的作用。虽然除了多神经病变外没有其他临床症状,但由于抗核抗体和抗Ro52抗体阳性,他还被诊断为Sjögren综合征,并患有自身免疫性涎腺炎。经过6个月的每月静脉注射免疫球蛋白和口服甲基强的松龙治疗后,患者的左脚能够背屈并在没有支持的情况下行走。结论:据我们所知,我们的病例是首例同时存在Sjögren综合征和CIDP的儿童病例。因此,我们建议调查儿童CIDP的潜在自身免疫性疾病,如Sjögren综合征。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Chronic inflammatory demyelinating polyradiculoneuropathy associated with Sjögren`s syndrome in a child.

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a peripheral nervous system disease associated with polyautoimmunity.

Case: We report a previously healthy 13-year old boy who was referred to our outpatient clinic with gait disturbance and distal lower limb weakness that had been increasing for six months. The patient had decreased deep tendon reflexes in the upper extremities and absence in the lower extremities, reduced muscle strength in the distal and proximal lower extremities, muscle atrophy, drop foot, and normal pinprick sensations. The patient was diagnosed with CIDP as a result of clinical findings and electrophysiological studies. Autoimmune diseases and infectious agents were investigated in terms of triggering CIDP. Although there was no clinical sign other than polyneuropathy, he was also diagnosed with Sjögren`s syndrome due to positive antinuclear antibodies and antibodies against Ro52, and with autoimmune sialadenitis. After six months of monthly intravenous immunoglobulin and oral methylprednisolone treatments, the patient was able to dorsiflex his left foot and walk without support.

Conclusions: To our knowledge, our case is the first pediatric case with the coexistence of Sjögren`s syndrome and CIDP. Therefore, we suggest investigating children with CIDP in terms of underlying autoimmune diseases such as Sjögren`s syndrome.

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来源期刊
CiteScore
1.40
自引率
0.00%
发文量
122
审稿时长
6-12 weeks
期刊介绍: The Turkish Journal of Pediatrics is a multidisciplinary, peer reviewed, open access journal that seeks to publish research to advance the field of Pediatrics. The Journal publishes original articles, case reports, review of the literature, short communications, clinicopathological exercises and letter to the editor in the field of pediatrics. Articles published in this journal are evaluated in an independent and unbiased, double blinded peer-reviewed fashion by an advisory committee.
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