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家族性地中海热的神经学表现

Neurological Face of Familial Mediterranean Fever.

作者信息

Bektaş Polat Cengiz, Kavas Tufan Aslı, Çetin Nuran, Yarar Coşkun, Carman Kürşat Bora

机构信息

Department of Pediatrics, Eskişehir Osmangazi University, Faculty of Medicine, Eskişehir, Turkey.

Department of Pediatric Nephrology, Eskişehir Osmangazi University, Faculty of Medicine, Eskişehir, Turkey.

出版信息

Turk Arch Pediatr. 2022 Sep;57(5):511-515. doi: 10.5152/TurkArchPediatr.2022.21368.

Abstract

OBJECTIVE

Familial Mediterranean fever is a systemic inflammatory disease characterized by recurrent attacks in the form of fever and inflammation of serous membranes. We aimed to search for neurological signs and symptoms of children with familial Mediterranean fever.

MATERIALS AND METHODS

Medical records database from 2010 to 2020 was screened retrospectively. In total, 625 children with familial Mediterranean fever were included in the study. Neurological symptoms and associated factors were searched.

RESULTS

The mean age at onset of familial Mediterranean fever symptoms and time to diagnosis was calculated as 5.12 ± 3.51 years and 7.27 ± 3.9 years, respectively. The neurological symptoms were present in 142 (23.5%) patients. Headache was the most common symptom. During follow-up, different neurologic diseases were diagnosed in 40 familial Mediterranean fever patients and epilepsy was the most frequent disease. The coexistent disease was present in 49.9% of children with familial Mediterranean fever. Juvenile idiopathic arthritis was found to be a risk factor for the neurologic symptom (P < .05). The frequency of neurological symptoms was higher in patients with E148Q mutation (P < .012).

CONCLUSION

The results of the present study revealed that patients with familial Mediterranean fever can present with various central nervous system manifestations. A multidisciplinary approach must be considered in the treatment of these children.

摘要

目的

家族性地中海热是一种全身性炎症性疾病,其特征为以发热和浆膜炎症形式反复发作为特点。我们旨在探寻家族性地中海热患儿的神经体征和症状。

材料与方法

回顾性筛查2010年至2020年的病历数据库。本研究共纳入625例家族性地中海热患儿。对神经症状及相关因素进行探寻。

结果

家族性地中海热症状出现的平均年龄和诊断时间分别计算为5.12±3.51岁和7.27±3.9岁。142例(23.5%)患者出现神经症状。头痛是最常见的症状。随访期间,40例家族性地中海热患者被诊断出不同的神经系统疾病,癫痫是最常见的疾病。49.9%的家族性地中海热患儿存在共存疾病。幼年特发性关节炎被发现是神经症状的一个危险因素(P<.05)。E148Q突变患者的神经症状发生率更高(P<.012)。

结论

本研究结果显示,家族性地中海热患者可出现各种中枢神经系统表现。对这些患儿的治疗必须考虑采用多学科方法。

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Neurological Face of Familial Mediterranean Fever.家族性地中海热的神经学表现
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本文引用的文献

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Prediction of More Severe MEFV Gene Mutations in Childhood.儿童中更严重的MEFV基因突变预测
Turk Arch Pediatr. 2021 Nov;56(6):610-617. doi: 10.5152/TurkArchPediatr.2021.21147.
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Update in familial Mediterranean fever.家族性地中海热的最新进展。
Curr Opin Rheumatol. 2021 Sep 1;33(5):398-402. doi: 10.1097/BOR.0000000000000821.
8
Coexisting Diseases in Patients with Familial Mediterranean Fever.家族性地中海热患者的并存疾病
Open Access Rheumatol. 2020 May 28;12:65-71. doi: 10.2147/OARRR.S252071. eCollection 2020.
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Autoinflammatory Diseases in Childhood.儿童自身炎症性疾病。
Balkan Med J. 2020 Aug 11;37(5):236-246. doi: 10.4274/balkanmedj.galenos.2020.2020.4.82. Epub 2020 Apr 27.

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