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福冈型先天性肌营养不良症患者血清肌酸激酶水平与感染相关下降。

Infection-associated decrease of serum creatine kinase levels in Fukuyama congenital muscular dystrophy.

机构信息

Department of Pediatrics, Yokohama City University Medical Center, 4-57 Urafune, Minami-ku, Yokohama 232-0024, Japan; Division of Child Neurology, Yokohama Medical and Welfare Center, Konan, 4-6-20 Konandai, Konan-ku, Yokohama 234-0054, Japan.

Department of Pediatrics, Yokohama City University Medical Center, 4-57 Urafune, Minami-ku, Yokohama 232-0024, Japan; Division of Child Neurology, Yokohama Medical and Welfare Center, Konan, 4-6-20 Konandai, Konan-ku, Yokohama 234-0054, Japan.

出版信息

Brain Dev. 2021 Mar;43(3):440-447. doi: 10.1016/j.braindev.2020.11.009. Epub 2020 Dec 2.

DOI:10.1016/j.braindev.2020.11.009
PMID:33277141
Abstract

BACKGROUND

Marked decreases in serum creatine kinase levels have been noted in Duchenne and Becker muscular dystrophies as rare complications of autoimmune or autoinflammatory diseases.

SUBJECTS AND METHODS

The influence of systemic inflammation on serum creatine kinase levels was reviewed from the charts of three subjects with Fukuyama congenital muscular dystrophy.

RESULTS

A total of 30 infectious events were identified. Elevated serum C-reactive protein levels coincided with decreased creatine kinase levels on 19 occasions. In one subject, administration of 2 mg/kg/d prednisolone for bronchial asthma resulted in a decrease in creatine kinase level on six other occasions.

CONCLUSION

Apart from an increase in endogenous cortisol secretion, certain inflammation-related molecules could play a role in mitigating muscle cell damage in Fukuyama congenital muscular dystrophy during febrile infectious episodes. Corticosteroids may be a promising agent for the treatment of muscular symptoms in this disorder.

摘要

背景

在杜氏肌营养不良症和贝克肌营养不良症等自身免疫或自身炎症性疾病的罕见并发症中,血清肌酸激酶水平明显降低。

方法

从三位先天性肌营养不良症患者的图表中回顾了全身性炎症对血清肌酸激酶水平的影响。

结果

共发现 30 次感染事件。在 19 次情况下,血清 C 反应蛋白水平升高与肌酸激酶水平降低同时发生。在一名患者中,因支气管哮喘给予 2mg/kg/d 泼尼松龙治疗,导致肌酸激酶水平在另外 6 次情况下降低。

结论

除了内源性皮质醇分泌增加外,某些与炎症相关的分子可能在发热性感染期间减轻先天性肌营养不良症的肌肉细胞损伤中发挥作用。皮质类固醇可能是治疗该疾病肌肉症状的有前途的药物。

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Infection-associated decrease of serum creatine kinase levels in Fukuyama congenital muscular dystrophy.福冈型先天性肌营养不良症患者血清肌酸激酶水平与感染相关下降。
Brain Dev. 2021 Mar;43(3):440-447. doi: 10.1016/j.braindev.2020.11.009. Epub 2020 Dec 2.
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引用本文的文献

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World J Clin Cases. 2024 May 16;12(14):2445-2450. doi: 10.12998/wjcc.v12.i14.2445.
2
UnloCKing the Role of Creatine Kinase in Childhood Asthma.揭示肌酸激酶在儿童哮喘中的作用
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