Serum Creatine Kinase and Transaminase Levels in Duchenne and Becker Muscular Dystrophies.

IF 3.1 3区医学 Q2 CLINICAL NEUROLOGY

Muscle & Nerve Pub Date : 2025-08-01 Epub Date: 2025-05-11 DOI:10.1002/mus.28431

Marie Rohlenová, Kateřina Machová, Jana Baranová, Lenka Mokrá, Livie Mensová, Radim Mazanec, Lenka Juříková, Jan Staněk, Petra Fuchsová, Barbora Lauerová, Markéta Kumhera, Jana Haberlová

{"title":"Serum Creatine Kinase and Transaminase Levels in Duchenne and Becker Muscular Dystrophies.","authors":"Marie Rohlenová, Kateřina Machová, Jana Baranová, Lenka Mokrá, Livie Mensová, Radim Mazanec, Lenka Juříková, Jan Staněk, Petra Fuchsová, Barbora Lauerová, Markéta Kumhera, Jana Haberlová","doi":"10.1002/mus.28431","DOIUrl":null,"url":null,"abstract":"Introduction/aims: The distinction between hepatic and muscular origin of transaminases in Duchenne (DMD) and Becker (BMD) muscular dystrophy is challenging. We aimed to describe the relations between creatine kinase (CK), lactate dehydrogenase (LDH), myoglobin, and alanine aminotransferase (ALT), aspartate aminotransferase (AST), gamma glutamyltransferase (GGT), and their evolution with age, the severity of the disease, and its complications (corticosteroid therapy, cardiac ejection fraction, body mass index [BMI]).Methods: Multiple regression analysis of the studied markers was performed in three cohorts: 1. Retrospective data of 212 DMD and BMD boys available in the national registry of muscular dystrophies (REaDY), and 2. Cross-sectional study supplemented by retrospective data of A. 64 DMD boys and B. 18 adults with either BMD or B/DMD carriers.Results: The estimation of normal levels of transaminases from CK levels was: ALT [UI/L] = 228.508 + 0.011*CK [UI/L] ± 341.822for DMD, and ALT [UI/L] = 124.498 + 0.011*CK [UI/L] ± 341.822for BMD. AST [UI/L] = 167.436 + 0.012*CK [UI/L]-6.323*Age ± 249.804 for both (all p < 0.001). In the smaller cohort, ALT was best predicted by LDH and physical performance, whereas AST was defined by CK and LDH combined (all p < 0.001). In DMD, all AST, ALT, CK, LDH, and myoglobin decreased with age (p < 0.001), while GGT increased with age and BMI (both p < 0.001).Discussion: Transaminases are dependent on CK and LDH in dystrophinopathy. There is an increase in GGT in DMD connected to BMI.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"240-249"},"PeriodicalIF":3.1000,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Muscle & Nerve","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1002/mus.28431","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/5/11 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Introduction/aims: The distinction between hepatic and muscular origin of transaminases in Duchenne (DMD) and Becker (BMD) muscular dystrophy is challenging. We aimed to describe the relations between creatine kinase (CK), lactate dehydrogenase (LDH), myoglobin, and alanine aminotransferase (ALT), aspartate aminotransferase (AST), gamma glutamyltransferase (GGT), and their evolution with age, the severity of the disease, and its complications (corticosteroid therapy, cardiac ejection fraction, body mass index [BMI]).

Methods: Multiple regression analysis of the studied markers was performed in three cohorts: 1. Retrospective data of 212 DMD and BMD boys available in the national registry of muscular dystrophies (REaDY), and 2. Cross-sectional study supplemented by retrospective data of A. 64 DMD boys and B. 18 adults with either BMD or B/DMD carriers.

Results: The estimation of normal levels of transaminases from CK levels was: ALT [UI/L] = 228.508 + 0.011*CK [UI/L] ± 341.822for DMD, and ALT [UI/L] = 124.498 + 0.011*CK [UI/L] ± 341.822for BMD. AST [UI/L] = 167.436 + 0.012*CK [UI/L]-6.323*Age ± 249.804 for both (all p < 0.001). In the smaller cohort, ALT was best predicted by LDH and physical performance, whereas AST was defined by CK and LDH combined (all p < 0.001). In DMD, all AST, ALT, CK, LDH, and myoglobin decreased with age (p < 0.001), while GGT increased with age and BMI (both p < 0.001).

Discussion: Transaminases are dependent on CK and LDH in dystrophinopathy. There is an increase in GGT in DMD connected to BMI.

查看原文本刊更多论文

杜氏和贝克尔肌营养不良症患者血清肌酸激酶和转氨酶水平。

Duchenne型（DMD）和Becker型（BMD）肌营养不良患者转氨酶的肝脏来源和肌肉来源的区别是具有挑战性的。我们旨在描述肌酸激酶（CK）、乳酸脱氢酶（LDH）、肌红蛋白和丙氨酸转氨酶（ALT）、天冬氨酸转氨酶（AST）、γ谷氨酰转移酶（GGT）之间的关系，以及它们随年龄、疾病严重程度及其并发症（皮质类固醇治疗、心脏射血分数、体重指数[BMI]）的演变。方法：对所研究的标志物进行多元回归分析，分为三个队列：1。国家肌营养不良症登记处（REaDY）中212名DMD和BMD男孩的回顾性数据；横断面研究补充了回顾性数据，其中A. 64名DMD男孩和B. 18名成年BMD或B/DMD携带者。结果：CK水平对转氨酶正常水平的估计为：ALT [UI/L] = 228.508 + 0.011*CK [UI/L]±341.822。DMD为ALT [UI/L] = 124.498 + 0.011*CK [UI/L]±341.822。AST [UI/L] = 167.436 + 0.012*CK [UI/L]-6.323*年龄±249.804（均p）讨论：肌营养不良患者转氨酶依赖于CK和LDH。与BMI相关的DMD中GGT增加。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Muscle & Nerve 医学-临床神经学

CiteScore

6.40

自引率

5.90%

发文量

287

审稿时长

3-6 weeks

期刊介绍： Muscle & Nerve is an international and interdisciplinary publication of original contributions, in both health and disease, concerning studies of the muscle, the neuromuscular junction, the peripheral motor, sensory and autonomic neurons, and the central nervous system where the behavior of the peripheral nervous system is clarified. Appearing monthly, Muscle & Nerve publishes clinical studies and clinically relevant research reports in the fields of anatomy, biochemistry, cell biology, electrophysiology and electrodiagnosis, epidemiology, genetics, immunology, pathology, pharmacology, physiology, toxicology, and virology. The Journal welcomes articles and reports on basic clinical electrophysiology and electrodiagnosis. We expedite some papers dealing with timely topics to keep up with the fast-moving pace of science, based on the referees'' recommendation.