马多重酰基辅酶a脱氢酶缺乏症(MADD)与季节性牧场肌病在美国中西部。

IF 2.6 2区 农林科学
B T Sponseller, S J Valberg, N E Schultz, H Bedford, D M Wong, K Kersh, G D Shelton
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引用次数: 33

摘要

背景:季节性牧场肌病(SPM)是一种高度致命的非劳力横纹肌溶解形式,发生在美国的牧场马在秋季或春季。在欧洲,一个类似的条件,非典型肌病(AM),是常见的。最近,在患有AM的马中发现了一种脂质代谢缺陷,即多酰基辅酶a脱氢酶缺乏症(MADD)。目的:确定美国的SPM是否由MADD引起。动物:根据病史、临床症状、血清肌酸激酶活性或死后发现诊断为SPM的六匹马。方法:回顾性描述性研究。在2009年4月至2010年1月期间,对提交给明尼苏达大学神经肌肉诊断实验室的材料进行了审查,以确定SPM病例。纳入标准为急性非劳力性横纹肌溶解,血清、尿液或肌肉样本可供分析。通过尿有机酸、血清酰基肉碱、肌肉肉碱或组织病理学来评估马的MADD。结果:6匹马有临床症状,并且(4/6匹马)尸检结果与SPM一致。受影响的肌肉(4/4)表现为变性,肌纤维内脂质积累,游离肉碱浓度降低,肉碱酯增加。血清酰基肉碱谱(3/3)显示短链和中链酰基肉碱升高,尿有机酸谱(3/3)显示乙基丙二酸、甲基琥珀酸和甘氨酸偶联物水平升高,与马的MADD一致。结论及临床意义:与AM类似,SPM的生化缺陷是MADD,引起肌肉脂质代谢缺陷,肌纤维脂质含量过高。诊断可通过评估血清酰基肉碱和尿有机酸谱。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Equine multiple acyl-CoA dehydrogenase deficiency (MADD) associated with seasonal pasture myopathy in the midwestern United States.

Background: Seasonal pasture myopathy (SPM) is a highly fatal form of nonexertional rhabdomyolysis that occurs in pastured horses in the United States during autumn or spring. In Europe, a similar condition, atypical myopathy (AM), is common. Recently, a defect of lipid metabolism, multiple acyl-CoA dehydrogenase deficiency (MADD), has been identified in horses with AM.

Objective: To determine if SPM in the United States is caused by MADD.

Animals: Six horses diagnosed with SPM based on history, clinical signs, and serum creatine kinase activity, or postmortem findings.

Methods: Retrospective descriptive study. Submissions to the Neuromuscular Diagnostic Laboratory at the University of Minnesota were reviewed between April 2009 and January 2010 to identify cases of SPM. Inclusion criteria were pastured, presenting with acute nonexertional rhabdomyolysis, and serum, urine, or muscle samples available for analysis. Horses were evaluated for MADD by urine organic acids, serum acylcarnitines, muscle carnitine, or histopathology.

Results: Six horses had clinical signs and, where performed (4/6 horses), postmortem findings consistent with SPM. Affected muscle (4/4) showed degeneration with intramyofiber lipid accumulation, decreased free carnitine concentration, and increased carnitine esters. Serum acylcarnitine profiles (3/3) showed increases in short- and medium-chain acylcarnitines and urinary organic acid profiles (3/3) revealed increased ethylmalonic and methylsuccinic acid levels, and glycine conjugates, consistent with equine MADD.

Conclusions and clinical importance: Similar to AM, the biochemical defect causing SPM is MADD, which causes defective muscular lipid metabolism and excessive myofiber lipid content. Diagnosis can be made by assessing serum acylcarnitine and urine organic acid profiles.

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来源期刊
Journal of Veterinary Internal Medicine
Journal of Veterinary Internal Medicine Veterinary-General Veterinary
自引率
11.50%
发文量
243
期刊介绍: The mission of the Journal of Veterinary Internal Medicine is to advance veterinary medical knowledge and improve the lives of animals by publication of authoritative scientific articles of animal diseases.
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