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慢性肾脏病中的肉碱缺乏:病理生理学、临床意义及治疗前景

Carnitine Deficiency in Chronic Kidney Disease: Pathophysiology, Clinical Implications, and Therapeutic Perspectives.

作者信息

Kaida Yusuke, Taguchi Kensei, Fukami Kei

机构信息

Division of Nephrology, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, Japan.

Research Institute of Medical Mass Spectrometry, Kurume University School of Medicine, Kurume 830-0011, Japan.

出版信息

Nutrients. 2025 Jun 23;17(13):2084. doi: 10.3390/nu17132084.

Abstract

Carnitine is essential for the mitochondrial transport of long-chain fatty acids and thus plays a pivotal role in energy metabolism, particularly in metabolically active organs, such as skeletal and cardiac muscle. In patients with dialysis, carnitine homeostasis is disrupted because of the reduced synthesis, impaired renal reabsorption, and carnitine loss during extracorporeal procedures. Carnitine deficiency is linked to a wide range of clinical manifestations, including muscle weakness, treatment-resistant anemia, intradialytic hypotension, mental disorder, and cardiovascular disease. This review provides a comprehensive overview of the physiological function of carnitine, elucidates the underlying mechanisms of carnitine deficiency in patients with dialysis, and explores the clinical consequences. Furthermore, the efficacy and limitations of L-carnitine supplementation in clinical practice are discussed based on the current literature. A better understanding of the pathophysiological and clinical relevance of carnitine deficiency may help facilitate personalized therapeutic strategies for patients with kidney diseases.

摘要

肉碱对于长链脂肪酸的线粒体转运至关重要,因此在能量代谢中起关键作用,尤其是在代谢活跃的器官,如骨骼肌和心肌中。在透析患者中,由于合成减少、肾脏重吸收受损以及体外操作过程中的肉碱丢失,肉碱稳态被破坏。肉碱缺乏与多种临床表现有关,包括肌肉无力、难治性贫血、透析中低血压、精神障碍和心血管疾病。本综述全面概述了肉碱的生理功能,阐明了透析患者肉碱缺乏的潜在机制,并探讨了临床后果。此外,基于当前文献讨论了补充左旋肉碱在临床实践中的疗效和局限性。更好地理解肉碱缺乏的病理生理和临床相关性可能有助于为肾病患者制定个性化治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5135/12251438/52424fbd8020/nutrients-17-02084-g001.jpg

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