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成人发病肉碱棕榈酰转移酶 II(CPT II)缺乏症伴横纹肌溶解和急性肾损伤。

Adult-onset carnitine palmitoyl transferase II (CPT II) deficiency presenting with rhabdomyolysis and acute kidney injury.

机构信息

Department of Pediatrics, Pediatric Metabolism Unit, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Department of Internal Medicine, Nephrology Unit, Osmangazi University Faculty of Medicine, Eskişehir, Turkey.

出版信息

CEN Case Rep. 2024 Apr;13(2):81-85. doi: 10.1007/s13730-023-00804-8. Epub 2023 Jun 21.

Abstract

Metabolic myopathies are among the treatable causes of rhabdomyolysis and myoglobinuria. Carnitine palmitoyl transferase 2 (CPT II) deficiency is one of the most common causes of recurrent myoglobinuria in adults. It is an inherited disorder of fatty acid oxidation pathway, commonly associated with elevated acylcarnitine levels. In this case report, we present a 49-year-old male patient who developed acute kidney injury after rhabdomyolysis and was thus diagnosed with CPT2 deficiency after his first episode of rhabdomyolysis. Inborn errors of metabolism should be kept in mind in patients with rhabdomyolysis. Acylcarnitine profile may be normal in CPT II deficiency, even during an acute attack, and molecular genetic diagnostics should be applied if there is high index of clinical suspicion.

摘要

代谢性肌病是横纹肌溶解症和肌红蛋白尿的可治疗病因之一。肉毒碱棕榈酰转移酶 2(CPT II)缺乏症是成人复发性肌红蛋白尿的最常见原因之一。它是脂肪酸氧化途径的一种遗传性疾病,通常与酰基肉碱水平升高有关。在本病例报告中,我们介绍了一位 49 岁男性患者,他在横纹肌溶解症后发生急性肾损伤,因此在首次横纹肌溶解症发作后被诊断为 CPT2 缺乏症。在横纹肌溶解症患者中应考虑到先天性代谢缺陷。即使在急性发作期间,CPT II 缺乏症的酰基肉碱谱也可能正常,如果临床怀疑指数高,应进行分子遗传学诊断。

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