Abstract
BackgroundThe level and profiles of blood free carnitine and acylcarnitines, obtained by acylcarnitine analysis using tandem mass spectrometry, reflect various metabolic conditions. We aimed to examine the level of free carnitine and acylcarnitines in liver cirrhosis patients by acylcarnitine analysis and determine the clinical and subjective factors associated with blood carnitine fraction levels in liver cirrhosis.MethodsWe compared blood carnitine fractions in 54 liver cirrhotic patients to other laboratory test results and questionnaire answers.ResultsIn almost all patients, the blood levels of free carnitine (C0) and acetylcarnitine (C2) were within the normal reference range. However, in some patients, the levels of long-chain acylcarnitines, such as C16 and C18:1-acylcarnitine, were higher than the normal reference range. Liver function, assessed by Child-Pugh score, was significantly correlated with the blood level of each carnitine fraction measured (C0, C2, C3, C4, C6, C10, C12, C12:1, C14:1, C16, C18:1, and C18:2-acylcarnitine). Cirrhotic symptom score was significantly correlated with C0, C2, C3, C16, and C18–1-acylcarnitine blood levels. Among the 36-item short-form health survey (SF-36) items, the physical component summary was significantly associated with C0, C2, and C18–1-acylcarnitine blood levels.ConclusionsCarnitine fraction levels were positively correlated with liver cirrhosis stage, particularly, long-chain acylcarnitines. Moreover, carnitine fraction levels were associated with various subjective physical symptoms in liver cirrhosis patients.
Highlights
The level and profiles of blood free carnitine and acylcarnitines, obtained by acylcarnitine analysis using tandem mass spectrometry, reflect various metabolic conditions
The acylcarnitine in which an acyl-base derived from long-chain fatty acid and carnitine are ester-linked is transported into mitochondria, where acylcarnitines are converted to acylCoA at the inner mitochondrial membrane, and are provided as the substrate for β-oxidation
Carnitine exists as free carnitine and acylcarnitine fractions in blood samples; the acylcarnitine fraction can be further subdivided into multiple compounds
Summary
The level and profiles of blood free carnitine and acylcarnitines, obtained by acylcarnitine analysis using tandem mass spectrometry, reflect various metabolic conditions. The acylcarnitine in which an acyl-base derived from long-chain fatty acid and carnitine are ester-linked is transported into mitochondria, where acylcarnitines are converted to acylCoA at the inner mitochondrial membrane, and are provided as the substrate for β-oxidation. It eliminates intracellular acyl-compounds, regulating the ratio of coenzyme A (CoA) to acyl-CoA [1, 2]. The amount of each acylcarnitine fraction in a blood sample was too small to accurately measure until recently Advances in technologies, such as MS/MS, have enabled detailed analyses of acylcarnitine fractions in blood; there have been few studies on the factors associated with blood acylcarnitines in patients with liver cirrhosis [8]
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