Abstract

Carnitine is a vitamin-like substance that regulates lipid metabolism and energy production. Carnitine homeostasis is mainly regulated by dietary intake and biosynthesis in the organs, including the skeletal muscle and the liver. Therefore, liver cirrhotic patients with reduced food intake, malnutrition, biosynthetic disorder, and poor storage capacity of carnitine in the skeletal muscle and liver are more likely to experience carnitine deficiency. In particular, liver cirrhotic patients with sarcopenia are at a high risk for developing carnitine deficiency. Carnitine deficiency impairs the important metabolic processes of the liver, such as gluconeogenesis, fatty acid metabolism, albumin biosynthesis, and ammonia detoxification by the urea cycle, and causes hypoalbuminemia and hyperammonemia. Carnitine deficiency should be suspected in liver cirrhotic patients with severe malaise, hepatic encephalopathy, sarcopenia, muscle cramps, and so on. Importantly, the blood carnitine level does not always decrease in patients with liver cirrhosis, and it sometimes exceeds the normal level. Therefore, patients with liver cirrhosis should be treated as if they are in a state of relative carnitine deficiency at the liver, skeletal muscle, and mitochondrial levels, even if the blood carnitine level is not decreased. Recent clinical trials have revealed the effectiveness of carnitine supplementation for the complications of liver cirrhosis, such as hepatic encephalopathy, sarcopenia, and muscle cramps. In conclusion, carnitine deficiency is not always rare in liver cirrhosis, and it requires constant attention in the daily medical care of this disease. Carnitine supplementation might be an important strategy for improving the quality of life of patients with liver cirrhosis.

Highlights

  • Carnitine is a vitamin-like biofactor involved in fatty acid and energy metabolism

  • There is a possibility that patients with liver cirrhosis (LC) are in a state of relative carnitine deficiency at the liver, skeletal muscle, and mitochondrial levels, even if the blood carnitine concentration is in the normal range or higher

  • In patients with LC, it is difficult to diagnose carnitine deficiency by measuring its blood concentration; it is necessary to make a comprehensive diagnosis based on clinical symptoms and general clinical laboratory findings

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Summary

Introduction

Carnitine is a vitamin-like biofactor involved in fatty acid and energy metabolism. The dysfunction of the liver causes several metabolic disorders because hepatocytes play a central role in glucose, lipid, and protein metabolism. Carnitine deficiency is frequently observed in patients with liver cirrhosis (LC), especially in those with sarcopenia and malnutrition. Recent clinical evidence has revealed the possibility that supplementation with carnitine is effective in improving the complications of LC, including hepatic encephalopathy, sarcopenia, and muscle cramps. We summarized the physiological function and homeostasis of carnitine. The effectiveness of carnitine in improving the complications of LC was investigated, based on the recent clinical trials

Physiological Function of Carnitine
Homeostasis of Carnitine and Liver Disease
Diagnosis of Carnitine Deficiency
Cardiac dysfunction
Chronic Liver Disease and Carnitine Deficiency
Carnitine Supplementation and LC
Carnitine Supplementation and Hepatic Encephalopathy
Carnitine Supplementation and Sarcopenia
Carnitine Supplementation and Muscle Cramps
10. Carnitine and Hepatocellular Carcinoma
11. Conclusions
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