Mechanism for development of malnutrition in primary biliary cholangitis

Abstract

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease that is associated with impaired biliary excretion processes. Along with the development of cholestasis, there is a deficient flow of bile acids into the intestinal lumen causing malnutrition (MN) that is manifested in deficiencies of both macro- and micronutrients. The mechanism for development of trophological insufficiency is multifactorial. However, the trigger of MN in PBC is impaired enterohepatic circulation of bile acids. The ingress of bile acids with a detergent effect into the general bloodstream, followed by elimination via the kidneys and skin, triggers a cascade of metabolic disturbances, which leads to the gradual development and progression of calorie MN. The latter gradually transforms into protein-calorie MN (PСM) (as marasmus) due to the insufficient entry of bile acids into the duodenum, which is accompanied by a decrease in the emulsification, hydrolysis, and absorption of fats and fat-soluble vitamins, as well as disturbance of intestinal motility and bacterial overgrowth. Fat-soluble vitamin deficiencies complement PСM with vitamin and mineral MN. The development of hepatocellular failure enhances the progression of PСM due to the impaired protein synthetic function of hepatocytes in the advanced stage of PBC, which results in deficiency of not only the somatic but also the visceral pool of proteins. A mixed PСM form of marasmus and kwashiorkor develops. Early recognition of energy, protein, micronutrient, and macronutrient deficiencies is of great importance because timely nutritional support can improve liver function and quality of life in patients with PBC. In this case, it is important to know what type (energy, protein-calorie, vitamin, and vitamin-mineral) and form (marasmus, marasmus-kwashiorkor) of MN is present in the patient and how it is associated with the stage of the disease. Therefore, it is recommended to screen all patients with PBC for MN, from the early asymptomatic stage of the disease in order to identify and avoid preventable complications, such as fatigue, malaise, performance decrement, sarcopenia, osteoporosis, and hepatic encephalopathy, which will be able to provide appropriate nutritional support for correction of the trophological status.

Keywords: Primary biliary cholangitis, Malnutrition, Calorie, Protein-calorie, Vitamin-mineral malnutrition, Marasmus and marasmus-kwashiorkor malnutrition

Core Tip: The review discusses the development of malnutrition in primary biliary cholangitis. It presents the factors contributing to the gradual progression of signs of malnutrition in these patients in different stages of the disease and considers the pathogenesis of energy, protein-calorie (marasmus), and protein (kwashiorkor) malnutrition as the disease progresses. By taking into account the mechanisms of different malnutrition signs and forms, the authors present the principles of diet therapy for primary biliary cholangitis.