Nutritional Management of Cholestatic Syndromes in Childhood

Cholestatic liver disease causes severe risk of malnutrition which includes protein-energy malnutrition and specific nutritional deficiencies. The nutritional status can be assessed based on anthropometric measurements, which can be misleading because of ascitis and peripheral edema. Biochemical determinations of lipid-soluble vitamin status are important to evaluate requirements. Based on nutritional status assessment, nutritional therapy should be planned according to a well-defined schedule. The basic principle of nutritional management is to correct the nutritional status as well as to reduce the risk of nutritional deficiencies. Children with cholestasis usually need extra energy supply that can be obtained by increasing energy density of feeds or addition of glucose polymers and lipids. For catch-up growth, usually, protein intake should be increased. Lipid-soluble vitamin supplementation deserves special attention and it is not easy to correct poor vitamin E status. For some children, parenteral administration of vitamin K is needed. Since recently, a water-soluble vitamin E (d -tocopheryl polyethylene glycol 1000 succinate) given by oral route is used with a good therapeutic effect. As the liver disease progresses to liver Prof. Piotr Socha Department of Gastroenterology, Hepatology and Immunology The Children’s Memorial Health Institute, Al. Dzieci Polskich 20 PL–04-730 Warsaw (Poland) E-Mail [email protected] © 2008 Nestec Ltd., Vevey/S. Karger AG, Basel 0517–8606/08/0663–0137$24.50/0 Accessible online at: www.karger.com/ane D ow nl oa de d by : 54 .7 0. 40 .1 1 10 /5 /2 01 7 1: 29 :0 9 P M Socha Ann Nestlé [Engl] 2008;66:137–147 138 liver insufficiency and require liver transplantation. Nutritional therapy seems to be one of the priorities and can be regarded as a ‘bridging’ for liver transplantation. Nutritional status is an important prognostic factor, and children with better nutritional status have fewer complications and lower mortality at liver transplantation [2] . This is the reason why we recently elaborated and published guidelines on nutrition of infants with cholestatic liver disease [3] . We have almost forgotten diseases related to specific nutrient deficits. Recently, Samonte et al. [4] described a young girl who underwent liver transplantation and had a long battle with complications presenting as end-stage renal disease and liver failure. She developed classical oral signs of scurvy which were confused with cyclosporine toxicity, skin changes suggested vasculitis and bone aches were regarded to be related to renal osteodystrophy or corticosteroid-induced osteoporosis. Finally, it appeared that the disease was caused by vitamin C deficiency. In the editorial to this publication, we indicate that there are still situations where we can find basic diseases, caused by deficiency of one nutritional component [5] . Severe liver disease with cholestasis is a very good example of these nutritional problems. In this chapter, causes of malnutrition, specific nutrient requirements and a nutritional approach to children with cholestatic liver disease are presented and discussed. Causes of Malnutrition Diminished bile flow leading to decreased intraluminal bile acid concentration seems to be the major pathomechanism responsible for malnutrition. Cholestasis leads to a significant decrease in lipid absorption and long-chain triglycerides require bile acids for utilization. Lipids significantly contribute to energy uptake, and thus, their malabsorption may cause a negative energy balance. High requirements of other nutrients like protein and carbohydrate are also documented. Many studies show that absorption and utilization of lipids and lipid-soluble vitamins is a process of several stages: dispersion in lipid emulsion, solubilization into mixed bile salt micelles, movement across the unstirred water layer adjacent to the microvilli, cellular uptake by intestinal mucosal cells, incorporation into chylomicrons, and secretion into the lymph. Absorption of some vitamins in cholestasis may be severely reduced, like vitamin E or -carotene; other vitamins are better utilized, and highdose supplementation can efficiently balance poor absorption. There are several other indicators of deficiency risk that should be evaluated: low caloric intake, unbalanced diet with low fat and vegetable consumption, increased oxidative status due to recurrent infections and some other chronic disease conditions complicating liver disease. For these high-risk groups, special attention is needed. Increased energy expenditure was also documented in cholestasis and it can increase up to 40% or more for body weight in malnutrition but it is normal if calculated for age [6, 7] . Anorexia is a common problem of advanced liver disease and it can be attributed to organomegaly or ascitis. Portal hypertension with naturally occurring shunts may have central effects of unidentified toxins. As important growth hormones like insulin-like growth factor 1 and its binding proteins are produced by the liver in response to circulating growth hormone, cholestasis and portal hypertension may lead to abnormal insulinlike growth factor 1 axis response. These abnormalities cannot be corrected by hyperalimentation [8] . Can We Use a Standard Approach to All Cholestatic