METABOLIC AND NUTRITIONAL REPERCUSSIONS OF LIVER DISEASE ON CHILDREN: HOW TO MINIMIZE THEM?

OBJECTIVE: To describe the metabolic and nutritional repercussions of chronic liver disease (CLD), proposing strategies that optimize nutritional therapy in the pre- and post-liver transplantation (LT) period, in order to promote favorable clinical outcomes and adequate growth and development, respectively. DATA SOURCES: Bibliographic search in the PubMed, Lilacs and SciELO databases of the last 12 years, in English and Portuguese; target population: children from early childhood to adolescence; keywords in Portuguese and their correlates in English: "Liver Transplant," "Biliary Atresia," "Nutrition Therapy," "Nutritional Status," and "Child"; in addition to Boolean logics "and" and "or," and the manual search of articles. DATA SYNTHESIS: Malnutrition in children with CLD is a very common condition and an important risk factor for morbidity and mortality. There is an increase in energy and protein demand, as well as difficulties in the absorption of carbohydrates, lipids and micronutrients such as fat-soluble vitamins and some minerals. An increase in the supply of energy, carbohydrates and proteins and micronutrients, especially fat-soluble vitamins, iron, zinc and calcium, is suggested, except in cases of hepatic encephalopathy (this restriction is indicated for a short period). CONCLUSIONS: Based on metabolic changes and anthropometric and body composition monitoring, a treatment plan should be developed, following the nutritional recommendations available, in order to minimize the negative impact of malnutrition on clinical outcomes during and after LT.

[Biliary atresia - signs and symptoms, diagnosis, clinical management].

Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis. The authors discuss the clinical presentation, diagnosis and management of biliary atresia.

Effectiveness of Enteral Versus Oral Nutrition With a Medium-Chain Triglyceride Formula to Prevent Malnutrition and Growth Impairment in Infants With Biliary Atresia.

OBJECTIVES: The aim of this study was to compare the effectiveness of oral (PO) versus enteral nutrition (EN) medium-chain triglyceride (MCT) containing-formula to prevent malnutrition and growth impairment in infants with biliary atresia (BA) waiting for a liver transplant. METHODS: A total of 15 infants, 3 to 9 months old with BA were included. They were randomly assigned to either PO or EN. For 12 weeks, both groups received an MCT formula fortified with glucose polymers and corn oil to reach a caloric density between 0.8 and 1 kcal/mL. The formula given to the PO group was administered ad libitum and that given via EN was infused through a nasogastric tube to reach 140% of the energy intake recommended by the Dietary Recommended Intake guidelines. Protein intake was adjusted to 4 to 5 g/kg present weight. Outcome variables were growth and nutritional status evaluated periodically by anthropometric indicators. Biochemical and hematological variables were evaluated through the study. RESULTS: Baseline clinical, nutritional, biochemical, and hematological variables showed no differences between the study groups. Baseline length/age was <-2 SD in 10 of the 15 patients; in the PO group, it fell <-3 SD, whereas in the EN group, it remained stable. Head circumference z score dropped 0.6 SD in the PO group, whereas in the EN group it remained stable. Triceps skinfold values improved in the infants taking EN, P < 0.001. The frequency of adverse effects--respiratory infection and diarrhea--was higher in the EN group. No biochemical or hematological differences were observed between the study groups throughout the study. CONCLUSIONS: A 12-week EN trial with an MCT-fortified formula prevented malnutrition and growth impairment in infants with BA waiting for a liver transplant.

Branched chain amino acids improve body composition and nitrogen balance in a rat model of extra hepatic biliary atresia.

Malnutrition and growth retardation remain a major complication in infants with extrahepatic biliary atresia associated cholestasis. The purpose of this study was to investigate whether oral supplementation with branched chain amino acids (BCAA) can correct malnutrition in a rat model of biliary atresia. Four groups of 15 rats, 30 d old, were used. Group A were shamoperated animals, given a normal laboratory diet (17.5% of caloric intake as proteins). Group B were cholestatic rats (biliary atresia) fed a diet enriched in BCAA (supplement of 8.5%, valine/leucine/isoleucine ratio 1:1:1). Group C were cholestatic mice fed a diet enriched in casein (supplement of 8.5%). Group D were cholestatic mice fed a normal diet. Thirty-two days after surgery, groups were compared for body weight, serum amino acid content, nitrogen balance, muscle mass, and carcass composition. The results showed that the weight of group B, C, and D animals was 85, 81, and 64% of group A (controls). Serum BCAA levels were markedly increased in group B animals. Nitrogen retention was similar in groups B and A, but reduced to 63 and 44% in groups C and D, respectively. Dry weights were similar in group A (39.1% of body weight) and B (37.7%), but reduced to 28.1 and 28.6% of body weight in groups C and D. Body proteins were higher in groups A (13.9%) and B (14.2%) than in group D (9.7%) rats. Mineral content of group B animals was 84% of those of group A, 50% in group C, and 23% in group D rats. It was concluded that an oral supplement of BCAA can correct growth, nitrogen retention, and body composition in experimental biliary atresia. Administration of BCAA supplements to cholestatic infants should be considered.

Short- and long-term vitamin A treatment in children with cholestasis.

Newborns have limited reserve supplies of vitamin A. Infants with chronic cholestasis are in a precarious nutritional state because of their limited ability to build these stores even though the vitamin is present in their diet. In this study, we investigated liver concentrations of vitamin A in 30 children with extrahepatic biliary atresia. We demonstrate that correction of the deficiency occurs after intramuscular administration of a water-miscible solution of retinyl palmitate (100,000 IU, or 30 mg retinol equivalent). Furthermore, we evaluated the effect of vitamin A injections on liver and blood concentrations in nine children with chronic cholestasis over a 1-y period. We conclude this treatment is efficient and is well tolerated.

Amino acid abnormalities in infants with extrahepatic biliary atresia and cirrhosis.

We measured fasting plasma amino acids in 26 children aged 6 months to 5 years with extrahepatic biliary atresia and cirrhosis and compared them with fasting values in 95 normal control children aged 4 months to 12 years. We found that the cirrhotic children had elevations of total free plasma amino acids implying reduced hepatic metabolism of amino acids and that the molar ratio of the branched chain amino acids (isoleucine, leucine, and valine) to the aromatic amino acids (phenylalanine and tyrosine) was significantly depressed. Methionine was also markedly elevated, and taurine concentrations were significantly decreased. Manipulation of the amino acid distribution in dietary protein to normalize plasma amino acids prior to orthotopic hepatic transplantation may be helpful in improving amino acid utilization.

Plasma and urine zinc in infants and children with extrahepatic biliary atresia.

The objective of this study was to undertake a pilot assessment of zinc status in infants and children with extrahepatic biliary atresia. Plasma zinc concentrations and urine zinc excretion rates were measured longitudinally in 45 infants and young children with this disorder. The mean (+/- SD) plasma zinc (298 observations) was 56.8 +/- 17.9 micrograms/dl (controls 85.6 +/- 10.8). Plasma zinc was not correlated with age and did not appear to be related to repeated surgical procedures or to episodes of cholangitis. Plasma zinc was only weakly correlated with serum albumin (r = 0.27, p less than 0.001). The 24-h urine zinc excretion rates were correlated with age but not so strongly as for normal children. Hyperzincuria was evident from the linear regression equation of 24-h urine zinc on age. On a body weight basis, urine zinc excretion rates were approximately 6 times normal for the first 2 years and remained high across the entire age range. To replace these losses, net zinc absorption would need to increase by 40%. The relationship of these data to the zinc nutritional status of these patients and to their underlying hepatic disease remains to be clarified by more definitive studies.