malnutrition in 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...

Several conditions, especially chronic liver diseases, can lead to cirrhosis in children and adolescents. Most cases in clinical practice are caused by similar etiologies. In infants, cirrhosis is most often caused by biliary atresia and genetic-metabolic diseases, while in older children, it tends to result from autoimmune hepatitis, Wilson's disease, alpha-1-antitrypsin deficiency and primary sclerosing cholangitis. The symptoms of cirrhosis in children and adolescents are similar to those of adults. However, in pediatric patients, the first sign of cirrhosis is often poor weight gain...

OBJECTIVE: To evaluate the growth of children in weight and height before and after liver transplantation and the relation between malnutrition and postoperative time. METHOD: Growth was assessed for children who received liver transplantation from July 2007 to December 2012 after operation during follow-up. Weight and height were measured for 51 children in May 30, 2013. Weight and height percentiles of each child were calculated in accordance with data surveying on physical development of children in nine provinces/municipalities...

Malnutrition is common in end-stage liver disease, but a correction after transplantation is expected. Body cell mass (BCM) assessment using total body potassium (TBK) measurements is considered the gold standard for assessing nutritional status. The aim of this study was to examine the BCM and, therefore, nutritional status of long-term survivors after childhood liver transplantation. This was a longitudinal nested cohort study of patients undergoing transplantation at <18 years of age and surviving >3 years with ongoing review at our center...

Malnutrition is a treatable complication in children with end-stage liver disease (ESLD). Biliary atresia and other cholestatic disorders are the most frequent cause of ESLD in children. No single variable provides adequate information about nutrition status, yet effective nutrition support is the one intervention known to improve pre- and posttransplant outcomes. A proactive approach consisting of screening anthropometry interpreted using appropriate growth references, recognition of clinical manifestations associated with micronutrient deficiency, and timely aggressive nutrition support is of a paramount importance to maximize anabolism and optimize outcomes...

This article reports the experience of the largest pediatric liver transplant (LT) program in México. Between June 1998 and May 2011, 76 LT were performed in 74 recipients, including 80% cadaveric-whole organ grafts and 20% segmental grafts, 12% of those coming from live donors and 8% from cadaver reduced donors. The most common indication for LT was biliary atresia (43%), followed by metabolic disorders (13%) and fulminant hepatitis (12%). Most of the recipients were infants or toddlers weighing <15 kg (age range 0...

DH is a rare complication following LT. This report presents three cases of right-sided DH after LT using a left-sided graft. All of the patients were younger than one yr of age, and they were critically ill owing to their original disease, characterized by biliary atresia, progressive familiar intrahepatic cholestasis, and acute liver failure. DH occurred with sudden onset within three months after LT. All of the cases were promptly diagnosed and treated. A literature review of 24 cases of DH identified four factors associated with DH: left-sided graft, right-sided DH, relatively delayed onset of DH, and age-specific chief complaint...

Growth retardation is one of the most prominent consequences of childhood cholestatic liver diseases. The pathogenesis of malnutrition is multifactorial and includes reduced calorie intake, fat malabsorption, abnormal protein metabolism, and increased energy expenditure. Poor growth is a typical feature of biliary atresia, cystic fibrosis, progressive familial intrahepatic cholestasis and Alagille syndrome. In some of these features it is not only directly related to impaired nutrient absorption. Liver transplantation is required if liver disease progresses to liver failure - improved nutritional status is achieved within weeks after surgery...

UNLABELLED: Malnutrition is a significant clinical problem in infants with biliary atresia. The natural history of poor growth and its potential association with early transplantation or death in children with biliary atresia was determined. Serial weight- and length-for-age z-scores were computed as part of a retrospective study of 100 infants who underwent hepatoportoenterostomy (HPE) for biliary atresia at 9 U.S. pediatric centers between 1997 and 2000. Poor outcome was defined as transplantation or death by 24 months of age (n = 46) and good outcome was defined as survival with native liver at 24 months of age with total serum bilirubin less than 6 mg/dL (n = 54)...

OBJECTIVE: Malnutrition frequently complicates end-stage liver disease and orthotopic liver transplantation (OLT) in pediatric patients. Pretransplant malnutrition has been associated with increased post-OLT mortality and length of stay in adults. The relationship between pre-OLT nutritional status and post-OLT outcomes in pediatric liver transplant recipients, however, is not well studied. METHODS: The records of 65 pediatric patients who underwent OLT at a single institution were reviewed...

In a 5.1-year-old girl who had been treated by surgical correction of biliary atresia and total orthotopic liver transplantation, extreme dietary selectivity was noted; this was treated by behaviour therapy. On entry in the rehabilitation centre, she manifested malnutrition along with a variety of gastro-enterologic complaints. The treatment consisted of a set of behavioural procedures such as stepwise expansion of the diet, verbal prompting, intermittent contingent attention and a list of agreements with a system of rewards; this led to the elimination of the dietary selectivity and to the consumption of a varied diet in a normal tempo...

BACKGROUND/PURPOSE: This report describes our experiences with 5 cases of biliary atresia associated with meconium peritonitis caused by perforation of small bowel atresia. METHODS: A review of medical records was undertaken in an effort to recognize cases of biliary atresia associated with meconium peritonitis. RESULTS: Five patients of 171 with biliary atresia (2.9%) were detected to have meconium peritonitis caused by perforation of small bowel atresia...

BACKGROUND: Carnitine is an essential cofactor in the transfer of long-chain fatty acids across the inner mitochondrial membrane for oxidation. As its synthesis is performed in the liver, alterations in carnitine metabolism is expected in liver diseases, especially in cirrhosis. METHODS: In this study, we investigated plasma and liver carnitine concentrations of 68 children with chronic liver disease, 36 of whom had cirrhosis as well. Carnitine level was determined by enzymatic method...

OBJECTIVE: Complications of childhood cirrhosis include abnormal growth and malnutrition, associated with abnormalities in circulating IGFs and IGFBPs. Controlled studies suggest that intensive enteral feeding enhances nutritional status. The aim was to ascertain whether nasogastric feeding improves nutritional status in clinical practice and to assess the effect of feeding on serum IGF-I and IGFBPs. PATIENTS: Thirty-three children (median age 0.6 years) with biliary atresia and failure to thrive who were treated with nasogastric feeding...

BACKGROUND: Malnutrition associated with chronic cholestasis in children often requires continuous enteral feeding through a nasogastric tube, which may be poorly tolerated. METHOD: Percutaneous endoscopic gastrostomy was performed in five children (age range, 20 months to 13 years) with severe cholestasis (Alagille syndrome in four; biliary atresia in one) and severe malnutrition (mean weight, -2.6 standard deviations; mean height, -2.7 standard deviations) who were awaiting liver transplantation...

BACKGROUND: The success of orthotopic liver transplantation as treatment for end-stage liver disease has prompted investigation of strategies to maintain or improve nutrition and growth in children awaiting transplantation, because malnutrition is an adverse prognostic factor. The purpose of this study was to evaluate the effect of recombinant human growth hormone therapy on body composition and indices of liver function in patients awaiting transplant. METHODS: The study was designed as a placebo-controlled, double-blind, crossover trial...

Long-chain polyunsaturated fatty acids are important for the growth and early development of the central nervous system. Cholestatic infants suffer from fat malabsorption and disturbed lipid metabolism and therefore may be at risk of developing polyunsaturated fatty acid depletion. The aims of this study were to determine essential fatty acid status in cholestatic infants and to study the relationship to disease severity, degree of undernutrition, antioxidant status and mode of feeding. Twenty-four-hour dietary records were obtained in 34 cholestatic infants, and measurements were taken of skin fold thicknesses, bilirubin levels, activities of serum alanine aminotransferase, alkaline phosphatase, gamma-glutamyl transpeptidase, prothrombin time, serum concentrations of albumin, bile acids, total lipids, phospholipids, cholesterol, vitamins A and E, the fatty acid composition of plasma phospholipids and plasma lipid peroxides expressed as thiobarbiturate reactive substance (TBARS)...

STUDY DESIGN: Forty children < 2 years of age receiving extrahepatic liver transplantation were tested with the Bayley Scales of Infant Development before transplantation and again at 3 and 12 months after transplantation. Neurodevelopmental status 1 year after transplantation was organized by a descriptive statistic of normal, suspect, or delayed. Disease and transplantation variables were investigated for association with delayed neurodevelopmental outcome. RESULTS: Before transplantation mental development was in the low-average range (92 +/- 13...

The paediatric liver transplantation programme was started, at our institution, in 1988 and since then 75 children have received 79 transplants, which corresponds to about 85% of all the paediatric liver transplantations performed in Italy. The main indications to transplantation were biliary atresia, familiar cholangiopathies and metabolic diseases. The patients came from all the regions of Italy, except for 2 of them who came from abroad. Patients with malnutrition and impaired growth underwent an aggressive nutritional programme whilst waiting for transplantation...

Malnutrition is a critical predictor of mortality and morbidity in children with biliary atresia who undergo orthotopic liver transplantation. Growth hormone (GH) enhances nitrogen retention in patients with chronic obstructive lung disease, sepsis, and in fasted adult volunteers. The goal of this study was to assess the acute response to recombinant human GH (rhGH) treatment in children with biliary atresia to determine whether GH therapy was likely to improve pretransplant nutritional status. Five children, aged 10-32 months, with biliary atresia and persistent cholestasis despite surgical attempts to reestablish bile flow, were studied...