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malnutrition in biliary atresia

Shikha S Sundaram, Cara L Mack, Amy G Feldman, Ronald J Sokol
Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation...
September 21, 2016: Liver Transplantation
Daniele Santetti, Maria Inês de Albuquerque Wilasco, Cristina Toscani Leal Dornelles, Isabel Cristina Ribas Werlang, Fernanda Urruth Fontella, Carlos Oscar Kieling, Jorge Luiz Dos Santos, Sandra Maria Gonçalves Vieira, Helena Ayako Sueno Goldani
AIM: To evaluate the nutritional status and its association with proinflammatory cytokines in children with chronic liver disease. METHODS: We performed a cross-sectional study with 43 children and adolescents, aged 0 to 17 years, diagnosed with chronic liver disease. All patients regularly attended the Pediatric Hepatology Unit and were under nutritional follow up. The exclusion criteria were fever from any etiology at the time of enrollment, inborn errors of the metabolism and any chronic illness...
August 7, 2015: World Journal of Gastroenterology: WJG
Andrea Carpenter, Vicky Lee Ng, Karen Chapman, Simon C Ling, Marialena Mouzaki
BACKGROUND AND OBJECTIVES: Malnutrition is common in children with end-stage liver disease (ESLD) and is associated with increased morbidity and mortality. The inability to accurately estimate energy needs of these patients may contribute to their poor nutrition status. In clinical practice, predictive equations are used to calculate resting energy expenditure (cREE). The objective of this study is to assess the accuracy of commonly used equations in pediatric patients with ESLD. METHODS: Retrospective study performed at the Hospital for Sick Children...
July 23, 2015: JPEN. Journal of Parenteral and Enteral Nutrition
Rocío Macías-Rosales, Alfredo Larrosa-Haro, Genaro Ortíz-Gabriel, Benjamín Trujillo-Hernández
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...
January 2016: Journal of Pediatric Gastroenterology and Nutrition
Raquel Borges Pinto, Ana Claudia Reis Schneider, Themis Reverbel da Silveira
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...
March 27, 2015: World Journal of Hepatology
Bin Zhang, Qiang Xia, Lirong Jiang, Jianjun Zhang, Xiaosong Chen, Zhaohui Deng, Wenjuan Chen
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...
August 2014: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Looi Cheng Ee, Rebecca Joanne Hill, Kerrie Beale, Charlton Noble, Jonathan Fawcett, Geoffrey John Cleghorn
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...
August 2014: Liver Transplantation
Sona Young, Elizabeth Kwarta, Ruba Azzam, Timothy Sentongo
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...
June 2013: Nutrition in Clinical Practice
Gustavo Varela-Fascinetto, J Alejandro Hernández-Plata, Jaime Nieto-Zermeño, J Manuel Alcántar-Fierros, Victor Fuentes-García, Pedro Castañeda-Martínez, Pedro Valencia-Mayoral, J Manuel Salgado-Ramírez
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...
September 2011: Revista de Investigación Clínica; Organo del Hospital de Enfermedades de la Nutrición
Takanobu Shigeta, Seisuke Sakamoto, Hiroyuki Kanazawa, Akinari Fukuda, Toshihiko Kakiuchi, Chiaki Karaki, Hajime Uchida, Naoto Matsuno, Hideaki Tanaka, Mureo Kasahara
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...
August 2012: Pediatric Transplantation
Joanna Pawlowska, Piotr Socha, Irena Jankowska
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...
January 2010: Annals of Transplantation: Quarterly of the Polish Transplantation Society
Patricia A DeRusso, Wen Ye, Ross Shepherd, Barbara A Haber, Benjamin L Shneider, Peter F Whitington, Kathleen B Schwarz, Jorge A Bezerra, Philip Rosenthal, Saul Karpen, Robert H Squires, John C Magee, Patricia R Robuck, Ronald J Sokol
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)...
November 2007: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Neal R Barshes, I-Fen Chang, Saul J Karpen, Beth A Carter, John A Goss
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...
July 2006: Journal of Pediatric Gastroenterology and Nutrition
J M de Moor, E Brons, F T Kokke, H Vos, J J Tolboom
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...
April 17, 2004: Nederlands Tijdschrift Voor Geneeskunde
S J Han, A Han, S H Choi, J T Oh, E H Hwang
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...
September 2001: Journal of Pediatric Surgery
M A Selimoglu, S Aydogdu, R V Yagci, A Huseyinov
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...
August 2001: Pediatrics International: Official Journal of the Japan Pediatric Society
R I Holt, J P Miell, J S Jones, G Mieli-Vergani, A J Baker
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...
February 2000: Clinical Endocrinology
M Duché, D Habès, A Lababidi, C Chardot, J Wenz, O Bernard
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...
July 1999: Journal of Pediatric Gastroenterology and Nutrition
R M Greer, P Quirk, G J Cleghorn, R W Shepherd
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...
August 1998: Journal of Pediatric Gastroenterology and Nutrition
P Socha, B Koletzko, E Swiatkowska, J Pawlowska, A Stolarczyk, J Socha
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)...
March 1998: Acta Paediatrica
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