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https://www.readbyqxmd.com/read/28333693/pediatric-intestinal-failure-associated-liver-disease
#1
Cathleen M Courtney, Brad W Warner
PURPOSE OF REVIEW: The goal of this review is to provide updates on the definition, pathophysiology, treatment, and prevention of intestinal failure-associated liver disease (IFALD) that are relevant to care of pediatric patients. RECENT FINDINGS: Current literature emphasizes the multifactorial nature of IFALD. The pathogenesis is still largely unknown; however, molecular pathways have been identified. Key to these pathways are proinflammatory cytokines involved in hepatic inflammation and bile acids synthesis such as Toll-like receptor 4 and farnesoid X receptor, respectively...
March 22, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28267037/intravenous-lipid-emulsions-in-pediatric-patients-with-intestinal-failure
#2
Olivier Goulet, Cécile Lambe
The incidence of cholestatic liver disease (CLD) in pediatric patients suffering intestinal failure (IF) is not well established. Due to persistent portal inflammation, about 20% of these patients will progress to end-stage intestinal failure associated liver disease (IFALD) leading to liver transplant or death. PURPOSE OF REVIEW: Premature babies as well as infants with short bowel syndrome (SBS) and repeated sepsis (catheter or small intestinal bacterial overgrowth related) are at risk of developing CLD...
April 2017: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/28201847/the-differentiation-of-intestinal-failure-associated-liver-disease-from-nonalcoholic-fatty-liver-and-nonalcoholic-steatohepatitis
#3
Alan L Buchman, Bita V Naini, Bert Spilker
Intestinal failure-associated liver disease (IFALD), formerly known as parenteral nutrition-associated liver disease has often been listed in textbooks as an example of nonalcoholic fatty liver disease (NAFLD). However, the etiology, pathophysiology, epidemiology, histology, and progression differ substantially between the conditions defined as NAFLD and the disease, IFALD. Therefore, IFALD should not be defined or considered as a type or a cause of nonalcoholic fatty liver or nonalcoholic steatohepatitis, but rather as a distinct disease...
February 2017: Seminars in Liver Disease
https://www.readbyqxmd.com/read/28052307/ultrashort-bowel-syndrome-outcome-in-children-treated-in-a-multidisciplinary-intestinal-rehabilitation-unit
#4
Mariela Dore, Paloma Triana Junco, Ane Andres Moreno, Vanesa Nuñez Cerezo, Martha Romo Muñoz, Alba Sánchez Galán, Alejandra Vilanova Sánchez, Gerardo Prieto, Esther Ramos, Francisco Hernandez, Leopoldo Martínez Martínez, Manuel Lopez Santamaria
Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Patients and Methods Retrospective study of patients with USBS (defined as < 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed...
February 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28028560/pathophysiology-prevention-treatment-and-outcomes-of-intestinal-failure-associated-liver-disease
#5
REVIEW
Noora H Al-Shahwani, David L Sigalet
BACKGROUND: Intestinal failure-associated liver disease (IFALD) remains a serious problem in the treatment of infants with nutritional problems and short bowel syndrome. METHODS: A review of the recent literature from 2010 to 2016, concentrating on articles related to the pathophysiology of IFALD and to outcomes of novel nutritional and pharmacological therapies for neonatal cholestasis in the post-surgical neonate. RESULTS: The pathophysiology of IFALD relates to an increase sensitivity of the neonatal liver to cholestasis in the non-fed state; prolonged cholestasis almost inevitably results in liver damage which will progress from fibrosis to cirrhosis...
April 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/27997531/redefining-short-bowel-syndrome-in-the-21st-century
#6
REVIEW
Valeria C Cohran, Joshua D Prozialeck, Conrad R Cole
In 1968, Wilmore and Dudrick reported an infant sustained by parenteral nutrition (PN) providing a potential for survival for children with significant intestinal resections. Increasing usage of TPN over time led to some patients developing Intestinal Failure Associated Liver Disease (IFALD), a leading cause of death and indication for liver/intestinal transplant. Over time, multidisciplinary teams called Intestinal Rehabilitation Programs (IRPs) began providing meticulous and innovative management. Usage of alternative lipid emulsions and lipid minimization strategies have resulted in the decline of IFALD and an increase in long-term and transplant-free survival, even in the setting of ultrashort bowel (< 20 cm)...
January 25, 2017: Pediatric Research
https://www.readbyqxmd.com/read/27979360/risk-of-post-procedural-bleeding-in-children-on-intravenous-fish-oil
#7
Prathima Nandivada, Lorenzo Anez-Bustillos, Alison A O'Loughlin, Paul D Mitchell, Meredith A Baker, Duy T Dao, Gillian L Fell, Alexis K Potemkin, Kathleen M Gura, Ellis J Neufeld, Mark Puder
BACKGROUND: Intestinal failure-associated liver disease (IFALD) can be treated with parenteral fish oil (FO) monotherapy, but practitioners have raised concerns about a potential bleeding risk. This study aims to describe the incidence of clinically significant post-procedural bleeding (CSPPB) in children receiving FO monotherapy. METHODS: A retrospective chart review was performed on patients at our institution treated with intravenous FO for IFALD. CSPPB was defined as bleeding leading to re-operation, transfer to the intensive care unit, re-admission, or death, up to one month after any invasive procedure...
December 1, 2016: American Journal of Surgery
https://www.readbyqxmd.com/read/27976737/altered-systemic-bile-acid-homeostasis-contributes-to-liver-disease-in-pediatric-patients-with-intestinal-failure
#8
Yong-Tao Xiao, Yi Cao, Ke-Jun Zhou, Li-Na Lu, Wei Cai
Intestinal failure (IF)-associated liver disease (IFALD), as a major complication, contributes to significant morbidity in pediatric IF patients. However, the pathogenesis of IFALD is still uncertain. We here investigate the roles of bile acid (BA) dysmetabolism in the unclear pathogenesis of IFALD. It found that the histological evidence of pediatric IF patients exhibited liver injury, which was characterized by liver bile duct proliferation, inflammatory infiltration, hepatocyte apoptosis and different stages of fibrosis...
December 15, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27856995/choline-protects-against-intestinal-failure-associated-liver-disease-in-parenteral-nutrition-fed-immature-rats
#9
Jie Zhu, Ting Lu, Fei Chen, Junkai Yan, Fan Chen, Qiaosen Zhang, Jifan Wang, Weihui Yan, Tingxi Yu, Qingya Tang, Wei Cai
BACKGROUND: Deficiency of choline, a required nutrient, is related to intestinal failure-associated liver disease (IFALD). Therefore, we aimed to investigate the effects of choline supplementation on IFALD and the underlying mechanisms. METHODS: Male Sprague-Dawley rats (4 weeks old) were fed AIN-93G chow and administered intravenous 0.9% saline (control), parenteral nutrition (PN), or PN plus intravenous choline (600 mg/kg) for 7 days. We evaluated body weight, hepatic histology, biochemical indicators, triglycerides, oxidative status, methylation levels of peroxisomal proliferator-activated receptor alpha (PPARα) gene promoter, expression of PPARα and carnitine palmitoyltransferase 1 (CPT1), and levels of choline metabolites...
November 17, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/27683392/pediatric-intestinal-failure-associated-liver-disease-challenges-in-identifying-clinically-relevant-biomarkers
#10
Kathleen M Gura, Andrew E Mulberg, Paul D Mitchell, John Yap, Clara Y Kim, Minjun Chen, Alexis Potemkin, Mark Puder
BACKGROUND: Intestinal failure-associated liver disease (IFALD) is complex and diagnosed by concurrent use of parenteral nutrition, clinical presentation, and alterations in hepatic biomarkers exclusive of other causes of liver disease. In comparison with individual measures, composite biomarkers may provide a more effective means for assessing disease progression and response to treatment than single parameters. Since IFALD is considered by some to be a type of drug-induced liver injury (DILI), those diagnostic criteria could potentially be used in this population...
September 28, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/27651372/the-administration-of-ghrelin-improved-hepatocellular-injury-following-parenteral-feeding-in-a-rat-model-of-short-bowel-syndrome
#11
Shun Onishi, Tatsuru Kaji, Waka Yamada, Kazuhiko Nakame, Tomoe Moriguchi, Koushirou Sugita, Koji Yamada, Takafumi Kawano, Motoi Mukai, Masakazu Souda, Sohsuke Yamada, Takako Yoshioka, Akihide Tanimoto, Satoshi Ieiri
PURPOSE: Long-term parenteral nutrition following massive bowel resection causes liver dysfunction, such as intestinal failure-associated liver disease (IFALD). IFALD includes two different states, cholestasis and steatosis, which represents a life-threatening complication. The previous reports have shown the protective role of ghrelin in the liver. The aim of this study was to evaluate the effects of the administration of ghrelin in the liver in a parenterally fed rat model of short bowel syndrome (SBS)...
December 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27510535/predictors-of-failure-of-fish-oil-therapy-for-intestinal-failure-associated-liver-disease-in-children
#12
Prathima Nandivada, Meredith A Baker, Paul D Mitchell, Alison A O'Loughlin, Alexis K Potemkin, Lorenzo Anez-Bustillos, Sarah J Carlson, Duy T Dao, Gillian L Fell, Kathleen M Gura, Mark Puder
BACKGROUND: Parenteral fish-oil (FO) therapy is a safe and effective treatment for intestinal failure-associated liver disease (IFALD). Patients whose cholestasis does not resolve with FO may progress to end-stage liver disease. OBJECTIVE: We sought to identify factors associated with the failure of FO therapy in treating IFALD to guide prognostication and referral guidelines. DESIGN: Prospectively collected data for patients treated with FO at Boston Children's Hospital from 2004 to 2014 were retrospectively reviewed...
September 2016: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/27484488/higher-doses-of-fish-oil-based-lipid-emulsions-used-to-treat-inadequate-weight-gain-and-rising-triene-tetraene-ratio-in-a-severely-malnourished-infant-with-intestinal-failure-associated-liver-disease
#13
Mary Riedy, Brittany DePaula, Mark Puder, Kathleen M Gura, Kevin A Sztam
Fish oil-based lipid emulsions (FOLEs) have been used to treat cholestasis in children with intestinal failure-associated liver disease (IFALD). When FOLEs are dosed at 1 g/kg/d, essential fatty acid (EFA) deficiency typically does not occur. We describe the clinical course of a severely malnourished parenteral nutrition-dependent infant with IFALD. Baseline EFA panels were normal upon starting FOLE at 1 g/kg/d. Despite biochemical improvement in IFALD, weight velocity was below target and biochemical EFA status worsened, even after correction for other factors affecting weight...
August 1, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/27109980/adult-intestinal-and-multivisceral-transplantation-experience-from-a-single-center-in-the-united-kingdom
#14
C S Rutter, I Amin, N K Russell, L M Sharkey, A J Butler, S J Middleton
Cambridge is one of two designated adult intestinal transplant centers in the United Kingdom and has performed 60 transplants on 54 patients since 2007; 52% of these were undertaken in the last 3 years. This increasing trend is in contrast with that reported worldwide; 27% were small bowel grafts (SBT), 15% modified multivisceral (MMVT), and 58% multivisceral (MVT). Median recipient age was 47 years; the female-to-male ratio was 27/33. Primary diseases included visceral arterial thromboses (17%), Crohn's disease (17%), motility disorders (12%), visceral venous thromboses (12%), familial adenomatous polyposis (FAP)/desmoids (8%), alcoholic cirrhosis (3%), nonalcoholic fatty liver disease (3%), ulcerative colitis (2%), and other (15%)...
March 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27109978/long-term-outcomes-of-intestinal-and-multivisceral-transplantation-at-a-single-center-in-argentina
#15
D Ramisch, C Rumbo, C Echevarria, L Moulin, S Niveyro, G Orce, A Crivelli, M I Martinez, L Chavez, M A Paez, J Trentadue, F Klein, A Fernández, H Solar, G E Gondolesi
BACKGROUND: Intestinal failure (IF) patients received parenteral nutrition (PN) as the only available therapy until intestinal transplantation (ITx) evolved as an accepted treatment. The aim of this article is to report the long-term outcomes of a series of ITx performed in pediatric and adult patients at a single center 9 years after its creation. PATIENTS AND METHODS: This is a retrospective analysis of the ITx performed between May 2006 and January 2015. Diagnoses, pre-ITx mean time on PN, indications for ITx, time on the waiting list for types of ITx, mean total ischemia time, and warm ischemia time, time until PN discontinuation, incidence of acute and chronic rejection, and 5-year actuarial patient survival are reported...
March 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27103984/reversal-of-intestinal-failure-associated-liver-disease-ifald-emphasis-on-its-multifactorial-nature
#16
Christian Hvas, Kamelia Kodjabashia, Emma Nixon, Stephen Hayes, Kirstine Farrer, Arun Abraham, Simon Lal
Patients with intestinal failure (IF) and home parenteral nutrition commonly develop abnormal liver function tests. The presentations of IF-associated liver disease (IFALD) range from mild cholestasis or steatosis to cirrhosis and decompensated liver disease. We describe the reversal of IFALD in an adult patient with IF secondary to severe Crohn's disease and multiple small bowel resections. The patient developed liver dysfunction and pathology consistent with IFALD. Multiple causal factors were implicated, including nutrition-related factors, catheter sepsis and the use of hepatotoxic medications...
April 2016: Frontline Gastroenterology
https://www.readbyqxmd.com/read/27030532/outcome-of-home-parenteral-nutrition-in-251-children-over-a-14-y-period-report-of-a-single-center
#17
Elie Abi Nader, Cécile Lambe, Cécile Talbotec, Bénédicte Pigneur, Florence Lacaille, Hélène Garnier-Lengliné, Laetitia-Marie Petit, Catherine Poisson, Amélia Rocha, Odile Corriol, Yves Aigrain, Christophe Chardot, Frank M Ruemmele, Virginie Colomb-Jung, Olivier Goulet
BACKGROUND: Parenteral nutrition (PN) is the main treatment for intestinal failure. OBJECTIVE: We aimed to review the indications for home parenteral nutrition (HPN) in children and describe the outcome over a 14-y period from a single center. DESIGN: We conducted a retrospective study that included all children who were referred to our institution and discharged while receiving HPN between 1 January 2000 and 31 December 2013. The indications for HPN were divided into primary digestive diseases (PDDs) and primary nondigestive diseases (PNDDs)...
May 2016: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/26962059/long-term-fish-oil-lipid-emulsion-use-in-children-with-intestinal-failure-associated-liver-disease
#18
Prathima Nandivada, Gillian L Fell, Paul D Mitchell, Alexis K Potemkin, Alison A O'Loughlin, Kathleen M Gura, Mark Puder
BACKGROUND: Fish oil lipid emulsion (FOLE) and multidisciplinary care for infants with intestinal failure (IF) have been associated with reduced morbidity and mortality due to IF-associated liver disease (IFALD). With increased survival, a greater proportion of infants with IF are now able to remain on parenteral nutrition (PN) in the long term. The purpose of this study was to examine outcomes in children with IFALD who have required long-term PN and FOLE therapy due to chronic IF. MATERIALS AND METHODS: A review of prospectively collected data was performed for children with IFALD who required at least 3 years of PN and FOLE therapy due to chronic IF...
March 9, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/26838529/preventing-the-progression-of-intestinal-failure-associated-liver-disease-in-infants-using-a-composite-lipid-emulsion-a-pilot-randomized-controlled-trial-of-smoflipid
#19
Ivan R Diamond, Robert C Grant, Paul B Pencharz, Nicole de Silva, Brian M Feldman, Peter Fitzgerald, David Sigalet, Bryan Dicken, Justine Turner, Valerie Marchand, Simon C Ling, Aideen M Moore, Yaron Avitzur, Paul W Wales
BACKGROUND: To examine whether SMOFlipid prevents progression of intestinal failure-associated liver disease (IFALD) in parenteral nutrition (PN)-dependent infants with early IFALD (conjugated bilirubin 17-50 µmol/L, 1-3 mg/dL). STUDY DESIGN: Pilot multicenter blinded randomized controlled trial comparing SMOFlipid with Intralipid. Patients received the trial lipid for up to 12 weeks, unless they achieved full enteral tolerance sooner. The primary clinical outcome was the serum conjugated bilirubin...
February 2, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/26826264/utility-of-aminotransferase-platelet-ratio-index-to-predict-liver-fibrosis-in-intestinal-failure-associated-liver-disease-in-pediatric-patients
#20
Carolina Rumbo, María Inés Martinez, Ana Cabanne, Julio Trentadue, Adriana Fernández, Gabriel Gondolesi
BACKGROUND: Intestinal failure-associated liver disease (IFALD) is a frequent indication for intestinal transplantation. Liver biopsy (LBX) is the gold standard test for its diagnosis. Identifying noninvasive markers of fibrosis progression would be of considerable clinical use. Aspartate aminotransferase/platelet ratio index (APRI) has a good correlation in adult patients with chronic liver disease; few studies have been performed in children with IFALD. AIM: To evaluate APRI in a cohort of children with IFALD...
January 29, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
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