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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
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)...
September 20, 2016: Pediatric Surgery International
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
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
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
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
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
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
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
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
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
Aparna Dalal
Intestinal transplantation is a complex and challenging surgery. It is very effective for treating intestinal failure, especially for those patients who cannot tolerate parenteral nutrition nor have extensive abdominal disease. Chronic parental nutrition can induce intestinal failure associated liver disease (IFALD). According to United Network for Organ Sharing (UNOS) data, children with intestinal failure affected by liver disease secondary to parenteral nutrition have the highest mortality on a waiting list when compared with all candidates for solid organ transplantation...
April 2016: Transplantation Reviews
Brenna S Fullerton, Eric A Sparks, Amber M Hall, Christopher Duggan, Tom Jaksic, Biren P Modi
PURPOSE: Patient selection for transplant evaluation in pediatric intestinal failure is predicated on the ability to assess long-term transplant-free survival. In light of trends toward improved survival of intestinal failure patients in recent decades, we sought to determine if the presence of biopsy-proven hepatic cirrhosis or the eventual achievement of enteral autonomy were associated with transplant-free survival. METHODS: After IRB approval, records of all pediatric intestinal failure patients (parenteral nutrition (PN) >90 days) treated at a single intestinal failure center from February 2002 to September 2014 were reviewed...
January 2016: Journal of Pediatric Surgery
Gillian Lam, Irina G Strogach, Nicole Baron, John F Thompson
OBJECTIVES: Lipid limitation, that is, ≤1 g · kg⁻¹ · day⁻¹ of soy oil lipid emulsion (SOLE), has been suggested as a method to reduce the risk of intestinal failure (IF)-associated liver disease (IFALD). There are limited data as to the effects of this strategy on growth and essential fatty acid (EFA) status. The aim of the study was to assess growth, prevalence of cholestasis, and EFA deficiency in patients with IF who were provided daily SOLE at a dose ≤1 g · kg⁻¹ · day⁻¹...
February 2016: Journal of Pediatric Gastroenterology and Nutrition
Katri Korpela, Annika Mutanen, Anne Salonen, Erkki Savilahti, Willem M de Vos, Mikko P Pakarinen
BACKGROUND: Intestinal failure (IF)-associated liver disease (IFALD) is the major cause of mortality in IF. The link between intestinal microbiota and IFALD is unclear. METHODS: We compared intestinal microbiota of patients with IF (n = 23) with healthy controls (n = 58) using culture-independent phylogenetic microarray analysis. The microbiota was related to histological liver injury, fecal markers of intestinal inflammation, matrix metalloproteinase 9 and calprotectin, and disease characteristics...
May 1, 2015: JPEN. Journal of Parenteral and Enteral Nutrition
Faraz A Khan, Jeremy G Fisher, Eric A Sparks, Alexis Potemkin, Christopher Duggan, Bram P Raphael, Biren P Modi, Tom Jaksic
OBJECTIVES: Intestinal failure-associated liver disease (IFALD) contributes to significant morbidity in pediatric patients with intestinal failure (IF); however, the use of parenteral nutrition (PN) with a fish oil-based intravenous (IV) emulsion (FO) has been associated with biochemical reversal of cholestasis and improved outcomes. Unfortunately, FO increases the complexity of care: because it can be administered only under Food and Drug Administration compassionate use protocols requiring special monitoring, it is not available as a 3-in-1 solution and is more expensive than comparable soy-based IV lipid emulsion (SO)...
March 2015: Journal of Pediatric Gastroenterology and Nutrition
Lisa M Sharkey, Susan E Davies, Arthur Kaser, Jeremy M Woodward
BACKGROUND: Intestinal failure-associated liver disease (IFALD) is the most serious consequence of long-term parenteral nutrition for intestinal failure. Little is known about the pathogenesis of IFALD, although many of the risk factors are also linked to endoplasmic reticulum stress (ERS). We propose that ERS may have a role in the development of IFALD. METHODS: Archived liver tissue from patients with early and late IFALD, as well as from normal controls, was used for RNA extraction and immunohistochemistry to demonstrate the presence of ERS markers...
March 2016: JPEN. Journal of Parenteral and Enteral Nutrition
Justine M Turner, Catherine J Field, Sue Goruk, Pamela Wizzard, Bryan J Dicken, Aisha Bruce, Paul W Wales
BACKGROUND: Fish oil monotherapy has been an advance for treating intestinal failure-associated liver disease (IFALD). However, such patients are at risk of bleeding complications from liver disease and because fish oil can inhibit thrombosis. We have previously reported abnormal platelet function in neonatal piglets given fish oil monotherapy during parenteral nutrition (PN). The purpose of this study was to determine if abnormal fatty acid composition of the platelets could explain the prior observed antiplatelet effect...
May 2016: JPEN. Journal of Parenteral and Enteral Nutrition
Annika Mutanen, Jouko Lohi, Päivi Heikkilä, Hannu Jalanko, Mikko P Pakarinen
BACKGROUND & AIMS: The pathogenesis of intestinal failure (IF) associated liver disease (IFALD) is uncertain, we therefore investigated the role of FGF19 and pro-inflammatory cytokines has on this disease state. METHODS: Serum FGF19, IL-6 and, TNF-α were measured in 52 IF patients at median age 6.0 years (IQR 2.2-13) after 10 months (4.1-39) on parenteral nutrition (PN). Thirty-nine patients underwent liver biopsies. RESULTS: In IF patients, FGF19 concentrations were lower and those of IL-6 and TNF-α higher compared to healthy matched controls (p ⩽ 0...
June 2015: Journal of Hepatology
Sanghoon Lee, Hyo Jung Park, Jihye Yoon, Seul Hee Hong, Chae-Youn Oh, Suk-Koo Lee, Jeong-Meen Seo
Intestinal failure-associated liver disease (IFALD) is a serious complication of parenteral nutrition (PN). Studies have shown that the amount and content of intravenous lipid emulsions (LEs) used is closely related to the development of IFALD. We report 2 cases of IFALD reversed by switching from a combination lipid emulsion containing fish oil to fish oil monotherapy (Omegaven; Fresenius Kabi Austria Gmbh, Graz, Austria). Patients initially received PN containing SMOFlipid 20% (SMOF; Fresenius Kabi Austria Gmbh, Graz, Austria), 2...
March 2016: JPEN. Journal of Parenteral and Enteral Nutrition
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