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https://www.readbyqxmd.com/read/29534292/prevalence-evolution-and-risk-factors-for-advanced-liver-fibrosis-in-adults-undergoing-intestinal-transplantation
#1
Geneviève Huard, M Isabel Fiel, Jang Moon, Kishore Iyer, Thomas D Schiano
INTRODUCTION: Intestinal failure-associated liver disease (IFALD) occurs commonly in intestinal transplant (ITx) candidates receiving parenteral nutrition (PN). The aim of this study is to establish the prevalence and risk factors for advanced liver fibrosis in adults at the time of ITx. METHODS: Retrospective chart review of all ITx was performed in adults between January 2000 and May 2014. Advanced liver fibrosis was defined as stage 3 or stage 4 fibrosis. RESULTS: Fifty-three patients met the inclusion criteria...
March 13, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29505154/characterization-of-fatty-acid-profiles-in-infants-with-intestinal-failure-associated-liver-disease
#2
Meredith A Baker, Paul D Mitchell, Alison A O'Loughlin, Alexis K Potemkin, Lorenzo Anez-Bustillos, Duy T Dao, Gillian L Fell, Kathleen M Gura, Mark Puder
BACKGROUND: The purpose of this study was to characterize fatty acid profiles (FAPs) in parenteral nutrition (PN)-dependent infants with intestinal failure-associated liver disease (IFALD) receiving soybean oil-based lipid emulsion (SO) doses of ∼3 and ∼1 g/kg/d. METHODS: Prospectively collected data were retrospectively reviewed. Serum FAPs of patients <1 year old who experienced development of IFALD while receiving standard PN with SO were examined before transitioning to a fish oil-based lipid emulsion for IFALD treatment...
January 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29498797/visceral-transplantation-in-patients-with-intestinal-failure-associated-liver-disease-evolving-indications-graft-selection-and-outcomes
#3
REVIEW
Jason S Hawksworth, Chirag S Desai, Khalid M Khan, Stuart S Kaufman, Nada Yazigi, Raffaele Girlanda, Alexander Kroemer, Thomas M Fishbein, Cal S Matsumoto
Intestinal failure (IF)-associated liver disease (IFALD) is widely recognized as a lethal complication of long-term parenteral nutrition. The pathophysiology of IFALD is poorly understood but appears to be multifactorial and related to the inflammatory state in the patient with IF. Visceral transplant for IFALD includes variants of intestine, liver, or combined liver-intestine allografts. Graft selection for an individual patient depends on the etiology of IF, abdominal and vascular anatomy, severity of IFALD, and potential for intestinal rehabilitation...
March 2, 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/29443401/pediatric-intestinal-failure-associated-liver-disease-challenges-in-identifying-clinically-relevant-biomarkers
#4
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...
February 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29402454/postoperative-enteral-nutrition-guidelines-reduce-the-risk-of-intestinal-failure-associated-liver-disease-in-surgical-infants
#5
Darla R Shores, Samuel M Alaish, Susan W Aucott, Janine E Bullard, Courtney Haney, Heidi Tymann, Bareng A S Nonyane, Kathleen B Schwarz
OBJECTIVE: To assess the effectiveness of postoperative feeding guidelines in reducing the incidence and severity of intestinal failure-associated liver disease (IFALD) among infants. STUDY DESIGN: Two cohorts of infants <6 months old undergoing intestinal surgery were compared: preguideline (retrospective data from 2007 to 2013; n = 83) and postguideline (prospective data from 2013 to 2016; n = 81). The guidelines included greater initial enteral nutrition volumes of 20 mL/kg/d and daily feeding advancement if tolerated...
February 2, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29382923/effect-of-a-fish-oil-based-lipid-emulsion-on-intestinal-failure-associated-liver-disease-in-children
#6
Tian Zhang, Nan Wang, Weihui Yan, Lina Lu, Yijing Tao, Fang Li, Ying Wang, Wei Cai
BACKGROUND/OBJECTIVES: The aim of this study was to assess the effects of a fish oil-based lipid emulsion on intestinal failure-associated liver disease (IFALD) in children. SUBJECTS/METHODS: From January 2014 through June 2017, we enrolled 32 children with IF on long-term parenteral nutrition (PN). When the levels of any three of seven liver indicators (TBA, alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase, gamma glutamyl transferase (γ-GT), total bilirubin (TB), or direct bilirubin (DB)) were two times higher than normal levels, we switched a 50:50 mix of soybean oil and medium-chain triglycerides (MCT) lipid emulsion (with an average dose of 1...
January 30, 2018: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29187040/parenteral-soybean-oil-induces-hepatosteatosis-despite-addition-of-fish-oil-in-a-mouse-model-of-intestinal-failure-associated-liver-disease
#7
Prathima Nandivada, Gillian L Fell, Amy H Pan, Vania Nose, Paul D Mitchell, Kathleen M Gura, Mark Puder
BACKGROUND: Replacement of parenteral soybean oil (SO) with fish oil (FO) is an effective therapy for intestinal failure-associated liver disease (IFALD) in children. However, practitioners remain concerned about the risk of essential fatty acid deficiency (EFAD) and sometimes treat IFALD with a combination of 50% SO and 50% FO emulsions. The purpose of this study was to determine if mixing 50% SO and 50% FO emulsions would prevent hepatosteatosis in a murine model of parenteral nutrition (PN)-induced hepatosteatosis...
February 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29148930/use-of-mixed-oil-fat-emulsion-to-improve-intestinal-failure-associated-liver-disease-in-long-term-home-parenteral-nutrition-a-case-report
#8
Ryan T Hurt, Manpreet S Mundi
Home parenteral nutrition (HPN) is a life-saving therapy for patients who are not able to use their gastrointestinal tract. There are a number of complications associated with HPN, including metabolic bone disease, intestinal failure-associated liver disease (IFALD), and catheter-related bloodstream infections. We present a case of a 32-year-old HPN patient who initially developed biopsy-proven IFALD (total bilirubin, 2.4 mg/dL) while on long-term HPN. His HPN was initiated due to myopathic intestinal dysmotility and pseudo-obstruction when he was 15 years old...
November 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29079315/a-comparison-of-lipid-minimization-strategies-in-children-with-intestinal-failure
#9
Jessica Gonzalez-Hernandez, Purvi Prajapati, Gerald Ogola, Van Nguyen, Nandini Channabasappa, Hannah G Piper
PURPOSE: The purpose of this study was to compare outcomes of lipid minimization with either Intralipid (IL) or Omegaven® in children with intestinal failure (IF) who developed intestinal failure-associated liver disease (IFALD) while receiving parenteral nutrition (PN). METHODS: A retrospective review of children with IF requiring PN who developed IFALD (direct bilirubin >2 mg/dL) while receiving IL (2009-2016) was performed. Clinical characteristics, nutritional, and laboratory values were compared between children treated with reduced IL or Omegaven®...
October 12, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29039050/the-protective-effect-of-fish-oil-lipid-emulsions-on-intestinal-failure-associated-liver-disease-in-a-rat-model-of-short-bowel-syndrome
#10
Seiro Machigashira, Tatsuru Kaji, Shun Onishi, Waka Yamada, Keisuke Yano, Koji Yamada, Ryuta Masuya, Takafumi Kawano, Kazuhiko Nakame, Motoi Mukai, Satoshi Ieiri
PURPOSE: Pediatric patients with intestinal failure need long-term parenteral nutrition (PN), but this nutritional support causes liver dysfunction, such as intestinal failure-associated liver disease (IFALD). Several studies have shown that the lipid emulsion produced by soybean oil (SO) is associated with the occurrence of IFALD. In this study, we evaluated the effect of SO and fish oil (FO) lipid emulsion on hepatic steatosis. METHODS: Sprague-Dawley rats underwent jugular vein catheterization and were divided into three groups: sham operation with normal chow (Sham group), 80% small bowel resection (80% SBR) + TPN with SO lipid emulsion (SO group), and 80% SBR + TPN with FO lipid emulsion (FO group)...
October 16, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28943136/the-extent-of-intestinal-failure-associated-liver-disease-in-patients-referred-for-intestinal-rehabilitation-is-associated-with-increased-mortality-an-analysis-of-the-pediatric-intestinal-failure-consortium-database
#11
Patrick J Javid, Assaf P Oron, Christopher Duggan, Robert H Squires, Simon P Horslen
BACKGROUND: The advent of regional multidisciplinary intestinal rehabilitation programs has been associated with improved survival in pediatric intestinal failure. Yet, the optimal timing of referral for intestinal rehabilitation remains unknown. We hypothesized that the degree of intestinal failure-associated liver disease (IFALD) at initiation of intestinal rehabilitation would be associated with overall outcome. METHODS: The multicenter, retrospective Pediatric Intestinal Failure Consortium (PIFCon) database was used to identify all subjects with baseline bilirubin data...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28719764/surgical-anatomy-does-not-affect-the-progression-of-intestinal-failure-associated-liver-disease-in-neonatal-piglets
#12
Celeste M Lavallee, Pamela R Wizzard, Marihan Lansing, Donna F Vine, Patrick N Nation, Jason Y Yap, Benjamin P Willing, Paul W Wales, Justine M Turner
BACKGROUND: Intestinal failure-associated liver disease (IFALD) causes significant morbidity in neonates with short bowel syndrome (SBS) dependent on parenteral nutrition (PN). Resected ileum, with loss of the ileocecal valve (ICV), is the most common anatomy in SBS, yet its impact on IFALD has not been adequately studied. METHODS: Neonatal piglets were randomized to 75% intestinal resection with jejunocolic anastomosis (JC, n = 12), 75% resection with jejunoileal anastomosis and intact ICV (JI, n = 13), PN-fed sham (sham, n = 14), or sow-fed control (SF, n = 8)...
July 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28521607/intravenous-fish-oil-and-pediatric-intestinal-failure-associated-liver-disease-changes-in-plasma-phytosterols-cytokines-and-bile-acids-and-erythrocyte-fatty-acids
#13
Kara L Calkins, Andrea DeBarber, Robert D Steiner, Martiniano J Flores, Tristan R Grogan, Susanne M Henning, Laurie Reyen, Robert S Venick
BACKGROUND: Soybean oil (SO) emulsions are associated with intestinal failure-associated liver disease (IFALD); fish oil (FO) emulsions are used to treat IFALD. SO and FO differ with respect to their fatty acid and phytosterol content. In children with IFALD whose SO was replaced with FO, we aimed to (1) quantify changes in erythrocyte fatty acids and plasma phytosterols, cytokines, and bile acids and (2) correlate these changes with direct bilirubin (DB). DESIGN: This study enrolled IFALD children who received 6 months of FO...
May 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28481229/parenteral-nutrition-and-intestinal-failure
#14
REVIEW
Barbara Bielawska, Johane P Allard
Severe short bowel syndrome (SBS) is a major cause of chronic (Type 3) intestinal failure (IF) where structural and functional changes contribute to malabsorption and risk of micronutrient deficiencies. Chronic IF may be reversible, depending on anatomy and intestinal adaptation, but most patients require long-term nutritional support, generally in the form of parenteral nutrition (PN). SBS management begins with dietary changes and pharmacologic therapies taking into account individual anatomy and physiology, but these are rarely sufficient to avoid PN...
May 6, 2017: Nutrients
https://www.readbyqxmd.com/read/28333693/pediatric-intestinal-failure-associated-liver-disease
#15
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...
June 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28267037/intravenous-lipid-emulsions-in-pediatric-patients-with-intestinal-failure
#16
REVIEW
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
#17
REVIEW
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
#18
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
#19
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
#20
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)...
April 2017: Pediatric Research
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