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Nutrition Support of Children with Chronic Liver Diseases

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Nutrition Support of Children with Chronic Liver Diseases SOCIETY PAPER Nutrition Support of Children With Chronic Liver Diseases: A Joint Position Paper of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition ÃMarialena Mouzaki, yJiri Bronsky, zGirish Gupte, §Iva Hojsak, jjJorg Jahnel, ôNikhil Pai, 09/27/2019 on BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3IJrtBKuSsQVsKRVaZGM1l4DvatJ7+0OA9ytY7fNKvII= by https://journals.lww.com/jpgn from Downloaded Downloaded #Ruben E. Quiros-Tejeira, ÃRenee Wieman, and ÃÃShikha Sundaram from https://journals.lww.com/jpgn ABSTRACT Chronic liver disease places patients at increased risk of malnutrition that What Is Known can be challenging to identify clinically and treat. Nutrition support is a key aspect of the management of these patients as it has an impact on their by Chronic liver disease is associated with malnutrition, BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3IJrtBKuSsQVsKRVaZGM1l4DvatJ7+0OA9ytY7fNKvII= quality of life, morbidity, and mortality. There are significant gaps in the which is an independent predictor of outcomes. literature regarding the optimal nutrition support for patients with different Careful monitoring to detect and address nutritional types of liver diseases and the impact of these interventions on long-term deficiencies in the child with chronic liver disease outcomes. This Position Paper summarizes the available literature on the is important. nutritional aspects of the care of patients with chronic liver diseases. Specifically, the challenges associated with the nutritional assessment of these subjects are discussed, and recently investigated approaches to deter- What Is New mining the patients’ nutritional status are reviewed. Furthermore, the pathophysiology of the malnutrition seen in the context of chronic liver There are significant gaps in the literature that disease is summarized and monitoring, as well as treatment, recommenda- addresses the optimal nutrition support of patients tions are provided. Lastly, suggestions for future research studies are with chronic liver diseases. described. This report proposes the use of a clinical algorithm to standardize the nutritional management of children Key Words: cirrhosis, frailty, malnutrition, metabolic bone disease, with end-stage liver disease. nutrient deficiencies (JPGN 2019;69: 498–511) hildhood is a vulnerable period of development during which patients. Initially, the malnutrition experienced by children with significant variations in nutritional needs can be complicated cholestasis may be due to maldigestion and malabsorption of byC underlying medical conditions. Malnutrition is a common nutrients, along with an increased metabolic demand. Later, as complication of cholestatic and end-stage liver diseases, which the disease progresses to end-stage liver disease, the underlying together may increase the morbidity and mortality of individual etiology of malnutrition becomes increasingly complex and on Received October 8, 2018; accepted June 19, 2019. 09/27/2019 From the ÃCincinnati Children’s Hospital Medical Center, University of Supplemental digital content is available for this article. Direct URL Cincinnati School of Medicine, Cincinnati OH, the yDepartment of citations appear in the printed text, and links to the digital files are Pediatrics, 2nd Faculty of Medicine, Charles University in Prague and provided in the HTML text of this article on the journal’s Web site Motol University Hospital, Prague, Czech Republic, the zLiver Unit, (www.jpgn.org). Birmingham Children’s Hospital, Birmingham, United Kingdom, the I.H. has received payment/honorarium for lectures: BioGaia, Nutricia, §Children’s Hospital Zagreb, University of Zagreb School of Medicine, Nestle, GM Pharma; payment/honorarium for consultation: Farmas, Zagreb, Croatia, the jjDepartment of Pediatrics and Adolescent Medicine, Chr Hansen. J.R. has received payment/honoraria for lectures or Medical University of Graz, Graz, Austria, the ôDepartment of Pediat- consultation and/or conference support from AbbVie, MSD, rics, McMaster University, Hamilton, Canada, the #Children’s Hospital Nutricia, Nestle´, Biocodex, Ferring, and Walmark (none related to and Medical Center, University of Nebraska Medical Center, Omaha NE, this work). and the ÃÃChildren’s Hospital of Colorado, University of Colorado Copyright # 2019 by European Society for Pediatric Gastroenterology, School of Medicine, Aurora, CO. Hepatology, and Nutrition and North American Society for Pediatric Address correspondence and reprint requests to Marialena Mouzaki, MD, Gastroenterology, Hepatology, and Nutrition MSc, Cincinnati Children’s Hospital Medical Center, University of DOI: 10.1097/MPG.0000000000002443 Cincinnati School of Medicine, 3333 Burnet Ave, Cincinnati, OH 45229 (e-mail: [email protected]). 498 JPGN Volume 69, Number 4, October 2019 Copyright © ESPGHAN and NASPGHAN. All rights reserved. JPGN Volume 69, Number 4, October 2019 Nutrition Support of Children With Chronic Liver Diseases • Disease related: anorexia, delayed gastric emptying, nausea/vomiting, abdominal distension (ascites, organomegaly), luid-restriction • Comorbidity related: dysgeusia secondary to nutritional deiciencies, poor dentition, Poor intake depression, dietary modiications in the context of renal impairment • Iatrogenic: enteral feeding cessation for procedures, lack of early nutrition re-initiation post procedures, suboptimal/inappropriate feeding approaches (e.g. restricting protein intake in acute liver failure), medication side effects • Increased energy expenditure: futile metabolic cycles (e.g. lipolysis of triglycerides to free fatty acids with subsequent re-esteriication to triglycerides), recurrent infections, Altered medications, altered bile acid signalling nutrient • Insulin resistance: peripheral lipolysis and suboptimal protein synthesis in liver and muscle metabolism • Abnormal Growth Hormone (GH) signalling: GH resistance, limited insulin like growth factor 1 release following GH signalling • Abnormal oxidation of macronutrients: e.g. increased leucine oxidation, which further limits protein synthesis; fat oxidation even if fasted state • Other: Limited glycogen stores • Maldigestion: limited intraluminal bile acids (e.g. in cholestasis or biliary diversion), pancreatic insufLiciency (primary or secondary, depending on underlying liver disease), rapid Maldigestion/ transit through the gastrointestinal tract malabsorption • Malabsorption: portal hypertension-related enteropathy, medication-related • Increased losses: diarrhea, electrolytes and minerals lost in renal impairment FIGURE 1. Pathophysiology of malnutrition seen in chronic and end-stage liver disease. includes factors such as anorexia, nausea and vomiting, abnormal Individual authors were responsible for specific sections of nutrient metabolism, increased energy expenditure, maldigestion/ the document, with all co-authors reviewing and editing each malabsorption and may also be iatrogenic (Fig. 1). In children with section in draft form. All recommendations were made based on chronic cholestatic and/or end-stage liver disease, there is a defini- the available literature; each author initially providing recommen- tive need to identify nutritional deficiencies early and to initiate dations for their section. These recommendations were modified on nutritional interventions to both optimize appropriate development further electronic and phone call communication that ultimately led and prevent further complications. This is particularly critical in to the final recommendations that were agreed upon by all the children with end-stage liver disease requiring transplantation, as authors (100% agreement was reached for all recommendations). optimized pre-transplant nutrition may hasten post-transplant recovery while simultaneously decreasing complications. Nutrition NUTRITIONAL STATUS ASSESSMENT support should ideally occur using a multifaceted approach, includ- This section will review general considerations for perform- ing detailed investigations of dietary intake and nutritional status, ing a nutritional status assessment on patients with cholestasis and personalized dietary prescriptions and ongoing assessments of cirrhosis. Disease-specific details regarding the nutritional aspects nutritional status to guide nutritional interventions. The objective of care are discussed later in this Position Paper. of this Position Paper is to summarize the available literature on the topic of nutrition in chronic pediatric liver disease, provide clin- icians with guidance regarding the diagnosis and management of History and Physical Examination the nutritional issues that occur in this context and highlight areas that require further research. A thorough nutritional assessment should start with the patient’s medical history as it pertains to the underlying liver disease and associated comorbidities. It is important to determine whether the METHODS patient suffers from conditions that may impact enteral intake, such as An outline of the desired content of this Position Paper was dental disease or dysphagia. A review of medications may identify developed by the authors, who are members of the Nutrition and side effects that affect intake. Lastly, socioeconomic factors, such as Hepatology Committees of the North American and European access to food and vitamins or supplements, should be explored, as Societies of Pediatric Gastroenterology,
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