Physical function in children and adolescents pre- and 1-year post-liver transplant.

BACKGROUND: Several studies describe poorer motor developmental motor outcomes post-liver transplant (LT) in younger children. Limited studies examine physical function in older children and adolescents pre- and post-LT. METHODS: Retrospective review of standard of care physical function outcome measures pre- and 1-year post-LT in children ≥6 years at LT. Measures include: 6-minute walk test (6MWT), grip strength, Bruininks-Oseretsky Test of Motor Proficiency-2 (BOT-2) components, Physical Activity Questionnaire (PAQ), and Paediatric Quality of Life Multidimensional Fatigue Scale. Association of medical variables with outcomes was explored. RESULTS: The study cohort included 23 (8 male, median (interquartile range) age 11.67 (8.25, 13.92) years at LT) participants. Top two primary diagnoses included biliary atresia (30.4%) and fulminant hepatic failure (21.7%). At 1-year post-LT, over one-third (36%) were overweight or obese. Compared with healthy norms, children had significantly lower pre-LT PAQ scores (p = .002), pre- and post-6MWT scores (p < .001) and post-LT BOT-2 strength and agility scores (p < .001). Pre-LT, lower balance scores were associated with abdominal distention/ascites (p = .009) and splenomegaly (p = .017). Lower pre-LT platelet count correlated with poorer balance (r = .532, p = .017) and lower strength and agility scores (r = .446, p = .043). Significant moderate inverse correlations were found between weight/body mass index z-scores and BOT-2 components. Post-LT children continue to demonstrate decreased levels of motor proficiency and functional capacity but report less fatigue and increased physical activity. CONCLUSIONS: Older children and adolescents undergoing LT are at risk of decreased physical function, highlighting the need for pre- and post-LT rehabilitation to optimize long term outcomes.

Equivalence of Paper and Electronic-Based Patient Reported Outcome Measures for Children: A Systematic Review.

Patient-reported outcome measures (PROMs) exist for a variety of chronic gastrointestinal disorders in children. The availability of electronic (e-)formats of PROMs enhance the accessibility of these tools. The International Society for Pharmacoeconomic and Outcomes Research (ISPOR) defines measurement equivalence (ME) as "comparability of the psychometric properties of data" obtained from the administration of original and adapted versions of PROMs. Consideration of proxy PROM versions is unique to pediatrics and must be included in ME evaluations. We conducted a systematic review (SR) of the literature evaluating ME of e-versions adapted from pediatric paper-based PROMs. A literature search was conducted through Medline, Embase, APA PsychInfo, and the Cochrane Library. Titles, abstracts, and manuscripts were reviewed by 2 independent reviewers. The search yielded 19 studies meeting pre-defined criteria. Just over half (52.6%) of 19 PROMs were disease-specific ones. ME between paper- and e-PROM versions was reported as present in all 19 studies evaluating 5653 participants under the age of 18 years. However, only 6 (31.6%) studies evaluated ME in proxy reported e-versions. Despite the use of PROMs for children with a variety of chronic gastrointestinal disorders, only 1 study evaluated a PROM in this population (IMPACT III for inflammatory bowel disease). Findings from this SR highlight strategic opportunities for the pediatric gastroenterologist to broaden the clinical and research armamentarium to include e-PROMs.

Feasibility of using a patient-reported outcome measure into clinical practice following pediatric liver transplantation: The Starzl Network experience.

BACKGROUND: Patient-reported outcome measures (PROMs) are not routinely used in clinical care by pediatric liver transplant (LT) teams. The Starzl Network for Excellence in Pediatric Transplantation (SNEPT) assessed feasibility of using a disease-specific Quality of Life (QoL) questionnaire in the ambulatory setting at 10 SNEPT sites. METHODS: A mixed methods feasibility project assessing administration processes, barriers, and user experiences with the Pediatric Liver Transplant Quality of Life (PeLTQL) tool. Iterative processes sought stakeholder feedback across four phases (Pilot, Extended Pilot, Development of a Mobile App PeLTQL version, and Pilot App use). RESULTS: A total of 149 patient-parent dyads completed the PeLTQL during LT clinic follow-up. Clinicians, parents, and patients evaluated and reported on feasibility of operationalization. Only two of 10 SNEPT sites continued PeLTQL administration after the initial two pilot phases. Reasons include limited clinical time and available personnel aggravated by the COVID-19 pandemic. In response, a mobile application version of the PeLTQL was initiated. Providing PeLTQL responses electronically was "very easy" or "easy" as reported by 96% (22/23) parents. CONCLUSIONS: Administration of a PROM into post-pediatric LT clinical care was feasible, but ongoing utilization stalled. Use of a mobile app towards facilitating completion of the PeLTQL outside of clinic hours may address the time and work-flow barriers identified.

North American Biliary Stricture Management Strategies in Children After Liver Transplantation: A Multicenter Analysis From the Society of Pediatric Liver Transplantation (SPLIT) Registry.

Biliary strictures affect 4%-12% of pediatric liver transplantations. Biliary strictures can contribute to graft loss if left untreated; however, there remains no consensus on the best course of treatment. Study objectives included analyses of outcomes associated with biliary stricture management strategies via percutaneous transhepatic cholangiography (PTC), endoscopic retrograde cholangiopancreatography (ERCP), or surgery. We identified pediatric liver transplantation recipients (2011-2016) with biliary strictures from the Society of Pediatric Liver Transplantation (SPLIT) registry and retrieved imaging, procedural, and operative reports from individual centers. Subanalyses were performed to specifically evaluate PTC and ERCP for "optimal biliary outcome" (OBO), defined as graft survival with stricture resolution and without recurrence or surgery. A total of 113 children with a median follow-up of 3.9 years had strictures diagnosed 100 days (interquartile range, 30-290) after liver transplantation; 81% were isolated anastomotic strictures. Stricture resolution was achieved in 92% within 101 days, more frequently with isolated anastomotic strictures (96%). 20% of strictures recurred, more commonly in association with hepatic artery thrombosis (32%). Patient and graft survival at 1 and 3 years were 99% and 98% and 94% and 92%, respectively. In a subgroup analysis of 79 patients with extrahepatic strictures managed by PTC/ERCP, 59% achieved OBO following a median of 4 PTC, and 75% following a median of 3 ERCP (P < 0.001). Among patients with OBO, those with ERCP had longer time intervals between successive procedures (41, 47, 54, 62, 71 days) than for PTC (27, 31, 36, 41, 48 days; P < 0.001). Allograft salvage was successful across all interventions. Stricture resolution was achieved in 92%, with 20% risk of recurrence. Resolution without recurrence was highest in patients with isolated anastomotic strictures and without hepatic artery thrombosis.

Standardized Feeding Protocol Improves Delivery and Acceptance of Enteral Nutrition in Children Immediately After Liver Transplantation.

Delivery of adequate nutrition after liver transplantation (LT) surgery is an important goal of postoperative care. Existing guidelines recommend early enteral nutrition after abdominal surgery and in the child who is critically ill but data on nutritional interventions after LT in children are sparse. We evaluated the impact of a standardized postoperative feeding protocol on enteral nutrition delivery in children after LT. Data from 49 children (ages 0-18 years) who received a LT prior to feeding protocol implementation were compared with data for 32 children undergoing LT after protocol implementation. The 2 groups did not differ with respect to baseline demographic data. After protocol implementation, enteral nutrition was started earlier (2 versus 3 days after transplant; P = 0.005) and advanced faster when a feeding tube was used (4 versus 8 days; P = 0.03). Protocol implementation was also associated with reduced parenteral nutrition use rates (47% versus 75%; P = 0.01). No adverse events occurred after protocol implementation. Hospital length of stay and readmission rates were not different between the 2 groups. In conclusion, implementation of a postoperative nutrition protocol in children after LT led to optimized nutrient delivery and reduced variability of care.

Engaging high school students about organ donation and transplantation: an evaluation of the High School Outreach Initiative (HSOI) program.

Adolescents can be influential in changing societal perceptions of organ donation and transplantation (ODT) but current studies on youth are limited. We sought to (1) assess the baseline knowledge in ODT among students in Toronto, Canada, and (2) evaluate the effectiveness of the High School Outreach Initiative (HSOI) program presentations in changing awareness and interest about ODT. Pre- and post-presentation surveys were administered to high school students about their knowledge of ODT, awareness of donor registration, importance of donation, intent to register, and willingness to talk to their families about donation. Descriptive statistics were used to characterize the students' baseline knowledge and interest. Wilcoxon and McNemar tests were used to analyze changes in perceptions before and after the presentation. A total of 449 HSOI presentations were delivered to 33,090 students at 102 high schools in the Greater Toronto Area between 2012 and 2019. Data from 3327 surveys completed by students before a presentation showed 46.5% were not knowledgeable about ODT. For the 2-year period between 2017 and 2019, 1224 matched pre- and post-presentation surveys were collected. The 49.8% of students who stated they were not knowledgeable about ODT prior to the presentation decreased to 3.8% after (p < 0.001). Those who were not willing to register decreased by half after the presentation (p < 0.001). The HSOI is an effective educational program in improving youth's attitudes and perceptions toward ODT. Further directions of the program include the expansion to other cities and the collection of demographic information of students.

Kidney disease in children with heart or liver transplant.

Over the past few decades, there has been increasing recognition of kidney disease in children with non-kidney solid organ transplantation. The risk of kidney disease in children undergoing heart or liver transplantation is higher than the general population as the underlying disease and its associated management may directly impair kidney function. Both heart and liver failures contribute to hypoperfusion and kidney ischemia before patients reach the point of transplant. The transplant surgery itself can often be complicated by acute kidney injury (AKI), which may be further exacerbated by a complicated postoperative course. In the short- and long-term post-transplant period, these children are at risk of acute illness, exposed to nephrotoxic medications, and susceptible to rare but severe infections and immunologic insults that may contribute to AKI and chronic kidney disease (CKD). In some, CKD can progress to kidney failure with replacement therapy (KFRT). CKD and KFRT are associated with increased morbidity and mortality in this patient population. Therefore, it is critical to monitor for and recognize the risk factors for kidney injury in this population and mitigate these risks. In this paper, the authors provide an overview of kidney disease pertaining to heart and liver transplantation in children with guidance on monitoring, diagnosis, prevention, and management.

Conversion from tacrolimus to sirolimus as a treatment modality in de novo allergies and immune-mediated disorders in pediatric liver transplant recipients.

De novo PTAID may develop in pediatric solid organ transplant recipients, have a diverse spectrum, and are occasionally treatment resistant. Previous reports showed resolution of immune cytopenias in solid organ transplant recipients following replacement of the calcineurin inhibitor tacrolimus with the mTOR inhibitor sirolimus. Herein we describe a retrospective review (2000-2017) of subjects who developed PTAID in whom immunosuppression was changed to sirolimus. Eight recipients (6 males) of either liver (n = 7) or multivisceral transplant (n = 1) suffered from severe, treatment-resistant PTAID and were switched from tacrolimus to sirolimus. The median age at transplant was 1 year (range 0.5-2.4 years). Six (75%) recipients developed de novo allergy and 2 immune-mediated diseases. The median age at presentation of PTAID was 2.7 (1.4-9) years at a median of 1.3 (0.25-8) years after transplantation. The median time from PTAID presentation to conversion to sirolimus was 1.8 (0.45-10) years. Complete resolution of symptoms was seen in 4 (50%) patients after a median of 12 (range 4-24) months including 2 patients with immune-mediated disease, 1 eczema, and 1 with eosinophilic colitis. One patient with multiple food allergies had a partial response and 3 (38%) had no response. None of the 8 recipients developed sirolimus-attributed adverse events or acute rejection during a median follow-up of 5 (0.6-8) years after the conversion. Immunosuppression conversion from tacrolimus to sirolimus can be an effective therapy in patients suffering severe or treatment-resistant PTAID, suggesting a potential role for tacrolimus in the pathogenesis of PTAID.

Neurodevelopmental Outcomes in Preschool and School Aged Children With Biliary Atresia and Their Native Liver.

OBJECTIVES: The aim of the study was to assess neurodevelopmental outcomes among children with biliary atresia (BA) surviving with their native liver at ages 3 to 12 years and evaluate variables that associate with neurodevelopment. METHODS: Participants (ages 3-12 years) in a prospective, longitudinal, multicenter study underwent neurodevelopmental testing with Weschler Preschool and Primary Scale of Intelligence, 3rd edition (WPPSI-III, ages 3-5 years) and Weschler Intelligence Scale for Children, 4th edition (WISC-IV, ages 6-12 years). Continuous scores were analyzed using Kolmogorov-Smironov tests compared with a normal distribution (mean = 100 ±â€Š15). Effect of covariates on Full-Scale Intelligence Quotient (FSIQ) was analyzed using linear regression. RESULTS: Ninety-three participants completed 164 WPPSI-III (mean age 3.9) and 51 WISC-IV (mean age 6.9) tests. WPPSI-III FSIQ (104 ±â€Š14, P < 0.02), Verbal IQ (106 ±â€Š14, P < 0.001), and General Language Composite (107 ±â€Š16, P < 0.001) distributions were shifted higher compared with test norms. WISC-IV FSIQ (105 ±â€Š12, P < 0.01), Perceptual Reasoning Index (107 ±â€Š12, P < 0.01), and Processing Speed Index (105 ±â€Š10, P < 0.02) also shifted upwards. In univariate and multivariable analysis, parent education (P < 0.01) was a significant predictor of FSIQ on WPPSI-III and positively associated with WISC-IV FSIQ. Male sex and higher total bilirubin and gamma glutamyl transferase (GGT) predicted lower WPPSI-III FSIQ. Portal hypertension was predictive of lower WISC-IV FSIQ. CONCLUSIONS: This cohort of children with BA and native liver did not demonstrate higher prevalence of neurodevelopmental delays. Markers of advanced liver disease (higher total bilirubin and GGT for age ≤5 years; portal hypertension for age ≥6) correlate with lower FSIQ and may identify a vulnerable subset of patients who would benefit from intervention.

Live vaccines after pediatric solid organ transplant: Proceedings of a consensus meeting, 2018.

Growing evidence suggests receipt of live-attenuated viral vaccines after solid organ transplant (SOT) has occurred and is safe and needed due to lapses in herd immunity. A 2-day consortium of experts in infectious diseases, transplantation, vaccinology, and immunology was held with the objective to review evidence and create expert recommendations for clinicians when considering live viral vaccines post-SOT. For consideration of VV and MMR post-transplant, evidence exists only for kidney and liver transplant recipients. For MMR vaccine post-SOT, consider vaccination during outbreak or travel to endemic risk areas. Patients who have received antiproliferative agents (eg. mycophenolate mofetil), T cell-depleting agents, or rituximab; or have persistently elevated EBV viral loads, or are in a state of functional tolerance, should be vaccinated with caution and have a more in-depth evaluation to define benefit of vaccination and net state of immune suppression prior to considering vaccination. MMR and/or VV (not combined MMRV) is considered to be safe in patients who are clinically well, are greater than 1 year after liver or kidney transplant and 2 months after acute rejection episode, can be closely monitored, and meet specific criteria of "low-level" immune suppression as defined in the document.

It takes a village: primary care of the pediatric liver transplant recipient.

PURPOSE OF REVIEW: Long-term survival is now the rule rather than the exception for infants and children who undergo liver transplantation for end-stage liver disease, metabolic liver conditions and a variety of other indications. Pediatricians and primary care providers play vital roles in the care and management of this patient population. The purpose of this review is to highlight key aspects important to the care of the pediatric liver transplant recipient. RECENT FINDINGS: Significant advances in immunosuppressive therapies and surgical techniques have contributed to improved graft and patient survival rates, shifting the focus beyond immediate survival to strategies to minimize comorbidities related to long-term immunosuppression during growing years, attend to patient and parent-reported outcomes and enhance quality of life. A multidisciplinary approach allows for monitoring and surveillance of both routine (growth, nutritional rehabilitation, cognitive development, mental and psychosocial health, contraception and daily activities) and transplant-related (adverse effects of immunosuppression, susceptible infections, extra-hepatic systems, transition from childhood to adolescence to adulthood) themes. SUMMARY: Effective communication between the primary care physician and the transplant team is imperative for optimizing best outcomes. The primary care provider should be aware of the multifacet nature of posttransplant management, which includes medication regimens, common complications and infections.

Barriers to ideal outcomes after pediatric liver transplantation.

Long-term survival for children who undergo LT is now the rule rather than the exception. However, a focus on the outcome of patient or graft survival rates alone provides an incomplete and limited view of life for patients who undergo LT as an infant, child, or teen. The paradigm has now appropriately shifted to opportunities focused on our overarching goals of "surviving and thriving" with long-term allograft health, freedom of complications from long-term immunosuppression, self-reported well-being, and global functional health. Experts within the liver transplant community highlight clinical gaps and potential barriers at each of the pretransplant, intra-operative, early-, medium-, and long-term post-transplant stages toward these broader mandates. Strategies including clinical research, innovation, and quality improvement targeting both traditional as well as PRO are outlined and, if successfully leveraged and conducted, would improve outcomes for recipients of pediatric LT.

Donor outcomes in anonymous live liver donation.

BACKGROUND & AIMS: Death rates on liver transplant waiting lists range from 5%-25%. Herein, we report a unique experience with 50 anonymous individuals who volunteered to address this gap by offering to donate part of their liver to a recipient with whom they had no biological connection or prior relationship, so called anonymous live liver donation (A-LLD). METHODS: Candidates were screened to confirm excellent physical, mental, social, and financial health. Demographics and surgical outcomes were analyzed. Qualitative interviews after donation examined motivation and experiences. Validated self-reported questionnaires assessed personality traits and psychological impact. RESULTS: A total of 50 A-LLD liver transplants were performed between 2005 and 2017. Most donors had a university education, a middle-class income, and a history of prior altruism. Half were women. Median age was 38.5 years (range 20-59). Thirty-three (70%) learned about this opportunity through public or social media. Saving a life, helping others, generativity, and reciprocity for past generosity were motivators. Social, financial, healthcare, and legal support in Canada were identified as facilitators. A-LLD identified most with the personality traits of agreeableness and conscientiousness. The median hospital stay was 6 days. One donor experienced a Dindo-Clavien Grade 3 complication that completely resolved. One-year recipient survival was 91% in 22 adults and 97% in 28 children. No A-LLD reported regretting their decision. CONCLUSIONS: This is the first and only report of the characteristics, motivations and facilitators of A-LLD in a large cohort. With rigorous protocols, outcomes are excellent. A-LLD has significant potential to reduce the gap between transplant organ demand and availability. LAY SUMMARY: We report a unique experience with 50 living donors who volunteered to donate to a recipient with whom they had no biological connection or prior relationship (anonymous living donors). This report is the first to discuss motivations, strategies and facilitators that may mitigate physical, social and ethical risk factors in this patient population. With rigorous protocols, anonymous liver donation and recipient outcomes are excellent; with appropriate clinical expertise and system facilitators in place, our experience suggests that other centers may consider the procedure for its significant potential to reduce the gap between transplant organ demand and availability.

Predicting ideal outcome after pediatric liver transplantation: An exploratory study using machine learning analyses to leverage Studies of Pediatric Liver Transplantation Data.

Machine learning analyses allow for the consideration of numerous variables in order to accommodate complex relationships that would not otherwise be apparent in traditional statistical methods to better classify patient risk. The SPLIT registry data were analyzed to determine whether baseline demographic factors and clinical/biochemical factors in the first-year post-transplant could predict ideal outcome at 3 years (IO-3) after LT. Participants who received their first, isolated LT between 2002 and 2006 and had follow-up data 3 years post-LT were included. IO-3 was defined as alive at 3 years, normal ALT (<50) or GGT (<50), normal GFR, no non-liver transplants, no cytopenias, and no PTLD. Heat map analysis and RFA were used to characterize the impact of baseline and 1-year factors on IO-3. 887/1482 SPLIT participants met inclusion criteria; 334 had IO-3. Demographic, biochemical, and clinical variables did not elucidate a visual signal on heat map analysis. RFA identified non-white race (vs white race), increased length of operation, vascular and biliary complications within 30 days, and duct-to-duct biliary anastomosis to be negatively associated with IO-3. UNOS regions 2 and 5 were also identified as important factors. RFA had an accuracy rate of 0.71 (95% CI: 0.68-0.74), PPV = 0.83, and NPV = 0.70. RFA identified participant variables that predicted IO-3. These findings may allow for better risk stratification and personalization of care following pediatric liver transplantation.

Superior Outcomes and Reduced Wait Times in Pediatric Recipients of Living Donor Liver Transplantation.

BACKGROUND: Living donor liver transplantation (LDLT) is increasingly used to bridge the gap between the current supply and demand imbalance for deceased donor organs to provide lifesaving liver transplantation. METHODS: Outcomes of 135 children who underwent LDLT were compared with 158 recipients of deceased donor liver transplantation (DDLT) at the largest pediatric liver transplant program in Canada. RESULTS: Recipients of LDLT were significantly younger than deceased donor recipients (P ≤ 0.001), less likely to require dialysis pretransplant (P < 0.002) and had shorter wait time duration when the primary indication was cholestatic liver disease (P = 0.003). The LDLT donors were either related genetically or emotionally (79%), or unrelated (21%) to the pediatric recipients. One-, 5-, and 10-year patient survival rates were significantly higher in LDLT (97%, 94%, and 94%) compared with DDLT (92%, 87%, and 80%; log-rank P = 0.02) recipients, as were graft survival rates (96%, 93%, and 93% for LDLT versus 89%, 81.4%, and 70%, respectively, for DDLT; log-rank P = 0.001). Medical and surgical complications were not statistically different between groups. Graft failure was higher in recipients of DDLT (odds ratio, 2.60; 95% confidence interval, 1.02, 6.58) than in the LDLT group after adjustment for clinical characteristics and propensity score. CONCLUSIONS: Living donor liver transplantation provides superior outcomes for children and is an excellent and effective strategy to increase the chances of receiving a liver transplant.

Corrigendum to "Assessment of School Readiness in Chronic Cholestatic Liver Disease: A Pilot Study Examining Children with and without Liver Transplantation".

[This corrects the article DOI: 10.1155/2017/9873945.].

Health-Related Quality of Life after Pediatric Liver Transplantation: A Qualitative Analysis of the Perspectives of Health Care Providers.

With improved survival outcomes after pediatric liver transplantation (LT), health-related quality of life (HRQoL) is an important outcome metric. Understanding the elements contributing to HRQoL after LT in children would enable more targeted strategies towards optimizing best outcomes. This qualitative study aimed to explore health care providers (HCP) perceptions about HRQoL after pediatric LT. Thirteen experienced HCP participated in two focus group discussions. Data analysis via a thematic analysis approach revealed 4 major themes: "LT as a facilitator of better HRQoL," "coping and adapting to LT," "living with a transplanted liver," and "the family context." HCP identified elements that both enhance (improved physical health, peer relationship, and activities of daily living) and challenge (need for immunosuppression, transplant follow-up, and restrictions) the multidimensional domains of HRQoL. HCP perceived LT to be a stressful life-changing event for children and their families. Patients and their parents' ability to cope and adjust positively to LT was perceived as a key contributor to better HRQoL. HCP perspective highlights the importance of promoting psychosocial support and a family-centered care delivery model towards the overarching goal of optimizing durable outcomes.

Assessment of School Readiness in Chronic Cholestatic Liver Disease: A Pilot Study Examining Children with and without Liver Transplantation.

Background. Assessment of school readiness evaluates physical, social-emotional, and neuropsychological domains essential for educational success. Cognitive testing of preschool aged children with chronic liver disease may guide more timely interventions and focused efforts by health care providers. Patients and Methods. Children with chronic cholestatic liver disease diagnosed as an infant and still with their native liver (NL) and children who received a liver transplant (LT) before age of 2 years underwent testing with a battery of well-validated pediatric psychometric measures. Results. Eighteen (13 LT, 5 NL) patients (median age of 4.45 and 4.05 years, resp.) were tested. Median Full-Scale IQ was 98 (range 102-116) for LT and 116 [(range 90-106), p = 0.35, NS] for NL subjects. LT recipients had significantly greater visual based difficulties, poorer caregiver rated daily living skills (p = 0.04), and higher levels of executive function based difficulties (e.g., inattention, inhibition). Conclusion. This pilot study highlights the risk of neuropsychological difficulties in early school age children who were under 2 years of age at time of LT. Comprehensive early school age assessment should integrate psychometric measures to identify children at greatest risk, thus allowing for proactive educational intervention.

Predictive Equations Are Inaccurate in the Estimation of the Resting Energy Expenditure of Children With End-Stage Liver Disease.

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. Clinical, laboratory, and indirect calorimetry data from children listed for liver transplant between February 2013 and December 2014 were reviewed. Calorimetry results were compared with cREE estimated using the Food and Agriculture Organization/World Health Organization/United Nations University (FAO/WHO/UNU), Schofield [weight], and Schofield [weight and height] equations. RESULTS: Forty-five patients were included in this study. The median age was 9 months, and the most common indication for transplantation was biliary atresia (64%). The Schofield [weight and height], FAO/WHO/UNU, and Schofield [weight] equations were compared with indirect calorimetry and found to have a mean (SD) difference of 48.8 (344.0), 59.3 (229.8), and 206.5 (502.6) kcal/d, respectively. The FAO/WHO/UNU, Schofield [weight], and Schofield [weight and height] equations introduced a mean error of 21%, 38%, and 76%, respectively. The FAO/WHO/UNU equation tended to underestimate, whereas the Schofield equations overestimated the REE. CONCLUSIONS: Commonly used predictive equations perform poorly in infants and young children with ESLD. Indirect calorimetry should be used when available to guide energy provision, particularly in children who are already malnourished.