Frailty in Children with Liver Disease: A Prospective Multicenter Study

Lurz, Eberhard; Quammie, Claudia; Englesbe, Michael J.; Alonso, Estella M.; Lin, Henry C.; Hsu, Evelyn; Furuya, Katryn N.; Gupta, Nitika; Venkat, Veena; Daniel, J.; Leonis, Mike A.; Miloh, Tamir; Telega, Grzegorz; Yap, Jason; Menendez, Javier A.; Book, Linda S.; Himes, Ryan; Sundaram, Shikha S.; Parekh, Rulan S.; Sonnenday, C.J.; Bucuvalas, John C.; Ng, Vicky L.; Kamath, Binita M.

doi:10.1016/j.jpeds.2017.10.066

Cited by 47 publications

(58 citation statements)

References 32 publications

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“…Though historically associated with poor outcomes, outcomes for pediatric patients with ALF have improved, both in medical management alone and after liver transplantation 27 . Furthermore, a recent study of children with ESLD due to chronic disease suggests that these children may be further disadvantaged by the presence of frailty, a marker of morbidity not adequately captured by conventional laboratory tests 28 . Frailty was shown to be present in 24% of their cohort of children and represents a possible mechanism by which children with chronic liver disease would experience a longer LOS compared to children with ALF.…”

Section: Discussionmentioning

confidence: 99%

Determinants of length of stay after pediatric liver transplantation

Covarrubias

Luo

Massie

et al. 2020

Pediatric Transplantation

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Background:We sought to identify factors that are associated with LOS following pediatric (<18 years) liver transplantation in order to provide personalized counseling and discharge planning for recipients and their families. Methods:We identified 2726 infants (≤24 months) and 3210 children (>24 months) who underwent pediatric liver-only transplantation from 2002-2017 using the Scientific Registry of Transplant Recipients. We used multilevel multivariable negative binomial regression to analyze associations between LOS and recipient and donor characteristics and calculated the MLOSR to quantify heterogeneity in LOS across centers. Results:In infants, the median LOS (IQR) was 19 (13-32) days. Hospitalization prior to transplant (ICU ratio: 1.46 1.59 1.70 ; non-ICU ratio: 1.08 1.16 1.23 ), public insurance (ratio: 1.03 1.09 1.15 ), and a segmental graft (ratio: 1.08 1.15 1.22 ) were associated with a longer LOS; thus, we would expect a 1.59-fold longer LOS in an infant admitted to the ICU compared to a non-hospitalized infant with similar characteristics. In children, the median LOS (IQR) was 13 (9-21) days. Hospitalization prior to transplant (ICU ratio: 1.49 1.62 1.77 ; non-ICU ratio: 1.34 1.44 1.56 ), public insurance (ratio: 1.02 1.07 1.13 ), a segmental graft (ratio: 1.20 1.27 1.35 ), a living donor graft (ratio: 1.27 1.38 1.51 ), and obesity (ratio: 1.03 1.10 1.17 ) were associated with a longer LOS. The MLOSR was 1.25 in infants and 1.26 in children, meaning if an infant received a transplant at another center with a longer LOS, we would expect a 1.25-fold difference in LOS driven by center practices alone. Conclusions:While center-level practices account for substantial variation in LOS, consideration of donor and recipient factors can help clinicians provide more personalized counseling for families of pediatric liver transplant candidates. K E Y W O R D S length of stay, liver transplantation, MELD, pediatric, PELD Approximately 600 children with ESLD will undergo liver transplantation annually in the United States, with priority given to the sickest candidates as determined by the PELD/MELD score. 1 Understanding hospital LOS is necessary to provide more personalized counseling and discharge planning for pediatric liver transplant recipients and their families. 2 From a more practical perspective, understanding LOS is important for families when considering how duration of hospitalization will affect other aspects of their lives, such as arranging childcare for siblings, requesting time off work, and coordinating travel between the transplant center and the patient's home. 3 Most importantly, a prolonged hospitalization places pediatric liver transplant recipient at risk for developing hospital-acquired infections.Finally, from a financial perspective, a longer LOS incurs more costs; a longer LOS in adult liver transplant recipients has been tied to increasing costs and resource utilization. 4A previous multi-center study from the pre-PELD/MELD era of pediatric liver transplantation found that recipients...

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Section: Discussionmentioning

confidence: 99%

Determinants of length of stay after pediatric liver transplantation

Covarrubias

Luo

Massie

et al. 2020

Pediatric Transplantation

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“…1. The majority of children are too young to undergo functional or performance-based (frailty) testing (47) before the time of LT, underscoring the special role and importance of muscle mass assessment in this vulnerable patient population. 2.…”

Section: Key Pointsmentioning

confidence: 99%

A North American Expert Opinion Statement on Sarcopenia in Liver Transplantation

et al. 2019

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Loss of muscle mass and function, or sarcopenia, is a common feature of cirrhosis and contributes significantly to morbidity and mortality in this population. Sarcopenia is a main indicator of adverse outcomes in this population, including poor quality of life, hepatic decompensation, mortality in patients with cirrhosis evaluated for liver transplantation (LT), longer hospital and intensive care unit stay, higher incidence of infection following LT, and higher overall health care cost. Although it is clear that muscle mass is an important predictor of LT outcomes, many questions remain, including the best modality for assessing muscle mass, the optimal cut-off values for sarcopenia, the ideal timing and frequency of muscle mass assessment, and how to best incorporate the concept of sarcopenia into clinical decision making. For these reasons, we assembled a group of experts to form the North American Working Group on Sarcopenia in Liver Transplantation to use evidence from the medical literature to address these outstanding questions regarding sarcopenia in LT. We believe sarcopenia assessment should be considered in all patients with cirrhosis evaluated for liver transplantation. Skeletal muscle index (SMI) assessed by computed tomography constitutes the best-studied technique for assessing sarcopenia in patients with cirrhosis. Cut-off values for sarcopenia, defined as SMI < 50 cm 2 /m 2 in male and < 39 cm 2 /m 2 in female patients, constitute the validated definition for sarcopenia in patients with cirrhosis. Conclusion: The management of sarcopenia requires a multipronged approach including nutrition, exercise, and additional pharmacological therapy as deemed necessary. Future studies should evaluate whether recovery of sarcopenia with nutritional management in combination with an exercise program is sustainable as well as how improvement in muscle mass might be associated with improvement in clinical outcomes.

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“…18 One recent study examined the utility of Fried Frailty Criteria in children with chronic liver disease. 19 Frailty scores were lower in children with end-stage liver disease than in those with compensated chronic liver disease and did not correlate with MELD-Na-suggesting that the score captured currently unaccounted for factors. However, children <5 years of age or with significant physical impairment had to be excluded due to expected inability to complete the tasks.…”

Section: Discussionmentioning

confidence: 90%

“…intrahepatic cholestatic syndromes, total parenteral nutrition cholestasis, primary sclerosing cholangitis or primary biliary cirrhosis, idiopathic cholestasis, congenital hepatic fibrosis, autoimmune hepatitis cirrhosis, drug toxicity, hepatitis C cirrhosis, nonalcoholic steatohepatitis cirrhosis, unknown cirrhosis, chronic rejection/graft failure, inborn errors in bile acid metabolism, and Wilson disease. 19 Frailty scores were lower in children with end-stage liver disease than in those with compensated chronic liver disease and did not correlate with MELD-Na-suggesting that the score captured currently unaccounted for factors. Acute liver failure includes diagnoses coded or text described as "acute liver failure," "fulminant liver failure," or "fulminant" without other specifying diagnosis.…”

Section: Functional Status and Waitlist Mortalitymentioning

confidence: 90%

Functional status at listing predicts waitlist and posttransplant mortality in pediatric liver transplant candidates

Perito

Bucuvalas²,

Lai

2019

American Journal of Transplantation

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Functional impairment is associated with mortality in adult liver transplant candidates. This has not been studied in pediatric liver transplant candidates. United Network for Organ Sharing Standard Transplant Analysis and Research files were used to investigate functional status, waitlist mortality, and posttransplant outcomes in children younger than 18 years who were waitlisted in 2006-2016 for primary liver transplant. Functional status was categorized, by using the Lansky Play-Performance Scale (LPPS), as normal/good (80-100), moderately impaired (50-70), or severely impaired (10-40) by center assessment. Among 3250 children not listed as Status 1A, 62% had an LPPS score of 80-100, 25% had a score of 50-70, and 13% had a score of 10-40 at listing. Children with an LPPS score of 10-40 at listing were more likely to die while on the waitlist (standardized hazard ratio 1.85, 95% confidence interval 1.09-3.13, P = .02) in analyses adjusting for being on a ventilator, breathing support, or dialysis and other illness severity measures. For the 2565 children transplanted, an LPPS score of 10-40 at listing drastically increased mortality risk by 1 year posttransplant (hazard ratio 5.77, 95% confidence interval 3.05-10.91, P < .0005). LPPS scores of 10-40 and 50-70 both increased the risk of graft loss by 1 year. Functional status is an independent predictor of waitlist and posttransplant mortality in pediatric liver transplant candidates. Validated tools for the assessment of functional status in these children would improve our ability to predict mortality risk-and to appropriately prioritize them for transplant. K E Y W O R D S clinical research/practice, health services and outcomes research, liver allograft function/ dysfunction, liver transplantation/hepatology, organ allocation, pediatrics, United Network for Organ Sharing (UNOS), waitlist management

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Frailty in Children with Liver Disease: A Prospective Multicenter Study

Cited by 47 publications

References 32 publications

Determinants of length of stay after pediatric liver transplantation

Determinants of length of stay after pediatric liver transplantation

A North American Expert Opinion Statement on Sarcopenia in Liver Transplantation

Functional status at listing predicts waitlist and posttransplant mortality in pediatric liver transplant candidates

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