Sarcopenia in Children With End‐Stage Liver Disease on the Transplant Waiting List

Abstract

Sarcopenia predicts morbidity and mortality in adults with end-stage liver disease (ESLD) and is determined by total psoas muscle area (tPMA) measurement from computed tomography (CT) imaging. Recently developed pediatric age-and sex-specific tPMA growth curves provide the opportunity to ascertain the prevalence and impact of sarcopenia in children awaiting liver transplantation (LT). We performed a retrospective single-center study to evaluate sarcopenia in children with ESLD between 1 and 16 years of age with a clinically indicated abdominal CT less than 3 months before first isolated LT. Sarcopenia was defined as a tPMA z score less than −2 measured at the intervertebral L4-5 level. Patient demographic, biochemical, and outcome data were recorded. tPMA was compared to other measures of nutritional and growth status using univariate and multivariate logistic analyses. Outcome measures included 1-year morbidity events and mortality after LT. CT images from 25 (64% female) children with a median age of 5.50 (interquartile range [IQR], 3.75, 11.33) years were reviewed. Of the 25 children with ESLD, 10 (40%) had a tPMA z score less than −2. Sarcopenia was associated with lower z scores for weight (odds ratio [OR], 0.38; P = 0.02) and height (OR, 0.32; P = 0.03) and nutritional support before LT (OR, 12.93; P = 0.01). Patients with sarcopenia had a longer duration of pediatric intensive care unit (PICU) stay (3.50 [IQR, 3.00, 6.00] vs. 2.00 [IQR, 2.00, 3.50] days; P = 0.03). Sarcopenia was prevalent in 40% of children with ESLD who underwent clinically indicated CT while awaiting LT, and lower tPMA z score was associated with anthropometrics and need for nutritional support before LT. Post-LT PICU duration was increased in children with sarcopenia, reflecting adverse outcomes associated with muscle loss. Further studies are needed to elucidate the underlying mechanisms of sarcopenia in children with ESLD. Sarcopenia, defined as a decrease in skeletal muscle mass and function, is a frequent finding in adults with cirrhosis. (1-3) In a recent consensus statement by the North American Working Group on Sarcopenia in Liver Transplantation, computed tomography (CT) assessment of total psoas muscle area (tPMA) was recommended as the gold-standard technique to assess sarcopenia in patients with cirrhosis. (4) Unaffected by ascites, tPMA is linearly related to whole body mass, providing an estimation of overall lean muscle mass in patients with end-stage liver disease (ESLD).

Malnutrition, Frailty, and Sarcopenia in Patients With Cirrhosis: 2021 Practice Guidance by the American Association for the Study of Liver Diseases

“…43 In children with end-stage liver disease, sarcopenia has been associated with adverse outcomes including growth failure, hospitalizations, infections, and motor delay. 151,155,156 Sarcopenic Obesity Sarcopenic obesity refers to the state of decreased muscle mass in the setting of increased fat mass. This phenotype presents a unique clinical challenge in that it can be difficult to detect without dedicated testing because fat mass can mask underlying muscle wasting.…”

Utility of Preoperative Computed Tomography–Based Body Metrics in Relation to Postoperative Complications in Pediatric Liver Transplantation Recipients

“…This study aimed to determine the relation between body metrics (PMI and SMI as measurements of sarcopenia, and ScFI as measurement of subcutaneous fat storage) derived from preoperative abdominal CT scans and postoperative short-term clinical outcomes and complications in children with cirrhotic liver disease undergoing LT. While these body metrics are advocated in adults for predicting postoperative outcomes (4,10), in children studies on tPSMA and PMI have shown varying results, and SMI and ScFI have not been previously studied (14)(15)(16)(17).…”

“…A recent study showed that psoas muscle index (PMI), skeletal muscle index (SMI), and fat indices could be reliably determined even in young children (median age 4.6 months) with biliary atresia (13). Recent pediatric studies have also shown potential clinical application for PMI and total psoas muscle surface area (tPMSA), but did not investigate SMI or fat indices (14)(15)(16)(17). In adults undergoing LT, sarcopenia diagnosed by CT-based body metrics is advocated as an independent predictor of higher waiting list and posttransplantation mortality, and higher perioperative complication rates (18,19).…”

Sequential Treatment of Biliary Atresia With Kasai Hepatoportoenterostomy and Liver Transplantation: Benefits, Risks, and Outcome in 393 Children

“…(3) accordingly, successful KP could contribute to save organs in the context of worsening donor shortage; and (4) even in those children where KP does not provide reversal of cholestasis and in whom LT will be eventually required, partial biliary drainage may allow to buy months and sometimes years with prolonged preservation of general and nutritional status, contributing to reduce the risks of LT (39). Data from single-center studies, as well as national or multicenter registries, reported that the 20-year native liver survival (NLS) rate after the Kasai operation ranges between 14 and 44% (12)(13)(14)(15)(16)(17).…”