Neurodevelopmental, nutritional, and clinical outcomes of infants and children with end-stage liver disease awaiting liver transplantation
Creators: Razcon Echeagaray, Andrea
Publisher: name: University of Alberta Library
Year: 2023

<p>Background/Aim: Patients with pediatric end-stage liver disease (ESLD) are at increased vulnerability for neurodevelopmental delay (NDD), due to exposure to risk factors such as malnutrition, hyperammonemia, and environmental deprivation. We hypothesized that NDD would be prevalent in infants and children awaiting LTx, particularly in the motor skills domain, and that NDD would be associated with pre-LTx malnutrition and adverse pre- and post-LTx clinical outcomes. Methods: A secondary analysis of previously collected data from a retrospective study in infants and children (31M/36F) who attended the Pediatric LTx Clinic at the Stollery Children’s Hospital (2009-2019) was conducted. The study encompassed six timepoints: LTx assessment, time of LTx, Intensive Care Unit (ICU) discharge, hospital discharge, 6-month follow-up and 12-month follow-up. NDD was assessed at LTx assessment using the Vineland Adaptive Behaviour Scales [motor skill, socialization, communication, adaptive behavior composite (ABC) scores]. The cohort was categorized as having an adequate adaptive level if the had an ABC score ≥85, and as having an inadequate adaptive level if their ABC score &lt;85. Nutritional data (nutritional status, route of nutritional delivery and intake) was collected at LTx assessment. Nutritional status was determined per the Subjective Global Nutritional Assessment (SGNA) tool, the McLaren criteria for wasting (based on percent ideal body weight, %IBW, and the World Health Organization’s criteria for stunting (height for age z-score &lt;-2). Growth parameters (daily weight/height gain, weight/height velocity SDS) were collected at all timepoints. Clinical outcomes [encephalopathy, hepato-pulmonary syndrome, hepato-renal syndrome, varices, presence of ascites, infections (total, fungal, bacterial, viral), and hospital visits (type/duration/frequency) were collected pre-LTx. Post-LTx outcomes included ICU/total hospital length of stay (LOS), ventilation dependency, mortality, infection/rejection (type, frequency), and major complications (vascular, biliary, others). Results: Neurodevelopment was predominantly characterized as adequate or low average. Seventy-two percent lacked age-appropriate gross motor skills. A below median motor skills score was associated with increased rates of pre-LTx encephalopathy (trend, p=0.15), post-LTx ICU LOS (trend, p=0.06), and ventilator dependency (p=0.05). SGNA was found to be the strongest predictor of neurodevelopmental outcomes, followed by age (p&lt;0.05). Malnutrition was prevalent (36% moderately malnourished, 55% severely malnourished) in the cohort when classified using the SGNA, but not McLaren and WHO criteria. When aggregating neurodevelopmental and nutritional status, the following phenotypes were identified (prevalence of malnutrition differed when using the 3 definitions): adequate adaptive level ± malnutrition [SGNA: 9% (well nourished) and 57% (malnourished); McLaren criteria: 54% (well nourished) and 11% (malnourished); WHO criteria: 53% (well nourished) and 9% (malnourished)] and inadequate adaptive level ± malnutrition [SGNA: 0% (well nourished) and 34% (malnourished); McLaren criteria: 28% (well nourished) and 8% (malnourished); WHO criteria: 33% (well nourished) and 5% (malnourished)]. An adequate adaptive level ± malnutrition (per any definition) was associated with improved growth parameters [daily weight/height gain (g/day and mm/day) and height velocity SDS] in the 6- and 12-month follow-ups (p&lt;0.05). A lower percentage of those with an inadequate adaptive level ± malnutrition had higher rates of participants achieving age-appropriate weight gain post-LTx (p&lt;0.05). Conclusions: Pediatric patients with ESLD have high rates of NDD, particularly in the motor skill domain. Worse scores (overall and domain-specific) are associated with adverse nutritional and clinical outcomes. Nevertheless, evidence shows that malnutrition secondary to ESLD plays a major role in neurodevelopment. NDD must be considered when developing intervention strategies pre- and post-LTx to achieve optimal outcomes and health-related quality of life in this population.</p>