Liver transplantation in children: state of the art and future perspectives

To determine physical activity (PA), aerobic fitness, muscle strength, health‐related quality of life (HRQOL), fatigue, and participation in children after liver transplantation. Children, 6‐12 years, at least one year after liver transplantation, participated in this cross‐sectional study. Measurements: Time spent in moderate to vigorous PA (MVPA) was measured using an accelerometer, and aerobic fitness (VO2 peak) was measured by cardiopulmonary exercise testing. Muscle strength was measured by hand‐held dynamometry. Fatigue was measured using the multidimensional fatigue scale, and HRQOL with the Pediatric Quality of life Core scales and leisure activities was measured using the Children's Assessment of Participation and Enjoyment. Outcomes (medians and interquartile range (IQR)) were compared to norm values. Twenty‐six children participated in this study (14 boys, age 9.7 years, IQR 7.7;11.4). Children spent 0.8 hours/d (IQR 0.6;1.1) on MVPA. One child met the recommendation of at least 1 hour of MVPA every day of the week. Aerobic fitness was similar to norms (VO2 peak 1.4 L/min, IQR 1.1;1.7, Z‐score −0.3). Z‐scores of muscle strength ranged between −1.4 and −0.4 and HRQOL and fatigue between −2.3 and −0.4. Participation was similar to published norms (Z‐scores between −0.6 and 0.6). Young children after liver transplantation have similar MVPA patterns and aerobic fitness compared to published norms. Despite lower HRQOL, more fatigue, and less muscle strength, these children have similar participation in daily activities. Although children do well, it remains important to stimulate PA in children after liver transplantation in the context of long‐term management.

Section: Methodsmentioning

confidence: 99%

“…New surgical techniques and immune-suppressive medication have improved treatment and survival of children after liver transplantation. 1 One-year survival of children undergoing liver transplantation is 93% and 5-year survival 88%. 2 In the Netherlands, 5-year survival has increased in the last 20 years from 71% to 83%.…”

Section: Introductionmentioning

confidence: 99%

Physical activity and aerobic fitness in children after liver transplantation

Bos

Lelieveld

Scheenstra

et al. 2019

“…For inborn errors of metabolism children (n = 9), the proportion with scores <70 on FSIQ, PIQ, VIQ, VMI, and GAC was 33 Others have found similar neurocognitive and HRQL outcomes in children after liver transplant. [4][5][6][7][8][9][10][11][12][15][16][17][18] In our study, the average FSIQ, PIQ, and VIQ were within one-half SD of population norms, suggesting no clinically discernible differences from the population as a group; however, there were much higher than expected proportions of patients below 1 and 2 SD of population means. The SPLIT longitudinal study found that FSIQ was 71-85 in 29% of those assessed and <70 in 7%.…”

Section: Times Expected Proportion (P-value)mentioning

confidence: 64%

“…4,15,17 HRQL outcomes often were lower than in healthy controls and comparable to those of children with other chronic diseases. 16,18 We previously reported neurocognitive outcomes at kindergarten entry in 33 children who had liver transplant at age under 3 years at our center. 17 That report was limited by the small number of patients that precluded multiple regression analyses for predictors of outcomes and lack of data for HRQL and functional outcomes.…”

Section: Introductionmentioning

confidence: 99%

Kindergarten‐age neurocognitive, functional, and quality‐of‐life outcomes after liver transplantation at under 6 years of age

Joffe

Wong

Bond

et al. 2019

Background We aimed to describe school‐entry age neurocognitive, functional, and HRQL outcomes and their predictors after liver transplant done at age <6 years. Methods A prospective cohort of all (n = 69) children surviving liver transplant from 1999 to 2014 were assessed at age 55.4 (SD 7.2) months and 38.6 (12.4) months after transplant. Assessment included: the Wechsler Preschool and Primary Scales of Intelligence, Beery‐Buktenica Developmental Test of VMI, Adaptive Behavior Assessment System caregiver‐completed questionnaire, and PedsQL 4.0 Generic Core Scales. Univariate and multiple linear regression determined predictors of outcomes at P < .05. Results Neurocognitive and functional outcomes were on average within 1 SD of population norms, although shifted to the left (P ≤ .03), with more patients than expected having scores >2 (3.7‐5.9 times more, P ≤ .007) SD below population norms. Total and Summary HRQL scores were statistically significantly lower than the healthy normative population (P ≤ .02) and a congenital heart disease group (P ≤ .02), but similar to children with other chronic health conditions; differences often exceeded the MCID and were lowest in the School functioning domain. There were few predictors on multiple linear regressions, and we could not confirm previous studies that suggested various inconsistent predictors of outcomes. Neurocognitive and functional outcomes scores were highly correlated with HRQL scores except for the School functioning domain, but did not fully explain them. Conclusions Long‐term follow‐up of this vulnerable population is important in order to facilitate support for the patient and family, and early intervention for any difficulties identified.

“…Liver transplantation is a well‐established treatment and a standard of care for children with end‐stage liver disease and liver‐based metabolic diseases. Improved surgical techniques, modern immunosuppressive regimens and highly developed peri‐ and postoperative intensive care increased success rates for long‐term patient survival up to 90% . Despite excellent survival rates, complications after pLT still occur.…”

Section: Introductionmentioning

confidence: 99%

Liver transplantation during infancy: No increased rate of neurological complications

Ameres¹,

Melter²,

Zant³

et al. 2018

pLT is a highly standardized therapy for children with end‐stage liver disease and liver‐based metabolic diseases. However, NCs after transplantation occur and especially younger children are considered as more vulnerable and susceptible to NCs. Up to now, detailed data particularly for the very young age group do not exist. We therefore retrospectively studied NCs in children after pLT under age of 24 months. Forty children aged between 19 days and 22 months were evaluated according to type of NC and potential risk factors. NCs occurred in 8/40 patients (20%). All experienced new‐onset seizures and in 1/6 surviving patients, seizures evolved into epilepsy. Other NCs were intracerebral abscess (1/8 patients) and subdural hemorrhage (1/8 patients). The overall 3‐year mortality rate was 10% (4/40 patients). Significant risk factors for NCs and therefore seizures were HAT (P = 0.020), total surgery time (P = 0.009), retransplantation (P < 0.001), period of catecholamine therapy (P = 0.024), period of mechanical ventilation (P = 0.014), and period of sedation (P = 0.010). Our study is the first to provide detailed information on NCs after pLT in children under 24 months of age. The incidence of NCs in this particular group of very young patients was not increased compared to previously published data of children of all ages. Main NC was new‐onset seizure. In the surviving infants, prognosis of seizure was excellent and the risk of developing epilepsy was low. Even more, the occurrence of NCs did not significantly affect mortality or survival in this particular age group.