Outcomes of pediatric liver transplant recipients who reach adulthood: Current perspectives and unmet needs

Table 1 Predictors of ideal long-term outcome after pediatric liver transplantation

Variables associated with ideal outcome at 10 years follow-up[9]	Prevalence1 (%)	Variables associated with AYA-ideal outcome at long term follow-up[8]	Prevalence (%)
Graft related issues
No re-transplantation	88	No late re-transplantation2	76.7
No chronic rejection	91	No chronic rejection	93.3
Normal liver enzymes (alanine aminotransferase)	89	Normal liver enzymes (alanine aminotransferase)	66.7
Normal liver enzymes (gamma-glutamyl transpeptidase)	85	Normal liver enzymes (gamma glutamyl transpeptidase)	74.2
Normal liver function (albumin)	98	Normal liver function (albumin)	87.5
Normal liver function (total bilirubin)	99
Immunosuppression-induced issues
No post-transplant lymphoproliferative disease	94	No malignancy2	95
No renal dysfunction (i.e., GFR < 90 mL/minute/1.73 m2)	91	No renal dysfunction (i.e., GFR < 90 mL/minute/1.73 m2)	61.7
Acceptable linear growth	93	No arterial hypertension2	68.3
No diabetes	99	No diabetes	99.2
Additional medications
No ongoing use of prednisone	81	No antiseizure medication	97.5
No use of antihypertensive agents	87	No treatment with steroids	80.8
No use of antiseizure medication	100

¹Prevalence of ideal outcome has been calculated from patients with all available data (n = 106/167; of these, 53 fulfilled criteria of ideal outcome since they fulfilled all the above-mentioned items).

²Differences between the two scores.

GFR: Glomerular filtration rate; AYA: Adolescent and young adult.

Table 2 Progress on the management of paediatric liver transplant recipients reaching adulthood and future actions

Topic	Progress on the management of paediatric LT recipients reaching adulthood	Future actions
pLT overall health during and beyond transition	Recognition of overall health as the ideal outcome to be achieved in the long-term follow-up	Change of current scores or development of novel composite scores, preferably derived from multicenter studies, capable of capturing overall health from both medical and psychosocial perspectives
Long-term allograft health	Increased awareness of importance of monitoring allograft health, especially beyond transition; lack of universal association between normal transaminases and absence of graft inflammation/immune-mediated injury; variable degree of histologically proven fibrosis after pLT without significant rates of progression over time, according to studies in paired biopsies	Identification of novel biomarkers capable of enabling early detection of immune-mediated graft injury and fibrosis, as well as predicting long-term clinical outcomes; integration of graft function-related biomarkers into algorithms for the assessment of overall health during long-term follow-up, either alongside or as a replacement for conventional transaminase measurements
Extrahepatic complications affecting long-term overall health	Implementation of CNI sparing/minimization strategies to reduce the risk of long-term CKD development; increased awareness of metabolic syndrome as a potentially dangerous complication after pediatric LT; increased awareness about reproductive health (including sexual dysfunction, infertility, contraception) as a key topic to address in the long-term follow-up	Prevention and aggressive correction of modifiable risk factors for CKD development; development of tailored (universal) surveillance algorithms for de novo cancer; evaluation of safety and effectiveness of available drugs for the general population of young adult LT recipients with metabolic syndrome; evaluation of effectiveness and safety of artificial reproductive technologies even in the setting of young adult LT recipients
Transition	Increased awareness of the importance of transition among many pediatric and adult transplant hepatologists; development and validation of tools to assess readiness for transition; development of structured transition pathways in many transplant centers, managed by qualified healthcare professionals	Development of a universal transition protocol applicable in all LT centers, to be tailored according to local expertise and needs; greater integration of psychosocial support into transition programs; systematic application of reliable endpoints to evaluate the quality and effectiveness of the transition process; long-term, multicenter data on the outcomes of structured transition programs to guide best practices

LT: Liver transplant; pLT: Pediatric liver transplantation; CNI: Calcineurin inhibitors; CKD: Chronic kidney disease.