Survival, growth and quality of life in children after orthotopic liver transplantation: A 5 year experience

Abstract

The aims of this study were to investigate outcome and to evaluate areas of potential ongoing concern after orthotopic liver transplantation (OLT) in children. Actuarial survival in relation to age and degree of undernutrition at the time of OLT was evaluated in 53 children (age 0.58-14.2 years) undergoing OLT for endstage liver disease. Follow-up studies of growth and quality of life were undertaken in those with a minimum follow-up period of 12 months (n = 26). The overall 3 year actuarial survival was 70%. Survival rates did not differ between age groups (actuarial 2 year survival for ages less than 1, 1-5 and greater than 5 years were 70, 70 and 69% respectively) but did differ according to nutritional status at OLT (actuarial 2 year survival for children with Z scores for weight less than -1 was 57%, greater than -1 was 95%; P = 0.004). Significant catch-up weight gain was observed by 18 months post-transplant, while height improved less rapidly. Quality of life (assessed by Vineland Adaptive Behaviour Scales incorporating socialization, daily living skills, communication and motor skills) was good (mean composite score 91 +/- 19). All school-aged children except one were attending normal school. Two children had mild to moderate intellectual handicap related to post-operative intracerebral complications. Satisfactory long-term survival can be achieved after OLT in children regardless of age but the importance of pre-operative nutrition is emphasized. Survivors have an excellent chance of a good quality of life and of satisfactory catch-up weight gain and growth.

Does Liver Transplantation Affect Growth Pattern in Alagille Syndrome?

“…33 This difference observed in linear growth after OLT appeared to be secondary to immunosuppression (prednisone), graft dysfunction, or other post-OLT complications. [26][27][28][29][30][31][32][33] Some series reported improved linear growth in children with AGS after OLT in the same pattern observed in children who underwent OLT for other kinds of cholestatic diseases. 24,25 Conversely, other series have shown that catch-up growth after OLT in AGS or metabolic or familial cholestatic disorders is not as good as that seen in other liver disorders.…”

“…[24][25][26][27][28][29][30][31][32][33] In a recent study, it was shown that catch-up growth after OLT does not achieve normal height compared with age-and sex-matched peers. 33 This difference observed in linear growth after OLT appeared to be secondary to immunosuppression (prednisone), graft dysfunction, or other post-OLT complications.…”

Liver Transplantation

“…Existem alguns estudos indicando que até 59% das crianças transplantadas não retomam o crescimento 27 . Estudos mais recentes, entretanto, têm demonstrado que a maioria dos pacientes (80%) vão crescer e se desenvolver normalmente 28,29 . O retardo no crescimento parece estar diretamente relacionado com o uso de esteróides, que vem diminuindo com o uso de Tacrolimus.…”

Body Composition and Components of Energy Expenditure in Children With End- Stage Liver Disease

“…The evidence suggests that poor nutrition and growth failure are major factors increasing the morbidity and mortality of children who undergo liver transplantation (3)(4)(5)(6). Because metabolic transformations in the liver are central in intermediary metabolism, whole body metabolism and energy expenditure may be very different in children with ESLD.…”