S. E. Chin

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

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 <1,1‐5 and >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 <−1 was 57%, >−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.

Malnutrition in children with chronic liver disease accepted for liver transplantation: Clinical profile and effect on outcome

Abstract  The nutritional profiles of 37 children (aged 0.5–14.0 years) with chronic liver disease at the time of acceptance for orthotopic liver transplantation (OLTP) have been evaluated using clinical, biochemical and body composition methods. Nutritional progress while waiting for a donor has been related to outcome, whether transplanted or not. At the time of acceptance, most children were underweight (mean standard deviation (s.d.) weight = 1.4±0.2) and stunted (mean s.d. height = ‐ 2.2±0.4), had low serum albumin (27/35) and had reduced body fat and depleted body cell mass (measured by total body potassium ‐ mean % expected for age = 58±5%, n= 15). Mean ad libitum nutrient intake was 63±5% of recommended daily intake (RDI). Those who died while waiting (n= 8) had significantly lower mean initial s.d. weight compared with those transplanted. The overall actuarial 1 year survival of those who were transplanted (mean waiting time = 75 days) was 81% but those who were initially well nourished (s.d. weight > ‐ 1.0) had an actuarial 1 year survival of 100%. There were no significant differences in actuarial survival in relationship to age, type of transplant (whole liver or segmental), liver biochemistry or the presence or absence of ascites. Of the total group accepted for OLTP, whether transplanted or not, the overall 1 year survival for those who were relatively well nourished was 88% and for those undernourished (initial s.d. weight > ‐ 1.0) was 38% (P<0.003). Declining nutritional status during the waiting period also adversely affected outcome. We conclude that malnutrition and/or declining nutritional status is a major factor adversely affecting survival in children awaiting OLTP. In transplant units where waiting time is greater than 40 days, earlier referral, prioritization of cases and the use of adult donor livers may reduce this risk and efforts to maintain or improve nutritional status deserve further study.

Glomerular abnormalities in children undergoing orthotopic liver transplantation

A prospective study of renal function was undertaken on an unselected group of 8 children with chronic progressive liver disease on whom a renal biopsy was performed subsequently at the time of orthotopic liver transplantation. Two patients had abnormal urinalyses and 2 elevated urinary albumin/creatinine ratios. The remainder had no clinical evidence of renal dysfunction. All had normal serum creatinine concentrations. Glomerular abnormalities were present in all renal biopsies and were of two types: hepatic glomerulosclerosis (n=5) and minor glomerular abnormalities (n=3). IgM immunofluorescence was present in all biopsies and IgA in 6. Elevated serum immunoglobulin levels were observed in all patients, with IgM elevation in 6, IgA in 4 and IgG in 6. C3 and/or C4 were reduced in 5 patients and increased circulating immune complexes containing IgM were noted in 4. The clinical significance of these cirrhosis-associated glomerular abnormalities can only be established by long-term follow-up studies after orthotopic liver transplantation.

Survival, Growth and Quality of Life After Liver-Transplant in Children

To investigate outcome and evaluate areas of potential ongoing concern after orthotopic liver transplantation (OLT) in children, overall actuarial survival rates in relation to age and degree of undernutrition at the time of OLT have been evaluated and followup studies of growth parameters and quality of life undertaken in a group of 48 children (age 0.5-14.2 yr) undergoing OLT for end-stage liver disease. Overall 3 yr actuarial survival was 68%. Survival rates did not differ between age groups (actuarial 1 year survival for ages 5-15 yr, 1-5 yr and <1 yr were 68%, 72% and 62% respectively) but did differ according to nutritional status at OLT (actuarial 1 year survival for children with Z scores for weight <-1 was 58%, >-1 was 94% p=0.01)- Significant catchup weight gain and growth were observed by 6, 12 and 18 months post transplant (n=17). Of 28 survivors studied 12 months after OLT, quality of life was assessed by adaptive behaviour and social skills indices. In all but 3 cases, quality of life was judged to be excellent and all school aged children bar one were attending normal school. Two cases had intracerebral perioperative complications with mild to moderate intellectual handicap.Satisfactory long term survival can be achieved after OLT in children regardless of age but the importance of preoperative nutrition is emphasized. Survivors have an excellent chance of good quality of life and cmchup growth by 1 year post-transplant.