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Objective and aim Liver disease is a potentially life-threatening complication of intravenous/parenteral nutrition (PN). Our aim was to determine the incidence, aetiology and outcome of intestinal failure-associated liver disease (IFALD) in hospitalised children treated with long-term PN (>27 days). Methods Over 4 years all long-term intestinal failure (IF) patients were reviewed for the possible predisposing factors of age, diagnosis, PN lipid, sepsis, length of PN treatment and length of hospitalisation. Outcome measures were IFALD incidence, severity and prognosis. Results Of 60/279 (22%) children aged 0-18 years who developed IFALD, 13 (5%) progressed to type 3/ end stage disease. IFALD was associated with younger age (p=0.03), longer treatment (p<0.001), longer hospitalisation (p=0.01), surgical diagnosis (p=0.005) and prematurity (p=0.03). IFALD was not associated with sepsis. Intestinal surgery was associated with IFALD independently of age (p=0.03). Survival was 86%, with three deaths attributed to IFALD (1% of all cases), all of which were surgical. Conclusion IFALD incidence was lower than previously reported in paediatric patients, with surgical neonates at greatest risk. [ABSTRACT FROM AUTHOR]

Objective: Outcome of children with intestinal failure (IF) has improved on treatment with parenteral nutrition (PN). The effects of PN and IF on body composition (BC) are unknown. The aim was to review BC in PN-treated children and those weaned off and to compare with reference data. Design: Children on long-term/home PN underwent measurement of regional fat mass (FM) and lean mass (LM) using dual energy X-ray absorptiometry. Underlying diseases were intestinal enteropathy, n=15, short bowel syndrome (SBS), n=8 and intestinal dysmotility, n=11. PN duration was median 10 years. Fat Mass Index (FMI) and Lean Mass Index (LMI) were compared in children with and without intestinal inflammation, steroid treatment and according to PN dependency. Results: 34 children aged 5-20 years were studied. They were short, mean height SD score (SDS) -1.8 (p<0.001) and light (mean weight SDS -0.86, p<0.001) with high body mass index (BMI) SDS: mean 0.4 (p=0.04) and low Limb LMI SDS -0.9 (p<0.001). Children with SBS had low FMI SDS -0.8 ( p=0.01). BC did not significantly differ between diagnostic groups or with steroid treatment. Patients with intestinal inflammation (n=20) had higher BMI SDS than those without, p=0.007. Totally, PN-dependent children, n=11 had higher BMI SDS, p=0.004, total body FMI SDS, p=0.008 and trunk FMI SDS, p=0.001 compared with patients partially dependent and off PN. Conclusions: Significantly low limb LM was seen in all patient groups with high FM in children on total PN. Children with IF requiring PN treatment >27 days may benefit from BC monitoring and PN adjustment according to results in order to maximise linear growth and health in later life. [ABSTRACT FROM AUTHOR]