Reversal of Intestinal Failure in Children With Tufting Enteropathy Supported With Parenteral Nutrition at Home

ABSTRACTObjectives:To review long-term outcome of Intestinal Epithelial Dysplasia(IED)/tufting enteropathy(TE) patients treated with parenteral nutrition(PN) at home managed by an intestinal failure(IF) rehabilitation serviceMethods:Infants presenting from 1986–2010 with IF, and TE histology were retrospectively reviewed for up to 30 years. Data collected included: outcome, presentation, nutrition(parenteral/enteral), country of residence, race, EpCAM gene, growth, bone age, occupation.Results:13 patients (6 male) in Malta and the UK with TE histology were established on home PN. Survival was 100% for UK children and 92% overall (one death aged 13 months). Six patients (50% of the surviving 12) weaned off PN. Overall PN requirements reduced with increasing age and 25 years. Two/12 cases weaned from PN by 10 years, 1/8 by 15 years, 3/7 by 20 years and 3/4 or 75% >25 years. Six Maltese patients homozygous for the same EPCAM gene abnormality had a similar outcome to the other cases.Weight, height, bone mineralisation, bone age and IGF-1 levels were low, but improved with age. Patients achieved educational levels of parents and were employed.Conclusions:IED cases should have >92% chance of long-term survival and >50% chance of enteral autonomy by/in early adult life and 75% by 25 years. Even if PN dependent s/he can gain employment. Patients with IED managed on PN at home by an IF rehabilitation service should avoid intestinal transplant. Objectives: To review long-term outcome of Intestinal Epithelial Dysplasia(IED)/tufting enteropathy(TE) patients treated with parenteral nutrition(PN) at home managed by an intestinal failure(IF) rehabilitation service Methods: Infants presenting from 1986–2010 with IF, and TE histology were retrospectively reviewed for up to 30 years. Data collected included: outcome, presentation, nutrition(parenteral/enteral), country of residence, race, EpCAM gene, growth, bone age, occupation. Results: 13 patients (6 male) in Malta and the UK with TE histology were established on home PN. Survival was 100% for UK children and 92% overall (one death aged 13 months). Six patients (50% of the surviving 12) weaned off PN. Overall PN requirements reduced with increasing age and 25 years. Two/12 cases weaned from PN by 10 years, 1/8 by 15 years, 3/7 by 20 years and 3/4 or 75% >25 years. Six Maltese patients homozygous for the same EPCAM gene abnormality had a similar outcome to the other cases. Weight, height, bone mineralisation, bone age and IGF-1 levels were low, but improved with age. Patients achieved educational levels of parents and were employed. Conclusions: IED cases should have >92% chance of long-term survival and >50% chance of enteral autonomy by/in early adult life and 75% by 25 years. Even if PN dependent s/he can gain employment. Patients with IED managed on PN at home by an IF rehabilitation service should avoid intestinal transplant. Address correspondence and reprint requests to Dr Susan Hill, BM, MRCP, MRCPCH, DM, Gastroenterology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH UK (e-mail: susan.hill@gosh.nhs.uk). Received 1 August, 2017 Accepted 22 December, 2017 Conflicts of Interests: None declared © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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