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

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Abstract

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 <7 infusions/week were needed by 10/12, 83% by 10 years, 6/8, 75% who had reached 15 years, 5/7, 71% who had reached 20 and all 4, 100% >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.

Results:

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.

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