Aims To review very long-term outcome with regard to growth, survival and intestinal function of children with intestinal epithelial cell dysplasia/tufting enteropathy and age when enteral autonomy gained.
Methods Twenty children who had presented in infancy with watery diarrhoea and severe intestinal failure (IF) requiring long-term parenteral nutrition (PN) treatment were reviewed. All cases had histological entrocyte tufting on small intestinal+/- colonic biopsies. Age, sex, dependence on PN, growth, survival and age of weaning PN were recorded
Results 11 cases were male and 9 female One patient died aged 2 years. The patients had all presented with diarrhoea and severe faltering growth. They were all commenced on treatment with long-term PN. In 15 cases parents were trained and PN was continued at home. Five children remained on long-term hospital PN. Height remained below the o.4th centile even when nutrition (including protein intake) was maximised and weight was in the normal range.
When reviewed the surviving 19 patients were aged from 3 – 27 (mean 13) years. Eight children were aged under 10 (3–9) years, 6 children were aged 10 – 20 years and 5 over 20 years. One of the 8 children under 10 years of age had weaned off PN. Two of 6 children aged 10–20 years had weaned off PN and 3 of 5 patients over 20 years had done so.
All 13 children still on PN also had significant enteral intake. Six/13 children had 7 infusions/week, 3/13 had 6 infusions/week, 3 others had 5/week and one, 2/week. The two patients on PN over 20 years were infusing 5 nights/week. Height had reached the 2nd centile after 18 years in the three oldest cases.
The six children (30%) with enteral autonomy gained it when aged 3–22 years. They are now aged 10–24 (mean 16.5) years and have remained off PN.
Conclusion Children with tufting enteropathy have an increasing chance of weaning off PN with increasing age ranging from 2/20 or 10% chance before 10 years to 40% aged 10–20 years and 3/5, 60% aged over 20 years. It is possible that more children could wean with best possible medical care and psychological support. Given good long-term outcome, intestinal transplant should be avoided whenever possible.
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