Abstract

Growth outcomes in children with intestinal failure (IF) after weaning from parenteral nutrition (PN) may be modified by primary diagnosis and interventions aimed at achieving enteral tolerance. We evaluated growth after weaning by diagnosis and intestinal transplant status and during treatment with the glucagon-like peptide-2 analog teduglutide. A two-center retrospective review was conducted on children diagnosed with IF at age <12 months. The z scores for weight and length/height were examined up to 5 years after PN weaning and in children who received teduglutide for >6 months. Data were reported as median and interquartile range (IQR). A total of 362 children (58% male and 72% White) were reviewed; 41% (n = 150) weaned from PN at age 1.5 years (IQR = 0.96-3). Weight and length/height data were available for 144 children; 46 received an intestinal transplant. Median weight and length/height z scores at weaning were -1.15 (IQR = -2.09 to -0.39) and -1.89 (IQR = -2.9 to -1.02), respectively. In those not transplanted, z scores remained stable (± 0.5 change). Children with small bowel atresia experienced accelerated linear growth (> +0.5 change) beginning in year 3. Most children transplanted experienced growth acceleration beginning in year 2. Fourteen children received teduglutide (median = 840 [IQR = 425-1530] days), and growth remained stable throughout treatment. Five were weaned from PN within 1 year. We observed stable growth with limited catch-up after PN weaning, with minimal variation by diagnosis, and during teduglutide therapy. Children who received an intestinal transplant experienced acceleration in weight and linear growth after weaning.

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