| dc.description.abstract | Background and aims: Intestinal failure (IF) is a condition of severe intestinal malabsorption resulting from obstruction, dysmotility, surgical resection, congenital defects or intestinal disease. Children with IF require highly specialised nutritional treatment, such as parenteral (PN) or enteral nutrition support (ENS). Even though IF in children is uncommon, the impact on the affected individuals, their families and the health care system is high. The primary aim was to assess nutritional status and quality of life (QoL) in paediatric IF patients. Associations between nutritional treatment strategies (PN or no PN), QoL, family impact and gastrointestinal (GI) symptoms were explored. Methods: This cross-sectional analysis included 27 eligible paediatric IF patients aged one to 18. Nutritional assessment included anthropometrics, clinical data, blood samples and dietary intake obtained with two 24-hours recalls. Dietary intake, ENS and PN support were registered and calculated in Dietist Net Pro. The group were divided into the patients receiving PN at baseline visit, and those who had weaned off PN. QoL, family impact and GI-symptoms were assessed by questionnaires. Results: 20 participants received PN support whereas seven children had weaned off PN. Most of the patients had a combination of PN, ENS and dietary intake. In the total group, growth failure was prevalent (30% below -2 Z-score length, 19% below -2 Z-score weight). The IF patients with PN support received a median of 55% of their estimated energy intake from PN. An adequate nutritional status and nutrient provision were found for most macro- and micronutrients, except for iron. Three participants had mild anaemia. A high supply of B12 were seen in the PN group (6.5 µg) and reflected in blood samples. The group without PN support had a lower intake of niacin and vitamin A compared to recommended daily intake (RDI) and the PN group. Self-reported QoL were good among the children. No statistical difference was seen between nutritional treatment strategies and QoL. Parents to children on PN support reported a higher negative impact on the family functioning than the group without PN (50 vs 83 points, p=0.038). The most reported GI symptoms were abdominal pain, gas and bloating, diarrhoea and vomiting but there were no significant differences between the group receiving PN and the no PN group. Conclusion: The study underlines the importance of tailoring the nutritional strategies in IF paediatric patients as well as taking care of the families with chronically ill children. A comprehensive understanding of the children with IF is crucial to maintain the best nutritional status and QoL as possible. | eng |