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Summary Background & aims Children with intestinal failure (IF) receive parental nutrition to ensure adequate growth and development. The aim of this study was to assess micronutrient status in paediatric IF patients receiving home parenteral nutrition (HPN) in comparison to a group of healthy children. Methods An observational cross-sectional study was performed at Oslo University Hospital and at the Department of Nutrition, University of Oslo from January to September 2017. All children with IF, aged two to 18 years, were invited to participate. A reference group of healthy children was recruited through social media advertisement. Dietary intake was assessed by a four-day food record, and enteral and parenteral provision was recorded. Blood samples were analysed for vitamins, minerals and haematology to assess iron status. Two spot urine samples from each subject were analysed for iodine concentration (UIC) and creatinine. Results Nineteen children with IF and 50 healthy children were included. The mean age of the participants was 10.0 years. IF-patients received a median of 76% of their estimated energy requirements from parenteral nutrition (PN). Recommended intake (RI) for iodine from the diet was reached by 16% of IF patients and 28% of healthy children. In the IF group there was a significant positive correlation between UIC and the percentage of iodine intake from oral diet and enteral nutrition support (r = 0.57, p = 0.03). Although the IF patients had a median parenteral iodine supply of more than twice the ESPGHAN recommendation, the median UIC was 89 μg/L indicating insufficient iodine status. This may suggest that the ESPGHAN recommendation for iodine in paediatric parenteral nutrition is too low. The healthy children had sufficient iodine status according to the median UIC (133 μg/L). IF patients had significantly lower total provision of iron compared to the healthy children, 4,9 vs 8,4 mg/day (p = 0.01) with 21% of IF patients and 28% of healthy children reaching RI for iron. The prevalence of anaemia was higher in IF patients than in the healthy children (40 vs.10%, p = 0.016). Conclusion The study indicates an insufficient iodine and iron status among paediatric IF patients. Iodine status was associated with enteral provision and patients had insufficient status even if they received the ESPGHAN recommendation of iodine. Trial identification number Clinical Trials AEV2017/1. 2016/391/REK sor-ost B.

Background & aims Patients with intestinal failure (IF) are dependent on long-term home parenteral nutrition (HPN) to ensure growth and development. The primary aim of the present study was to assess bone mineral density (BMD) and vitamin D status in paediatric IF patients on HPN and a group of healthy children aged 2–18 years. Secondary aims were to assess growth, body composition, nutrient provision and physical activity. Methods An observational cross-sectional study was performed at Oslo University Hospital and at the Department of Nutrition, University of Oslo, from January to September 2017. Dual energy x-ray absorptiometry (DXA; Lunar Prodigy in IF patients and Lunar iDXA in healthy subjects) was performed to assess BMD and body composition. BMD z-score (BMDz) was calculated for total body and lumbar spine L2-L4 based on the integrated reference population in the software. Weight and height were measured for growth assessment. Nutrient provision was assessed by a 4-day food record. Blood samples were analysed for 25-hydroxy-vitamin D (25(OH)D) and 1,25-dihydroxyvitamin D (1,25(OH)2D). Physical activity was reported by a questionnaire. Results Nineteen IF patients and 50 healthy children were included. The mean age of participants was 10.0 years. The aetiology of IF patients was paediatric intestinal pseudo-obstruction (58%), short bowel syndrome (26%), and intestinal enteropathy (16%). Lower median BMDz for total body (−0.4 vs 1.1, P < 0.001) and lumbar spine L2-L4 (−0.9 vs 0.2, P = 0.01) were found in the IF group compared with the healthy children. Vitamin D provision was significantly higher in IF patients (17 μg/d vs 5.3 μg/d, P < 0.001). Both groups were sufficient in 25(OH)D (IF patients 71 nmol/L vs healthy 81 nmol/L). Nevertheless, IF patients had significantly lower 1,25(OH)2D than healthy children (71 pmol/L vs 138 pmol/L, P < 0.001). The IF group was significantly shorter (height for age z-score −1,5 vs 0,1, P = 0.001) and lighter (weight for age z-score −1,0 vs 0,1, P = 0.009) compared with the healthy subjects. BMIz did not differ; however, body fat percentage was significantly higher in IF patients compared with healthy children (34% vs 25%, P = 0.02). A lower frequency of physical activity was found in the IF group compared with the healthy group (P = 0.001). Conclusions Paediatric IF patients on HPN had lower BMD, impaired growth, and higher body fat percentage in comparison with the healthy children. Despite a higher total supply of vitamin D in the IF group, the levels of 25(OH)D did not differ. Nevertheless, a significantly lower level of 1,25(OH)2D was found in IF patients. The results raise questions regarding differences between oral and parenteral vitamin D provision and whether intestinal function is important for the metabolism of vitamin D. publishedVersion

OBJECTIVES We aimed to study whether the incidence of pediatric celiac disease (CD) in South-Eastern Norway changed from 2000 to 2010. We also examined whether there was a change in symptoms and histopathological morphology in the duodenal biopsies during the same period. METHODS In 3 hospitals in South-Eastern Norway, records from pediatric patients (0-14.9 years) diagnosed with CD during two 3-year periods (2000-2002 and 2008-2010) were reviewed. Only cases with a duodenal biopsy diagnosis of CD classified as Marsh grade 2 and 3a-c were included. Frequencies of symptoms, anthropometric data, and laboratory results were compared, in addition to re-examinations of histological sections from one of the hospitals. RESULTS A total of 400 cases were diagnosed with a female to male ratio of 1.5:1. The incidence rate for 2000 to 2002 was 15.9 cases per 100,000 person-years (95% confidence interval 12.8-19.4), compared with 45.5 cases per 100,000 person-years during 2008 to 2010 (95% confidence interval 40.5-50.9), P

Background: Epidemiological studies have shown an association between gastro-oesophageal reflux disease (GORD) and asthma, and oesophageal acid perfusion may cause bronchial constriction. However, no causative relation has been proven. Aim: To assess whether acid suppression would lead to reduced asthma symptoms in children with concomitant asthma and GORD. Methods: Thirty eight children (mean age 10.8 years, range 7.2–16.8; 29 males) with asthma and a reflux index ⩾5.0 assessed by 24 hour oesophageal pH monitoring were randomised to 12 weeks of treatment with omeprazole 20 mg daily or placebo. The groups were similar in age, gender, mean reflux index, and asthma severity. Primary endpoints were asthma symptoms (daytime wheeze, symptoms at night, in the morning, and during exercise) and quality of life (PAQLQ). Secondary endpoints were changes in lung function and the use of short acting bronchodilators. At the end of the study a repeated pH study was performed to confirm the efficacy of acid suppression. Results: The change in total symptom score did not differ significantly between the omeprazole and the placebo group, and decreased by 1.28 (95% CI −0.1 to 2.65) and 1.28 (95% CI −0.72 to 3.27) respectively. The PAQLQ score increased by 0.62 (95% CI 0.29 to 0.95) in the omeprazole group compared to 0.50 (95% CI 0.29 to 0.70) in the placebo group. Change in lung function and use of short acting bronchodilators were similar in the groups. The acid suppression was adequate (reflux index Conclusion: Omeprazole treatment did not improve asthma symptoms or lung function in children with asthma and GORD.

Transforming growth factor (TGF)-α1 (encoded by TGFB1) is the prototypic member of the TGF-β family of 33 proteins that orchestrate embryogenesis, development and tissue homeostasis(1,2). Following its discovery(3), enormous interest and numerous controversies have emerged about the role of TGF-β in coordinating the balance of pro- and anti-oncogenic properties(4,5), pro- and anti-inflammatory effects(6), or pro- and anti-fibrinogenic characteristics(7). Here we describe three individuals from two pedigrees with biallelic loss-of-function mutations in the TGFB1 gene who presented with severe infantile inflammatory bowel disease (IBD) and central nervous system (CNS) disease associated with epilepsy, brain atrophy and posterior leukoencephalopathy. The proteins encoded by the mutated TGFB1 alleles were characterized by impaired secretion, function or stability of the TGF-β1-LAP complex, which is suggestive of perturbed bioavailability of TGF-β1. Our study shows that TGF-β1 has a critical and nonredundant role in the development and homeostasis of intestinal immunity and the CNS in humans.