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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

Summary Background & aims The aim was to study the influence of Nissen fundoplication on rate of gastric emptying and postprandial symptoms in relation to protein source in liquid meals in children with cerebral palsy. Methods Ten children with cerebral palsy and Nissen fundoplication and ten with cerebral palsy without Nissen fundoplication were studied. Patients had gastrostomy and received two meals, double-blinded, in random order, on separate days. Meals contained a standardised carbohydrate and fat base plus one of two protein modules (Meal A: 100% casein; Meal B: 40% casein/60% whey). The 13 C octanoic acid breath test was used to assess gastric emptying. Postprandial symptoms were recorded. Results are given as median. Results For meal A and B, respectively, time until 50% of the meal had emptied ( T 1/2 ) was 110 in the Nissen fundoplication- and 181 min in the non-Nissen fundoplication group, ( p = 0.35) and 50 and 85 min ( p = 0.25). Seven in the Nissen fundoplication group reported postprandial symptoms to meal B, none in the non-Nissen fundoplication group ( p Conclusions Compared with cerebral palsy-children without Nissen fundoplication, those with Nissen fundoplication have postprandial symptoms more frequently after receiving a rapid emptying meal. Gastric emptying alone, however, does not seem to explain the symptom occurrence. ClinicalTrials.gov: UUSKBK 28200706.

UNLABELLED Using an investigation protocol, the aim of this study was to determine the frequency of organic abnormalities in children with recurrent abdominal pain, as new diagnostic approaches may reveal a higher prevalence of organic disease in this group than has been found in most studies. Included in the study were 44 children (mean age 8.3 y; 2-15) with more than three bouts of abdominal pain severe enough to affect the daily activities of the child and lasting more than 3 mo. The investigation covered a detailed medical story, a physical examination, blood, urine and stool samples. The somatic investigation was completed by abdominal X-ray and ultrasound, lactose-breath-hydrogen test and 24-h pH monitoring in the lower oesophagus. A Child Behaviour Checklist was completed to assess psychosocial aspects of the illness. The blood, urine and stool samples were normal, and abdominal ultrasound did not give any results related to the symptoms. Constipation was diagnosed in 7 patients (16%); 9 patients (21%) had gastro-oesophageal reflux and oesophagitis was found in another 3 children. One child had nodular antral gastritis with colonization by Helicobacter pylori, and three children had pathological lactose-breath-hydrogen tests. Twenty-four children (55%) did not have any signs of organic disease. The total score for the CBCL was in the normal range in 89%. CONCLUSION Our observations indicate a higher proportion of organic abnormalities in recurrent abdominal pain than has been found in most previously reported studies, though a multicausal approach seems important.

Objectives: Few prospective population-based studies have been carried out on the incidence of inflammatory bowel disease (IBD). In a population-based study of pediatric IBD in southeastern Norway, patients Methods: From 1990 to 1993 new cases of IBD were registered in a population of 174,482 children aged less than 16 years. The patients’ diagnoses were systematically evaluated 1 year after diagnosis and the patients were followed up clinically for up to 5 years after diagnosis. Results: Sixteen cases of Crohn’s disease (CD), 14 cases of ulcerative colitis (UC) and 3 cases of indeterminate colitis (IND) were initially registered. After 1 year IND were reclassified as UC (n = 2) or CD (n = 1). Altogether, 18% (6/33) had their diagnosis changed during the 5 years of follow-up, which yielded a mean annual incidence of 2.7/100,000 for CD and 2.0/100,000 for UC. Of the children with CD, more than 80% had relapses during the 5-year period, and 6 of 18 had surgery. Two-thirds of the children with UC had relapses during the 5-year period, and 3 patients underwent colectomy. Conclusions: An incidence of 4.7/100,000 is comparable to that found in most other studies made in Europe. The relationship between UC and CD in children was found to differ from that in the adult population. One of 5 patients had their diagnosis changed during the follow-up period. Pediatric UC seems to have a more serious course of disease than in the adult IBD population, which may be explained by the higher risk of pancolitis at diagnosis.

To explore the long-term prognosis for children referred for recurrent abdominal pain (RAP), 44 children investigated for RAP 5 y ago were compared to a group of controls (n=88). The former RAP patients reported RAP, headache and school absence more frequently than controls. Conclusion: A high proportion of children referred with RAP have persistent symptoms, with more headache and school absence than controls. The diagnostic subgroup did not predict persistence of abdominal pain.

Dysmotility, nausea and vomiting are common among children with cerebral palsy. This study aimed to evaluate influence of protein composition on rate of gastric emptying and study the relation between gastric emptying and postprandial gastrointestinal symptoms.15 children with cerebral palsy, using gastrostomy, received four liquid test meals on separate days in random order. The meals contained a standard carbohydrate and fat base plus one of four protein modules (100% casein (A), hydrolysed whey (B), amino acids (C) and 40% casein/60% whey (D)) with a total energy of 1 kcal/ml. The (13)C octanoic acid breath test was applied to assess gastric emptying.When comparing half emptying time (T(1/2)) of the fast emptying meals (meal B, C and D) with the slowest emptying meal (meal A), more rapid emptying was demonstrated for meal D (p 0.001). For meal D, emptying was significantly faster in children with postprandial symptoms than in those without (p 0.01).In children with cerebral palsy using gastrostomy, gastric emptying is influenced by type of protein in the meal. The present results also suggest that there is a relation between rapid gastric emptying and postprandial gastrointestinal symptoms. CLINICALTRIALS.GOV: UUSKBK 28200706.

Aim To explore the prevalence of symptoms suggestive of gastro-oesophageal reflux disease (GERD) in asthmatics and controls, and to control for the possible effect of overweight. Methods The prevalence of GERD symptoms was assessed using a questionnaire about reflux symptoms in children with asthma (n=872, mean age 10.4 y, 65% males) compared to non-asthmatic controls (n=264, mean age 10.8 y, 48% males), and a symptom score was calculated. The association between GERD symptoms and overweight (age-adjusted BMI > 25) was assessed independently. Results A positive reflux symptom score was found in 19.7% of the asthmatics compared to 8.5% of the non-asthmatic control group (odds ratio (OR) 2.6, 95% CI 1.7-4.2). Overweight children reported GERD symptoms more frequently than children with normal weight (OR 1.8, 95% CI 1.2-2.6). Asthma and obesity remained significant predictors when analysed simultaneously by logistic regression analysis. One hundred and fifty-two children with asthma consented to an oesophageal pH study, and an abnormal pH study result (reflux index > 5.0) correlated positively with overweight (OR 4.9, 95% CI 2.2-11.0). Conclusion The prevalence of symptoms associated with gastro-oesophageal reflux was increased in children with asthma and in overweight children. Overweight and asthma were independently associated with GERD symptoms, and overweight did not explain the higher frequency of GERD in asthma patients.

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.

The prevalence of symptoms associated with gastroesophageal reflux disease (GERD) in patients with abnormal results of pH monitoring has been investigated in adults and infants. A questionnaire suitable for children between 7 and 16 years of age has been proposed, but this tool has so far not been validated. In the present study the items of the questionnaire are validated against results from an esophageal 24-h study of pH.Ninety-nine children aged from 7 to 16 years referred from two outpatient clinics for suspected GERD completed the 7-point questionnaire regarding symptoms during the week prior to a pH study. The frequency of symptoms was investigated in patients with abnormal versus normal pH (reflux index/5.0). A group of healthy children (n = 284) served as controls to estimate the frequency of symptoms in the normal population.It was found that 37/99 (37%) of patients had an abnormal pH study result. Regurgitation/vomiting yielded the best symptom discrimination, and was reported by 46% with abnormal versus 24% with normal pH-study results (p = 0.029). A weighted score including the five best discriminating symptoms was positive in 75% versus 44% (OR 3.78, CI 1.52-9.37, p = 0.006). In a comparison of children with abnormal pH studies and healthy controls, a correct diagnosis based on five symptoms could be obtained in 75% and 94%, respectively.A relatively weak association was found between reflux symptoms and a positive pH study in 7-16-year-old children referred for pH monitoring. Thus, the questionnaire is not a diagnostic tool, and its potential use is limited to epidemiological studies.

To explore the long-term prognosis for children referred for recurrent abdominal pain (RAP), 44 children investigated for RAP 5 y ago were compared to a group of controls (n=88). The former RAP patients reported RAP, headache and school absence more frequently than controls. A high proportion of children referred with RAP have persistent symptoms, with more headache and school absence than controls. The diagnostic subgroup did not predict persistence of abdominal pain.

Since Apley, more than 40 years ago, concluded that less than 10% of cases with recurrent abdominal pain (RAP) are of organic origin, medical technology has improved, the knowledge has expanded and new methods of investigation have been developed. The lack of organic findings in many children with RAP has led to the conclusion that psychological factors are important.Forty-four children with RAP underwent an investigation programme to find organic abnormalities that might explain the symptoms. Current criteria for irritable bowel syndrome (IBS) in children were used to find out what proportion fulfilled these criteria, irrespective of the organic findings on clinical investigation. A standardized questionnaire, the CBCL (Child Behaviour Checklist), was used to evaluate emotional and behavioural disturbances in children referred for RAP.Thirteen out of 26 (50%) children with no signs of organic disease fulfilled the IBS criteria as opposed to 7 out of 18 (39%) children in the group with organic findings (P = 0.68). The total score for the CBCL was in the normal range for 32 out of 36 of the children.We found a high proportion of children fulfilling the IBS criteria in both groups, thus organic abnormalities have to be excluded before making the IBS diagnosis. The results of the CBCL forms did not show any difference between children with organic versus those with non-organic abnormalities, both groups within the normal range.

We describe a 3 1/2-year-old boy with disseminated histiocytic disease probably induced by Mycoplasma pneumoniae. In this patient, acid phosphatase was elevated in serum and was also detected histochemically in the infiltrating histiocytes. The serum acid phosphatase levels increased as his his tiocytosis progressed, apparently mirroring the activity of the disease. This observation suggests that serum acid phosphatase levels should be evaluated further to determine whether they will be a useful indicator of disease in children with different histiocytosis syndromes. © 1993 Wiley-Liss, Inc.

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.