Background and Aim Exclusive enteral nutrition (EEN) is recognized internationally as the first line of treatment for children with active Crohn's disease (CD). A survey conducted a decade ago demonstrated that 40% of Australian pediatric gastroenterologists did not think EEN to be an appropriate treatment for CD. This study aimed to explore the current attitudes of Australian and New Zealand (NZ) pediatric gastroenterologists toward the use of EEN in children with inflammatory bowel disease (IBD). Methods All practicing pediatric gastroenterologists in Australia and NZ were invited via an existing email network to complete an anonymous online questionnaire. Results The questionnaire was completed by 37 respondents (54% response rate), 31 from Australia and 6 from NZ. All respondents felt that EEN definitely or probably has a role in inducing remission for children with newly diagnosed CD. Australian gastroenterologists were more likely to use EEN for relapsed CD or IBD‐unclassified than NZ doctors (P < 0.05). Adherence was reported to be the greatest disadvantage of EEN. Dietitians were believed to play the most crucial role in EEN administration. Variations in EEN protocols included the use of flavorings or fluids during EEN and different patterns of food reintroduction. Conclusions These Australia and NZ pediatric gastroenterologists felt that EEN plays an important role in the induction of remission in children with newly diagnosed CD. However, the perceived role of EEN use in other types of IBD varied. EEN protocols varied widely between centers. Attitudes toward the roles of EEN have altered greatly across Australasia over the last decade.

Background Infliximab pharmacokinetics in steroid-refractory [SR] ulcerative colitis [UC] suggest a need for higher dosing, but data concerning efficacy of intensification in this setting are lacking in children and inconsistent overall. Methods Paediatric patients [N = 125] treated with infliximab for SR or steroid-dependent UC were retrospectively reviewed. Outcomes [clinical response and remission, colectomy, mucosal healing, safety] with standard vs intensified induction [mean induction dose ≥7 mg/kg or interval ≤5 weeks between doses 1 and 3] were compared. Results Among 125 patients [median age 14 years, median UC duration 0.7 years, 74 SR], 73 [58%] received standard induction and 52 [42%] received intensified induction. Overall, 73 [58%] achieved remission (judged by physician global assessment [PGA] and paediatric UC activity index [PUCAI]≤10]. Among patients in remission, 7 [10%] experienced secondary loss of response by a median of 0.7 [IQR 0.4–1.0] years. Of the 74 SR patients, 17 [23%] underwent colectomy, and of the 51 steroid-dependent patients, 12 [24%] underwent colectomy. Intensified induction in SR patients was associated with a higher chance of remission (hazard ratio [HR] 3.2, p = 0.02) and a lower chance of colectomy [HR 0.4, p = 0.05], but did not improve outcomes in steroid-dependent patients. During follow-up, 46/73 [63%] patients in remission had regimen individualization, with similar rates of return to standard dosing after 1 year between those with initial intensified or standard induction. Follow-up endoscopy, performed in 35/73 patients in remission, demonstrated mucosal healing for 66%. Adverse events were rare, despite use of intensified regimens. Conclusions These data suggest a benefit from intensified infliximab induction specifically among children with steroid-refractory UC. Prospective studies comparing dosing regimens and incorporating therapeutic drug monitoring should be undertaken.

<b><i>Introduction:</i></b> Although collecting faeces and blood samples are considered non-invasive methods of monitoring Crohn’s disease (CD), these methods are less preferred by some patients. This study utilized urine as an alternative to evaluate four disease biomarkers in young adults with active CD before and after exclusive enteral nutrition (EEN) therapy. <b><i>Methods:</i></b> Urine samples collected at baseline (W0) and after 8 weeks (W8) of EEN therapy were assayed by ELISA for levels of intestinal fatty acid-binding protein (I-FABP), liver fatty acid-binding protein (L-FABP), claudin-3, and calprotectin. Levels of each biomarker were also compared with standard clinical parameters, including disease indexes, nutrient, and inflammatory markers. <b><i>Results:</i></b> Of the paired urine samples from 14 patients, 10 were female and 12 were newly diagnosed with CD. Urinary I-FABP:Cr (standardized to urine Cr) levels were significantly reduced, while urinary L-FABP:Cr levels increased following EEN therapy. Urinary L-FABP:Cr correlated positively with serum insulin-like growth factor 1 (IGF-1) (<i>r</i> = 0.60, <i>p</i> = 0.02). Urinary CLND3:Cr and calprotectin:Cr levels were not significantly different after treatment. <b><i>Conclusion:</i></b> I-FABP is a potential urinary biomarker of disease activity in adults with CD, while urinary L-FABP may be an indirect marker of nutritional status in adults with CD. CLND3 and calprotectin do not appear to have roles as urinary biomarkers in CD. These findings warrant further investigations using a larger sample size.

Background The application of the European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) celiac disease (CeD) guidelines by pediatric gastroenterologists in Australia and New Zealand (Australasia) is unknown. Similarly, long-term management practices for patients with CeD are also unknown in this region. Aims This study aimed to explore the perceptions and practices of Australasian pediatric gastroenterologists in diagnosing and managing patients with CeD. Methods Australasian pediatric gastroenterologists and trainees were invited to complete an anonymous online survey over a 3-week period. Results The survey was completed by 28 respondents, 24 from Australia and four from New Zealand. Tissue transglutaminase antibody IgA was the most frequently ordered initial serologic test. Fifteen (54%) respondents relied on duodenal biopsies for the confirmation of CeD, six (21%) followed the ESPGHAN guidelines and the remaining seven offered either biopsy confirmation or no-biopsy diagnosis according to the parents' wishes. Following diagnosis, five (18%) respondents discharged patients from care, three (11%) discharged patients after one follow-up visit, one (4%) reviewed patients for 12 months, six (21%) reviewed patients until celiac antibodies normalized and children were clinically asymptomatic, and 13 (46%) reviewed patients until transition to adult care. Conclusion Tissue transglutaminase antibody IgA was the most common initial serologic test ordered by this group of Australasian pediatric gastroenterologists. Half of these physicians rely solely on duodenal biopsy for the confirmation of CeD diagnosis: a minority routinely use the ESPGHAN guidelines. Physicians reported a wide range of CeD follow-up practices. Supplementary Information The online version contains supplementary material available at 10.1007/s10620-021-06988-2.

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To circumvent the need for an endoscopic biopsy to establish the diagnosis of coeliac disease (CD), the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) introduced a non-biopsy pathway for selected children in 2012. This pathway was recently updated to utilise anti-tissue transglutaminase IgA (anti-TTG IgA), 10× upper limit of normal (ULN) and positive endomysial antibodies (EMA). This study focused on the retrospective application of these guidelines in children from two regions of New Zealand. Methods: Children aged <18 years who had anti-TTG IgA measured and underwent oesophagogastroduodenoscopy over a 30-month period were identified retrospectively. Medical records were reviewed to determine whether patients subsequently had biopsy-proven CD (Marsh ≥2). Results: One hundred and thirty-six children, with a mean age (±standard deviation) of 9.9 ± 4.2 years, fulfilled the study criteria and 101 (74%) of these children had positive anti-TTG IgA. Eighty-two of 136 (60%) children had biopsy-proven CD. Positive anti-TTG IgA and EMA were highly sensitive in diagnosing CD, 96.3 and 98.6%, respectively. Anti-TTG-IgA ≥10× ULN alone, and combined anti-TTG IgA ≥10× ULN with positive EMA, both provided positive predictive values of 100% in diagnosing CD. Nineteen of 103 (18%) children could have been diagnosed with CD based on the ESPGHAN non-biopsy criteria. Conclusion: A proportion of New Zealand children with CD can potentially be diagnosed using the latest ESPGHAN non-biopsy criteria. However, prospective studies are required to validate this conclusion.

Introduction: Fecal calprotectin (FC) is a useful non-invasive screening test but elevated levels are not specific to inflammatory bowel disease (IBD). The study aimed to evaluate the sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of FC alone or FC in combination with other standard blood tests in the diagnosis of IBD.Methods: Children aged &lt;17 years who had FC (normal range &lt;50 μg/g) measured and underwent endoscopy over 33 months in Christchurch, New Zealand were identified retrospectively (consecutive sampling). Medical records were reviewed for patient final diagnoses.Results: One hundred and two children were included; mean age was 12.3 years and 53 were male. Fifty-eight (57%) of the 102 children were diagnosed with IBD: 49 with Crohn's disease, eight with ulcerative colitis and one with IBD-unclassified. FC of 50 μg/g threshold provided a sensitivity of 96.6% [95% confident interval (CI) 88.3–99.4%] and PPV of 72.7% (95% CI 61.9–81.4%) in diagnosing IBD. Two children with IBD however were found to have FC &lt;50 μg/g. Sensitivity in diagnosing IBD was further improved to 98.3% (95% CI 90.7–99.1%) when including FC &gt;50 μg/g or elevated platelet count. Furthermore, PPVs in diagnosing IBD improved when FC at various thresholds was combined with either low albumin or high platelet count.Conclusion: Although FC alone is a useful screening test for IBD, a normal FC alone does not exclude IBD. Extending FC to include albumin or platelet count may improve sensitivity, specificity, PPV and NPV in diagnosing IBD. However, prospective studies are required to validate this conclusion.

Extrahepatic biliary atresia classically presents in the neonatal period with jaundice and pale stools. The lack of bile pigment in stool can be unrecognised, delaying diagnosis and surgical treatment. Vitamin K is given at birth to reduce the risk of haemorrhagic disease of the newborn, but this may be inadequate to prevent the development of coagulopathy secondary to fat soluble vitamin malabsorption. We present the case of a 3 month old infant who presented with an intracerebral haemorrhage and coagulopathy thought to be secondary to fat malabsorption resulting from delayed diagnosis of extrahepatic biliary atresia. This was despite the perinatal administration of intramuscular vitamin K. His parents did not recognise the stool pallor as being abnormal. This case illustrates the importance of educating parents on the significance of pale stools, and also the risk of coagulopathy in extrahepatic biliary atresia despite perinatal intramuscular vitamin K.