Children With Cystic Fibrosis Have Elevated Levels of Fecal Chitinase-3-like-1.

Although chitinase-3-like-1 (CHI3L1), predominately produced by epithelial cells and macrophages, is relevant to pulmonary disease in cystic fibrosis (CF), fecal levels have not yet been assessed in children with CF. Fecal CHI3L1 was measured with a commercial immunoassay using fecal samples provided by children with CF and healthy control (HC) children. Higher median (interquartile range) fecal CHI3L1 levels were seen in the 52 children with CF than in the 35 controls: 15.97 (3.34-50.53) ng/g versus 2.93 (2.13-9.27) ng/g ( P = 0.001). Fecal CHI3LI did not differ according to sex. In the children with CF, fecal CHI3L1 levels did not correlate with growth parameters nor were the levels affected by pancreatic insufficiency. Children with CF had higher fecal CHI3L1 levels, suggesting underlying gut inflammation. Further work is required to confirm the current findings and to ascertain the longer-term significance of elevated CHI3L1.

Australasian Pediatric Gastroenterologists' Perspectives and Practices of Celiac Disease Diagnosis and Management.

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.

Patient perceptions of current and potential inflammatory bowel disease diagnostic and monitoring tests.

BACKGROUND: Less invasive inflammatory bowel disease (IBD) tests are increasingly being incorporated into clinical practice as adjuncts to endoscopy to monitor disease activity and guide optimal care. Little is known about patient perspectives of these tests. AIMS: To assess patient perspectives of the current IBD tests and potential future tests, such as saliva, urine or breath. METHODS: New Zealand adults with IBD were invited to complete an anonymous online survey. Experiences relating to blood or faecal tests, medical imaging (abdominal ultrasound, abdominal computed tomography and magnetic resonance enterography) and colonoscopy were collected. RESULTS: Of the 117 completed surveys, 112 respondents provided background details. The majority (85%) of participants were female and 88% were aged <55 years with a mean disease duration of 8.98 years. General blood tests were reported as the most prescribed, completed, comfortable test and the least worrying test results. Imaging tests were reported as the least ordered and the most refused tests. Colonoscopy was rated as the least comfortable and generated the most worry. Test explanations by a referring clinician significantly impacted on respondents' levels of understanding across all tests, but did not affect the level of worry when receiving test results. Saliva, blood and breath tests were chosen as the most preferred disease monitoring tests. CONCLUSIONS: Patients with IBD preferred any tests less invasive than colonoscopy. An explanation by referring doctor enhanced patients' understanding, but did not necessarily influence their level of comfort or worry.

Australasian paediatric gastroenterologist practices of coeliac disease diagnosis before and during the COVID-19 pandemic.

AIM: To explore the perceptions and practices of Australasian paediatric gastroenterologists in diagnosing coeliac disease (CD) before and during the COVID-19 pandemic. METHODS: Paediatric gastroenterologists in Australasia were invited via email to complete an anonymous online questionnaire over a 2-week period in 2021. RESULTS: The questionnaire was completed by 39 respondents: 33 from Australia and six from New Zealand (NZ) equating to a 66% response rate. Thirty-four (87%) of the 39 respondents reported they currently practised non-biopsy diagnosis of CD in eligible children, while the rest diagnosed CD using biopsy confirmation only. All NZ respondents practised non-biopsy CD diagnosis. A majority of responders (76%) who practised non-biopsy CD diagnosis followed the 2020 European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) guidelines. Twenty-two (56%) respondents reported that they started using a non-biopsy CD diagnosis protocol before the pandemic and did not change their practice during the pandemic, 10 (26%) started diagnosing non-biopsy CD during the pandemic, 5 (13%) stated their practices of CD were not impacted by the pandemic and 2 (5%) did not respond on whether the pandemic changed their practice. CONCLUSION: The majority of Australasian gastroenterologist respondents reported they routinely utilised the 2020 ESPGHAN diagnostic criteria in eligible children; half of them started prior to the pandemic and another quarter started this approach due to the pandemic. A minority of practitioners routinely rely only on biopsy confirmation to diagnose CD.

The Role of Gastrointestinal-Related Fatty Acid-Binding Proteins as Biomarkers in Gastrointestinal Diseases.

The fatty acid-binding proteins play a major role in intracellular transportation of long-chain fatty acids. Nine fatty acid-binding proteins have been identified, with each having individual tissue-specific functions in addition to regulation of fatty acids. This review focuses on the three fatty acid-binding proteins found in the gastrointestinal tract and discusses their role as diagnostic or disease monitoring markers in neonatal necrotizing enterocolitis, acute mesenteric ischemia, celiac disease, and inflammatory bowel disease. Of these three fatty acid-binding proteins, intestinal fatty acid-binding protein is of the most interest due to its exclusive expression in the gastrointestinal tract. The elevation of intestinal fatty acid-binding protein in blood and urine reflects enterocyte damage, regardless of the underlying cause. The short half-life of intestinal fatty acid-binding protein also means it is a relatively sensitive marker. In contrast, there is currently less evidence to support liver fatty acid-binding protein and ileal bile acid-binding protein as sensitive biomarkers in these conditions. More extensive studies with specific endpoints are required to validate the roles of these fatty acid-binding proteins in gastrointestinal diseases.

Burkholderia multivorans septicemia in a pediatric liver transplant patient.

"Cepacia syndrome", caused by Burkholderia cepacia complex and often associated with cystic fibrosis, carries a high mortality rate. It is rare for Burkholderia multivorans, a species within the B. cepacia complex, to cause cepacia syndrome even among patients with cystic fibrosis. This is the first reported fatal case of cepacia syndrome caused by B. multivorans occurring in a pediatric liver transplant recipient who does not have cystic fibrosis. We describe the unique characteristics of this pathogen among the non-cystic fibrosis population and the importance of early recognition and treatment.

Yellow is pale: the complications and challenges of late diagnosis of extrahepatic biliary atresia.

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.

Gastro-oesophageal reflux in children--what's the worry?

BACKGROUND: Gastro-oesophageal reflux is common and benign in children, especially during infancy. Distinguishing between gastrooesophageal reflux, gastro-oesophageal reflux disease and other illnesses presenting as chronic vomiting can be difficult. The general practitioner has a key role to play in identifying if a child requires referral for further investigation. OBJECTIVE: This article outlines the main differential diagnoses to be considered in children presenting with chronic vomiting and/ or regurgitation. We also discuss key management decisions regarding gastro-oesophageal reflux disease in children and when to refer to a specialist for further investigation. DISCUSSION: Chronic vomiting and regurgitation frequently occurs in infancy and is most commonly due to simple, benign gastrooesophageal reflux, which is usually self limiting without requirement for further investigation. In contrast, gastrooesophageal reflux disease requires considered management and may be a presenting symptom of food allergy requiring more intensive therapy than simple acid suppression. Regular review by the general practitioner to ascertain warning signs will ensure that other serious illnesses are not overlooked and that appropriate investigation and specialist referral are made.

Fecal Calprotectin in Combination With Standard Blood Tests in the Diagnosis of Inflammatory Bowel Disease in Children.

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 <17 years who had FC (normal range <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 <50 µg/g. Sensitivity in diagnosing IBD was further improved to 98.3% (95% CI 90.7-99.1%) when including FC >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.

Exclusive enteral nutrition in children with inflammatory bowel disease: Physician perspectives and practice.

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.