Formula modifications to the Crohn's disease exclusion diet do not impact therapy success in paediatric Crohn's disease.

OBJECTIVES: The Crohn's disease exclusion diet (CDED) + partial enteral nutrition (PEN) is an emerging diet used to induce clinical remission in children with active Crohn's disease (CD). This study aims to determine the effectiveness of using the CDED+PEN to induce clinical remission in an Australian group of children with active CD using different PEN formulas and incorporating patient dietary requirements. METHODS: We retrospectively collected data from children (both newly diagnosed and with existing CD while on therapy) with active CD (Paediatric Crohn's Disease Activity Index [PCDAI] ≥10) and biochemical evidence of active disease (elevated C-reactive protein [CRP], erythrocyte sedimentation rate [ESR] or faecal calprotectin [FC]) who completed at least phase 1 (6 weeks) of the CDED+PEN to induce clinical remission. Data were collected at baseline, Week 6 and Week 12. The primary endpoint was clinical remission at Week 6 defined as PCDAI < 10. RESULTS: Twenty-four children were included in phase 1 analysis (mean age 13.8 ± 3.2 years). Clinical remission at Week 6 was achieved in 17/24 (70.8%) patients. Mean PCDAI, CRP, ESR and FC decreased significantly after 6 weeks (p < 0.05). Formula type (cow's milk based, rice based, soy based) did not affect treatment efficacy. A greater than 50% decrease in FC was achieved in 14/21 (66.7%) patients who completed phase 1 and 12/14 (85.7%) patients who completed phase 2 of the CDED+PEN. CONCLUSIONS: Formula modifications to the CDED+PEN do not impact the expected treatment efficacy in Australian children with active luminal CD.

Short Message Service Reminder Nudge for Parents and Influenza Vaccination Uptake in Children and Adolescents With Special Risk Medical Conditions: The Flutext-4U Randomized Clinical Trial.

Importance: Children with chronic medical conditions are at increased risk of severe influenza. Uptake of influenza vaccination in children and adolescents with these identified special risk medical conditions (SRMCs) is suboptimal. Objective: To assess the effectiveness of Flutext-4U, a parent short message service (SMS) reminder nudge intervention, in increasing influenza immunization in children and adolescents with SRMCs. Design, Setting, and Participants: This randomized clinical trial was conducted at a tertiary pediatric hospital in Adelaide, South Australia, from April 15 to September 30, 2021. Children and adolescents aged 6 months to younger than 18 years with SRMCs and a subspecialist outpatient appointment over a 5-month period during the Australian seasonal influenza vaccination season (April-August 2021) were eligible to participate. Follow-up was until September 30, 2021. Interventions: Participants were randomly assigned (1:1 ratio) to control: clinician nudges (hospital vaccine availability, ease of access, and recommendation from hospital subspecialists) or SMS intervention (control conditions plus an additional SMS reminder nudge to parents), with randomization stratified by age group (<5 years, 5-14 years, or >14 to <18 years). Main Outcomes and Measures: The primary outcome was influenza vaccination, as confirmed by the Australian Immunisation Register. Results: A total of 600 participants (intervention group: 298 [49.7%]; mean [SD] age, 11.5 [4.6] years; 162 female participants [54.4%]; control group: 302 [50.3%]; mean [SD] age, 11.4 [4.7] years; 155 female participants [51.3%]) were included. Influenza vaccination was 38.6% (113 of 293) in the SMS intervention group compared with 26.2% (79 of 302) in the control group (adjusted odds ratio [aOR], 1.79; 95% CI, 1.27-2.55; P = .001). Time to vaccine receipt was significantly lower among SMS participants (adjusted hazard ratio, 1.67; 95% CI, 1.25-2.22; P < .001). For participants randomly assigned by June 15, a significantly greater proportion receiving the SMS intervention were vaccinated during the optimal delivery period April to June 30 (SMS group: 40.0% [76 of 190] vs 25.4% [50 of 197]; aOR, 1.97; 95% CI, 1.28-3.06; P = .002). Conclusions and Relevance: Results of this randomized clinical trial suggest that an additional SMS reminder nudge for parents delivered in the tertiary care hospital setting to children and adolescents with SMRCs resulted in higher influenza vaccine uptake compared with clinician nudges alone. Trial Registration: ANZCTR Identifier: ACTRN12621000463875.

The clinical and genetic features of hereditary pancreatitis in South Australia.

OBJECTIVE: To characterise the clinical phenotypes and genetic variants of hereditary pancreatitis in people diagnosed in South Australia. DESIGN, SETTING, PARTICIPANTS: Cross-sectional study of people who received molecular diagnoses of hereditary pancreatitis from one of four major diagnostic services in South Australia, 1 January 2006 - 30 June 2021. MAIN OUTCOME MEASURES: Genotypic and clinical features of people with hereditary pancreatitis, including age at onset, attack frequency, pain indices, use of opioid medications, and physical and mental health impact of hereditary pancreatitis. RESULTS: We identified 44 people from ten families who received molecular diagnoses of hereditary pancreatitis during 2006-21 (including 25 Indigenous people [57%] and 27 women [61%]): 36 with PRSS1, five with SPINK1, and three with PRSS1 and SPINK1 mutations (determined by whole exome sequencing). Symptom onset before the age of ten years was reported by 37 people (84%). Pancreatitis-related pain during the preceding four weeks was described as moderate or high by 35 people (79%); 38 people regularly used opioids (86%). Fifteen patients had diabetes mellitus (34%), and eight had undergone pancreatic surgery (18%). The estimated prevalence of hereditary pancreatitis was 1.1 (95% CI, 0.72-1.4) cases per 100 000 population for non-Indigenous and 71 (95% CI, 66-77) cases per 100 000 population for Indigenous South Australians. Among people with adult-onset chronic pancreatitis admitted to South Australian public hospitals during 2001-2019, the proportions of Indigenous people (12%) and women (38%) were smaller than we report for hereditary pancreatitis. CONCLUSION: The estimated prevalence of hereditary pancreatitis in South Australia is higher than in Europe. PRSS1 gene mutations are important causes, particularly among Indigenous young people.

Using provider-parent strategies to improve influenza vaccination in children and adolescents with special risk medical conditions: a randomised controlled trial protocol.

INTRODUCTION: Influenza immunisation is a highly cost-effective public health intervention. Despite a comprehensive National Immunisation Program, influenza vaccination in children and adolescents with special risk medical conditions (SRMCs) is suboptimal. Flutext-4U is an innovative, multi-component strategy targeting paediatric hospitals, general practice and parents of children and adolescents with SRMC. The Flutext-4U study aims to assess the impact of Flutext-4U to increase influenza immunisation in children and adolescents with SRMC. METHODS AND ANALYSIS: This is a randomised controlled trial involving parents of children and adolescents (aged >6 months to <18 years) with SRMC receiving tertiary care at the Women's and Children's Hospital (WCH), Adelaide, South Australia, who are eligible for funded influenza immunisation with a hospital appointment between the start of the seasonal influenza vaccination season and 31 July 2021, their treating general practitioners (GPs), and WCH paediatric specialists.Parents (of children/adolescents with SRMC) are randomised (1:1 ratio) to standard care plus intervention (SMS reminder messages to parents; reminders (written correspondence) for their child's GP from the hospital's Paediatric Outpatients Department) or standard care (hospital vaccine availability, ease of access and reminders for WCH subspecialists) with randomisation stratified by age-group (<5, 5-14, >14 to <18 years).The primary outcome is influenza vaccination, as confirmed by the Australian Immunisation Register.The proportion vaccinated (primary outcome) will be compared between randomised groups using logistic regression, with adjustment made for age group at randomisation. The effect of treatment will be described using an OR with a 95% CI. ETHICS AND DISSEMINATION: The protocol and all study materials have been reviewed and approved by the Women's and Children's Health Network Human Research Ethics Committee (HREC/20/WCHN/5). Results will be disseminated via peer-reviewed publication and at scientific meetings, professional and public forums. TRIAL REGISTRATION NUMBER: Australian New Zealand Clinical Trials Registry (ACTRN12621000463875).

The Epidemiology and Burden of Childhood Chronic Pancreatitis in South Australia.

OBJECTIVE: To assess longitudinal, population-based data on the prevalence and impact of chronic pancreatitis in children. STUDY DESIGN: Administrative data linkage was used to ascertain an index cohort consisting of all individuals who had an initial diagnosis of chronic pancreatitis before age 19 years in the South Australian public hospital system between June 2000 and June 2019. Age- and sex-matched controls were drawn from the general population of South Australia, children with type 1 diabetes, and children with type 2 diabetes. Main outcomes and measures included hospital visits, days in hospital, emergency department (ED) visits, intensive care unit (ICU) admissions, education comparators, and incidence and prevalence estimates. RESULTS: A total of 73 incident cases were identified. The crude prevalence and incidence of pediatric chronic pancreatitis were estimated at 6.8/100 000 and 0.98/100 000 per year, respectively. Of the index cohort, 24 cases (32.8%) of pediatric chronic pancreatitis were identified as occurring in children of Aboriginal and/or Torres Strait Islander descent. Compared with matched general population controls, children with chronic pancreatitis averaged 11-fold more hospital visits, 5-fold more ED visits, and 9-fold more ICU admissions; spent 10-fold more days in the hospital; and had a 2-fold higher rate of absence from school (P < .001 for all). Similarly, children with chronic pancreatitis used substantially more health resources than children with type 1 or 2 diabetes. CONCLUSIONS: Pediatric patients with chronic pancreatitis consume a high volume of public health services and are significantly impacted in their ability to engage in education.

Paediatric Gastrointestinal, Hepatic and Pancreatic Inflammatory Myofibroblastic Tumours, A Single Centre Experience.

ABSTRACT: Inflammatory myofibroblastic tumours (IMTs) are rare soft tissue tumours. Reports of gastrointestinal tract, liver and pancreas tumours are limited. The objective of this study is to identify presenting features, contributing prognostic / etiological factors and any variability in outcomes in the context of different historical treatments. We retrospectively reviewed the records of seven children treated at our hospital between 2006 and 2019 and assessed the demographic, presentation, treatment, immunohistochemistry, and outcomes of their tumours. Age range at presentation was 4 months-15 years with a male predominance. Presentations were typically due to local mass effect or incidental discovery. Systemic symptoms were rare. Outcomes were good with six out of seven stable or in remission irrespective of treatment. Surgical resection where possible is the treatment of choice. Medical therapy had good outcomes with chemotherapy acting as first line treatment when required. The only negative prognostic factor identified was local spread at the time of presentation.

Multichannel impedance monitoring for distinguishing nonerosive reflux esophagitis with minor changes on endoscopy in children.

OBJECTIVES: There are reports describing the relationship between baseline impedance level and esophageal mucosal integrity at endoscopy, such as erosive and nonerosive reflux esophagitis. However, many children with symptoms of gastroesophageal reflux disease have normal findings or minor changes on esophagogastroduodenoscopy. We aimed to examine whether modest changes at esophagogastroduodenoscopy can be evaluated and correlated with esophageal multichannel intraluminal impedance monitoring. METHODS: Patients (ages 0-17 years) with upper gastrointestinal symptoms who underwent combined esophagogastroduodenoscopy and multichannel intraluminal impedance monitoring at the Women's and Children's Hospital, Adelaide, Australia, between 2014 and 2016 were retrospectively studied and the following data were collected and used for analysis: demographics, multichannel intraluminal impedance data, included baseline impedance. Endoscopic findings were classified by modified Los Angeles grading, Los Angeles N as normal, Los Angeles M as with minimal change such as the erythema, pale mucosa, or friability of the mucosa following biopsy. Patients on proton pump inhibitor were excluded. RESULTS: Seventy patients (43 boys; 61%) were enrolled with a mean age of 7.9 years (range 10 months to 17 years). Fifty-one patients (72.9%) were allocated to Los Angeles N, while Los Angeles M was evident in 19 patients (27.1%). Statistically significant differences were observed in the following parameters: frequency of acid and nonacid reflux and baseline impedance in channels 5 and 6. The median values of the data were 18.3 episodes, 16.0 episodes, 2461.0 Ω, 2446.0 Ω in Los Angeles N, 36.0 episodes, 31.0 episodes, 2033.0 Ω, 2009.0 Ω in Los Angeles M, respectively. CONCLUSION: Lower baseline impedance is helpful in predicting minimal endoscopic changes in the lower esophagus. A higher frequency of acid and nonacid reflux episodes was also predictive of minimal endoscopic change in the lower esophagus.

New Pathogenic Mutations Associated with Diacylglycerol O-Acyltransferase 1 Deficiency.

Diacylglycerol O-acyltransferase 1 deficiency is a recently discovered, rare congenital diarrheal disorder. We report 2 patients with newly described pathogenic mutations in diacylglycerol O-acyltransferase 1 with compound heterozygous inheritance and unusual phenotypes. This included a macrophage activation syndrome-like response seen in one patient, ameliorated with low dietary fat.

Future of paediatric gastroenterology.

This article explores what an 'ideal' paediatric gastroenterology department in the future should look like and what it could potentially provide if given carte blanche by health funds.

High-resolution esophageal manometry in pediatrics: Effect of esophageal length on diagnostic measures.

BACKGROUND: High-resolution esophageal manometry (HREM), derived esophageal pressure topography metrics (EPT), integrated relaxation pressure (IRP), and distal latency (DL) are influenced by age and size. Combined pressure and intraluminal impedance also allow derivation of metrics that define distension pressure and bolus flow timing. We prospectively investigated the effects of esophageal length on these metrics to determine whether adjustment strategies are required for children. METHODS: Fifty-five children (12.3 ± 4.5 years) referred for HREM, and 30 healthy adult volunteers (46.9 ± 3.8 years) were included. Studies were performed using the MMS system and a standardized protocol including 10 × 5 mL thin liquid bolus swallows (SBM kit, Trisco Foods) and analyzed via Swallow Gateway (www.swallowgateway.com). Esophageal distension pressures and swallow latencies were determined in addition to EGJ resting pressure and standard EPT metrics. Effects of esophageal length were examined using partial correlation, correcting for age. Adult-derived upper limits were adjusted for length using the slopes of the identified linear equations. KEY RESULTS: Mean esophageal length in children was 16.8 ± 2.8 cm and correlated significantly with age (r = 0.787, P = .000). Shorter length correlated with higher EGJ resting pressure and 4-s integrated relaxation pressures (IRP), distension pressures, and shorter contraction latencies. Ten patients had an IRP above the adult upper limit. Adjustment for esophageal length reduced the number of patients with elevated IRP to three. CONCLUSIONS & INFERENCES: We prospectively confirmed that certain EPT metrics, as well as potential useful adjunct pressure-impedance measures such as distension pressure, are substantially influenced by esophageal length and require adjusted diagnostic thresholds specifically for children.

Laparoscopic Adjustable Gastric Banding in Australian Adolescents: Should It Be Done?

OBJECTIVE: There are very few studies on laparoscopic adjustable gastric banding (LAGB) in obese adolescents with follow up for more than 36 months, let alone good prospective data beyond 24 months in Australian adolescents. We aimed to evaluate medium term (>36 months) safety and efficacy of LAGB in adolescents with severe obesity. METHODS: This is a prospective cohort study (March 2009-December 2015) in one tertiary referral hospital including obese adolescents (14-18 years) with a body mass index (BMI) >40 (or ≥35 with comorbidities) who consented to have LAGB. Exclusion criteria were syndromal causes of obesity, depression and oesophageal motility disorders. Main outcome measures include change in weight and BMI at 6, 12, 24, 36 and 48 months post LAGB; postoperative complications; and admissions. RESULTS: Twenty-one adolescents (median [interquartile range (IQR)] 17.4 [16.5-17.7] years, 9 males, mean ± SD BMI 47.3 ± 8.4 kg/m2) had a median follow up of 45.5 [32-50] months post LAGB. Follow up data were available for 16 adolescents. Weight and BMI improved significantly at all follow up times (all p < 0.01). The median maximum BMI loss was 10 [7.1-14.7] kg/m2. There were four minor early complications. Seven bands were removed due to weight loss failure/regain (two had also obstructive symptoms). CONCLUSIONS: We have shown in the longest prospective LAGB postoperative follow up study of Australian adolescents that LAGB improves BMI in the majority of adolescents without significant comorbidities. LAGB is still a reasonable option to be considered as a temporary procedure to manage severe obesity during adolescence.

Kasai hepatoportoenterostomy in South Australia: a case for 'centralized decentralization'.

BACKGROUND: Recent follow-up studies have demonstrated significant improvement in overall survival as well as survival with native liver following geographic centralization of services to three centres in the UK. However, this model has not been replicated in countries with relatively low population density such as Australia and Canada. METHODS: Retrospective evaluation of all patients born with biliary atresia (BA) in South Australia from 1989 to 2010 was performed. Thirty-one patients with BA were discovered. Two patients were excluded because the initial Kasai procedure (KP) was performed interstate. Outcome parameters measured were (i) clearance of jaundice (bilirubin of less than 20 µmol/L, by 6 months); (ii) survival with native liver; and (iii) overall survival. Kaplan-Meier survival curves were plotted for both survival with native liver and overall survival. RESULTS: The incidence of BA in South Australia between 1989 and 2010 was 7.48 per 100,000 live births. Following KP, clearance of jaundice was achieved in 42.9% of patients. Five-year actuarial survival with native liver was 55.2%, and overall 5-year actuarial survival was 89.3%. CONCLUSIONS: The results of KP performed at Women's and Children's Hospital from 1989 to 2010 can be considered comparable with international benchmarks. Based on these results, we propose the creation of a 'centralized' pool of surgeons in Australia to help continue providing 'decentralized' care of BA.

Cystic fibrosis: an update for clinicians. Part 2: hepatobiliary and pancreatic manifestations.

This paper, the second in the series, will build on the first and explore the importance of liver and pancreatic manifestations of cystic fibrosis (CF) and the effect on morbidity and mortality of this multifaceted genetic condition. It will also further develop the critical role of the gastroenterologist as part of the multidisciplinary group of clinicians and allied health staff in the effective management of patients with CF.