Rising Incidence of Inflammatory Bowel Disease in South Asian Children in New Zealand-A Retrospective Population-Based Study.

OBJECTIVES: High rates of inflammatory bowel disease (IBD) are reported in children of South Asian (SA) descent in some western countries. This population-based study describes the incidence and clinical course of IBD in SA children compared to non-South Asian (NSA) children in New Zealand (NZ). METHODS: Children (≤15 years) with new-onset IBD presenting to a centralized tertiary referral center in Auckland, NZ from 2010 to 2020 were identified. Disease phenotype, clinical characteristics, response to exclusive enteral nutrition, clinical remission rates at 3 and 12 months, biologic use, corticosteroid exposure, and disease complications were compared by ethnicity; IBD incidence was calculated. RESULTS: There were 127 (26 SA; 101 NSA) children with Crohn disease, 41 (10 SA; 31 NSA) with ulcerative colitis, and 10 (3 SA; 7 NSA) with IBD-unclassified. IBD incidence in SA and NSA children was 14.1 per 100,000 and 4.3 per 100,000 respectively ( P < 0.001). IBD incidence increased by 5.6% per year ( P = 0.022), due to a greater rise in incidence in SA (SA 16.8% per year, P = 0.015; NSA 4.5% per year, P = 0.317). At presentation, SA children had worse biochemical parameters, severe colitis, and vitamin D deficiency. SA children had lower rates of remission following exclusive enteral nutrition (28.5% vs 65.0%, P < 0.001) or biologic induction (35.7% vs 70.8%, P = 0.020), at 3-month (35.3% vs 69.8%, P < 0.001) and 12-month follow-up (29.4% vs 55.0%, P = 0.005). No significant differences were found in disease location or corticosteroid burden. CONCLUSIONS: Increasing incidence of IBD was disproportionately represented by SA children with more severe disease and lower remission rates following exclusive enteral nutrition or biologic therapy.

The cost of investigating weight-related comorbidities in children and adolescents in Aotearoa/New Zealand.

AIM: Expert recommendations for child/adolescent obesity include extensive investigation for weight-related comorbidities, based on body mass index (BMI) percentile cut-offs. This study aimed to estimate the cost of initial investigations for weight-related comorbidities in children/adolescents with obesity, according to international expert guidelines. METHODS: The annual mean cost of investigations for weight-related comorbidities in children/adolescents was calculated from a health-funder perspective using 2019 cost data obtained from three New Zealand District Health Boards. Prevalence data for child/adolescent obesity (aged 2-14 years) were obtained from the New Zealand Health Survey (2017/2018), and prevalence of weight-related comorbidities requiring further investigation were obtained from a previous New Zealand study of a cohort of children with obesity. RESULTS: The cost of initial laboratory screening for weight-related comorbidities per child was NZD 28.36. Based on national prevalence data from 2018/2019 for children with BMI greater than the 98th percentile (obesity cut-off), the total annual cost for initial laboratory screening for weight-related comorbidities in children/adolescents aged 2-14 years with obesity was estimated at NZD 2,665,840. The cost of further investigation in the presence of risk factors was estimated at NZD 2,972,934. CONCLUSIONS: Investigating weight-related comorbidities in New Zealand according to international expert guidelines is resource-intensive. Ways to further determine who warrants investigation with an individualised approach are required.

Prospective Evaluation of the ESPGHAN Guidelines for Diagnosis of Celiac Disease in New Zealand Children.

OBJECTIVE: The 2012 European Society of Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) guideline for diagnosis of celiac disease (CD) questioned the requirement for intestinal biopsy to confirm the diagnosis. The guideline recommends that in symptomatic patients with consistent human leukocyte antigen (HLA) subtypes, the diagnostic accuracy of strongly positive serology is sufficient to confirm the diagnosis. We prospectively assessed these guidelines in a "real-life" clinical setting. METHODS: One hundred and four children referred for evaluation of possible CD were prospectively recruited. Following informed consent, blood was drawn for serological testing and HLA analysis at upper gastrointestinal endoscopy. Histological findings according to Marsh criteria were correlated with blood results and the accuracy of the guideline analyzed.The study also examined the role of deamidated gliadin peptide (DGP) in the diagnosis of CD. RESULTS: For symptomatic patients with consistent HLA subtypes, strongly positive serology (as described in the ESPGHAN guidelines) accurately predicted biopsy-proven CD in >95% of cases. DGP was positive in fewer patients than anti-TG2 or EMA. Incorporation of DGP as a second confirmatory serological test in place of EMA was associated with maintained predictive value of guideline, but fewer patients fulfilling criteria for biopsy-free diagnosis. CONCLUSIONS: The ESPGHAN guideline performs well in our population. Adoption of the guideline would reduce the number of patients requiring endoscopy without compromise in diagnostic accuracy. The involvement of pediatric gastroenterological expertise, however, remains key to diagnosis of CD.

Fatal hyperammonemia associated with disseminated Serratia marcescens infection in a pediatric liver transplant recipient.

Hyperammonemia is a rare and important complication post-liver transplantation. We review a case of a 5-month-old boy with biliary atresia who received a split liver transplant following a variceal bleed. The transplant was complicated by recurrent portal vein thrombosis. Colonized with Serratia marcescens pretransplant, he developed disseminated infection associated with very high levels of ammonia that led to his death. It is important to be aware of serum ammonia levels in patients with portal vein thrombosis, particularly in the setting of gastrointestinal bleeding and sepsis.

Prospective Incidence of Paediatric Inflammatory Bowel Disease in New Zealand in 2015: Results From the Paediatric Inflammatory Bowel Disease in New Zealand (PINZ) Study.

BACKGROUND: The global incidence of paediatric inflammatory bowel disease (IBD) is increasing. Much of the evidence attesting to this has arisen from North America and Europe. There is a relative paucity of information on the epidemiology of paediatric IBD in the Southern Hemisphere. The present study aimed to document the prospectively collected incidence of paediatric IBD in New Zealand in 2015. METHODS: All patients younger than 16 years of age and diagnosed with IBD in New Zealand between 1 January 2015 and 31 December 2015 were identified. Demographic and disease phenotypic details were collected and entered into a secure database. Age-specific population data for New Zealand were obtained and national incidence rates for IBD and its subtypes were calculated. RESULTS: The prospectively calculated incidence of paediatric IBD, Crohn disease, ulcerative colitis (UC), and IBD unclassified in New Zealand in 2015 were 5.2 (95% confidence interval 3.9-6.8), 3.5 (2.4-4.8), 1.0 (0.5-1.8), and 0.7 (0.3-1.4) per 100,000 children, respectively. CONCLUSIONS: Incidence rates of paediatric IBD in New Zealand are comparable to the highest rates published in the literature from Western Europe and North America. Ongoing prospective ascertainment of the incidence of paediatric IBD is required to better understand the environmental factors, which are accounting for this increase in disease burden.

Point Prevalence of Pediatric Inflammatory Bowel Disease in New Zealand in 2015: Initial Results from the PINZ Study.

BACKGROUND: The incidence of pediatric inflammatory bowel disease (IBD) around the world is increasing. However, there is a scarcity of data on the epidemiology of pediatric IBD in the Southern Hemisphere. This study aimed to document the point prevalence of pediatric IBD in New Zealand on June 30, 2015. METHODS: All patients in New Zealand, under 16 years of age, with a diagnosis of IBD on June 30, 2015 were identified. Demographic and disease phenotypic details were collected and entered into a secure database. Age-specific population data for New Zealand were obtained and national and regional prevalence rates were calculated. RESULTS: The point prevalence of pediatric IBD, Crohn's disease, ulcerative colitis, and inflammatory bowel disease unclassified in New Zealand on June 30, 2015 was (95% confidence intervals) 21.7 (18.9-24.8), 16.5 (14.0-19.2), 3.3 (2.2-4.6), and 1.9 (1.2-3.0) per 100,000 children, respectively. There was a striking disparity between the prevalence rates in the North and South Islands. CONCLUSIONS: The point prevalence of pediatric IBD in New Zealand represents the first-ever national, population-based prevalence rates of pediatric IBD published. Results from the Paediatric IBD in New Zealand (PINZ) study are also the first to show markedly higher prevalence rates of IBD in the southern part of a country compared with its northern counterpart. Ongoing prospective ascertainment of the incidence of pediatric IBD is required.

Utilization of Reflex Testing for Direct Bilirubin in the Early Recognition of Biliary Atresia.

BACKGROUND: Delayed diagnosis of biliary atresia is an important cause of pediatric end-stage liver failure and liver transplantation. We sought to determine whether direct bilirubin is underutilized by retrospectively reviewing patients with biliary atresia. Further, we aimed to determine the role of reflex testing for direct bilirubin in patients suspected for jaundice. METHODS: The time intervals between total bilirubin and direct bilirubin measurements were retrospectively reviewed in patients with biliary atresia. We also audited the results of two major laboratories that had implemented reflex testing for direct bilirubin. We evaluated the clinical impact and cost of reflex testing in infants with increased direct bilirubin (>1.5 mg/dL; >25 µmol/L). RESULTS: In patients with known biliary atresia, an isolated total bilirubin measurement preceded direct bilirubin measurement in 46% (40/87) of patients; with a median delay of 19 days (interquartile range 3-44 days). In the community setting, direct bilirubin had a higher clinical specificity for biliary atresia than in the hospital setting. Reporting direct bilirubin results in 1591 infants younger than 2 weeks of age in the community was associated with three admissions to the hospital, one of whom was diagnosed with biliary atresia. The cost for the two laboratories for direct-bilirubin testing was estimated at US$3200 (NZ$4600) for each newly diagnosed case of biliary atresia. CONCLUSIONS: We identified underutilization of direct bilirubin as a cause of delay in the recognition of biliary atresia and show that reflex testing for direct bilirubin in jaundiced infants is a cost-effective solution.

Diagnostic utility of modified gliadin peptide antibody assays in New Zealand children.

OBJECTIVE: The aim of the present study was to evaluate a panel of different antibody assays, including second-generation antigliadin kits, in a local paediatric population thought to be at risk for coeliac disease (CD). METHODS: Seventy-nine children, who tested positive for immunoglobulin A (IgA) antibodies to tissue transglutaminase (TG), underwent duodenal biopsy. At endoscopy, serum was collected from all of the patients, and 9 different coeliac antibody assays were performed, both as isolated assays and in combination. These included immunoglobulin A (IgA) anti-tissue transglutaminase (TGA), and IgA plus IgG anti-deamidated gliadin peptide (DGPAG). A diagnosis of CD was made if the biopsies showed Marsh grade 3 lesions. RESULTS: Twenty-four of 79 children had CD confirmed histologically. Only 39 of 79 were positive for Inova TGA, and 35 of 79 were positive for Inova DGPAG. Twenty-four of 39 who were TGA positive and 24 of 35 who were DGPAG positive had confirmed CD on biopsy. There was good correlation between TGA and DGPAG-positive predictive values. None of the modified gliadin tests produced false-negative results, and neither did the TGA. CONCLUSIONS: The Inova DGPAG and TGA assays have similar use in predicting CD in a selected paediatric population; however, in children who are positive for TGA when screened for CD, more than half have negative TGA serology when repeat testing is done at the time of biopsy. Those with persistent TGA positivity have only a 61.5% probability of having histologic CD, compared with 68.6% of those children positive for DGPAG.

Live donor liver transplantation in New Zealand: a report on the first 20 cases.

BACKGROUND: Liver transplantation (LT) is established treatment for adults and children with acute or chronic liver failure, however there are insufficient donor organs to meet demand and 14% of New Zealand patients have died waiting or were de-listed due to deterioration whilst on the waiting list. Live donor liver transplantation (LDLT) offers an alternative graft source that enables timely transplantation, but also carries the risk of morbidity and mortality for the donor. AIM: To report the initial experience with LDLT in New Zealand. METHODS: Review of donor and recipient outcomes for the first 20 cases. RESULTS: 129 potential live liver donors were assessed for 68 recipients. Donors were evaluated according to a multi-step protocol including independent donor advocacy. Twenty LDLT were performed on 7 adults and 13 paediatric recipients using 5 right lobe, 2 extended left lobe, 2 left lobe, and 11 left lateral section grafts. Five donors (25%) experienced postoperative complications, none of which were life-threatening. Four recipients had acute liver failure and 16 had chronic liver disease including one retransplant. There was a high rate of recipient biliary complications (40%) but graft and recipient survival is 100% to date. CONCLUSION: LDLT has been successfully introduced in New Zealand with good donor and recipient outcomes.

Paediatric inflammatory bowel disease in New Zealand.

AIM: To determine the incidence, presentation, and initial management of paediatric inflammatory bowel disease in New Zealand. METHODS: A prospective study in collaboration with the New Zealand Paediatric Surveillance Unit was undertaken between 2002-2003. Paediatricians and healthcare professionals working with children were surveyed monthly for cases of paediatric inflammatory bowel disease. RESULTS: There were 52 cases(30 males); 34 (66%) Crohn's disease, 9 (17%) ulcerative colitis, and 9 (17%) inflammatory bowel disease type unclassified. The estimated incidence of paediatric inflammatory bowel disease, Crohn's disease, and ulcerative colitis were 2.9, 1.9, and 0.5 per 100,000 per year respectively. Mean age at diagnosis was 11 years with a delay of 8.4 months from clinical presentation to diagnosis. 85% were European, while no Maori or Pacific Islanders had Crohn's disease or ulcerative colitis. The most common symptoms at presentation were abdominal pain (63%), rectal bleeding (57%), diarrhoea (55%), and weight loss (43%). 39% of Crohn's disease patients had perianal disease at presentation. Only 18% of the Crohn's disease patients presented with the classic triad of symptoms-abdominal pain, weight loss, and diarrhoea. Haematological laboratory abnormalities were more common in Crohn's disease. 5-aminosalicylic acid agents were the most common initial therapy followed by systemic steroids. 25% of the paediatric inflammatory bowel disease cohort received immunomodulators. CONCLUSIONS: The incidence of paediatric inflammatory bowel disease in New Zealand is comparable but at the lower end relative to North America and United Kingdom. There is more Crohn's disease than ulcerative colitis and only a minority of Crohn's disease patients presented with the classic triad of abdominal pain, weight loss, and diarrhoea. 5-aminosalicylic acid preparations and steroids as first line treatment of Crohn's disease were much more common than nutritional therapy. It is rare for New Zealand Polynesian children to develop paediatric inflammatory bowel disease.

Coeliac disease diagnosed at Starship Children's Hospital: 1999-2002.

AIM: To retrospectively review the clinical presentation and serological testing of children diagnosed with coeliac disease at Starship Children's Hospital (Auckland, New Zealand) over a 4-year period between January 1999 and December 2002. METHODS: A review of Starship Hospital medical records of all children diagnosed with coeliac disease by small bowel biopsy between January 1999 and December 2002 was conducted. Patients had anti-gliadin, endomysial, and tissue transglutaminase antibodies performed prior to small bowel biopsy. RESULTS: There were 48 patients, median age of 6.9 years (range 1.6 to 15.7 years). Comparing symptomatic age groups older and younger than 5 years, the former age group presented significantly more often with abdominal pain (p=0.005) and the latter age group presented significantly more often with failure to thrive (p=0.02). Screening at-risk groups yielded nine children (19%) with asymptomatic disease. Thirty-three of 36 (92%) patients tested positive for the anti-endomysial IgA antibody, and 26 of 27 (96%) patients tested positive with the anti-tissue transglutaminase IgA antibody. Three patients (aged 3, 4, and 6 years of age) were negative for anti-endomysial antibodies (including one also negative for anti-tissue transglutaminase antibody), but all three were positive for anti-gliadin antibody. CONCLUSIONS: Our study found that children with coeliac disease are being diagnosed at an older age. Older children also presented with more abdominal pain while younger children presented with more failure to thrive. At-risk groups for coeliac disease may be asymptomatic and form a significant group of patients diagnosed with coeliac disease. Anti-endomysial and tissue transglutaminase antibodies are reliable tests for coeliac disease. However, in younger patients or if there is a high clinical index of suspicion of coeliac disease, small bowel biopsy should be performed even if the anti-endomysial and tissue transglutaminase antibody tests are negative.