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OBJECTIVES: This study described disease characteristics and long-term outcomes in patients diagnosed with very early onset inflammatory bowel disease (VEOIBD) (diagnosed before 6 years of age) and infantile-IBD (before 2 years). METHODS: Cases from 21 centers worldwide diagnosed with VEOIBD (2008-2018), with minimum 2 years of follow-up, were retrospectively reviewed. RESULTS: The cohort included 243 patients (52% males, median follow-up of 5.8 [range 2-18] years, including 69 [28%]) with infantile-IBD. IBD subtypes included Crohn's disease (CD), ulcerative colitis (UC), or IBD-unclassified (IBDU) in 30%, 59%, and 11%, respectively. Among patients with CD, 94% had colonic involvement, and among patients with UC/IBDU, 75% had pancolitis. Patients with infantile-IBD presented with higher rates of IBDU, lower hemoglobin and albumin levels, and higher C-reactive protein, and had lower response rates to first-induction therapy and corticosteroids therapy (P < .05 for all). Colectomy and diversion surgeries were performed in 11% and 4%, respectively, with no significant differences between age groups. Corticosteroid-free remission rates were 74% and 78% after 3 and 5 years, respectively, and 86% at end of follow-up. Genetic testing was performed in 96 (40%) patients. Among tested population, 15 (16%) were identified with monogenic disease. This group demonstrated lower response rates to induction therapies, higher rates of surgical intervention, and higher rates of major infections (P < .05 for all). CONCLUSIONS: Patients with VEOIBD, including infantile-IBD, exhibit low rate of complications and surgical interventions at the long term. Patients with monogenic IBD are at risk for more severe disease course. [ABSTRACT FROM AUTHOR]

Celiac disease (CeD) is likely to be associated with growth impairment and poor weight gain. However, long-term growth patterns following diagnosis are poorly characterized. We evaluated long-term anthropometric changes in a large cohort of pediatric patients with CeD. A retrospective chart review of patients diagnosed with CeD between 1999 and 2018 was conducted. Demographic and clinical data were collected, and anthropometrics were analyzed from diagnosis and throughout follow-up. The study included 500 patients (59.8% females, median (IQR) age at diagnosis 5.7 (3.7–8.9) years), with a mean follow-up of 5.5 (range 1.5–16.2) years. Weight, height, and BMI Z-score-for-age (WAZ, HAZ, and BMIZ) increased significantly from a mean (± SD) of − 0.82 (± 1.21), − 0.73 (± 1.16), and − 0.32 (± 1.11) at diagnosis to − 0.41 (± 1.23), − 0.45(± 1.16), and − 0.17 (± 1.14) at last follow-up, respectively (p < 0.001 for WAZ and HAZ and p = 0.002 for BMIZ). The largest improvements were observed in patients diagnosed before 3 years of age (p < 0.01). Patients for whom the final adult height was available (n = 86) improved from HAZ mean (± SD) − 0.89 ± 1.37 at diagnosis to − 0.51 ± 1.28 at adulthood measurement, p < 0.05. Wasting was present in 19.7% and stunting in 16.4% of the cohort at diagnosis and normalized in 77.3% and 64.8%, respectively, within a median (IQR) time of 0.79 (0.42–4.24) and 2.3 (0.72–6.02) years, respectively. Gluten-free diet adherence and frequency of visits were not associated with normalization of wasting or stunting in all age groups. Conclusion: Over a long-term follow-up, pediatric patients with CeD demonstrate significant increases in weight, height, and BMI-for-age. Younger age at diagnosis is associated with greater improvement in weight and linear growth, emphasizing the importance of early diagnosis of CeD. What is Known: • Celiac disease (СeD) is likely to be associated with growth impairment and poor weight gain. • Long-term changes in anthropometric indices after diagnosis of CeD are not well characterized. What is New: • Over a long-term follow-up, pediatric patients with CeD demonstrate significant increases in weight, height, and BMI-for-age. • Young age at diagnosis is associated with larger improvement in weight and linear growth. [ABSTRACT FROM AUTHOR]

Current professional guidelines enable diagnosing pediatric Celiac Disease (CeD) without a biopsy, when tissue transglutaminase (TTG) IgA antibodies are > × 10 the upper limit of normal (ULN) and anti-endomysial antibodies (EMA) are positive in a second sample. We compared baseline characteristics and serology normalization in children diagnosed with or without biopsies. A retrospective study of pediatric patients diagnosed with CeD during 2020: group A, no biopsy and group B, biopsy-based diagnosis. Baseline characteristics included demographics, anthropometrics, symptoms, family history, and celiac serology. Follow-up at 6-month intervals, up to 18 months, included dietary compliance, symptoms, and serology. Of 145 children diagnosed with CeD, 42 (29%) and 103 (71%) were from group A and B respectively. Mean age was 7.8 years (range 2.4–17.9 y), 91 (62.8%) females. Baseline symptoms or signs were present in 93 (64.1%) children, without significant difference between the groups. Baseline TTG levels were > × 10 ULN in all patients in group A and 71 (68.9%) in group B. Among these patients, the rate of TTG decline during follow-up did not differ at any time point between patients diagnosed with and without biopsy, and between patients with and without symptoms. At the last follow-up visit, 24 (57%) children in group A and 46 (65%) in group B had TTG < × 3 ULN without significant difference between the groups. Conclusion: Rate of TTG decline did not differ between CeD patients diagnosed with and without biopsy, suggesting that, at least in short term, no biopsy approach may not change patients’ adherence and families’ attitude towards treatment. What is Known: • Based on current guidelines, there is a rise in the incidence of pediatric celiac disease (CeD) diagnosis without an intestinal biopsy. • There is insufficient data regarding patients’ adherence to treatment, including pattern of serology decline, based on the method of CeD diagnosis. What is New: • Children with CeD have similar baseline characteristics, including presence or absence of symptoms, whether diagnosed with or without biopsies. • During 18-month follow-up, the rate of celiac serology decline, and the reported adherence to treatment, do not differ between patients diagnosed based on biopsy or no biopsy approaches. [ABSTRACT FROM AUTHOR]

Aim: Children with inflammatory bowel disease (IBD) are prone to low bone mineral density (BMD). Our aim was to assess longitudinal changes in BMD in this population.Methods: A retrospective longitudinal study of children with IBD, treated at two tertiary centres in Israel, who underwent two BMD measurements by dual-energy X-ray absorptiometry (DXA). Changes in lumbar spine BMD (∆L1-4 z-scores) were examined for correlations with clinical characteristics.Results: The cohort included 41 patients (age at diagnosis 12.1 ± 3.5 years, 23 females).The mean interval between the scans was 3.4 ± 2.0 years. There was a trend towards improvement in L1-4 z-scores (-1.64 ± 1.02 vs -1.45 ± 0.83, P = .12). ∆L1-4 z-scores correlated positively with ∆weight-standard deviation scores (SDS), ∆height-SDS and ∆BMI-SDS, and with age at the second scan (R = .55, P < .01; R = .42, P < .01; R = .42, P = .01; R = .35, P = .02, respectively); and negatively with L1-4 z-scores at the first scan (R = -.63, P < .01). Stepwise linear regression analysis identified the first scan L1-4 z-scores and ∆weight-SDS as independent predictors of ∆L1-4 z-scores. An L1-4 z-score ≤-2 at the first DXA scan was associated with significant improvement at the second scan.Conclusion: Improvement in BMD was more pronounced in children who gained weight or whose BMD was low at the first scan. [ABSTRACT FROM AUTHOR]

We aimed to assess the correlation between clinical findings, serology, endoscopic findings, and histology in children diagnosed with celiac disease. Medical records of children diagnosed with celiac disease (2010–2017) at the Schneider Children's Hospital were reviewed retrospectively. Correlation between serologic measures anti-tissue transglutaminase (anti-tTG)/anti-endomysial antibodies (EMA) and other variables including mucosal damage, endoscopic findings (scalloping of duodenal folds), and clinical findings (abdominal pain, diarrhea, and anemia) was assessed. Out of 686 patients, 432 patients fulfilled the inclusion criteria (females 262, 61%; median age 6.0; interquartile range 4.0–9.0 years). Distribution of histopathology findings was Marsh IIIa 4%, Marsh IIIb 25%, and Marsh IIIc 71% with 313 (73%) patients having anti-tTG titer of ≥ 10 times the upper normal limit. Anti-tTG titer (but not EMA) positively correlated with Marsh grades, scalloping of duodenal folds and anemia. Anti-tTG ≥ 10 times the upper normal limit was associated with Marsh IIIc changes with an adjusted odds ratio of 4.5 (95% confidence interval, 1.7–12.1). Diarrhea and abdominal pain were not associated with serologic, endoscopic, or histologic markers of disease severity. Conclusion: Anti-tTG titers correlated with macroscopic and microscopic mucosal damage, with anemia but not with diarrhea or abdominal pain in children with celiac disease. What is Known: • Tissue transglutaminase antibody titers were shown to correlate with the degree of mucosal damage in patients with celiac disease. • There is a limited evidence regarding the association of celiac serologies with endoscopic and clinical measures. What is New: • Higher titers of tissue transglutaminase but not anti-endomysial antibodies are associated with more severe histologic and endoscopic damage and with the presence of anemia. • Symptoms do not correlate with the severity of mucosal damage such as scalloping of duodenal folds and histopathology changes according to Marsh classification or with serologic markers. [ABSTRACT FROM AUTHOR]