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European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) guidelines enable the diagnosis of celiac disease (CD) without biopsies in patients with immunoglobulin A (IgA)‐antibodies against tissue transglutaminase (TGA‐IgA) ≥ 10× the upper limit of normal (ULN) and positivity of endomysial antibodies in a second blood sample. Limited data exist comparing the biopsy versus the nonbiopsy diagnostic approach regarding long‐term outcomes in CD patients. Our study aimed to investigate the influence of the diagnostic approach on adherence to gluten‐free diet (GFD), serological remission (defined as normalization of TGA‐IgA during follow‐up (FU)) and clinical remission in CD patients with TGA‐IgA ≥ 10× ULN. Retrospective multicenter study. Patients with CD and TGA‐IgA ≥ 10× ULN at diagnosis were included in the study. Patients with confirmed diagnosis by biopsy were compared to patients diagnosed by nonbiopsy approach using univariate analysis, Kaplan–Meier survival curve, and logistic regression models. A total of 282 CD patients (192 [68.1%] in the biopsy group; 90 [31.9%] in the nonbiopsy group) were analyzed. The median time to normalization of TGA‐IgA was 16.5 months [interquartile range, IQR: 13, 28] in the biopsy and 15 months [IQR: 12, 26] in the nonbiopsy group; p= 0.14). Rates of normalized TGA‐IgA at first to third‐year FU were comparable between both groups. Adherence to GFD did not seem to be influenced by the diagnostic approach. The nonbiopsy approach is not inferior to the biopsy approach in terms of adherence to GFD and serological remission in patients with CD. Latest guidelines of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition of 2020 recommend the nonbiopsy approach for all patients with immunoglobulin A (IgA)‐antibodies against tissue transglutaminase (TGA‐IgA) ≥ 10× the upper limit of normal (ULN) and positive endomysial antibodies in a second independent blood sample.Prospective trials have shown the reliability of antibody tests in diagnosing celiac disease (CD) in pediatrics. Latest guidelines of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition of 2020 recommend the nonbiopsy approach for all patients with immunoglobulin A (IgA)‐antibodies against tissue transglutaminase (TGA‐IgA) ≥ 10× the upper limit of normal (ULN) and positive endomysial antibodies in a second independent blood sample. Prospective trials have shown the reliability of antibody tests in diagnosing celiac disease (CD) in pediatrics. Outcome of patients during follow‐up is independent of the diagnostic approach in CD patients with TGA‐IgA ≥ 10× ULN.The nonbiopsy approach is not inferior to the biopsy approach in terms of adherence to gluten‐free diet and serological remission. Outcome of patients during follow‐up is independent of the diagnostic approach in CD patients with TGA‐IgA ≥ 10× ULN. The nonbiopsy approach is not inferior to the biopsy approach in terms of adherence to gluten‐free diet and serological remission.

Inflammatory bowel disease (IBD) requires long-term drug therapy in most patients, posing a risk for adverse drug events with the need for discontinuation. In this study, we investigated adverse events (AE) necessitating drug discontinuation in pediatric and adolescent IBD patients. We used data prospectively collected from IBD patients below the age of 18 enrolled in the Swiss Inflammatory Bowel Disease Cohort Study (SIBDCS), namely demographic variables, medical characteristics, drug treatments, and related AE. We analyzed the frequency, type, and risk factors for AE necessitating drug discontinuation. A total of 509 pediatric IBD patients fulfilled the inclusion criteria of which 262 (51.5%) were diagnosed with Crohn disease (CD), 206 (40.5%) with ulcerative colitis (UC), and 41 (8%) with IBD-unclassified (IBD-U). In total, 132 (25.9%) presented with at least 1 drug-related AE that required drug cessation. Immunomodulators [methotrexate 29/120 (24.2%), azathioprine 57/372 (15.3%)] followed by tumor necrosis factor (TNF)-alpha antagonists [adalimumab 8/72 (11.1%), infliximab 22/227 (9.7%)] accounted for the highest proportions of AE necessitating treatment discontinuation. Treatment schemes with at least 3 concomitant drugs significantly amplified the risk for development of drug-related AE [odds ratio = 2.50, 95% confidence interval (1.50–4.17)] in all pediatric IBD patients. Drug-related AE necessitating discontinuation are common in pediatric and adolescent IBD patients. Caution needs to be taken in the case of concomitant drug use.

We report a case of an infant surviving aortoesophageal fistula secondary to lithium cell battery ingestion. In the setting of a delayed vascular complication, computed tomography and magnetic resonance imaging are essential to establishing the correct diagnosis and surgical management. Management of children after battery ingestion must be guided by a high index of clinical suspicion.

There is a paucity of data on extraintestinal manifestations (EIM) and their treatment in pediatric patients with inflammatory bowel disease (IBD). Since 2008, the Pediatric Swiss IBD Cohort Study has collected data on the pediatric IBD population in Switzerland. Data on 329 patients were analyzed retrospectively. A total of 55 patients (16.7%) experienced 1–4 EIM (39 Crohn disease, 12 ulcerative colitis, and 4 IBD-unclassified patients). At IBD onset, presence of EIM was more frequent than in the adult population (8.5% vs 5.0%, P= 0.014). EIM were more frequent in Crohn disease when compared to ulcerative colitis/IBD-unclassified (22.5% vs 10.3%, P= 0.003). The most prevalent EIM were peripheral arthritis (26/329, 7.9%) and aphthous stomatitis (24/329, 7.3%). Approximately 27.6% of all EIM appeared before IBD diagnosis. Median time between IBD diagnosis and occurrence of first EIM was 1 month (-37.5–149.0). Thirty-one of the 55 patients (56.4%) were treated with 1 or more anti–tumor necrosis factor (TNF) agents. IBD patients with EIM were more likely to be treated with anti-TNF compared to those without (56.4% vs 35.0%, P= 0.003). Response rates to anti-TNF depended on underlying EIM and were best for peripheral arthritis (61.5%) and uveitis (66.7%). In a cohort of pediatric patients with IBD, EIM were frequently encountered. In up to 30%, EIM appeared before IBD diagnosis. Knowledge of these findings may translate into an increased awareness of underlying IBD, thereby decreasing diagnostic delay. Anti-TNF for the treatment of certain EIM is effective, although a substantial proportion of new EIM may present despite ongoing anti-TNF therapy.

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Because the acinar cells of the exocrine pancreas in patients with Shwachman-Diamond syndrome (SDS) are severely depleted, we hypothesized that a similar deficiency may be present in acinar cells of the parotid gland.

Because the acinar cells of the exocrine pancreas in patients with Shwachman-Diamond syndrome (SDS) are severely depleted, we hypothesized that a similar deficiency may be present in acinar cells of the parotid gland. We determined serum pancreatic isoamylase and parotid amylase activities in 16 patients with SDS, 13 healthy controls, and 13 disease controls (cystic fibrosis or fibrosing pancreatitis). Parotid amylase and electrolyte concentrations were measured in stimulated parotid gland secretions. Starch digestion was assessed by breath hydrogen testing in patients with SDS (with and without enzyme supplements) and healthy controls. Serum pancreatic and parotid isoamylase values were lower in the patients with SDS than in the healthy controls (P< 0.0001 and P= 0.0002, respectively). Serum pancreatic isoamylase, but not parotid isoamylase, was significantly lower in the disease controls than in the healthy controls (P< 0.0001 and P= 0.17, respectively). Secreted parotid gland amylase concentration (units per milligram of protein) in patients with SDS was lower than that in the healthy controls (P= 0.04), whereas the disease controls were comparable to the healthy subjects (P= 0.09). Secreted parotid chloride concentration was inversely correlated with amylase concentration in the patients with SDS (P= 0.01), but no correlation was seen in the healthy controls or disease controls. When patients with SDS ingested starch without enzyme supplementation, their breath hydrogen excretion was significantly higher than that in the healthy controls (P= 0.009). Following starch ingestion with enzymes, breath hydrogen in the patients with SDS was lower (P< 0.05) than with no enzyme treatment, and no different from controls (P= 0.37). Mutations in the SBDSgene cause a generalized functional abnormality of exocrine acinar cells.

With the recognition of the close link between nutritional status and pulmonary function in cystic fibrosis (CF), treatment and prevention of malnutrition have become a major focus in the modern therapeutic approach for patients with CF. Thereby, pancreatic enzyme replacement therapy plays a central role. This article reviews key publications on important aspects of pancreatic enzyme replacement therapy contained in the literature over the last 12 months. New insights into the pathogenesis of exocrine pancreatic disease, efficacy and dosing of pancreatic enzyme preparations, occurrence of fibrosing colonopathy, enzyme replacement in the context of enteral nutrition, and assessment of pancreatic function are addressed.