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Objectives: To assess the short- and long-term efficacy of proton pump inhibitor (PPI) therapy for pediatric eosinophilic esophagitis (EoE) in real-world practice with a step-down strategy, and to evaluate factors predictive of PPI responsiveness., Methods: We collected data regarding the efficacy of PPIs during this cross-sectional analysis of the prospective nationwide RENESE registry. Children with EoE treated with PPI monotherapy were included. Histological remission was defined as a peak eosinophilic count of <15 eosinophils (eos)/high-power field (hpf). Factors associated with PPI responsiveness were identified using multivariate logistic regression analysis., Results: After induction therapy, histological and clinico-histological remission were observed in 51.4% (n = 346) and 46.5% of children, respectively. Normal endoscopic appearance of the esophagus was associated with a higher possibility [odds ratio (OR), 9.20; 95% confidence interval (CI), 2.10-40.16], and fibrostenotic phenotype was associated with a lower possibility (OR, 0.36; 95% CI, 0.18-0.74) of histological remission. Long-term therapy with a step-down strategy effectively maintained histological remission in 68.5% and 85.3% of children at 7 months (n = 108) and 16 months (n = 34), respectively. Complete initial histological remission (≤5 eos/hpf) was associated with a higher possibility of sustained histological remission (OR, 5.08; 95% CI, 1.75-14.68). Adverse events were infrequent and mild., Conclusions: We confirmed the efficacy of PPIs for a large cohort of children with EoE with sustained histological remission using a step-down strategy. Children with fibrostenotic phenotypes are less likely to respond to induction therapy. Furthermore, patients with complete initial histological remission are more likely to experience long-term histological remission., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Objectives: Few pediatric data on phenotypic aspects of eosinophilic esophagitis (EoE) are available. The pEEr registry was developed to prospectively characterize children with EoE from Europe and Israel., Methods: pEEr is an ongoing prospective registry enrolling children with esophageal eosinophilia (≥15 eos/HPF). Anonymized data were collected from 19 pediatric centers. Data regarding demographics, clinical manifestations, endoscopy, histology, and therapies were collected., Results: A total of 582 subjects (61% male) were analyzed. The median age at diagnosis was 10.5 years [interquartile range (IQR): 5.7-17.7], whereas the age at symptom onset was 9.2 years (IQR: 4.3-16.4), resulting in a median diagnostic delay of 1.2 years (IQR: 0.7-2.3). The diagnostic delay was longer below age <6 years. Shorter diagnostic delays were associated with the presence of food allergy or a family history for EoE. Symptoms varied by age with dysphagia and food impaction more common in adolescents, while vomiting and failure to thrive more common in younger children ( P < 0.001). Among endoscopic findings, esophageal rings were more common in adolescents, whereas exudates were more frequent in younger children( P < 0.001). Patients who responded to proton pump inhibitors (PPIs) were more likely to be older, males, and less often presented severe endoscopic findings. Patients unresponsive to PPIs received topical steroids (40%), elimination diet (41%), or a combined therapy (19%)., Conclusions: EoE findings vary according to age in pediatric EoE. Young children are commonly characterized by non-specific symptoms, atopic dermatitis, food allergy, and inflammatory endoscopic lesions. Adolescents usually have dysphagia or food impaction, fibrostenotic lesions, and a better PPI response., Competing Interests: Noam Zevit: consultation fees – Dr Falk Pharma, Adare Pharmaceuticals; speaker fees – Rafa inc and Sanofi. Salvatore Oliva: consultation fees – Medtronic, Ocean Farma; speaker fees – Medtronic. Marcus Karl-Heinz Auth – consultation fees: Dr Falk Pharma; educational grants: Nutricia, Mead Johnson. Alexandra Papadopoulou: research grants from Abbvie, United Pharmaceuticals, Dr Falk Pharma GmbH, Takeda, AstraZeneca; speaker or consultation fees from Adare Pharmaceuticals, Dr Falk Pharma GmbH, Specialty Therapeutics, Uni-Pharma Pharmaceuticals Laboratories S.A., Cross Pharmaceuticals, Petsiavas, Nestle, Touch Independent Medical Education, and Sanofi-Aventis. The remaining authors report no conflicts of interest., (Copyright © 2022 by European Society for European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: The role of systemic steroids in the treatment of esophageal strictures in children with Eosinophilic Esophagitis (EoE) is poorly defined., Aims: To describe a cohort of children with EoE-associated esophageal strictures responding to systemic steroids., Methods: Retrospective review of medical records of children with EoE and moderate (<9 mm) to severe (<6 mm) strictures, who responded clinically and endoscopically to systemic steroids., Results: Twenty children (median age 10.6 ± 4.2 years; 17 males) from nine centers in six countries were included in the analysis; 16 had moderate and four, severe strictures; 18 had dysphagia or bolus impaction; median diagnostic delay was 8 months (IQR 3.5-35). Eighteen patients received oral systemic steroids (mean dose 1.4 mg/kg/day) for a median of 4 weeks, while two initially received IV steroids. All patients showed clinical improvement and 15/20 became asymptomatic. Stricture resolution at endoscopy was found in 19/20, while histological resolution of EoE (<15 eos/hpf) in 13/20. Only minor side effects were reported: hyperphagia (10/20); weight gain (5/20); hyperactivity (2/20) and acne (1/20). Esophageal dilation was required in 3/20 patients during a median follow-up of 48.5 months (IQR 26.7-73.2)., Conclusion: Children with EoE and esophageal strictures, may benefit from the use of a short course of systemic steroids, avoiding mechanical dilation., Competing Interests: Declaration of Competing Interest Manuscript title: Systemic Steroids Have a Role in Treating Esophageal Strictures in Pediatric Eosinophilic Esophagitis List of coauthors: Assaf Hoofien, Francesca Rea, Maria do Céu Espinheira, Jorge Amil Dias, Claudio Romano, Salvatore Oliva, Marcus Karl-Heinz Auth, Tsili Zangen, Nicolas Kalach, Gloria Domínguez-Ortega, Paola De Angelis Noam Zevit. This study was supported by an unconditional gift to the research fund of Dr. Noam Zevit at Schneider Medical Center by Mrs. Martha White, in loving memory of Dr. Seymor White and Dr. Judah White. Dr. Noam Zevit reports receiving consultation fees from Adare Pharmaceuticals and Dr. Falk Pharma ltd. All other authors report no relevant conflicts of interest., (Copyright © 2020 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Background/objectives: The paediatric reference range of fecal calprotectin (FC) has not been decisively established and previous studies show a wide within-age variability, suggesting that other factors like anthropometric data or type of feeding can influence FC. Our aims were to establish the normal levels of FC in healthy children grouped by age and analyze whether sex, gestational age, birth weight, type of delivery, type of feeding, or anthropometric data influence FC values., Methods: This multicentre, cross-sectional, and observational study enrolled healthy donors under 18 years of age who attended their Primary Health Care Centre for their routine Healthy Child Program visits. The exclusion criteria were: (i) immunodeficiency, (ii) autoimmune or (iii) gastrointestinal disease; (iv) medication usage; (v) gastrointestinal symptoms; or (vi) positive finding in the microbiological study., Results: We enrolled 395 subjects, mean age was 4.2 years (range 3 days to 16.9 years), and 204 were male. The median FC was 77.0 mcg/g (interquartile range 246). A negative correlation between age and FC was observed (Spearman's rho = -0.603, P<0.01), and none of the other factors analyzed were found to influence FC levels., Conclusions: Normal FC values in healthy children (particularly in infants) are higher than those considered to be altered in adults and show a negative correlation with age. It is necessary to reconsider the upper limits of FC levels for paediatric patients according to age, with further studies required to determine other factors that influence FC during infancy., (© The Author(s) 2019. Published by Oxford University Press on behalf of the Canadian Paediatric Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Eosinophilic oesophagitis is an emerging and chronic disorder mediated by the immune system, and is characterised by symptoms of oesophageal dysfunction and inflammation with isolated eosinophil infiltration in the oesophagus. It is more common in males and in atopic subjects, and the symptoms vary with age. In younger children, there is vomiting, abdominal pain and dietary problems, with dysphagia and food impaction in older children and adolescents. The diagnosis is based on the presence of symptoms and oesophageal inflammation with ≥ 15 eosinophils / high power field, and after ruling out other causes of oesophageal eosinophilia. Without treatment, the disease usually persists and can progress to fibrostenotic forms more common in adults. The treatment options included proton pump inhibitors, empirical elimination diets, and swallowed topical corticosteroids. Maintenance therapy is advisable after the induction treatment. Diet is the only treatment that is directed at the cause of the disease, on identifying the triggering food or foods. The response to the treatments requires a histological assessment due to the poor agreement between the symptoms and the oesophageal inflammation. The practical management of Eosinophilic oesophagitis presents with challenges, due to, among other causes, the current lack of availability of specific drugs, and to its approach with, occasionally complex, diet treatments. The present document, prepared by the Working Group on Eosinophilic Gastrointestinal Disorders of the Spanish Society of Paediatric Gastroenterology, Hepatology and Nutrition, has as its objective to help in the diagnostic and therapeutic approach to paediatric eosinophilic oesophagitis, based on the recent evidence-based consensus guidelines., (Copyright © 2020 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.)

Objective: The aim of the study was to describe clinical, epidemiological, and management characteristics of food protein-induced enterocolitis syndrome (FPIES) cases in Spain., Patients and Methods: Multicenter observational retrospective study. FPIES cases diagnosed in specialized units in Spain over 12 months in 2017 (January-December) according to the recently published international diagnostic criteria were included., Results: One hundred twenty patients (53.3% boys) were included. The majority were acute cases (111) with mild-to-moderate severity (76.7%). Triggering foods were cow's milk (48/120), fish (38), egg (13), rice (12), and soy (1). The majority (84.2%) of the patients had FPIES to 1 food only. In addition to vomiting (100%), pallor (89.2%), and altered behavior (88.3%) were most frequently observed in acute forms. On the contrary, diarrhea (70%), abdominal distension (33.3%), and blood in stools (44.4%) were more frequently observed in chronic cases. Oral challenge was performed in 18.9% of the acute forms compared to 44.4% of the chronic forms. The most common treatment was intravenous fluids followed by ondansetron. Corticosteroids were used in 6 patients (5 with acute symptoms and 1 chronic). Seven patients were treated with antibiotics for suspicion of infection. Most cases of cow's milk FPIES were treated with extensively hydrolyzed formulas (69.8%)., Conclusions: FPIES is not uncommon in our units. Unlike other published series, fish and egg are important triggers in our country. A greater knowledge and diffusion of the international consensus criteria will allow a better characterization of the cases and a standardization of their management.

Introduction: Lumbar puncture (LP) is a commonly performed procedure in paediatrics. Performing this technique properly can avoid the most common associated complications., Objective: To assess whether paediatricians and paediatric residents in Spain follow current recommendations for the LP technique., Material and Methods: A cross-sectional study was conducted by sending a questionnaire by mail through the Spanish Society of Paediatric Emergencies, collecting demographic information and responses to multiple choice questions about LP technique., Results: A total of 206 questionnaires were analysed, of which 143 (69.5%) were answered by paediatricians, and 63 (30.5%) by paediatric residents. The majority (128; 62.1%) of physicians did not allow parents to be present during LP, 198 (96.1%) routinely use analgesia and sedation; 84 (42%) only used local anaesthesia. The majority of respondents used standard Quincke needles (126; 62.7%). The bevel was correctly positioned when puncturing the dura mater by 22 residents (36.1%) and 21 paediatricians (15.1%), a variation that was statistically significant (P=.001). For neonatal lumbar punctures, 63 paediatricians (46%) and 19 paediatric residents used a butterfly needle which did not contain a stylet, and this difference was also statistically significant (P=.035). Of those surveyed, 190 (92.2%) re-inserted the stylet when re-orientating the needle, and 186 (93%) re-oriented this when removing it. The recommendation of bed rest was made by 195 (94.7%) physicians., Conclusions: The majority of paediatricians orient the bevel wrongly when inserting the needle during LP, and still use "butterfly" needles in newborns, despite warnings to the contrary. Paediatric residents and less experienced paediatricians follow the recommendations more frequently., (Copyright © 2011 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)