Diagnosis and management of eosinophilic esophagitis in children: An update from the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN).

INTRODUCTION: Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus characterized by symptoms of esophageal dysfunction and histologically by predominantly eosinophilic infiltration of the squamous epithelium. European Society for Pediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) published a guideline in 2014; however, the rapid evolution of knowledge about pathophysiology, diagnostic criteria, and therapeutic options have made an update necessary. METHODS: A consensus group of pediatric gastroenterologists from the ESPGHAN Working Group on Eosinophilic Gastrointestinal Diseases (ESPGHAN EGID WG) reviewed the recent literature and proposed statements and recommendations on 28 relevant questions about EoE. A comprehensive electronic literature search was performed in MEDLINE, EMBASE, and Cochrane databases from 2014 to 2022. The Grading of Recommendations Assessment, Development and Evaluation system was used to assess the quality of evidence and formulate recommendations. RESULTS: A total of 52 statements based on the available evidence and 44 consensus-based recommendations are available. A revision of the diagnostic protocol, options for initial drug treatment, and the new concept of simplified empiric elimination diets are now available. Biologics are becoming a part of the potential armamentarium for refractory EoE, and systemic steroids may be considered as the initial treatment for esophageal strictures before esophageal dilation. The importance and assessment of quality of life and a planned transition to adult medical care are new areas addressed in this guideline. CONCLUSION: Research in recent years has led to a better understanding of childhood EoE. This guideline incorporates the new findings and provides a practical guide for clinicians treating children diagnosed with EoE.

Helicobacter pylori infection found during upper endoscopy performed for the diagnosis of celiac, inflammatory bowel diseases, and eosinophilic esophagitis: A multicenter pediatric European study.

BACKGROUND: Helicobacter pylori may be found during upper gastrointestinal endoscopy (UGE) performed to diagnose celiac disease (CeD), inflammatory bowel disease (IBD), and eosinophilic esophagitis (EoE). We aimed to describe the frequency of H. pylori in children undergoing UGE for CeD, IBD, and EoE and the number of children receiving eradication treatment. MATERIALS AND METHODS: A retrospective multicenter study from 14 countries included pediatric patients diagnosed with CeD, IBD, and EoE between January 2019 and December 2021. DATA COLLECTED: age, gender, hematologic parameters, endoscopic, histologic, and H. pylori culture results, and information on eradication treatment. RESULTS: H. pylori was identified in 349/3890 (9%) children [167 (48%) male, median 12 years (interquartile range 8.1-14.6)]. H. pylori was present in 10% (173/1733) CeD, 8.5% (110/1292) IBD and 7.6% (66/865) EoE patients (p = NS). The prevalence differed significantly between Europe (Eastern 5.2% (28/536), Southern 3.8% (78/2032), Western 5.6% (28/513)) and the Middle East 26.6% (215/809) [odds ratio (OR) 7.96 95% confidence interval (CI) (6.31-10.1) p < 0.0001]. Eradication treatment was prescribed in 131/349 (37.5%) patients, 34.6% CeD, 35.8% IBD, and 56.1% EoE. Predictors for recommending treatment included erosions/ulcers [OR 6.45 95% CI 3.62-11.47, p < 0.0001] and nodular gastritis [OR 2.25 95% CI 1.33-3.81, p 0.003]. Treatment rates were higher in centers with a low H. pylori prevalence (<20%) [OR 3.36 95% CI 1.47-7.66 p 0.004]. CONCLUSIONS: Identifying H. pylori incidentally during UGE performed for the most common gastrointestinal diseases varies significantly among regions but not among diseases. The indications for recommending treatment are not well defined, and less than 40% of children received treatment.

The Variable Response to Teduglutide in Pediatric Short Bowel Syndrome: A Single Country Real-Life Experience.

OBJECTIVES: The glucagon-like peptide-2 analog Teduglutide has been shown to enhance intestinal absorption and decrease parenteral nutrition (PN) requirements in short bowel syndrome (SBS). As data in children is limited, we evaluated nationwide real-life experience and treatment outcome in children with SBS. METHODS: Longitudinal data of children treated with Teduglutide for ≥3 months was collected. Data included demographic and medical background, anthropometrics, laboratory assessments and PN requirements. Treatment response was defined as >20% reduction in PN requirement. RESULTS: The study included 13 patients [54% males, median (interquartile range {IQR}) age of 6 (4.7-7) years]. The most common SBS etiology was necrotizing enterocolitis (38%), and median (IQR) small bowel length was 20 (15-40) cm. Teduglutide treatment ranged between 3 and 51 months [median (IQR) of 18 (12-30) months], with 10 patients (77%) treated >1 year. Response to treatment was observed in 8 patients (62%), with a mean [±standard deviation (SD)] treatment duration of 5.9 (±3.2) months. Among responders, 2 patients were weaned off PN and additional 4 decreased PN needs by >40%. There was a median (IQR) reduction in PN volume/kg of 36% (15%-55%) and in PN energy/kg of 27% (6%-58%). Response was not associated with patients' background, and no correlation was found with bowel length or PN dependency at baseline. CONCLUSIONS: Real-life response to Teduglutide is highly variable among children with SBS. While most patients did reach 20% reduction in PN, less achieved further significant reduction or enteral autonomy. No predictive factors of response to treatment were identified, and large multicenter studies are needed to elucidate predictive factors and long-term outcome.

Systemic steroids have a role in treating esophageal strictures in pediatric eosinophilic esophagitis.

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 ±â€¯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.

Partial enteral nutrition with a Crohn's disease exclusion diet is effective for induction of remission in children and young adults with Crohn's disease.

BACKGROUND: Exclusive enteral nutrition is effective for inducing remission in active pediatric Crohn's disease. Partial enteral nutrition (PEN) with free diet is ineffective for inducing remission, suggesting that the mechanism depends on exclusion of free diet. We developed an alternative diet based on PEN with exclusion of dietary components hypothesized to affect the microbiome or intestinal permeability. METHODS: Children and young adults with active disease defined as a pediatric Crohn's disease activity index >7.5 or Harvey-Bradshaw index ≥4 received a 6-week structured Crohn's disease exclusion diet that allowed access to specific foods and restricted exposure to all other foods, and up to 50% of dietary calories from a polymeric formula. Remission, C-reactive protien, and erythrocyte sedimentation rate were reevaluated at 6 weeks. The primary endpoint was remission at 6 weeks defined as Harvey-Bradshaw index ≤3 for all patients and pediatric Crohn's disease activity index <7.5 in children. RESULTS: We treated 47 patients (mean age, 16.1 ± 5.6 yr; 34 children). Response and remission were obtained in 37 (78.7%) and 33 (70.2%) patients, respectively. Mean pediatric Crohn's disease activity index decreased from 27.7 ± 9.4 to 5.4 ± 8 (P < 0.001), Harvey-Bradshaw index from 6.4 ± 2.7 to 1.8 ± 2.9 (P < 0.001). Remission was obtained in 70% of children and 69% of adults. Normalization of previously elevated CRP occurred in 21 of 30 (70%) patients in remission. Seven patients used the diet without PEN; 6 of 7 obtained remission. CONCLUSIONS: Dietary therapy involving PEN with an exclusion diet seems to lead to high remission rates in early mild-to-moderate luminal Crohn's disease in children and young adults.

Role reversal method for treatment of food refusal associated with infantile feeding disorders.

OBJECTIVES: Infantile feeding disorders (IFDs) are common causes of food refusal and failure to thrive, and are frequently encountered by primary care physicians and specialists. We have published the Wolfson criteria for IFD, which have eased the approach to the diagnosis of IFDs. Along with and complementary to the Wolfson criteria, we have also developed the role reversal treatment method for IFD, which has been briefly described earlier. The aim of this study was to validate the role reversal treatment method on a cohort of infants diagnosed as having IFD and to present a detailed description of this method for the first time. METHODS: Parents of infants and children diagnosed as having IFD were invited to participate in the study; they were handed over a questionnaire comprising 6 categories of questions related to patient and parents behaviors, attitudes, and perceptions, which was completed at initiation and at the end of treatment. Full response was defined as improved normative feeding, cessation of abnormal parental feeding, and improved or normal growth patterns. A partial response was defined as success with two-third categories. RESULTS: We enrolled 38 patients, and 32 patients completed the study. Improved feeding occurred in 78%, full recovery was documented in 53% of infants by 6 months, and partial response was observed in another 25%. All forms of pathological feeding improved significantly (mechanistic, nocturnal, persecutory, forced feeding, and distraction). CONCLUSIONS: The role reversal treatment method is a simple and effective approach to the treatment of food refusal associated with IFD.

Celiac-associated peptic disease at upper endoscopy: how common is it?

OBJECTIVE: Recently, several publications in adults have shown an increased incidence of non-Helicobacter pylori (HP), non-non-steroidal anti-inflammatory drug (NSAID) peptic disease (PD). There are only a few case reports linking celiac disease (CD) to PD. We therefore aimed to review our experience of CD presenting with PD. MATERIAL AND METHODS: We retrospectively reviewed all the endoscopies performed for children and young adults diagnosed with CD between 1 January, 2004 and 31 October, 2008. The diagnosis of CD was based on accepted guidelines. Patients with a doubtful diagnosis of CD were excluded. RESULTS: We had 240 patients with the diagnosis of CD. We had 29 (12.0%) patients [15 males (52%), 14 females (48%)] for whom the diagnosis of PD was ascertained. The age range was 1-50 years (mean 16.9 +/- 12.1 years). Twenty-three of the 29 patients (79%) were HP-negative. Duodenal PD was noted in 22 patients (76%) and 16 (73%) were HP-negative. Gastric PD was noted in eight patients (28%) and 7 (87%) were HP-negative. The PD group was significantly older at diagnosis (p < 0.001) compared to the whole CD group. CONCLUSIONS: PD is not uncommon in the presentation of CD. It is more likely to be found in the second decade of life. CD should be included in the differential diagnosis of patients with non-HP PD and we suggest routine CD serology and small bowel biopsy in patients with unexplained PD.

Diagnostic clues for identification of nonorganic vs organic causes of food refusal and poor feeding.

OBJECTIVES: Food refusal, poor feeding, and somatic symptoms such as vomiting, gagging, irritability and failure to thrive (FTT) are commonly found in both infantile feeding disorders (IFD) and common treatable medical conditions. Present diagnostic classifications for diagnosing IFD are complex and difficult to apply in daily practice, leading to underdiagnosis and delay in diagnosis of IFD. We attempted to identify parental and infantile behaviour patterns or symptoms that could help distinguish between organic or behavioural causes for these symptoms. METHODS: We screened 226 children with poor feeding. After exclusion criteria, we divided the remaining 151 into 2 groups. The nonorganic group (n=83) included patients with onset of symptoms before age 2, persistent food aversion longer than 1 month, and a response to behavioural intervention. The second group consisted of children (n=68) presenting with similar characteristics, who responded to medical or nutritional therapy in which a final diagnosis of gastro-esophageal reflux disease, milk allergy, or idiopathic or nutritional FTT was made. RESULTS: Poor intake, poor weight gain, or vomiting did not discriminate between organic and nonorganic causes. Factors indicating the presence of a behavioural cause included food refusal, food fixation, abnormal parental feeding practices, onset after a specific trigger, and presence of anticipatory gagging (P<0.0001 for all). CONCLUSIONS: Integration of a few structured questions regarding infant behaviour, parental feeding practices, infant symptoms, and triggers for the onset of symptoms may help clinicians distinguish between organic and nonorganic causes for food refusal or low intake FTT.

Post-infectious gastroparesis: clinical and electerogastrographic aspects.

AIMS: Post-infectious gastroparesis (PIGP) is a subgroup of idiopathic gastroparesis. The aim of this study was to identify post-viral gastroparesis and to characterize clinical and electrogastrographic aspects of the disease and their usefulness as a diagnostic tool. METHODS: Patients diagnosed with gastroparesis were defined as PIGP if they had a clear history of an acute viral illness prior to the development of their symptoms. All patients underwent evaluation of gastric emptying and electrogastrography (EGG). RESULTS: Seven patients met the criteria for diagnosis of PIGP. Patients' age ranged from 3 months to 47 years. A specific virus was identified in two patients (one cytomegalovirus [CMV] and one Epstein-Barr virus [EBV]). EGG was pathological in six out of seven patients. In four out of seven patients, symptoms resolved spontaneously within 4 weeks to 12 months, three patients had improved but were still symptomatic at the time of the writing of this work. CONCLUSION: We conclude that post-infectious gastroparesis is an uncommon and often over looked condition. It is self-limiting in most cases. EGG is pathological in most patients.

Gastrointestinal motility and sensory abnormalities may contribute to food refusal in medically fragile toddlers.

BACKGROUND: In chronically ill children who refuse to eat, surgery to correct anatomic problems and behavioral treatments to overcome oral aversion often succeed. A few patients fail with standard treatments. The aims of the study were to: 1) investigate motility and gastric sensory abnormalities and 2) describe treatment that was individualized based on pathophysiology in children who failed surgery and behavioral treatments. METHODS: We studied 14 patients (age 1.5-6; mean 2.5; M/F: 7/7). All had a lifelong history of food aversion and retching or vomiting persisting after feeding therapy and fundoplication. All were fed through gastrostomy or gastro-jejunostomy tubes. We studied esophageal and antroduodenal manometry, and gastric volume threshold for retching. We identified when gastric antral contractions were associated with retching and pain. A multidisciplinary treatment program included a variable combination of continuous post-pyloric feedings, drugs to decrease visceral pain, drugs for motility disorders, and behavioral, cognitive, and family therapy. We interviewed parents 2-6 months following testing to evaluate symptoms, mode of feeding and emotional health. RESULTS: We found a motility disorder alone in 2, decreased threshold for retching alone in 5 and both motility and sensory abnormalities in 7. After treatment, 6 of 14 (43%) began eating orally and 80% had improved emotional health. Retching decreased from 15 episodes per day to an average of 1.4 per day (p <0.01). CONCLUSIONS: Upper gastrointestinal motor and/or sensory disorders contributed to reduced quality of life for a majority of children and families with persistent feeding problems. A multidisciplinary approach improved symptoms and problems in these children

Water load test in children.

OBJECTIVE: The purpose of this study was to estimate values for the water load test in healthy elementary school children. METHODS: We measured the volume of water consumed by 176 children (71 boys and 105 girls) using the water load test at school. Children completed a questionnaire, which included self-efficacy and abdominal pain history. Then subjects drank water for 3 minutes or until full. RESULTS: Children drank 630 +/- 260 ml. Water consumption correlated with age, weight, and height. Children who believed they could drink a lot, drank more than those who thought they could not, < 0.01. CONCLUSION: The water load test is a simple and inexpensive method to estimate onset of satiety and may be useful in future studies now that there are values for healthy children.