Liver disease in germline mutations of telomere-related genes: Prevalence, clinical, radiological, pathological features, outcome, and risk factors.

BACKGROUND AND AIM: Germline mutations of telomere-related genes (TRG) induce multiorgan dysfunction, and liver-specific manifestations have not been clearly outlined. We aimed to describe TRG mutations-associated liver diseases. APPROACH AND RESULTS: Retrospective multicenter analysis of liver disease (transaminases > 30 IU/L and/or abnormal liver imaging) in patients with TRG mutations. Main measurements were characteristics, outcomes, and risk factors of liver disease in a TRG mutations cohort. The prevalence of liver disease was compared to a community-based control group (n = 1190) stratified for age and matched 1:3 for known risk factors of liver disease. Among 132 patients with TRG mutations, 95 (72%) had liver disease, with associated lung, blood, skin, rheumatological, and ophthalmological TRG diseases in 82%, 77%, 55%, 39%, and 30% of cases, respectively. Liver biopsy was performed in 52/95 patients, identifying porto-sinusoidal vascular disease in 48% and advanced fibrosis/cirrhosis in 15%. After a follow-up of 21 months (12-54), ascites, hepato-pulmonary syndrome, variceal bleeding, and HCC occurred in 14%, 13%, 13%, and 2% of cases, respectively. Five-year liver transplantation-free survival was 69%. A FIB-4 score ≥ 3·25 and ≥1 risk factor for cirrhosis were associated with poor liver transplantation-free survival. Liver disease was more frequent in patients with TRG mutations than in the paired control group [80/396, (20%)], OR 12.9 (CI 95%: 7.8-21.3, p < 0.001). CONCLUSIONS: TRG mutations significantly increase the risk of developing liver disease. Although symptoms may be mild, they may be associated with severe disease. Porto-sinusoidal vascular disease and cirrhosis were the most frequent lesions, suggesting that the mechanism of action is multifactorial.

High hydrostatic pressure is similar to Holder pasteurization in preserving donor milk antimicrobial activity.

BACKGROUND: The microbiological safety of donor milk (DM) is commonly ensured by Holder pasteurization (HoP, 62.5 °C for 30 min) in human milk banks despite its detrimental effects on bioactive factors. We compared the antimicrobial properties of DM after Holder pasteurization treatment or High Hydrostatic Pressure processing (HHP, 350 MPa at 38 °C), a non-thermal substitute for DM sterilization. METHODS: We assessed lactoferrin and lysozyme concentrations in raw, HHP- and HoP-treated pools of DM (n = 8). The impact of both treatments was evaluated on the growth of Escherichia coli and Group B Streptococcus in comparison with control media (n = 4). We also addressed the effect of storage of HHP treated DM over a 6-month period (n = 15). RESULTS: HHP milk demonstrated similar concentrations of lactoferrin compared with raw milk, while it was significantly decreased by HoP. Lysozyme concentrations remained stable regardless of the condition. Although a bacteriostatic effect was observed against Escherichia coli at early timepoints, a sharp bactericidal effect was observed against Group B Streptococcus. Unlike HoP, these results were significant for HHP compared to controls. Stored DM was well and safely preserved by HHP. CONCLUSION: Our study demonstrates that this alternative sterilization method shows promise for use with DM in human milk banks. IMPACT: Antimicrobial activity of donor milk after High Hydrostatic Pressure treatment has not been clearly evaluated. Donor milk lactoferrin is better preserved by High Hydrostatic Pressure than conventional Holder pasteurization, while lysozyme concentration is not affected by either treatment. As with Holder pasteurization, High Hydrostatic Pressure preserves donor milk bacteriostatic activity against E. coli in addition to bactericidal activity against Group B Streptococcus. Donor milk treated by High Hydrostatic Pressure can be stored safely for 6 months.

Proton pump inhibitors in esophageal atresia: A systematic review and meta-analysis.

Gastroesophageal reflux disease (GERD) is frequent and prolonged in esophageal atresia (EA) pediatric patients requiring routine use of proton pump inhibitors (PPIs). However, there are still controversies on the prophylactic use of PPIs and the efficacy of PPIs on GERD and EA complications in this special condition. The aim of the study is to assess the prophylactic use of PPIs in pediatric patients with EA and its complications. We, therefore, performed a systematic review including all reports on the subject from 1980 to 2022. We conducted meta-analysis of the pooled proportion of PPI-and no PPI groups using random effect model, meta-regression, and estimate heterogeneity by heterogeneity index I2 . Thirty-eight reports on the topic met the criteria selection, representing a cumulative 6044 patients with EA. Prophylactic PPI prescription during the first year of life does not appear to prevent GERD persistence at follow-up and is not associated with a significantly reduced rate of antireflux surgical procedures (ARP). PPIs improve peptic esophagitis and induce remission of eosinophilic esophagitis at a rate of 50%. Their effect on other GERD outcomes is uncertain. Evidence suggests that PPIs do not prevent anastomotic stricture, Barrett's esophagus, or respiratory complications. PPI use in EA can improve peptic and eosinophilic esophagitis but is ineffective on the other EA complications. Side effects of PPIs in EA are almost unknown.

Donor human milk treated by high-pressure processing improves the body growth of growth-restricted mice pups.

INTRODUCTION: Pasteurized human donor milk (DM) is frequently used for feeding preterm newborns and extrauterine growth-restricted (EUGR) infants. Most human milk banks performed a pasteurization of DM using the standard method of Holder pasteurization (HoP) which consists of heating milk at 62.5°C for 30 min. High hydrostatic pressure (HHP) processing was proposed to be an innovative nonthermal method to pasteurize DM. However, the effect of different modes of DM pasteurization on body growth, intestinal maturation, and microbiota has never been investigated in vivo during the lactation. OBJECTIVES: We aimed to study these effects in postnatally growth-restricted (PNGR) mice pups daily supplemented with HoP-DM or HHP-DM. METHODS: PNGR was induced by increasing the number of pups per litter (15 pups/mother) at postnatal Day 4 (PND4). From PND8 to PND20, mice pups were supplemented with HoP-DM or HHP-DM. At PND21, the intestinal permeability was measured in vivo, the intestinal mucosal histology, gut microbiota, and short-chain fatty acids (SCFAs) level were analyzed. RESULTS: HHP-DM pups displayed a significantly higher body weight gain than HoP-DM pups during lactation. At PND21, these two types of human milk supplementations did not differentially alter intestinal morphology and permeability, the gene-expression level of several mucosal intestinal markers, gut microbiota, and the caecal SCFAs level. CONCLUSION: Our data suggest that HHP could be an attractive alternative to HoP and that HHP-DM may ensure a better body growth of preterm and/or EUGR infants.

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.

Over-the-scope clip for closure of persistent gastrocutaneous fistula after gastrostomy tube removal: a multicenter pediatric experience.

BACKGROUND: Percutaneous endoscopic gastrostomy is commonly used for enteral nutritional access, but gastrocutaneous fistulae (GCF) may persist after tube removal, posing clinical challenges. The use of endoscopic closure devices, including over-the-scope clips (OTSC), has shown promise in managing non-healing fistulae, although data in the pediatric population are limited. METHODS: A retrospective multicenter study analyzed pediatric patients who underwent GCF closure following gastrostomy tube removal. Data from seven centers across multiple countries were collected, including patient demographics, procedural details, complications, and outcomes. Closure techniques were compared between OTSC and surgical closure. RESULTS: Of 67 pediatric patients included, 21 underwent OTSC closure and 46 had surgical closure. Surgical closure demonstrated a higher success rate (100%) compared to OTSC closure (61.9%, P < 0.001). While procedural duration was shorter for OTSC closure (25 vs. 40 min, P = 0.002), complications, and scar quality were comparable between techniques. A subsequent sub-analysis did not reveal differences based on center experience. CONCLUSION: OTSC closure is feasible and safe in pediatric patients, but surgical closure remains superior in achieving sustained GCF closure, although OTSC offers benefits, such as shorter procedural duration, potentially reducing the duration of general anesthesia exposure. Non-operative approaches, including OTSC, may be a valuable alternative to surgical closure.

Interplay of the Mediterranean diet and genetic hypertension risk on blood pressure in European adolescents: Findings from the HELENA study.

Early-life onset of high blood pressure is associated with the development of cardiovascular diseases in adulthood. In adolescents, limited evidence exists regarding the association between adherence to the Mediterranean Diet (MedDiet) and normal blood pressure (BP) levels, as well as its potential to modulate genetic predisposition to HTN. This study investigated the interaction between a MedDiet score and a recently developed HTN-genetic risk score (HTN-GRS) on blood pressure levels in a European adolescent cohort. The MedDiet score was derived from two non-consecutive 24-h dietary recalls and ranged from 0 (indicating low adherence) to 9 (indicating high adherence). Multiple linear regression models, adjusted for covariates, were employed to examine the relationship between the MedDiet score and BP z-scores and to assess the interaction effects between the MedDiet score and HTN-GRS on BP z-scores. MedDiet score showed a negative association with z-systolic BP (SBP) (ß = -0.40, p < 0.001) and z-diastolic BP (DBP) (ß = -0.29, p = 0.001). Additionally, a significant interaction effect was identified between the MedDiet score and HTN-GRS on z-SBP (ß = 0.02, p < 0.001) and z-DBP (ß = 0.02, p < 0.001). The modulatory effect of the MedDiet was more pronounced in females than in males, and HTN-GRS exhibited a stronger influence on DBP than on SBP.   Conclusion: The study suggests that higher adherence to the MedDiet is associated with reduced BP levels in adolescents and provides evidence of a genetic-diet interaction influencing BP in adolescents. What is Known: • Adherence to the Mediterranean diet may reduce BP levels. What is New: • It is the first study to assess the connection between adherence to a Mediterranean diet, a hypertension genetic risk score, and how they interact in influencing blood pressure. • It is conducted within a multicenter cohort of European adolescents.

The body mass index increases the genetic risk scores' ability to predict risk of hepatic damage in European adolescents: The HELENA study.

BACKGROUND: Hepatic disorders are often complex and multifactorial, modulated by genetic and environmental determinants. During the last years, the hepatic disease has been progressively established from early stages in life. The use of genetic risk scores (GRS) to predict the genetic susceptibility to a particular phenotype among youth has gained interest in recent years. Moreover, the alanine aminotransferase (ALT) blood biomarker is often considered as hepatic screening tool, in combination with imaging techniques. The aim of the present study was to develop an ALT-specific GRS to help in the evaluation of hepatic damage risk in European adolescents. METHODS: A total of 972 adolescents (51.3% females), aged 12.5-17.5 years, from the Healthy Lifestyle in Europe by Nutrition in Adolescence study were included in the analyses. The sample incorporated adolescents in all body mass index (BMI) categories and was divided considering healthy/unhealthy ALT levels, using sex-specific cut-off points. From 1212 a priori ALT-related single nucleotide polymorphisms (SNPs) extracted from candidate gene selection, a first screening of 234 SNPs univariately associated was established, selecting seven significant SNPs (p < .05) in the multivariate model. An unweighted GRS (uGRS) was developed by summing the number of reference alleles, and a weighted GRS (wGRS), by multiplying each allele to its estimated coefficient. RESULTS: The uGRS and wGRS were significantly associated with ALT (p < .001). The area under curve was obtained integrating BMI as clinical factor, improving the predictive ability for uGRS (.7039) and wGRS (.7035), using 10-fold internal cross-validation. CONCLUSIONS: Considering BMI status, both GRSs could contribute as complementary tools to help in the early diagnosis of hepatic damage risk in European adolescents.

Clinical and physical characteristics of thinness in adolescents: the HELENA study.

PURPOSE: Thinness in adolescence has not been studied as extensively as overweight or obesity. The aim of this study was to assess the prevalence, characteristics, and health impacts of thinness in a European adolescent population. METHODS: This study included 2711 adolescents (1479 girls, 1232 boys). Blood pressure, physical fitness, sedentary behaviors, physical activity (PA), and dietary intake were assessed. A medical questionnaire was used to report any associated diseases. A blood sample was collected in a subgroup of the population. Thinness and normal weight were identified using the IOTF scale. Thin adolescents were compared with adolescents of normal weight. RESULTS: Two hundred and fourteen adolescents (7.9%) were classified as being thin; the prevalence rates were 8.6% in girls and 7.1% in boys. Systolic blood pressure was significantly lower in adolescents with thinness. The age at the first menstrual cycle was significantly later in thin female adolescents than in those with normal weight. Upper-body muscular strength measured in performance tests and time spent in light PA were significantly lower in thin adolescents. The Diet Quality Index was not significantly lower in thin adolescents, but the percentage of adolescents who skipped breakfast was higher in adolescents with a normal weight (27.7% vs 17.1%). Serum creatinine level and HOMA-insulin resistance were lower and vitamin B12 level was higher in thin adolescents. CONCLUSIONS: Thinness affects a notable proportion of European adolescents with no physical adverse health consequences.

Relative validity of the Planetary Health Diet Index by comparison with usual nutrient intakes, plasma food consumption biomarkers, and adherence to the Mediterranean diet among European adolescents: the HELENA study.

PURPOSE: The EAT-Lancet Commission proposed an evidence-based global reference diet to improve human health within planetary boundaries. Recently, the Planetary Health Diet Index (PHDI) was developed based on the EAT-Lancet recommendations and validated among Brazilian adults. However, the relative validity of the PHDI in adolescents has yet to be assessed. Thus, we aimed to evaluate the relative validity of the PHDI in European adolescents. METHODS: We used cross-sectional data from 1804 adolescents (12.5-17.5 years) enrolled in the Healthy Lifestyle in Europe by Nutrition in Adolescence (HELENA) study. The PHDI (0-150 points) was calculated based on dietary intake data from two non-consecutive 24-h dietary recalls. Associations between the PHDI and usual nutrient intakes, plasma food consumption biomarkers, and adherence to the Mediterranean diet were evaluated using multivariable-adjusted mixed-effects linear regression models. RESULTS: Higher PHDI score was associated with greater intakes of nutrients predominantly from plant-source foods, such as vegetable protein, vitamin E, and folate and with lower intake of nutrients predominately from animal-source foods, such as total and saturated fat, cholesterol, and animal protein. Furthermore, a higher PHDI score was also positively associated with plasma ß-carotene, vitamin C, vitamin D, folate, and ferritin concentrations, while negatively associated with trans-fatty acids concentration. Moreover, higher PHDI was related to a greater adherence to the Mediterranean dietary pattern. CONCLUSIONS: The PHDI showed good relative validity among adolescents in the HELENA study. Hence, future research should assess adherence to the PHDI and long-term health outcomes.

Validation of the IMPACT-III Questionnaire in French Children With Inflammatory Bowel Disease.

OBJECTIVES: This study assessed the reliability and validity of the IMPACT-III questionnaire, a health-related quality of life (HRQoL) instrument, in French children with inflammatory bowel disease (IBD). METHODS: Eighty-four children and adolescents (45 boys, aged 14.3 ± 2.7 years) were included in a validation study of the IMPACT-III questionnaire. Patients completed 2 questionnaires for measuring HRQoL: the IMPACT-III and the Pediatric Quality of Life Inventory 4.0 Generic Core Scale (PedsQL). Internal consistency was assessed using Cronbach α. Factor analysis was performed on data from the IMPACT-III to help construct domains. Concurrent validity was assessed by calculating Spearman correlation coefficients. RESULTS: Cronbach α for the PedsQL total score was good (0.89). The most robust factor solution was a 3-domain structure: (a) Concerns, (b) Body Image and Physical Condition, and (c) Symptoms and Socializing. All domains had good reliability (0.674-0.863). Only 2 items had to be removed. Discriminant validity was demonstrated by significant differences ( P < 0.001) in median IMPACT-III scores between inactive and active disease for the total score (83.3 vs 72.0), and for Concerns ( P < 0.002) and Symptoms and Socializing ( P < 0.001). CONCLUSIONS: These results suggest that IMPACT-III appears to be a useful instrument for measuring HRQoL in French children with IBD.

Refeeding Syndrome in Pediatric Age, An Unknown Disease: A Narrative Review.

Refeeding syndrome (RS) is characterized by electrolyte imbalances that can occur in malnourished and abruptly refed patients. Typical features of RS are hypophosphatemia, hypokalemia, hypomagnesemia, and thiamine deficiency. It is a potentially life-threatening condition that can affect both adults and children, although there is scarce evidence in the pediatric literature. The sudden increase in food intake causes a shift in the body's metabolism and electrolyte balance, leading to symptoms such as weakness, seizures, and even heart failure. A proper management with progressive increase in nutrients is essential to prevent the onset of this condition and ensure the best possible outcomes. Moreover, an estimated incidence of up to 7.4% has been observed in pediatric intensive care unit patients receiving nutritional support, alone or as an adjunct. To prevent RS, it is important to carefully monitor feeding resumption, particularly in severely malnourished individuals. A proper strategy should start with small amounts of low-calorie fluids and gradually increasing the calorie content and amount of food over several days. Close monitoring of electrolyte levels is critical and prophylactic use of dietary supplements such as thiamine may be required to correct any imbalances that may occur. In this narrative review, we aim to provide a comprehensive understanding of RS in pediatric clinical practice and provide a possible management algorithm.

Endoscopic management of esophageal mucosal bridges in children with esophageal atresia.

BACKGROUND AND STUDY AIMS: Esophageal mucosal bridge (EMB) may be diagnosed at the anastomotic site in children operated on for esophageal atresia (EA) but so far only a few cases (n = 4) have been reported. This study aimed to characterize EMB in children with EA, risk factors, and treatment. PATIENTS AND METHODS: This retrospective multicenter study recorded patient's characteristics, EMB diagnosis circumstances, endoscopic management, follow-up, and EMB recurrence in children with EA aged less than 18 years, compared with paired EA patients without EMB. RESULTS: Thirty patients were included (60% male, 90% EA/tracheoesophageal fistula, 43% associated malformations). Compared to 44 paired controls, EMB was associated with a history of nasogastric tube feeding (31% vs. 9.1%, p = 0.02) and severe gastroesophageal reflux disease (history of fundoplication: 41.4% vs. 13.6%, p < 0.01). 77% had symptoms (food impaction and/or dysphagia). Endoscopic management was performed in 53% of patients (83% electrocoagulation) with no technical difficulties or complications. 80% of the symptomatic patients with EMB improved after endoscopic treatment, independently of anastomotic stricture dilatation or not. CONCLUSION: EMB endoscopic management by electrocoagulation is safe and often leads to symptom improvement.

Complications of one-step button percutaneous endoscopic gastrostomy in children.

To assess the complications of one-step button percutaneous endoscopic gastrostomy (B-PEG) and determine risk factors for developing stomal infections or gastropexy complications. A retrospective study of 679 children who underwent a B-PEG procedure in a single tertiary care center over a 10-year period to December 2020 was conducted. Patient characteristics, early complications (occurring ≤ 7 days after the procedure), late complications (> 7 days after the procedure), and outcomes were collected from medical records. A list of potential risk factors, including age at procedure, prematurity, underlying neurological disease, and undernutrition, was determined a priori. At least 1 year of follow-up was available for 513 patients. Median follow-up duration was 2.8 years (interquartile range 1.0-4.9 years). Major complications were rare (< 2%), and no death was related to B-PEG. Early complications affected 15.9% of the study population, and 78.0% of children presented late complications. Development of granulation tissue was the most common complication followed in frequency by tube dislodgment and T-fastener complications. Only 24 patients (3.5%) presented stomal infections. Young age at the time of PEG placement (odds ratio (OR) 2.34 [1.03-5.30], p = .042) was a risk factor for developing peristomal infection. T-fastener migration occurred in 17.3% of children, and we found underlying neurological disease was a protective factor (OR 0.59 [0.37-0.92], p = .019).  Conclusion: B-PEG is a safe method and associated with a low rate of local infection. However, T-fasteners are associated with significant morbidity and require particular attention in young and premature infants. What is Known: • Percutaneous endoscopic gastrostomy (PEG) is the preferred method to provide long-term enteral nutrition in children to prevent malnutrition. The Pull-PEG method is still the most commonly used with complications , such as stomal infection. Since its description, only a few studies have reported postoperative complications of one-step button PEG (B-PEG). What is New: • T-fastener complications were not rare, and underlying neurologic disease was a protective factor. A very low rate of stomal infection was described, and young age at the time of PEG placement was a risk factor. The B-PEG is a safe method with fewer major complications than P-PEG in children.

Changes in physical activity patterns from adolescence to young adulthood: the BELINDA study.

Physical activity (PA) is recognized as a marker of health. The aim was to investigate PA differences from adolescence to young adulthood. European adolescents included in the HELENA study were invited to participate in a follow-up study, 10 years later. The present study included 141 adults (25.0 ± 1.4 years) for whom valid accelerometer data were available in adolescence and adulthood. Changes in PA by sex, weight and maternal education level were explored with interactions. Time spent in sedentary activity, light PA (LPA) and moderate PA (MPA) increased by 39.1, 59.6 and 6.6 min/day, respectively, whereas the time spent in vigorous PA (VPA) decreased by 11.3 min/day compared with adolescent VPA (p < 0.05). Increases in MPA were greater on weekends compared with weekdays, but we found a greater decrease in VPA on weekdays compared with weekends. Moderate-to-vigorous PA (MVPA) decreased significantly on weekdays (-9.6 min/day; 95%CI, -15.9 to -3.4), while it increased on weekends (8.4 min/day; 95%CI, 1.9 to 14.8). Significant heterogeneity was found across sexes for VPA and MVPA, with a stronger decrease in VPA in males compared with females and a significant decrease in MVPA (-12.5 min/day; 95%CI, -20.4 to -4.5) in males but not in females (1.9 min/day; 95%CI, -5.5 to 9.2). No significant heterogeneity was found to be linked to maternal education level or weight, irrespective of PA level.  Conclusion: Our data suggest that the transition from adolescence to young adulthood is a critical period for lifestyle PA habits. A decline in VPA and an increasingly sedentary time were observed. The observed changes are worrying and may increase the risk of developing adverse health consequences later in life. What is Known: • The transition from adolescence to adulthood is marked by many life changes affecting lifestyle habits. Most studies tracking physical activity from adolescence to adulthood were done using PA questionnaires, which is a subjective method. What is New: • Our study bring first data on objective changes in PA patterns between adolescence and young adulthood, taking account of BMI, sex and maternal educational level. Our results suggest that the transition from adolescence to young adulthood is a critical period for lifestyle PA habits, especially for time spent on sedentary activities.

Endoscopic Gastrojejunostomy in Infants and Children.

OBJECTIVE: To evaluate the feasibility of endoscopic gastrojejunal tube (GJT) placement in infants and children. STUDY DESIGN: All children undergoing endoscopic GJT placement between January 2010 and December 2019 were included in this single-center retrospective study. Difficulties with and failure of GJT placement, complication rates, and device longevity, efficacy, and duration were assessed. RESULTS: A total of 107 children, median age 10 months (IQR, 5.0-23.0 months) and median weight 6.6 kg (IQR, 5.3-9.5 kg), underwent endoscopic GJT placement using the gastric stoma to introduce the endoscope (one step: n = 36 of 107; 33.6%). Endoscopic placement was successful in 99%. Eight periprocedure complications occurred, including 1 pneumoperitoneum requiring exsufflation, 2 acute pulmonary hypertension episodes leading to death in 1 case, and 5 episodes of bronchospasm. Minor complications were frequent and mostly mechanical (79%), whereas major complications were rare (5.6%): intussusception (n = 4), intestinal perforation (n = 1), and pneumoperitoneum (n = 1). Ten patients died. Of the 97 patients who lived, 85 (87%) were weaned from jejunal feeding at a median of 179 days (IQR, 69-295 days) after initiation. Among them, 30 (35.2%) required fundoplication. Weight for age z-score was significantly higher at weaning. CONCLUSIONS: GJT placement is feasible in children, even low-weight infants. Complications are frequent but are mostly minor.

Comparison of Endoscopic Dilatation and Heller's Myotomy for Treating Esophageal Achalasia in Children: A Multicenter Study.

OBJECTIVE: To compare the efficacy of, and complications from, the 2 main treatments for achalasia: endoscopic dilatation and surgical cardiomyotomy (Heller's myotomy). STUDY DESIGN: We retrospectively collected data on children treated for achalasia over an 11-year period from 8 tertiary pediatric centers. A line of treatment was defined as performing either Heller's myotomy or 1-3 sessions of endoscopy dilatation over 3 months. Treatment success was a priori defined as clinical improvement and no need for new treatment. RESULTS: Ninety-seven children (median age, 12 years; 57% boys) were included. The median time to diagnosis was 10.5 months, and the median follow-up period was 27 months. Thirty-seven children were treated by Heller's myotomy and 60 by endoscopy dilatation as the first-line treatment. After adjustment for potentially confounding factors, Heller's myotomy was significantly more successful than endoscopy dilatation (hazard ratio, 3.93 [1.74; 8.88]; P = .001), with a median survival without failure of 49 and 7 months, respectively, and with no significant difference in the occurrence of complications (35.2% for Heller's myotomy, 29.7% for endoscopy dilatation, P = .56). Hydrostatic dilatation was as successful as pneumatic dilatation (hazard ratio, 1.35 [0.56; 3.23]; P = .50). CONCLUSIONS: Heller's myotomy is more successful than endoscopy dilatation, with no significant difference in the occurrence of serious complications. This raises the potential role of peroral endoscopic myotomy as an alternative treatment to Heller's myotomy.

Prevalence of acid gastroesophageal reflux disease in infants with esophageal atresia/tracheoesophageal fistula.

BACKGROUND: Given the high prevalence and complication risks of acid gastroesophageal reflux (GERD) in the first months of life in infants with esophageal atresia, the ESPGHAN/NASPGHAN consensus statement recommends systematic treatment with proton pump inhibitors (PPIs) until the age of 1 year and checking for acid GERD thereafter. However, these recommendations have not been evaluated. METHODS: This prospective study was conducted from 2007 to 2016. We evaluated the prevalence of acid GERD in 100 consecutive infants presenting with esophageal atresia/tracheoesophageal fistula after the age of 18 months when PPI treatment was stopped. The diagnosis of acid GERD was based on positive pH-metry and/or evidence of complications (e.g., peptic esophagitis, need for jejunal nutrition, or antireflux surgery). Those with acid GERD at a median age of 18 months received a control examination every year or adapted to their clinical situation. RESULTS: The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up (median age 65 months).There is no risk factor for acid GERD identified. CONCLUSIONS: This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped. IMPACT: Acid gastroesophageal reflux disease (GERD) is a frequent complication of esophageal atresia in infants. The ESPGHAN/NASPGHAN consensus, which is based on expert opinion, recommends systematic treatment of children with PPI until the age of 1 year. The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up. This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped.

Management of Gastroesophageal Reflux Disease in Esophageal Atresia Patients: A Cross-Sectional Survey amongst International Clinicians.

OBJECTIVES: After surgical repair, up to 70% of esophageal atresia (EA) patients suffer from gastroesophageal reflux disease (GERD). The ESPGHAN/NASPGHAN guidelines on management of gastrointestinal complications in EA patients were published in 2016. Yet, the implementation of recommendations on GERD management remains poor.We aimed to assess GERD management in EA patients in more detail, to identify management inconsistencies, gaps in current knowledge, and future directions for research. METHODS: A digital questionnaire on GERD management in EA patients was sent to all members of the ESPGHAN EA working group and members of the International network of esophageal atresia (INoEA). RESULTS: Forty responses were received. Thirty-five (87.5%) clinicians routinely prescribed acid suppressive therapy for 1-24 (median 12) months. A fundoplication was considered by 90.0% of clinicians in case of refractory GERD with persistent symptoms despite maximal acid suppressive therapy and in 92.5% of clinicians in case of GERD with presence of esophagitis on EGD. Half of clinicians referred patients with recurrent strictures or dependence on transpyloric feeds. Up to 25.0% of clinicians also referred all long-gap EA patients for fundoplication, those with long-term need of acid suppressants, recurrent chest infections and feedings difficulties. CONCLUSIONS: Respondents' opinions on the optimal duration for routine acid suppressive therapy and indications for fundoplication in EA patients varied widely. To improve evidence-based care for EA patients, future prospective multicenter outcome studies should compare different diagnostic and treatment regimes for GERD in patients with EA. Complications of therapy should be one of the main outcome measures in such trials.

Changes in (poly)phenols intake and metabolic syndrome risk over ten years from adolescence to adulthood.

BACKGROUND AND AIMS: (Poly)phenols might contribute to prevent cardiovascular disease, but limited prospective studies exist among adolescents. This study aimed to evaluate within-subject longitudinal changes in (poly)phenols intakes and food group contributors while also exploring the association with metabolic syndrome risk (MetS) during 10 years of follow up in European adolescents becoming young adults. METHODS AND RESULTS: In 164 participants (58% girls, 13-18 y at baseline) from Ghent, Zaragoza and Lille, longitudinal data (2006-2016) on (poly)phenol intake was retrieved via 2 or 3 24 h recalls. Linear and logistic longitudinal regression tested the association of (poly)phenols intake (total and classes) with Mets risk or its components (waist-height-ratio, HDL cholesterol, LDL cholesterol, triglycerides, blood pressure and insulin resistance index), adjusted for age, sex, country and other nutrient intakes. The total (poly)phenols intake was 421 ± 107 mg/day (192 mg/1000 kcal/day) at baseline, while 610 ± 101 mg/day (311 mg/1000 kcal/day) at follow-up. The three major food sources for (poly)phenols were 'chocolate', 'fruit and vegetable juices', 'cakes and biscuits' during adolescence and 'coffee', 'tea' and 'chocolate' during adulthood. Phenolic acid intake was associated with less LDL increase over time, while stilbene intake with a steeper increase in triglycerides over time. CONCLUSIONS: Differences in major (poly)phenols contributors over time were partially explained by age-specific dietary changes like increased coffee and tea during adulthood. Some significant (poly)phenols-MetS associations might argue for nutrition-based disease prevention during adolescence, especially since adolescents had low (poly)phenols intake.