Journal of Pediatric Gastroenterology and Nutrition最新文献

{"title":"Incidence, diagnostics, therapeutic management and outcomes of paediatric intestinal pseudo-obstruction in the Netherlands: A 20-year retrospective cohort study.","authors":"Aysenur Demirok, Sjoerd C J Nagelkerke, Malou Veldt, Ramon Gorter, Justin R de Jong, Gerard M Damen, Barbara A E de Koning, Caroline Meijer, Patrick F van Rheenen, Victorien M Wolters, Marc A Benninga, Merit M Tabbers","doi":"10.1002/jpn3.12400","DOIUrl":"https://doi.org/10.1002/jpn3.12400","url":null,"abstract":"<p><strong>Objectives: </strong>To describe incidence, clinical course, diagnostic and therapeutic management and long-term follow-up of paediatric intestinal pseudo-obstruction (PIPO) in the Netherlands between 2000 and 2020.</p><p><strong>Methods: </strong>Multicenter, national, retrospective, observational study including patients aged <18 years diagnosed with PIPO and treated between 2000 and 2020 in Dutch academic medical centres. Outcomes included demographics, incidence, symptoms, diagnostic- and treatment methods used during follow-up, number of hospital admissions and mortality.</p><p><strong>Results: </strong>Between 2000 and 2020, 43 children (median age 120 months, range 13 - 301, 54% female) were diagnosed with PIPO in the Netherlands. Mean incidence was 0.008/100,000/years (range 0/100 000-0.029/100 000). Twenty-six patients developed PIPO in the neonatal period. Initial symptoms were vomiting (n = 21/35, 60%) and abdominal distension (n = 14/35, 40%). Diagnostic strategies included imaging, manometry, histopathology, metabolic- and genetic screening, endoscopy and exploratory surgery. Treatment was divided in nutritional support, pharmacotherapy, colonic irrigation and surgical interventions, of which nutrition and surgery were the cornerstones for care. During the observed study period, the median number of hospital admissions was 22.5 (range 1-176) with a median of 157.5 days (range 3-840) during 20-year follow-up. Two patients (6%) died: one from sepsis and one due to a severe underlying neurological disease. Heterogeneity in diagnostic- en treatment methods existed between patients.</p><p><strong>Conclusions: </strong>PIPO is a rare, long-lasting complex disease requiring a high number of diagnostic and therapeutic interventions and hospital admissions. However, mortality rate is relatively low. Based on our results, we recommend centralization and standardization of care for this complex rare disease.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142564037","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Removal of an ingested battery-containing toothbrush that had caused esophageal obstruction in an 11-month-old male.","authors":"Thomas L Ratchford, Jessica Van Beek-King, Amanda Palm, Kelly McGregory","doi":"10.1002/jpn3.12405","DOIUrl":"https://doi.org/10.1002/jpn3.12405","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142564035","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Complementary feeding approaches and risk of choking: A systematic review.","authors":"Lia Correia, Ana R Sousa, Carolina Capitão, Ana R Pedro","doi":"10.1002/jpn3.12298","DOIUrl":"10.1002/jpn3.12298","url":null,"abstract":"<p><p>There are two main complementary feeding (CF) approaches: traditional spoon-feeding (TSF) and baby-led weaning (BLW). Many parents and healthcare professionals have concerns about the risk of choking associated with BLW. Since asphyxia is one of infants' main causes of death, this study aims to understand the influence of the CF approach adopted by caregivers on infants' risk of choking. A systematic review was performed. The search was conducted through PubMed, Scopus, and Web of Science databases. We included randomized controlled trials or observational studies published between January 2010 and November 2023, with a clear definition of the intervention and directly assessing the risk of choking. After the selection procedure, 7 of the 165 studies initially identified were included. No study reported statistically significant differences in the risk of choking between babies following BLW, baby-led introduction to solids (BLISS), and TSF. In five studies, although not statistically significant, infants in the TSF group had more choking episodes than those in the BLW or BLISS groups. The risk of choking does not seem to be associated with the CF approach. Instead, it may be related to the familiarity of the baby with each texture and the parent's understanding of the information about how to minimize the risk of choking. Recall bias may be present in all included studies. Advice on how to modify foods to make them safer needs to be clearer and reinforced to all parents.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"934-942"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141468853","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The association between erythema nodosum and pyoderma gangrenosum and pediatric inflammatory bowel disease.","authors":"Miranda L Yousif, Andrew Ritchey, Lucia Mirea, Ashish S Patel, Harper Price, Judith O'Haver, Jenna Rudo-Stern, Lili Montoya, Lucia Gonzalez-Llanos, Jamie Smith, Kathy Zeblisky, Brad Pasternak","doi":"10.1002/jpn3.12370","DOIUrl":"10.1002/jpn3.12370","url":null,"abstract":"<p><strong>Objectives: </strong>The objectives of this study is to estimate rates and identify factors associated with erythema nodosum (EN) and pyoderma gangrenosum (PG) in pediatric patients with inflammatory bowel disease (IBD).</p><p><strong>Methods: </strong>This cohort study examined longitudinal visits of patients aged ≤ 21 years from the ImproveCareNow (ICN) registry. We evaluated the association of factors at the patient-level (demographics and IBD diagnosis age) and visit-level (IBD severity scores, markers and phenotypes, comorbidities, and treatment) with the presence of EN and PG, using longitudinal logistic regression models adjusted for time and within-patient clustering.</p><p><strong>Results: </strong>A total of 285,913 visits from 32,497 patients aged ≤ 21 years from the ICN registry were analyzed. The occurrence of EN was 1.57% (95% confidence interval [95% CI]: 1.43%-1.71%) and the occurrence of PG was 0.90% (95% CI: 0.80%-1.00%). Co-occurrence of EN and PG was reported in 0.30% (95% CI: 0.25%-0.37%) patients. Both EN and PG were associated (p < 0.0001) with worse intestinal disease, lower remission, higher inflammatory markers, and extraintestinal manifestations (EIMs) arthritis and uveitis.</p><p><strong>Conclusions: </strong>EN and PG were associated with increased disease severity and other noncutaneous EIMs (arthritis and uveitis). A small subset of patients had developed both EN and PG.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"1009-1016"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142154448","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Sirolimus to treat chronic and steroid-resistant allograft rejection-related fibrosis in pediatric liver transplantation.","authors":"Jesús Quintero Bernabeu, Javier Juamperez Goñi, Maria Mercadal Hally, Cristina Padrós Fornieles, Juan Ortega López, Mauricio Larrarte King, José A Molino Gahete, María T Salcedo Allende, Ernest Hidalgo Llompart, Itxarone Bilbao Aguirre, Ramon Charco Torra","doi":"10.1002/jpn3.12286","DOIUrl":"10.1002/jpn3.12286","url":null,"abstract":"<p><p>This study aimed to report our experience with the use of Sirolimus (SRL) in pediatric liver transplant patients with chronic rejection or steroid-resistant rejection with hepatic fibrosis, focusing on their histological evolution. All pediatric liver transplant recipients who received off-label treatment with SRL for chronic ductopenic rejection or cortico-resistant rejection between July 2003 and July 2022 were included in the study. All nine patients included in the study showed improvement in liver enzymes and cholestasis parameters as soon as 1-month after post-SRL introduction. A decrease in fibrosis stage was observed in 7/9 (77.7%) patients at 36 months. All but one patient experienced an improvement in the Rejection Activity Index and ductopenia at 12 months. A single patient had to discontinue SRL treatment owing to nephrotic proteinuria. In conclusion, SRL may be a safe and effective treatment for chronic and steroid-resistant rejection and may improve allograft rejection-related fibrosis and ductal damage.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"962-968"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141555071","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Disorders of gut-brain interaction are common diagnoses among infants and toddlers in gastroenterology practices in Latin America.","authors":"Carlos A Velasco-Benítez, Maura Alvarez-Baumgartner, Claudia J Ortiz-Rivera, Daniela A Velasco-Suárez, Fátima A Reynoso Zarzosa, Mariana X Espriu Ramírez, Jorge A Macías-Flores, Roberto A Zablah Córdova, Ricardo A Chanis Águila, Milton D Mejía Castro, Yunuen Rivera Suazo, Amber N Balda, Miguel Saps","doi":"10.1002/jpn3.12359","DOIUrl":"10.1002/jpn3.12359","url":null,"abstract":"<p><strong>Objectives: </strong>To assess the relative frequency and associated factors of disorders of gut-brain interaction (DGBIs) in outpatient gastrointestinal (GI) clinics in young children of Latin America.</p><p><strong>Methods: </strong>Cross-sectional study in 10 pediatric GI outpatient clinics (private and public) in five countries of Latin America (El Salvador, México, Colombia, Panamá, and Nicaragua). Parents of patients 1 month 4 years of age from outpatient clinics complete/d a diagnostic questionnaire for DGBIs per Rome IV criteria (QPGS-IV, Spanish version). We conducted descriptive analysis, two-sample t-tests and chi-square tests, univariate analyses, and logistic regression to evaluate risk factors.</p><p><strong>Results: </strong>We collected data from 783 children. In total, 34.5% had a DGBI. Overall, functional constipation (FC) was the most common diagnosis (23.4%) in children of all ages (infants, 16.1%, 1-4-years-old, 32.7%). In infants, the second most common DGBI was regurgitation (6.6%) and in 1-4-years-old and cyclic vomiting syndrome (4.1%). The diagnosis of a DGBI was significantly associated with a family history of DGBIs (odds ratio [OR] 2.97, 95% confidence interval [CI] 1.61-5.57, p = 0.0001). Patients who identified as black (OR 2.25, 95% CI 1.28-3.92, p = 0.0021) or mixed race (OR 1.76, 95% CI 1.25-2.48, p = 0.0006) were also significantly associated with a higher likelihood of DGBIs.</p><p><strong>Conclusions: </strong>DGBIs are a common diagnosis in pediatric GI clinics of Latin America. Overall, FC was the most common DGBI.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"969-975"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142133030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Metabolomic comparison of postprandial distress syndrome patients with and without duodenal eosinophilia.","authors":"Stuti Dalal, Deepa Ajit, Chance S Friesen, Valentina Shakhnovich, Meenal Singh, Jennifer M Colombo, Jennifer V Schurman, Craig A Friesen","doi":"10.1002/jpn3.12351","DOIUrl":"10.1002/jpn3.12351","url":null,"abstract":"<p><strong>Objective: </strong>In functional dyspepsia patients, duodenal mucosal eosinophilia has been associated with early satiety but is not present in all patients suggesting varied pathways to symptom generation. The objective of the current study was to explore metabolic differences comparing those with duodenal mucosal eosinophilia to those without eosinophilia.</p><p><strong>Methods: </strong>This study was conducted utilizing an existing biorepository. Patients had plasma samples obtained at the time of endoscopy. All had undergone endoscopy for dyspepsia and reported early satiety. Two groups were identified including those with peak duodenal mucosal eosinophil densities above 30/high power field (N = 28) and those below 30 (N = 16). The fasting plasma samples were analyzed by liquid chromatography/high-resolution mass spectrometry. Significant differences between groups were determined.</p><p><strong>Results: </strong>The eosinophilia group demonstrated significant elevations in several gamma-glutamyl amino acids. The eosinophilia group had elevations of metabolites associated with oxidative stress including glutathione metabolites (cysteinlyglycine and cys-gly oxidized), and metabolites related to nitric oxide synthesis (arginine, citrulline, ornithine, and dimethylarginine). Eosinophilia was also associated with alterations in lipid metabolism including several long-chain acylcarnitine conjugated fatty acids. Carnitine levels were lower in the eosinophilia group. Lastly, vanillymandelate, a derivative of norepinephrine and epinephrine was elevated in the eosinophilia group.</p><p><strong>Conclusions: </strong>In patients with dyspepsia and early satiety, duodenal mucosal eosinophilia is associated with metabolites levels which are consistent with increased oxidative stress and alterations in lipid metabolism. Eosinophilia was also associated with lower carnitine levels. These alterations may contribute to pathophysiology and represent therapeutic targets.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"991-999"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141988194","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Feasibility of biliary atresia newborn screening in an integrated health network.","authors":"Stephen L Guthery, M Kyle Jensen, M Sean Esplin, Elizabeth O'Brien, Jake Krong, Rajendu Srivastava","doi":"10.1002/jpn3.12367","DOIUrl":"10.1002/jpn3.12367","url":null,"abstract":"<p><p>Diagnostic delay remains a barrier to improving biliary atresia (BA) outcomes. We tested the implementation feasibility of a two stage BA newborn screening program in an integrated healthcare system.</p><p><strong>Methods: </strong>Under a waiver of consent, we measured direct bilirubin (DB) levels in well newborns undergoing standard of care hyperbilirubinemia screening at four hospitals. Initial DB was measured by modifying nursery admission electronic medical record order sets. Second-stage DB was obtained at ~2 weeks of age under parental permission/informed consent (PP/IC). Implementation measures included the proportions of (1) eligible newborns that were screened before nursery discharge, (2) newborns undergoing second stage screening at ~2 weeks of age, and (3) newborns that underwent clinical evaluation for persistently elevated DB.</p><p><strong>Results: </strong>A total of 12,276 newborns met eligibility criteria for screening, of which 12,055 (98.2%) underwent first-stage screening in the newborn nursery. Ninety-four (0.78%) had elevated positive initial screens. Ninety newborns (95.7%) underwent second-stage screening (n = 20) or contact was made with the primary care provider to recommend second-stage screening (n = 70). Among all screened newborns, 15 (0.12%) had abnormal second screens. All had follow-up clinical evaluation for potential cholestatic liver disease. No BA cases were identified through screening, though two infants who met exclusion criteria (admission to the newborn intensive care unit) were subsequently diagnosed with BA during the screening period.</p><p><strong>Conclusions: </strong>BA newborn screening is feasible in an integrated health network. Low consent rates have implications for future studies. Program infrastructure is required for implementation success and sustainability.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"954-961"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142154447","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Four intestinal ultrasound scores and bowel wall thickness alone correlated well with pediatric ulcerative colitis disease activity.","authors":"Alexandra S Hudson, Daniela M Isaac, Henry Ma, Christine Lo, Justin Kim, Anna Kuc, Kerri L Novak, Matthew W Carroll, Eytan Wine, Hien Q Huynh","doi":"10.1002/jpn3.12358","DOIUrl":"10.1002/jpn3.12358","url":null,"abstract":"<p><strong>Objectives: </strong>Intestinal ultrasound (IUS) is a noninvasive tool in ulcerative colitis (UC), but scoring systems have mostly been developed for adults, Crohn's disease, and flaring UC. Our aim was to evaluate the performance of bowel wall thickness (BWT) and four IUS scores in pediatric patients with newly diagnosed UC.</p><p><strong>Methods: </strong>Patients <18 years old with suspected UC were prospectively enrolled. Baseline IUS was done, and ulcerative colitis intestinal ultrasound score (UC-IUS), Milan criteria, simple pediatric activity ultrasound score (SPAUSS), and Civatelli Index were calculated. Mayo endoscopic segment subscore, pediatric ulcerative colitis activity index (PUCAI), and biomarkers were correlated with IUS using nonparametric and receiver operating characteristic analyses.</p><p><strong>Results: </strong>Fifty-two patients (56% male, median age 13.9 years, interquartile range [IQR] 11.2-16.3) with 206 colon segments were included. Patients who needed hospitalization (n = 27/52) had significantly worse IUS (BWT and all scores) compared to those not hospitalized. For all patients, IUS scores and BWT significantly correlated with baseline endoscopic, clinical, and biochemical disease activity (p = 0.32-0.67, p < 0.05). BWT (τ_b = 0.53), UC-IUS (τ_b = 0.55), and Milan (τ_b = 0.52) had the strongest endoscopic correlations. For differentiating between endoscopic disease severity, BWT, UC-IUS, and Milan, had the highest areas under the curve (0.89-0.93). Using BWT alone, a thinner cut-off had improved sensitivity while maintaining high specificity: ≥2.5 mm for moderate/severe endoscopic inflammation (sensitivity 66%; specificity 94%) and ≥3.5 mm for severe endoscopic inflammation (sensitivity 92%; specificity 86%).</p><p><strong>Conclusions: </strong>BWT and all four IUS scores correlated well with endoscopic, clinical, and biochemical disease activity, and was another useful marker of severity in identifying patients needing hospitalization. Pediatric patients needed a thinner BWT cut-off, which should be accounted for when developing pediatric-specific scores. BWT alone may be just as clinically useful as composite US scores.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"1000-1008"},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142080698","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Direct cholangioscopy supporting diagnosis and conservative surgery of a biliary rhabdomyosarcoma in a child.","authors":"Valerio Balassone, Simona Faraci, Mario Brusco, Claudia Mandato, Giuseppe Maria Milano, Marco Spada, Paola De Angelis","doi":"10.1002/jpn3.12404","DOIUrl":"https://doi.org/10.1002/jpn3.12404","url":null,"abstract":"","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142563852","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}