JPGN reports最新文献

Objectives: This single-center, cross-sectional study aims to elucidate the clinical presentation, diagnostic evaluation, and outcomes in a subset of pediatric patients with atypical and/or challenging presentations of Meckel's diverticulum.

Methods: We conducted a single-center cross-sectional study on children diagnosed with Meckel's diverticulum at Children's Health in Dallas, Texas between 2010 and 2022. We identified 11 patients aged 0-17-years-old with confirmed Meckel's diverticulum who presented with atypical symptoms and/or a challenging diagnostic course. Patient demographics, symptoms at presentation, diagnostic workup, time to diagnosis, management, and outcomes were collected. Descriptive statistics were utilized.

Results: Eleven patients (n = 8, 73% male) were included in the study with an average age of 10.5 years (range: 1-17 years). The mean time interval from initial presentation of symptoms to diagnosis was 8 months (range: 0-33 months). Barriers to diagnosis of Meckel's diverticulum identified in our study included atypical presentations, negative or nonconfirmatory Meckel scan results, negative surgical findings, and competing differential diagnoses.

Conclusion: Meckel's diverticulum is a challenging diagnosis and should be considered even if initial evaluation is negative as certain patients exhibit atypical presentations that necessitate surgical intervention for diagnosis.

Menetrier's disease (MD) is a protein-losing gastropathy characterized by acute generalized edema due to hypoalbuminemia. MD is rare in childhood, and it is commonly associated with cytomegalovirus infection. We reported two children, who presented with a history of generalized edema after some days of abdominal pain and diarrhea. Laboratory tests showed hypoalbuminemia with no proteinuria. Thoracic and abdominal ultrasound (US) revealed respectively pleural and pericardial effusion and ascites. A specific gastric echography showed gastric wall thickening (>3 mm) and upper gastrointestinal endoscopy revealed prominent folds in the gastric body and fundus, with a subsequent histological confirmation of Menetrier diagnosis. They were discharged after several albumin infusions. A US follow-up confirmed the remission of the disease after 1 and 6 months. Gastric US revealed accurate in the diagnosis of this rare condition and in its follow-up. avoiding a second endoscopy in the short term.

Several states have recently enacted laws permanently granting all public school students access to free breakfast and lunch. However, children with dietary restrictions, such as celiac disease (CeD), may encounter barriers to participation in these meal programs. We surveyed caregivers of school-aged children with CeD to study barriers to universal school meals. More than half of the children with CeD did not participate in school meal programs due to concerns about the cafeteria's ability to prepare gluten-free (GF) meals safely. Moreover, among those who were food insecure and GF food insecure, 50% had never consumed free school lunch and breakfast. Parental perception of nutritional quality, communication regarding GF options, and safety of school kitchens emerged as common obstacles to participation in these programs. Addressing these concerns is paramount to ensuring equitable access to nutritious meals for all students.

Eosinophilic esophagitis (EoE) is an immunoinflammatory disease of the esophagus attributable to a complex interaction between genetic and environmental factors. While several genetic risk variants have been linked with EoE, we report a novel association between B-cell lymphoma/leukemia 11B genetic mutation in a child with dysmorphic facies, developmental delays, atopic comorbidities, and difficult-to-treat EoE. After a prolonged course of EoE and multiple esophagogastroduodenoscopies with biopsies, this patient achieved clinical and histologic remission on a combination of swallowed topical steroids and high-dose proton pump inhibitor (PPI) therapy. However, her EoE relapsed when we attempted to wean her off PPI, and it was finally controlled after adding PPI back to her regimen. This report underscores the importance of genetic testing in patients with unusual clinical features and difficult-to-treat EoE. Relevant to real-world clinical practice, this case also raises the question of the treatment goals in children with EoE and underlying genetic mutation(s).

Objectives: Challenges regarding feeding difficulties and nutrition in children with esophageal atresia (EA) have been sparsely studied. The aim of this study was to explore parent-reported feeding difficulties in children with EA by applying Montreal Children's Hospital-Feeding Scale (MCH-FS), and to further explore associations between feeding difficulties and clinical factors, growth and nutritional intake.

Methods: Parents of EA children born between 2012 and 2017 were invited. Clinical data were collected from medical records. In a prospective cohort-study parent-reported feeding difficulties (by MCH-FS) were reported at two assessments, and at the second assessment, dietary data were collected by using the 24-h food-recall method.

Results: Out of 55 eligible participants, we evaluated 53 children at median age of 1.6 years (Q1:Q3 1.0:2.9) (first assessment) and 38 at median age of 4.2 years (Q1:Q3 1.0:2.9) (second assessment). Feeding difficulties were reported by 34% and 31% of the parents, respectively, but no particular profile of concerns could be identified. Children's energy intake and weight-for-age were correlated with feeding difficulties (MCH-FS total score) (p < 0.02).

Conclusion: Parent-reported feeding difficulties were identified in one-third of children with EA and related to low energy intake and low weight-for-age, but not to clinical factors. This implies that feeding difficulties must be screened for during follow-up in all EA children and may facilitate early detection of challenges and intervention if needed.

An infant with biliary atresia had normal-range ('low') serum gamma-glutamyltranspeptidase (GGT) activity, exceptional because GGT generally is elevated in biliary atresia. Mechanisms underlying low-GGT cholestasis in biliary atresia are not defined, but the phenomenon is associated with worse clinical outcome. Testing in our patient revealed no variants in genes mutated in several disorders also associated with poor prognosis and with low-GGT cholestasis; indeed, at age 14 months she has stable disease with unremarkable biomarker values. Nonetheless, we recommend extended investigations in such patients, including genetic testing, to detect coexistent disorders and to expand understanding of GGT in biliary atresia.

Exploratory laparotomy in neonates is typically performed via a transverse laparotomy incision. However, this incision may be complicated by poor cosmesis and scar contracture. In three patients, primary gastroenterologists identified significant scar contractures that resulted in pain and limitations with physical activity, necessitating surgical referrals. All patients required subsequent surgical revision of their scar, which involved creation of skin flaps, repair of abdominal wall hernias if present, and reapproximation of the subcutaneous tissue. We describe this phenomenon and the resultant need for surgical management to raise awareness of these late complications and suggest subcutaneous tissue reapproximation should be performed when possible during abdominal wall closure.

Idiopathic myointimal hyperplasia of the mesenteric veins (IMHMV) is a rare entity reported in adults, frequently mimicking inflammatory bowel disease (IBD). We report the first case of intestinal ischemia secondary to IMHMV presenting as IBD in a pediatric patient with Down syndrome. Chronic intestinal ischemia is rare in children, and this case emphasizes the need to consider this in the differential, when histopathology is suggestive. Clinical findings and colonoscopy in these patients may mimic IBD. The mucosal biopsies do not show classic features of IBD. Instead, there can be variable extent of mucosal changes such as dilated small capillaries in the lamina propria, edema, and early fibrosis. These changes may in fact represent early chronic ischemia. These findings should alert for vascular imaging and a full-thickness biopsy to assess submucosal and subserosal larger vessels since medium- and large-caliber veins in the colonic wall and mesentery are affected in IMHMV.

Objectives: Payer mandates have resulted in children with inflammatory bowel disease (IBD) switching from originator Remicade® (O-Rem) to an infliximab biosimilar (B-IFX). Patients and families are fearful of switching because disease has been well controlled on O-Rem. Real-world data documenting clinical outcomes after such switches in pediatric patients are limited. The aim of this project was to examine 1 year of follow-up in a large adolescent/young adult IBD cohort who changed from O-Rem to B-IFX.

Methods: We identified patients with IBD at Connecticut Children's receiving O-Rem for at least 1 year, who were either in clinical remission or had low disease activity, and who were subsequently switched to B-IFX. An age, gender, IBD-subtype, and duration since diagnosis cohort that continued on O-Rem was then matched to the switch cohort and served as a comparator group (1: switch vs. 2: no-switch). B-IFX was Inflectra® in all cases.

Results: Two hundred and seventy-nine patients (mean age 18.7 years, Crohn's disease = 243, ulcerative colitis = 36) were studied (switch, n = 93, no-switch, n = 186). Mean time since diagnosis was >6 years in both groups, and mean duration of anti-tumor necrosis factor use was >5 years. There were no significant changes in hemoglobin, albumin, C-reactive protein, erythrocyte sedimentation rate, or disease activity in either group over 1 year. Dosing modifications as well as the frequency of low-level antibodies to infliximab were similar in both groups over the study period.

Conclusion: Switching from O-Rem to B-IFX has no impact on clinical or laboratory parameters over the subsequent year. Clinicians can reliably reassure patients and families that switching is safe.

Incidental diagnosis of asymptomatic appendicitis is exceptionally rare, even more so when identified endoscopically. This is among the first reported cases of appendicitis incidentally diagnosed during colonoscopy in a pediatric patient. Most notably, the identification of subclinical appendicitis allowed for early referral to pediatric surgery for management, which in turn may have prevented progression to acute symptomatic appendicitis.