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An ever-increasing number of disturbances in glycosylation have been described to underlie certain unexplained liver diseases presenting either almost isolated or in a multi-organ context. We aimed to update previous literature screenings which had identified up to 23 forms of congenital disorders of glycosylation (CDG) with associated liver disease. We conducted a comprehensive literature search of PubMed and Google Scholar databases looking at articles published during the last 20 years (January 2000 – October 2020). Eligible studies were case reports/series reporting liver involvement in CDG patients. Our systematic review led us to point out 41 forms of CDG where liver is primarily affected (n = 7) or variably involved in a multisystem disease with mandatory neurological abnormalities (n=34). Herein we summarize individual clinical and laboratory presentation characteristics of these 41 CDGs and outline their main presentation and diagnostic cornerstones with the aid of two synoptic tables. Dietary suppe case reports/series reporting liver involvement in CDG patients. Our systematic review led us to point out 41 forms of CDG where liver is primarily affected (n = 7) or variably involved in a multisystem disease with mandatory neurological abnormalities (n=34). Herein we summarize individual clinical and laboratory presentation characteristics of these 41 CDGs and outline their main presentation and diagnostic cornerstones with the aid of two synoptic tables. Dietary supplementation strategies have hitherto been investigated only in 7 of these CDG types with liver disease, with a wide range of results. In conclusion the systematic review recognized a liver involvement in a somewhat larger number of CDG variants corresponding to about 30% of the total of CDG so far reported, and it is likely that the number may increase further. This information could assist in an earlier correct diagnosis and a possibly proper management of these disorders.
Given the importance of scholarly work in academic medicine, better understanding of the manuscript review process (MRP) is useful for authors, reviewers, and editorial boards. We aim to describe the MRP at the Journal of Pediatric Gastroenterology and Nutrition (JPGN), assess the correlation between editor decisions and reviewer recommendations, and provide transparency to this process.
All manuscripts submitted in 2018 to JPGN were included in this analysis. Data included reviewers’ manuscript scores and recommendations, time spent on each review by reviewers, the editor’s rating of the reviewers’ reviews, the editor’s first decision, and final outcome. Data were collated using the JPGN manuscript submission website, Editorial Manager.
1023 manuscripts were submitted to JPGN in 2018 and included in this analysis. Of these, 486 manuscripts had at least two peer reviewers. The recommendations of the two reviewers were in agreement 43% of the time. Intra-class correlation (ICC) between the two reviewers suggests moderate agreement (ICC = 0.40). When both reviewers agreed to Not Reject (289/486), the editor agreed in 93% of cases (269/289). When both reviewers agreed to Reject (55/486), the editor agreed 100% of the time (55/55). The reviewers disagreed in about one-third of submissions (142/486), and the editor recommended to Reject in two-thirds of these cases (95/142). Overall, inter-reviewer agreement strongly correlated with the editor’s initial decision (p < 0.001).
The editor most often agreed with reviewers’ assessments when there was concordance between the two reviewers’ recommendations. About a third of peer reviews result in discordant recommendations between the two reviewers.
The editor most often agreed with reviewers’ assessments when there was concordance between the two reviewers’ recommendations. About a third of peer reviews result in discordant recommendations between the two reviewers.
Understanding coronavirus disease 2019 (COVID-19) in pediatric inflammatory bowel disease (PIBD) is important. We describe a single-center cohort of COVID-19 PIBD patients where seroconversion against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was examined.Immunosuppressed PIBD patients at Texas Children’s Hospital who tested positive for SARS-CoV-2 by nasopharyngeal reverse transcriptase polymerase chain reaction were included in the study. The clinical course of IBD, concurrent medications, COVID-19 related symptoms, SARS-CoV-2 testing date, and SARS-CoV-2 immunoglobulin G (IgG) antibody testing date and result were examined. Of 14 SARS-CoV-2 positive PIBD patients, 12 were tested for qualitative anti-SARS-CoV-2 IgG (seven with transient COVID-19 symptoms, five asymptomatic). All symptomatic (7/7) and 60% of asymptomatic (3/5) patients seroconverted. Selleck MS41 No patients required hospitalization attributed to COVID-19.High rates of COVID-19 seroconversion occurred in immunosuppressed and symptomaibuted to COVID-19.High rates of COVID-19 seroconversion occurred in immunosuppressed and symptomatic PIBD patients. More research to evaluate the significance of COVID-19 seroconversion is needed.
To present the clinical characteristics, treatment and outcomes of pediatric collagenous gastritis (CG).
This is a retrospective cohort study. Patients were identified via query of the institutional pathology database. Clinical data was obtained by review of medical records.
40 patients (57.5% female) were identified, mean age 11.3 ± 3.7 years (2-16years). Isolated CG was present in 66.7%, coexisting collagenous duodenitis in 17.5%, collagenous colitis in 7.5% and collagenous ileitis in 2.5%. Atopic comorbidities were found in 25.0%, autoimmune comorbidities in 12.5%.
Abdominal pain (77.5%), vomiting (65.0%), anemia (57.5%), nausea (55.5%), diarrhea (32.5%), anorexia (25.0%), weight loss (25.0%), gastrointestinal bleed (22.5%), poor growth (20.0%), poor weight gain (12.5%).
All had abnormal endoscopic findings on esophago-gastro-duodenoscopy (EGD), most commonly gastric nodularity (77.5%), visible blood (20.0%), erosions/superficial ulcerations (10.0%), ulcers (7.5%). Histologically, all patients had increased subepithelial collagen deposition.