Harnessing Electronic Medical Record Tools to Provide Proceduralist Feedback on Pediatric Endoscopy Quality Metrics.

In 2022, the Pediatric Endoscopy Quality Improvement Network published quality metrics related to pediatric endoscopy. We utilized electronic medical record (EMR) tools to collect pediatric endoscopy quality metrics (PEQM) and to standardize proceduralist feedback. EMR tools were created to capture and display PEQM: (1) an endoscopy documentation template, (2) nursing documentation of events during endoscopy for timed calculations, and (3) a data dashboard. Dashboard metrics provided individualized PEQM feedback relative to group performance and ideals where available. Utilization of the endoscopy documentation tools and data dashboard was measured. Utility was assessed using a survey based on the Technology Adoption Model. Adoption of documentation tools has been nearly universal with positive survey outcomes. Robust dashboard visualization has been demonstrated. Use of EMR documentation tools standardized PEQM collection. Future capture and sharing of common PEQM data across institutions could help determine PEQM benchmarks.

Adolescents' and Young Adults' Satisfaction with and Understanding of Medical Notes from a Pediatric Gastroenterology Practice: A Cross-Sectional Cohort Study.

Medical note sharing enhances patient-physician relationships, increases medication adherence, and improves self-care. However, many institutions do not release medical notes to adolescents, citing poor understanding and patient harm concerns. We evaluated the results of medical note sharing among adolescents with chronic disease and found high satisfaction and adequate comprehension.

A pilot study of S-adenosylmethionine in treatment of functional abdominal pain in children.

CONTEXT: Functional abdominal pain (FAP) is one of the most common functional gastrointestinal disorders (FGIDs) in children. Currently, medical practitioners widely use tricyclic antidepressants to treat FAP. Those antidepressants, however, have been associated with an increased risk of suicidal ideation, and the accompanying side effects often limit the benefits. S-adenosylmethionine (SAM-e) is a dietary supplement that has efficacy as an antidepressant and as a treatment for chronic pain. OBJECTIVE: The research team hypothesized that during SAM-e exposure (1) participants' pain reports would significantly improve over time, (2) participants' reported quality of life would significantly improve over time, and (3) toxicity measures (liver-function tests and mania and depression scales) would not change significantly. DESIGN: The research team performed an open-label, doseescalation trial of oral SAM-e among children with FAP. Participants came to the research facility for measurements at baseline and after 2 wk, 1 mo, and 2 mo. The research team monitored participants for potential toxicities (liver toxicity, mania, and depression) throughout the trial. SETTING: The trial was conducted at the University of California, San Diego. PARTICIPANTS: The research team recruited children and adolescents with FAP via advertisement at several community general pediatric clinics and at the research team's subspecialty pediatric gastrointestinal clinic at a tertiary care center. The eight resulting participants were children with a median and mean age of 14 y. INTERVENTION: To treat persistent abdominal pain, all participants received SAM-e at an initial dose of 200 mg/d, with escalation to a maximum dose of 1400 mg/d over the period of 2 mo. OUTCOME MEASURES: The primary outcomes were the participants' self-reports of pain and quality of life. The research team used the multidimensional measure for recurrent abdominal pain (MM-RAP), Wong-Baker FACES Pain Rating Scale, and the PedsQL for those measurements. The team used repeated measures analyses to analyze the data. RESULTS: Six participants completed the study. The research team demonstrated an improvement in self-pain reports over the 2-mo follow-up period (P = .004). The median dose of SAM-e that participants took at the 2-mo follow-up period was 1400 mg (interquartile range: 950-1400 mg) daily. Liver function tests and assessments for mania and depression did not change over the study period. CONCLUSIONS: Oral SAM-e demonstrates promise in reducing abdominal pain among children with FAP, with minimal toxicity. The research team needs to conduct larger, placebo-controlled trials to support its initial findings.

AN 8-YEAR-OLD CALIFORNIA GIRL WITH ASYMPTOMATIC HEPATIC CYSTS.

Echinococcus infections are rare in the United States but may present a growing public health threat. We present the case of an 8-year-old female patient from Southern California who was diagnosed with hepatic echinococcosis after the incidental discovery of large hepatic cysts.

Coordinate down-regulation of adenylyl cyclase isoforms and the stimulatory G protein (G(s)) in intestinal epithelial cell differentiation.

The intestinal epithelium is dynamic, with proliferation of undifferentiated crypt cells balanced by terminal differentiation and cell death at the colon surface or small intestinal villus tips. Cyclic AMP, induced by agonists such as prostaglandin E(2) and vasoactive intestinal polypeptide, promotes proliferation and ion secretion and suppresses apoptosis in intestinal epithelial cells. Here, we show that cell differentiation in a model intestinal epithelium leads to attenuation of cAMP production in response to G protein-coupled receptor and receptor-independent agonists. Concomitantly, key components of the cAMP cascade, the alpha subunit of the stimulatory G protein, G(s), and adenylyl cyclase (AC) isoforms 3, 4, 6, and 7 are down-regulated. By contrast, AC1, AC2, AC8, and AC9, and the receptors for prostaglandin E(2) and vasoactive intestinal polypeptide, are not expressed or not affected by differentiation. We confirmed key findings in normal murine colon epithelium, in which the major AC isoforms and G(s)alpha are markedly down-regulated in differentiated surface cells. Suppression of AC isoforms and G(s)alpha is functionally important, because their constitutive expression completely reverses differentiation-induced cAMP attenuation. Thus, down-regulation of AC isoforms and G(s)alpha is an integral part of the intestinal epithelial differentiation program, perhaps serving to release cells from cAMP-promoted anti-apoptosis as a prerequisite for cell death upon terminal differentiation.

Hereditary Spherocytosis as an Atypical Presentation of Anemia in Ulcerative Colitis.

Anemia is encountered in up to two-thirds of all patients with inflammatory bowel disease (IBD). We are reporting a case of a 9-year-old female with history of very early onset IBD ulcerative colitis, and primary sclerosing cholangitis who was found to have hereditary spherocytosis as the etiology of her anemia. Despite good clinical response to IBD therapy, she continued to have persistent normocytic anemia. Liver biopsy and magnetic resonance cholangiopancreatography for uptrending liver transaminases demonstrated iron deposition which led to a T2-weighted magnetic resonance imaging study that quantified significant iron deposition in her liver and kidneys. Without any history of blood transfusions, these findings were concerning for hereditary hemochromatosis, but the hereditary hemochromatosis gene test was negative. Whole genome sequencing identified a pathogenic de novo variant consistent with hereditary spherocytosis. Table of Contents Summary: A novel presentation of anemia in inflammatory bowel disease.

Expanding the genotypic spectrum of ACTG2-related visceral myopathy.

Visceral myopathies (VMs) encompass a spectrum of disorders characterized by chronic disruption of gastrointestinal function, with or without urinary system involvement. Pathogenic missense variation in smooth muscle γ-actin gene (ACTG2) is associated with autosomal dominant VM. Whole-genome sequencing of an infant presenting with chronic intestinal pseudo-obstruction revealed a homozygous 187 bp (c.589_613 + 163del188) deletion spanning the exon 6-intron 6 boundary within ACTG2 The patient's clinical course was marked by prolonged hospitalizations, multiple surgeries, and intermittent total parenteral nutrition dependence. This case supports the emerging understanding of allelic heterogeneity in ACTG2-related VM, in which both biallelic and monoallelic variants in ACTG2 are associated with gastrointestinal dysfunction of similar severity and overlapped clinical presentation. Moreover, it illustrates the clinical utility of rapid whole-genome sequencing, which can comprehensively and precisely detect different types of genomic variants including small deletions, leading to guidance of clinical care decisions.

What do pediatric primary care providers think are important research questions? A perspective from PROS providers.

OBJECTIVE: To describe what pediatric primary care providers involved in the Pediatric Research in Office Settings (PROS) research network think are important yet inadequately addressed questions in pediatric primary care research. METHODS: A total of 1785 pediatric primary care providers in the PROS network were asked what they thought were important yet inadequately addressed areas of primary care research. We used a single, open-ended question in a mail survey. Written answers to this question were analyzed by qualitative methods to determine the main themes of interest to pediatric primary care providers. RESULTS: Overall survey response rate was 48.7%; the open-ended question yielded 1109 individual answers. Six lines of inquiry were identified as being important to these providers: (1) effective counseling techniques to use in anticipatory guidance; (2) strategies to prevent and treat obesity; (3) the effectiveness of well-child care; (4) ongoing management of patients with attention-deficit/hyperactivity disorder; (5) the role of the primary care provider in caring for children with mental health needs; and (6) optimal organization of office practices. CONCLUSIONS: The translation of research into practice may be improved by a better understanding of the needs and interests of those who see pediatric patients in the primary care setting.

IL-6-dependent mucosal protection prevents establishment of a microbial niche for attaching/effacing lesion-forming enteric bacterial pathogens.

Enteric infections with attaching/effacing lesion-inducing bacterial pathogens are a worldwide health problem. A murine infection model with one such pathogen, Citrobacter rodentium, was used to elucidate the importance of the pleiotropic immune regulator, IL-6, in the pathogenesis of infection. IL-6 was strongly induced in colonic epithelial cells and macrophages upon C. rodentium infection and was required for effective host defense, because mice lacking IL-6 failed to control bacterial numbers 2-3 wk after infection and exhibited increased mortality. IL-6 was not needed for mounting effective T and B cell responses to the pathogens, nor was it important for induction of IFN-gamma or TNF-alpha, cytokines involved in host defense against the bacteria, or the antibacterial effector, NO. Instead, IL-6 played a key role in mucosal protection, since its absence was associated with marked infection-induced apoptosis in the colonic epithelium and subsequent ulcerations. Cell culture studies confirmed that IL-6 protected colon epithelial cells directly against inducible apoptosis, which was accompanied by increased expression of an array of genes encoding antiapoptotic proteins, including Bcl-x(L), Mcl-1, cIAP-2, and Bcl-3. Ulcerations appeared to be pathogenetically important, because bacteria localized preferentially to those regions, and chemically induced colonic ulcerations promoted bacterial colonization. Furthermore, blood components likely present in ulcer exudates, particularly alanine, asparagine, and glycine, promoted bacterial growth. Thus, IL-6 is an important regulator of host defense against C. rodentium by protecting the mucosa against ulcerations which can act as a microbial niche for the bacteria.