Lack of standardization and faculty development in pediatric colonoscopy: A qualitative study.

A standard curriculum for pediatric colonoscopy training has neither been required nor universally implemented in North American fellowship programs. This qualitative study assessed the needs of colonoscopy training in pediatric gastroenterology to determine the standardized components of procedural teaching. Focus groups with pediatric gastroenterology attendings, fellows, procedural nurses, and interviews with advanced endoscopists, all practicing at a single institution, were conducted between March and June 2018. Data were analyzed using thematic analysis principles. Four themes emerged: (1) lack of standardization of colonoscopy performance, (2) lack of professional development of procedure teaching skills, (3) need for teaching behaviors that promote learner's performance, and (4) barriers to effective teaching and learning. A conceptual framework was created for developing a standardized "train-the-trainer" curriculum. Our needs assessment supports expansion of efforts to make this comprehensive training available to all pediatric gastroenterologists involved in procedure teaching.

Creating a health equity and inclusion office in an academic pediatric medical center: priorities addressed and lessons learned.

BACKGROUND: Over the last two decades, medical schools and academic health centers have acknowledged the persistence of health disparities in their patients and the lack of diversity in their faculty, leaders and extended workforce. We established an Office of Health Equity and Inclusion (OHEI) at our pediatric academic medical center after a thorough evaluation of prior diversity initiatives and review of faculty development data. OBJECTIVE: To describe the lessons learned at a pediatric academic medical center in prioritizing and implementing health equity, diversity and inclusion (EDI) initiatives in creating the OHEI. MATERIALS AND METHODS: We reviewed internal administrative data and faculty development data, including data related to faculty who are underrepresented in medicine, to understand the role of our EDI initiatives in the strategic priorities addressed and lessons learned in the creation of the OHEI. RESULTS: The intentional steps taken in our medical center's strategic approach in the creation of this office led to four important lessons to improve pediatric health equity: (1) board, senior executive and institutional prioritization of EDI initiatives; (2) multi-specialty and interprofessional collaboration; (3) academic approach to EDI programmatic development; and (4) intentionality with accountability in all EDI initiatives. CONCLUSION: The key lessons learned during the creation of an Office of Health Equity and Inclusion can provide guidance to other academic health centers committed to implementing institutional priorities that focus their EDI initiatives on the improvement of pediatric health equity.

North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Position Paper on Entrustable Professional Activities: Development of Pediatric Gastroenterology, Hepatology, and Nutrition Entrustable Professional Activities.

Quality training in pediatric gastroenterology, hepatology, and nutrition is essential for the future of our specialty from advancing the science through research to providing clinical care for children with gastrointestinal, hepatic and nutritional disorders. As educational theory has developed, both the American Board of Pediatrics (ABP) and the Accreditation Council for Graduate Medical Education (ACGME) have commissioned projects to better define training including core competencies, and milestones with the goal of competency-based assessment. Seeking to provide a clinical context for these competencies and milestones, the ABP commissioned a project for each pediatric subspecialty to develop entrustable professional activities (EPA) while at the same time developing EPAs that are common to all pediatric subspecialties. North American Society for Pediatric Gastroenterology, Hepatology, Nutrition (NASPGHAN) commissioned an EPA Task Force to develop the pediatric gastroenterology, hepatology and nutrition EPAs. This document serves as an introduction to EPAs, including their historical background, underlying educational theory, and the process used to develop the pediatric gastroenterology, hepatology and nutrition EPAs in the United States of America.

Pediatric Gastroenterology, Hepatology, and Nutrition Entrustable Professional Activities: Development of an Assessment Tool and Curricular Resources.

BACKGROUND: Entrustable professional activities (EPAs) are critical activities performed by medical professionals, which can be observed and assessed. Adding on to common EPAs for all pediatric subspecialty trainees, specialty-specific EPAs for pediatric gastroenterology, hepatology, and nutritional fellowship were developed by the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) EPA Task Force. METHODS: Having developed specialty-specific EPAs, building EPA assessments is the next logical step, as EPAs are included under a larger umbrella of competency-based assessment. Thus, the NASPGHAN EPA Task Force and Training Committee collaborated on an assessment tool and associated curricular resources to aid in tracking trainees' progression to entrustment within individual EPAs and readiness for independent practice. RESULTS: This manuscript reports the development of an EPA assessment tool, including guiding principles and the theory behind the assessment tool, with a focus on simple, meaningful assessments that can provide crucial performance feedback to trainees. In addition, curricular resources were developed, based on the assessment tool, to support training. Ultimately, it is the hope of the NASPGHAN EPA Task Force and Training Committee that this tool can aid training programs in providing formative feedback for trainees, and can be used by training programs and clinical competency committees for summative evaluation.

Lack of Follow-up of Pediatric Patients With Celiac Disease.

Celiac disease (CD) is often diagnosed in childhood, and the treatment is a lifelong gluten-free diet (GFD).1,2 It may take several years to gain competence in the skills required to follow a GFD successfully. Inadequately treated CD is associated with bone fractures, nutritional deficiencies, and lymphoma.3,4 Healthcare providers are key resources for patients with CD. Consultation with a dietitian with GFD expertise at diagnosis and annual disease-specific follow-up care are recommended.2,5 The primary objective of this study was to evaluate adherence to guidelines for dietitian consultation and follow-up for children with CD. A secondary objective was to identify factors associated with loss to follow-up.

Red Spot Lesions in the Duodenal Bulb Are a Highly Specific Endoscopic Sign of Celiac Disease: A Prospective Study.

We have recognized red spot lesions (RSLs) in the duodenal bulb in children with celiac disease (CD) and believe they may represent an underappreciated and distinct endoscopic sign of CD. A total of 171 pediatric patients undergoing esophagogastroduodenoscopy with duodenal biopsy for symptoms consistent with CD were prospectively recruited. There were 75 patients who met criteria for CD and the remaining 96 patients served as symptomatic controls. As compared to endoscopic markers frequently mentioned in literature, RSLs had comparable sensitivity, specificity, positive predictive value, and negative predictive value of 31%, 94%, 80%, and 64%, respectively. If RSLs are noted during endoscopy in a patient with gastrointestinal symptoms that might be the result of CD, then sufficient duodenal biopsies to make the diagnosis of CD should be obtained.

Value of IgA tTG in Predicting Mucosal Recovery in Children With Celiac Disease on a Gluten-Free Diet.

OBJECTIVES: Our objective was to determine the rate of mucosal recovery in pediatric patients with celiac disease on a gluten-free diet. We also sought to determine whether immunoglobulin A tissue transglutaminase (tTG) correlates with mucosal damage at the time of a repeat endoscopy with duodenal biopsy in these patients. METHODS: We performed a retrospective chart review of 103 pediatric patients, younger than 21 years, with a diagnosis of celiac disease defined as Marsh 3 histology, and who underwent a repeat endoscopy with duodenal biopsy at least 12 months after initiating a gluten-free diet. RESULTS: We found that 19% of pediatric patients treated with a gluten-free diet had persistent enteropathy. At the time of the repeat biopsy, tTG was elevated in 43% of cases with persistent enteropathy and 32% of cases in which there was mucosal recovery. Overall the positive predictive value of the autoantibody tTG was 25% and the negative predictive value was 83% in patients on a gluten-free diet for a median of 2.4 years. CONCLUSIONS: Nearly 1 in 5 children with celiac disease in our population had persistent enteropathy despite maintaining a gluten-free diet and immunoglobulin A tTG was not an accurate marker of mucosal recovery. Neither the presence of symptoms nor positive serology were predictive of a patient's histology at the time of repeat biopsy. These findings suggest a revisitation of monitring and management criteria of celiac disease in childhood.

Early-onset Crohn's disease and autoimmunity associated with a variant in CTLA-4.

OBJECTIVE: IBD is a group of complex, systemic disorders associated with intestinal inflammation and extraintestinal manifestations. Recent studies revealed Mendelian forms of IBD, which contributed significantly to our understanding of disease pathogenesis and the heritability of IBD. DESIGN: We performed exome sequencing in a family with Crohn's disease (CD) and severe autoimmunity, analysed immune cell phenotype and function in affected and non-affected individuals, and performed in silico and in vitro analyses of cytotoxic T lymphocyte-associated protein 4 (CTLA-4) structure and function. RESULTS: A novel missense variant was identified in CTLA4 encoding CTLA-4, a coinhibitory protein expressed by T cells and required for regulation of T cell activation. The residue affected by the mutation, CTLA-4 Tyr60, is evolutionarily highly conserved, and the identified Y60C variant is predicted to affect protein folding and structural stability and demonstrated to cause impaired CTLA-4 dimerisation and CD80 binding. Intestinal inflammation and autoimmunity in carriers of CTLA-4 Y60C exhibit incomplete penetrance with a spectrum of clinical presentations ranging from asymptomatic carrier status to fatal autoimmunity and intestinal inflammation. In a clinically affected CTLA-4 Y60C carrier, T cell proliferation was increased in vitro and associated with an increased ratio of memory to naive T cells in vivo, consistent with impaired regulation of T cell activation. CONCLUSIONS: Our results support the concept that variants in CTLA4 provide the basis for a novel Mendelian form of early-onset CD associated with systemic autoimmunity. Incomplete penetrance of autoimmunity further indicates the presence of other genetic and/or environmental modifiers.

Graduate medical education in humanism and professionalism: a needs assessment survey of pediatric gastroenterology fellows.

The deterioration of humanism and professionalism during graduate medical training is an acknowledged concern, and programs are required to provide professionalism education for pediatric fellows. We conducted a needs assessment survey in a national sample of 138 first- and second-year gastroenterology fellows (82% response rate). Most believed that present humanism and professionalism education met their needs, but this education was largely informal (eg, role modeling). Areas for formal education desired by >70% included competing demands of clinical practice versus research, difficult doctor-patient relationships, depression/burnout, angry parents, medical errors, work-life balance, and the patient illness experience. These results may guide curricula to formalize humanism and professionalism education in pediatric gastroenterology fellowships.

Lost in the pandemic: COVID-19's impact on health professions educators.

BACKGROUND: Minimal research has explored the pandemic's impact on health professions educators (HPEs). Given that health professions educator academies provide centralised support and professional development to HPEs through communities of practice and promoting education at their institutions, it is important to examine how academies met HPEs' needs during the pandemic. This study investigates the COVID-19 pandemic's effects on HPEs and examines how academies supported HPEs' educational roles during the pandemic. METHODS: Using a mixed-methods approach, the authors surveyed United States educator academy members on changes in HPEs' activities, emphasising clinical and educational tasks and work-life integration. Participants shared their academies' innovations and support responses. Data were analysed using chi-square and content analyses. FINDINGS: Twenty percent of 2784 recipients (n = 559) completed the survey. Most respondents indicated the pandemic caused them to spend more time on clinical and education leadership/administration than before the pandemic. HPEs integrated innovative instructional strategies, yet many shifted away from teaching, mentoring and scholarship. Over half were dissatisfied with work-life integration during the pandemic. Females, especially, reported that professional work was compromised by personal caregiving. Academies increased their range of member services; however, they did not fully meet their members' needs, including providing expanded professional development and advocating on HPE's behalf for increased protected time dedicated to educator responsibilities. DISCUSSION: HPEs faced unprecedented challenges in their personal and professional lives during the COVID-19 pandemic. Neglecting the needs of HPEs amidst global crises poses a substantial threat to the quality of education for upcoming generations of health care professionals.

Multicenter study on season of birth and celiac disease: evidence for a new theoretical model of pathogenesis.

OBJECTIVE: To investigate whether season of birth is associated with celiac disease (CD). STUDY DESIGN: We performed a medical record review of 1964 patients with biopsy-proven CD at 3 teaching hospitals (2 pediatric centers and 1 adult center) between 2000 and 2010. The first positive small intestinal biopsy result defined age of diagnosis. The observed proportions of births in each season (spring [March-May], summer [June-August], fall [September-November], and winter [December-February]) were compared with the expected proportions using binomial probability tests. RESULTS: The mean age at diagnosis was 9.8 ± 5.0 years in the 2 pediatric centers and 43.6 ± 15.8 years in the adult center. The cohort was predominately female (69%). Overall, more patients were born in spring (27%) than in any other season: summer (25%), fall (25%), and winter (23%). In patients diagnosed before age 15 years, the spring birth excess was present in boys (33%; P = .0005), but not in girls (26%; P = .43). The sex difference in season of birth was less striking in patients with CD diagnosed at age ≥15 years. CONCLUSION: Season of birth is an environmental risk factor for CD, particularly in boys diagnosed before age 15 years. The results are consistent with a new theoretical model that integrates potential environmental factors (eg, gluten introduction, ultraviolet-B exposure, vitamin D status) and acute viral gastrointestinal infections in early childhood.

Beyond the Workshop: Results From a Longitudinal, Interprofessional Teaching Certificate Program.

OBJECTIVES: While most hospital-based, healthcare professionals are expected to teach and supervise, few receive training in education. We designed, implemented, and evaluated an interprofessional, hospital-based teaching certificate program based on experiential learning and reflective practice for healthcare professionals with little or no formal training in education. METHODS: Participants attended educational seminars; incorporated new concepts, skills, and behaviors in their teaching; and submitted written reflections. Participants also met with an education coach, received feedback from a trained observer, and observed a "master teacher." We used descriptive statistics to analyze a survey distributed to the 2017-2019 cohort. We also analyzed written reflections to determine whether participants described a new teaching skill, concept, or behavior, and how they applied these to their teaching. RESULTS: Survey completion rate was 15/20 (75%). Participants described feeling connected to an educator community, establishing educational alliances with senior educators, and learning teaching strategies from other certificate members outside their own profession. Participants indicated they are more likely to pursue educational innovation, leadership, and scholarship. In the reflections, 88% described incorporating a new concept in their teaching. CONCLUSION: Participants in an interprofessional teaching certificate program engaged in a curriculum of professional development in education. Graduates of the program reported knowledge gain, behavior change, and establishment of educational alliances and a community of practice.

Navigating the gluten-free diet in college.

Factors that affect adherence to the gluten-free diet (GFD) are reported in children and adults; however, there is little data regarding young adults. The objective of the present study is to explore adherence challenges experienced by young adults in college. Responses from the online survey (N = 50), interview (N = 21), and focus group (N = 7) indicate students were motivated to adhere but experience challenges related to dining services and social situations. Dining services from 6 colleges reported a variety of accommodations for students with celiac disease, but request increased student involvement. Tools and strategies that facilitate communication between students and dining services may improve adherence.

Cytotoxic T Lymphocyte Antigen 4 Haploinsufficiency Presenting As Refractory Celiac-Like Disease: Case Report.

Primary immunodeficiency may present with treatment-refractory enteropathy. We present two patients with celiac/celiac-like disease diagnosed in early childhood and refractory to the gluten-free diet. One patient had features of multi-system autoimmunity, whereas the other had celiac-like disease as an isolated clinical finding. Both patients underwent genetic testing given disease refractoriness and were ultimately diagnosed with cytotoxic T lymphocyte antigen 4 (CTLA4) haploinsufficiency. They are both now in complete clinical and endoscopic remission on abatacept. CTLA4 haploinsufficiency has incomplete penetrance and significant phenotypic heterogeneity but should be considered in the differential diagnosis of refractory celiac/celiac-like disease, as treatment implications are significant.

Non-responsive celiac disease in children on a gluten free diet.

BACKGROUND: Non-responsive celiac disease (NRCD) is defined as the persistence of symptoms in individuals with celiac disease (CeD) despite being on a gluten-free diet (GFD). There is scant literature about NRCD in the pediatric population. AIM: To determine the incidence, clinical characteristics and underlying causes of NRCD in children. METHODS: Retrospective cohort study performed at Boston Children's Hospital (BCH). Children < 18 years diagnosed with CeD by positive serology and duodenal biopsies compatible with Marsh III histology between 2008 and 2012 were identified in the BCH's Celiac Disease Program database. Medical records were longitudinally reviewed from the time of diagnosis through September 2015. NRCD was defined as persistent symptoms at 6 mo after the initiation of a GFD and causes of NRCD as well as symptom evolution were detailed. The children without symptoms at 6 mo (responders) were compared with the NRCD group. Additionally, presenting signs and symptoms at the time of diagnosis of CeD among the responders and NRCD patients were collected and compared to identify any potential predictors for NRCD at 6 mo of GFD therapy. RESULTS: Six hundred and sixteen children were included. Ninety-one (15%) met criteria for NRCD. Most were female (77%). Abdominal pain [odds ratio (OR) 1.8 95% confidence interval (CI) 1.1-2.9], constipation (OR 3.1 95%CI 1.9-4.9) and absence of abdominal distension (OR for abdominal distension 0.4 95%CI 0.1-0.98) at diagnosis were associated with NRCD. NRCD was attributed to a wide variety of diagnoses with gluten exposure (30%) and constipation (20%) being the most common causes. Other causes for NRCD included lactose intolerance (9%), gastroesophageal reflux (8%), functional abdominal pain (7%), irritable bowel syndrome (3%), depression/anxiety (3%), eosinophilic esophagitis (2%), food allergy (1%), eating disorder (1%), gastric ulcer with Helicobacter pylori (1%), lymphocytic colitis (1%), aerophagia (1%) and undetermined (13%). 64% of children with NRCD improved on follow-up. CONCLUSION: NRCD after ≥ 6 mo GFD is frequent among children, especially females, and is associated with initial presenting symptoms of constipation and/or abdominal pain. Gluten exposure is the most frequent cause.

Variability of histopathological changes in childhood celiac disease.

OBJECTIVES: Adult studies of celiac disease (CD) have shown that duodenal mucosal histopathological changes may be patchy, and the diagnostic utility of duodenal bulb biopsies is believed to be limited. Few related pediatric data exist. METHODS: We assessed the prevalence of variable biopsy findings and duodenal bulb involvement in children with CD, as well as its association with clinical parameters. A total of 198 consecutive cases of CD diagnosed at the Children's Hospital during 2001-2005 were analyzed. All biopsies were scored by a pathologist blinded to the clinical data using the Marsh criteria. Mucosal changes were classified as focal if changes consistent with CD and normal mucosa were found within a single biopsy fragment. Patchiness was defined as variation of at least one Marsh grade between separate fragments in a biopsy set. RESULTS: The median age was 9.3 years; 62% were female. An average of 3.6 biopsy samples was obtained per case. In 101 cases, biopsy samples were obtained from the duodenal bulb and the second portion of the duodenum. Focality was present in biopsy samples collected from 36 (18%) cases. Patchiness was found in 105 (53%) cases, and at least 1 normal biopsy fragment was present in 71 (36%) cases. In 10 cases, only the bulb biopsies were diagnostic of CD. There was no association with the clinical features examined. CONCLUSIONS: Duodenal involvement in pediatric CD is frequently patchy and may show variable severity even within a single biopsy fragment. Variability cannot be predicted by clinical characteristics. Multiple endoscopic biopsies, including the duodenal bulb, should be obtained in suspected pediatric CD cases to maximize diagnostic yield.