ACG Clinical Report and Recommendations on Transition of Care in Children and Adolescents With Hereditary Polyposis Syndromes.

Transition of care (TOC) in adolescents and young adults (AYAs) with chronic gastrointestinal disorders has received increased attention, especially in those with inflammatory bowel disease. AYAs with hereditary polyposis syndromes are a heterogeneous group of patients with overlapping and complex medical needs. These patients are particularly vulnerable because of the risk of loss of continuity of care and subsequent poor disease outcomes. The Pediatric Committee of the American College of Gastroenterology commissioned a report with recommendations on TOC in AYAs with hereditary polyposis syndromes. This report aims at achieving best practice by both pediatric and adult gastroenterologists despite the paucity of published evidence in this population reflected in the included PRISMA report. Therefore, the group extrapolated findings from the literature related to other chronic gastrointestinal disorders, and a high degree of expert consensus was scored for all recommendations. The report addresses TOC through identifying shared domains followed by specific recommendations in disease management, including models of care, providers and patient and socioeconomic factors relevant to TOC. Areas of strong emphasis include the need for early planning, flexibility in the transition process to maintain continuity during major surgical procedures, patient and family psychological readiness, liaison among team members addressing transition, and changing insurance coverage in this population.

Dietary Interventions Ameliorate Infectious Colitis by Restoring the Microbiome and Promoting Stem Cell Proliferation in Mice.

Decreases in short-chain-fatty-acids (SCFAs) are linked to inflammatory bowel disease (IBD). Yet, the mechanisms through which SCFAs promote wound healing, orchestrated by intestinal stem cells, are poorly understood. We discovered that, in mice with Citrobacter rodentium (CR)-induced infectious colitis, treatment with Pectin and Tributyrin diets reduced the severity of colitis by restoring Firmicutes and Bacteroidetes and by increasing mucus production. RNA-seq in young adult mouse colon (YAMC) cells identified higher expression of Lgr4, Lgr6, DCLK1, Muc2, and SIGGIR after Butyrate treatment. Lineage tracing in CR-infected Lgr5-EGFP-IRES-CreERT2/ROSA26-LacZ (Lgr5-R) mice also revealed an expansion of LacZ-labeled Lgr5(+) stem cells in the colons of both Pectin and Tributyrin-treated mice compared to control. Interestingly, gut microbiota was required for Pectin but not Tributyrin-induced Lgr5(+) stem cell expansion. YAMC cells treated with sodium butyrate exhibited increased Lgr5 promoter reporter activity due to direct Butyrate binding with Lgr5 at -4.0 Kcal/mol, leading to thermal stabilization. Upon ChIP-seq, H3K4me3 increased near Lgr5 transcription start site that contained the consensus binding motif for a transcriptional activator of Lgr5 (SPIB). Thus, a multitude of effects on gut microbiome, differential gene expression, and/or expansion of Lgr5(+) stem cells seem to underlie amelioration of colitis following dietary intervention.

A Conceptual Framework for Rescheduling Elective Pediatric Gastroenterology Procedures Following COVID-19 Pandemic Lockdown.

Elective surgical and endoscopic procedures were suspended nationwide during the March 2020 COVID-19 pandemic to minimize exposure and healthcare resource utilization. This resulted in an unprecedented backlog of procedures in most clinical practices including pediatrics. Our group developed an internal process toward the rational development of an algorithm prioritizing elective procedures. This was based on patient disease severity defined by the presence of alert symptoms, symptom severity for dysphagia and abdominal pain, and diagnostic investigation findings. The underlying rationale is to prioritize patients in whom suspected disease course would be greatest impacted by endoscopy. We developed a nurse phone call-based process utilizing REDCap®, identifying relevant symptoms categorized by severity, and a validated functional impairment questionnaire for abdominal pain. We abstracted key laboratory and radiological findings also categorized by severity. The order of priority of procedures was established on the basis of a 4-tiered system factoring both presence and severity of symptoms or prior diagnostic testing results. We present the framework that we have adopted toward prioritizing procedures with the assumption that it offers an objective methodology and that can be efficiently and more broadly applied to other similar practice scenarios. Our tool may have wide-ranging implications both in the current COVID-19 pandemic and in other scenarios of limited resource allocation and deserves further investigation.

Pediatric Elective Diagnostic Procedure Complications: A Multicenter Cohort Analysis.

BACKGROUND AND AIM: Increased access to endoscopic procedures have entrenched these investigative tools in routine pediatric gastroenterology practice. Patient outcomes following endoscopy therefore are topical in the decision toward endoscopy. We studied the likelihood and patient characteristics of children admitted following ambulatory endoscopy. METHODS: Hospitalization data were obtained from the Pediatric Hospital Information System including 49 tertiary children's hospitals in the USA. Children who underwent ambulatory diagnostic endoscopy between October 1, 2005 and September 25, 2015 were included. The primary outcomes were post-procedure events resulting in unplanned admission (not for inflammatory bowel disease management) or emergency room visit within 5 days. Unadjusted, univariate analyses were followed by multivariable analysis of the associations between patient characteristics and outcome using the R statistical package, v. 3.2.3. RESULTS: During the study period, 217 817 patients underwent diagnostic endoscopy; 101 (0.05%) patients were admitted directly; 1314 (0.60%) were admitted to the same facility's emergency department with either a respiratory or a gastrointestinal complication as a primary diagnosis within 5 days. None of the procedures resulted in death; female patients were more likely to experience adverse outcomes (P < 0.001), as were patients from an urban setting (P = 0.0004), whereas White, non-Hispanic patients were less likely to represent (P < 0.0001). Patients with chronic comorbidities were more likely to experience complications. The most frequent diagnoses at admission were abdominal pain (30.5%), other gastroenterologic processes (26.8%), respiratory disorders (17.1%), gastrointestinal hemorrhage (8.3%), and fever (4.5%). CONCLUSIONS: Ambulatory pediatric endoscopy is safe; significant adverse outcomes are rare but more likely in female, non-White or Hispanic patients and in patients with significant chronic comorbidities.

Polyp Characteristics of Nonsyndromic and Potentially Syndromic Juvenile Polyps: A Retrospective Cohort Analysis.

BACKGROUND: Juvenile polyps (JPs) are the most common gastrointestinal polyps diagnosed in children. There is paucity of evidence differentiating polyp burden groups and the presence and significance of neoplastic changes. METHODS: A retrospective chart review of patients, ages birth through 18 years with nonsyndromic JPs was performed from 2003 to 2017. Abstracted data included basic demographics, age, clinical presentation, colonoscopy findings, and pathology report. Slides of polyps with neoplasia were reviewed by a pathologist. RESULTS: A total of 213 subjects underwent 326 procedures and 435 polypectomies. Subjects with positive family history, positive gene mutations, or numerous (>10) polyps were excluded. Groups were defined by polyp number (1, 2-4, 5-10). Polyp recurrence on repeat colonoscopy was significantly related to polyp burden (1 polyp: 1.5%/2-4 polyps 19.2%/5-10 polyps 82.6%: P < 0.001). Polyp distribution was significantly different amongst different groups with isolated polyps favoring a distal distribution. JPs harboring adenomatous foci were reported in 26 (12%) patients. JPs harboring adenomatous foci were significantly more likely to be proximally distributed but the presence of adenomatous transformation within the polyps did not correlate with polyp number or the likelihood of polyp recurrence on repeat colonoscopy. CONCLUSIONS: JP recurrence is positively and significantly related to polyp burden. JP harbored adenomatous changes independent of polyp number, underscoring a possible malignant potential in JPs. In the absence of a consistent genotype or pedigree, the presence of adenomatous transformation within JPs cannot be construed as a biomarker for syndromic juvenile polyposis.

Pediatric elective therapeutic procedure complications: A multicenter cohort analysis.

BACKGROUND AND AIM: Current understanding of specific, therapeutic procedure-associated complications in pediatric patients remains limited. This study aims to determine the frequency of significant complications in pediatric age-range subjects following the principal therapeutic endoscopic procedures. METHODS: This study used retrospective multi-institutional, ICD-9-CM, Clinical Transaction Classification, and Current Procedural Terminology based database (Pediatric Hospital Information System) analysis. This study included demographic, chronic comorbidity, procedure type, and post-procedure outcomes defined through mortality, unplanned direct admission, emergency room, and inpatient admission and inpatient therapeutic events. RESULTS: During the study period, 18 018 patients underwent therapeutic endoscopy; 132 required direct (0.16%) or emergency room/inpatient (0.58%) admission within 5 days following the procedure; mortality was 0.01%. Most (50.75%) complications presented on the day of or 1 day post-procedure. Hispanic race and coexisting chronic comorbidities, especially gastrointestinal conditions, were identified risk factors for significant complications. Endoscopic dilatation and variceal ablation were most frequently associated with complications. Abdominal pain, gastrointestinal bleeding, and esophageal stricture were the most common diagnoses: 9.0% required intravenous antibiotics, 36.63% underwent chest imaging, 27.27% abdominal imaging, and 0.75% required blood transfusion. Readmission following esophageal dilatation was most likely to result in prolonged admission. CONCLUSION: In the pediatric population undergoing therapeutic endoscopy in the ambulatory setting, significant postoperative complications resulting in unplanned admission are rare. Complications can be anticipated in medically frail patients especially with gastrointestinal chronic illness. Procedures involving variceal ablation and esophageal dilatation entail the highest risk.

Management of Juvenile Polyposis Syndrome in Children and Adolescents: A Position Paper From the ESPGHAN Polyposis Working Group.

The European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Polyposis Working Group developed recommendations to assist clinicians and health care providers with appropriate management of patients with juvenile polyposis. This is the first juvenile polyposis Position Paper published by ESPGHAN with invited experts. Many of the published studies were descriptive and/or retrospective in nature, consequently after incorporating a modified version of the GRADE system many of the recommendations are based on expert opinion. This ESPGHAN Position Paper provides a guide for diagnosis, assessment, and management of juvenile polyposis syndrome in children and adolescents, and will be helpful in the appropriate management and timing of procedures in children and adolescents. The formation of international collaboration and consortia is proposed to monitor patients prospectively to advance our understanding of juvenile polyposis conditions.

Clinical and Genetic Study of Children With Peutz-Jeghers Syndrome Identifies a High Frequency of STK11 De Novo Mutation.

OBJECTIVES: The present study aims to identify the genotype-phenotype correlation in children with Peutz-Jeghers Syndrome (PJS) through the analysis of STK11 gene mutations in the context of clinical and pathological characteristics. METHOD: In this observational cohort study, the clinical characteristics of 18 families diagnosed with pediatric PJS were collected. Genomic DNA from the peripheral blood of affected children and their family members was collected. The coding region of STK11 was amplified by PCR and screened for mutation by Sanger sequencing. The families that were negative for STK11 mutation were further assessed by multiplex ligation-dependent probe amplification (MLPA). RESULT: Initial presentation in affected children was at 1.6 to 14.2 years and included anemia in 8 patients whereas 6 presented for screening by virtue of family history. All patients underwent endoscopy, colonoscopy, and polypectomy. Polyps were distributed throughout the gastrointestinal (GI) tract, including the small intestine, stomach, colon, and rectum.In the 18 pediatric PJS families, STK11 mutations were detected in 8 families by Sanger sequencing, and large deletions were detected in 3 by MLPA, respectively. Nine of the 11 STK11 mutations were de novo, 3 were novel (c.419T>C:p.L140P, c.314T>G:p.L105X), and (c.488_489insACGG p.L164fs). CONCLUSIONS: Although the main clinical features of pediatric PJS were similar to those of PJS cases in adults, a high frequency of STK11 de novo mutations were encountered in our population of patients with PJS.

One-day oral polyethylene glycol based cleanout is effective for pre-colonoscopy preparation in children.

BACKGROUND: The adequacy of pre-procedure preparation is the principal determinant of the quality of colonoscopy in pediatric as in adult patients. There is a lack of consensus, among providers on a standard pre-procedure regimen. Professional society guidelines include the use of Polyethylene glycol (PEG). Herein we report on the provider-assessed adequacy of a one day, age-categorized dosing, PEG based cleanout regimen in children undergoing colonoscopy in a tertiary institution. METHODS: The standard bowel preparation regime at our institution includes an age dependent minimum PEG dosing regimen in addition to clear liquids the day prior to the procedure. We retrospectively abstracted relevant indices including patient demographics, prep quality, procedure impairment, duration and completion from an institutional quality monitoring survey tool between 2015 and 2016 and similarly abstracted prospectively recorded indices that included the dataset above as well as additional fields for procedure deviations and additional laxative use. RESULTS: A total of 642 procedures (mean age 12.2 years; F: 380) were accrued, nonadherence to the cleanout regimen (7.3%) and additional laxative use (3.1%) were observed in a small proportion of the prospective dataset subjects, adequate cleanout defined as thin or thick liquid but no solids present was reported in 79.5% and 15.8% of cases and impaired study from inadequate cleanout was reported in 11.8% of studies albeit the cecum was reached and the terminal ileum was intubated in 97.8 and 93.6% of studies. The duration of the study was significantly longer with the presence of a fellow trainee assisting in the procedure. Patient age and gender did not correlate with prep adequacy or cecal and ileal intubation rates, inadequate cleanout was significantly associated with impairment and incomplete studies. CONCLUSION: A one day, single agent, osmotic laxative (Polyethylene glycol) based cleanout regimen is effective in routine pre-procedure cleanout for standard colonoscopy in pediatric age range patients.

Pediatric Endoscopic Procedure Complications.

PURPOSE OF REVIEW: This review summarizes the current body of research, define high-risk patients and endoscopic processes, and outline evidence-based countermeasures aimed at minimizing the incidence of complications during endoscopy in children. RECENT FINDINGS: Significant complications of endoscopy requiring emergency department or inpatient admission in otherwise healthy children are unusual, but more common with therapeutic procedures; risk from procedures increases incrementally with preoperative coexisting conditions. Duodenal hematoma is predominantly a pediatric endoscopic complication and is more likely in hematology-oncology patients. Air embolism is a well-defined endoscopic retrograde cholangiopancreatography (ERCP) complication in adults and is likely to increase in children with increased performance of pediatric ERCP. Increased physician expertise is the most often proposed countermeasure, especially in the context of endoscopy complications in the higher-risk patient and procedure. Endoscopy in children remains a very safe group of procedures, although a more detailed understanding of risk factors and ideal training and practice organization is lacking.

Readmission after Gastrointestinal Bleeding in Children: A Retrospective Cohort Study.

INTRODUCTION: To compare the demographic, clinical, and therapeutic characteristics in a cohort of patients discharged following acute gastrointestinal bleeding, representing to the emergency department (ED) and readmitted within 30 days of discharge with the characteristics of non-readmitted patients. STUDY DESIGN: Hospitalization data was obtained from the Pediatric Hospital Information System including 49 tertiary children's hospitals in the US. Children 1-21 years of age diagnosed with acute gastrointestinal bleeding, admitted between January 2007 and September 2015 were included. The primary outcomes in this study were 30-day inpatient readmission through the ED and 30-day return to the ED only. Unadjusted, univariate followed by multivariable analysis of the associations between patient characteristics and treatment course at the index encounter using the R statistical package, v. 3.2.3. RESULTS: During the study period, 9902 patients were admitted with acute gastrointestinal bleeding; in the following month, 1460 (16.1%) represented to the ED and 932 (9%) were readmitted; 68.7% within 14 days from discharge. Readmission was most frequently associated with portal hypertension or esophageal variceal hemorrhage. There was a decreased likelihood of readmission with endoscopy (OR 0.77, 95% CI, 0.661, 0.906) and with Meckel scan (OR 0.513, 95% CI 0.362, 0.727) during the initial admission. Multiple comorbidities, longer initial stay and the early proton pump inhibitor therapy were associated with higher likelihood of readmission. DISCUSSION: Readmission following acute gastrointestinal bleeding is common and is more likely following variceal hemorrhage, long initial admission, and chronic comorbidities.

Genetic Counselor Practices Involving Pediatric Patients with FAP: an Investigation of their Self-Reported Strategies for Genetic Testing and Hepatoblastoma Screening.

Familial adenomatous polyposis (FAP) is a cancer predisposition syndrome that causes early-onset polyposis and is associated with an increased risk for hepatoblastoma. There is currently a lack of consensus on when to order APC (adenomatous polyposis coli) gene testing or implement surveillance for hepatoblastoma. An online questionnaire was completed by 62 genetic counselors to capture their current practices regarding these questions. Extracolonic findings associated with FAP that were most likely to prompt APC testing in an otherwise asymptomatic 10 year-old child with a negative family history were multiple desmoid tumors, congenital hypertrophy of the retinal pigment epithelium (CHRPE), jaw osteomas, and hepatoblastoma. For hepatoblastoma screening, the majority did recommend this in children less than age five years with known APC mutations. An interval of every 3-6 months was most commonly suggested; however, responses extended to screening on a less than annual basis. These results highlight the need for further investigation into why some genetic counselors do not recommend APC testing in young at-risk children and what factors influence views about the ideal age and indication for APC testing. Studies of these issues would help to define the best clinical practice model for genetic testing and hepatoblastoma screening in pediatric patients with FAP.

Present Standard in Pediatric Gastroenterology Fellowship Training in the Interpretation of Capsule Endoscopy.

Consensuses on fellowship training in wireless capsule endoscopy (WCE) interpretation have been published for adult gastroenterology (GI) but not in pediatric GI training. A questionnaire has been sent to 64 pediatric and 45 adult GI fellowship programs to compare their present training approach. Adult GI programs reported having a formal GI capsule endoscopy module in 38% and required to attend hands-on course in 27% as compared with 4% and 8% in pediatric programs, respectively. A more formalized approach to WCE training may be required for credentialing pediatric trainees to be aligned with expectations in adult GI programs.

Economic Assessment of Supercritical CO2 Extraction of Waxes as Part of a Maize Stover Biorefinery.

To date limited work has focused on assessing the economic viability of scCO2 extraction to obtain waxes as part of a biorefinery. This work estimates the economic costs for wax extraction from maize stover. The cost of manufacture (COM) for maize stover wax extraction was found to be € 88.89 per kg of wax, with the fixed capital investment (FCI) and utility costs (CUT) contributing significantly to the COM. However, this value is based solely on scCO2 extraction of waxes and does not take into account the downstream processing of the biomass following extraction. The cost of extracting wax from maize stover can be reduced by utilizing pelletized leaves and combusting the residual biomass to generate electricity. This would lead to an overall cost of € 10.87 per kg of wax (based on 27% combustion efficiency for electricity generation) and €4.56 per kg of wax (based on 43% combustion efficiency for electricity generation). A sensitivity analysis study showed that utility costs (cost of electricity) had the greatest effect on the COM.

Enhanced microwave assisted pyrolysis of waste rice straw through lipid extraction with supercritical carbon dioxide.

A combination of supercritical carbon dioxide (scCO2) extraction and microwave-assisted pyrolysis (MAP) have been investigated for the valorisation of waste rice straw. ScCO2 extraction of rice straw led to a 0.7% dry weight yield of lipophilic molecules, at elevated temperatures of 65 °C and pressures of 400 bar. Lipid compositions (fatty acids, fatty alcohol, fatty aldehydes, steroid ketones, phytosterols, n-alkanes and wax esters) of the waxes obtained by scCO2 were comparable to those obtained Soxhlet extraction using the potentially toxic solvent n-hexane. ScCO2 extraction positively influenced the pyrolysis heating rate, with a rate of 420 K min-1 for particles of 500-2000 µm, compared to 240 K min-1 for the same particle size of untreated straw. Particle size significantly affected cellulose decomposition and the distribution of pyrolysis products (gaseous, liquid and char), highlighting the importance of selecting an adequate physical pre-treatment. TG and DTG of the original rice straw and resulting biochar produced indicated that cellulose was completely decomposed during the MAP. While a rapid pressure change occurred at ∼120 °C (size > 2000 µm) and ∼130 °C (size 500-2000 µm) during MAP and was associated with the production of incondensable gas during cellulose decomposition, this takes place at significantly lower temperatures than those observed with conventional pyrolysis, 320 °C. Wax removal by scCO2 influences the dielectric properties of the straw, enhancing microwave absorption with rapid heating rates and elevated final pyrolysis temperatures, illustrating the benefits of combining these sustainable technologies within a holistic rice straw biorefinery.

Polyps and Polyposis Syndromes in Children: Novel Endoscopic Considerations.

Polypectomy is the most common therapeutic endoscopic intervention in children. Management of sporadic juvenile polyps is limited to polypectomy to resolve symptoms, whereas polyposis syndromes pose a multidisciplinary challenge with broader ramifications. In preparation for polypectomy, there are key patient, polyp, endoscopy unit, and provider characteristics that factor into the likelihood of success. Younger age and multiple medical comorbidities increase the risk of adverse outcomes, classified as intraoperative, immediate postoperative, and delayed postoperative complications. Novel techniques, including cold snare polypectomy, can significantly decrease adverse events but a more structured training process for polypectomy in pediatric gastroenterology is needed.

Don't Look Back and Spray! Risk of Endoscope Adhesion with Hemospray Application in Retroflexion in a Pediatric Patient.

Although the vast majority of recognized pediatric upper gastrointestinal bleeding (GIB) resolves spontaneously, gastrointestinal hemorrhage is the most common indication for urgent or emergent therapeutic endoscopy in pediatric practice. The application of hemostatic powders, including TC-325 (Hemospray, Cook Medical, Winston-Salem, NC, USA), has shown considerable impact on the control of acute bleeding, with the advantage of potentially covering an extensive area and requiring less technical expertise. We report a case of transient adherence of an esophagogastroduodenoscopy following Hemospray application in a 22-month-old with upper GIB. Our experience does not detract from the significant gains in the management of pediatric GIB from Hemospray; however, it does raise a cautionary note toward the application technique utilized.