Genetic and acquired sucrase-isomaltase deficiency: A clinical review.

Genetic sucrase-isomaltase deficiency (GSID) is an inherited deficiency in the ability to digest sucrose and potentially starch due to mutations in the sucrase-isomaltase (SI) gene. Congenital sucrase-isomaltase deficiency is historically considered to be a rare condition affecting infants with chronic diarrhea as exposure to dietary sucrose begins. Growing evidence suggests that individuals with SI variants may present later in life, with symptoms overlapping with those of irritable bowel syndrome. The presence of SI genetic variants may, either alone or in combination, affect enzyme activity and lead to symptoms of different severity. As such, a more appropriate term for this inherited condition is GSID, with a recognition of a spectrum of severity and onset of presentation. Currently, disaccharidase assay on duodenal mucosal tissue homogenates is the gold standard in diagnosing SI deficiency. A deficiency in the SI enzyme can be present at birth (genetic) or acquired later, often in association with damage to the enteric brush-border membrane. Other noninvasive diagnostic alternatives such as sucrose breath tests may be useful but require further validation. Management of GSID is based on sucrose and potentially starch restriction tailored to the individual patients' tolerance and symptoms. As this approach may be challenging, additional treatment with commercially available sacrosidase is available. However, some patients may require continued starch restriction. Further research is needed to clarify the true prevalence of SI deficiency, the pathobiology of single SI heterozygous mutations, and to define optimal diagnostic and treatment algorithms in the pediatric population.

Relationships among intragastric meal distribution during gastric emptying scintigraphy, water consumption during water load satiety testing, and symptoms of gastroparesis.

Gastric emptying scintigraphy (GES) measures total gastric retention after a solid meal and can assess intragastric meal distribution (IMD). Water load satiety test (WLST) measures gastric capacity. Both IMD immediately after meal ingestion [ratio of proximal gastric counts after meal ingestion to total gastric counts at time 0 (IMD0)] and WLST (volume of water ingested over 5 min) are indirect measures of gastric accommodation. In this study, IMD0 and WLST were compared with each other and to symptoms of gastroparesis to gauge their clinical utility for assessing patients with symptoms of gastroparesis. Patients with symptoms of gastroparesis underwent GES to obtain gastric retention and IMD0, WLST, and filled out patient assessment of upper GI symptoms. A total of 234 patients with symptoms of gastroparesis were assessed (86 patients with diabetes, 130 idiopathic, 18 postfundoplication) and 175 (75%) delayed gastric emptying. Low IMD0 <0.568 suggesting initial rapid transit to the distal stomach was present in 8% and correlated with lower gastric retention, less heartburn, and lower volumes consumed during WLST. Low WLST volume (<238 mL) was present in 20% and associated with increased severity of early satiety, postprandial fullness, loss of appetite, and nausea. Low IMD0 is associated with less gastric retention and less heartburn. Volume of water consumed during WLST, while associated with IMD0, has associations with early satiety, postprandial fullness, loss of appetite, and nausea. Thus, IMD0 and WLST appear to overlap somewhat in their assessment of gastric physiology in adults with symptoms of gastroparesis but relate to different dyspeptic symptoms.NEW & NOTEWORTHY IMD0 and WLST were assessed for their clinical utility in assessing patients with symptoms of gastroparesis. Low IMD0 is associated with less gastric retention and less heartburn. Volume of water consumed during WLST, while associated with IMD0, has associations with early satiety, postprandial fullness, loss of appetite, and nausea. IMD0 and WLST appear to overlap somewhat in their assessment of gastric physiology in adults with symptoms of gastroparesis but relate to different dyspeptic symptoms.

Functional response to a microbial synbiotic in the gastrointestinal system of children: a randomized clinical trial.

BACKGROUND: Oral microbial therapy has been studied as an intervention for a range of gastrointestinal disorders. Though research suggests that microbial exposure may affect the gastrointestinal system, motility, and host immunity in a pediatric population, data have been inconsistent, with most prior studies being in neither a randomized nor placebo-controlled setting. The aim of this randomized, placebo-controlled study was to evaluate the efficacy of a synbiotic on increasing weekly bowel movements (WBMs) in constipated children. METHODS: Sixty-four children (3-17 years of age) were randomized to receive a synbiotic (n = 33) comprising mixed-chain length oligosaccharides and nine microbial strains, or placebo (n = 31) for 84 days. Stool microbiota was analyzed on samples collected at baseline and completion. The primary outcome was a change from baseline of WBMs in the treatment group compared to placebo. RESULTS: Treatment increased (p < 0.05) the number of WBMs in children with low baseline WBMs, despite broadly distinctive baseline microbiome signatures. Sequencing revealed that low baseline microbial richness in the treatment group significantly anticipated improvements in constipation (p = 0.00074). CONCLUSIONS: These findings suggest the potential for (i) multi-species-synbiotic interventions to improve digestive health in a pediatric population and (ii) bioinformatics-based methods to predict response to microbial interventions in children. IMPACT: Synbiotic microbial treatment improved the number of spontaneous weekly bowel movements in children compared to placebo. Intervention induced an increased abundance of bifidobacteria in children, compared to placebo. All administered probiotic species were enriched in the gut microbiome of the intervention group compared to placebo. Baseline microbial richness demonstrated potential as a predictive biomarker for response to intervention.

Many pediatric patients with gastroparesis do not receive dietary education.

BACKGROUND: Gastroparesis is delayed gastric emptying in the absence of obstruction; dietary modifications are first-line treatment. However, we do not know the factors related to provision of dietary recommendations. METHODS: We sought to determine how often pediatric patients with gastroparesis receive dietary education (from a gastroenterology provider vs dietitian), the recommendations given, and factors related to these outcomes. We performed a retrospective chart review of children 2- to 18-years-old managed by pediatric gastroenterology providers at our institution. Patient demographics and clinical data, dietary advice given (if any), and dietitian consultation (if any), practice location, and prokinetic use were captured. An adjusted binomial regression model identified factors associated with dietary education provision, dietitian consultation, and diet(s) recommended. RESULTS: Of 161 patients who met criteria, 98 (60.8%) received dietary education and 42 (26.1%) met with a dietitian. The most common recommendation by gastroenterology providers and dietitians was diet composition adjustment (26.5% and 47.6%, respectively). Patients with nausea/vomiting were less likely to receive dietary education or be recommended to adjust diet composition. Patients with weight loss/failure to thrive were more likely to receive dietitian support. Patients seen in the community vs medical center outpatient setting were more likely to be recommended a low-fat diet. CONCLUSIONS: Only a little over half of children with gastroparesis receive dietary education and use of a dietitian's expertise is much less frequent. Symptoms and clinical setting appear related to what, where, and by whom guidance is provided.

Diagnostic Utility of Mucosal Biopsies Taken During Colonoscopy-Guided Colonic Manometry Catheter Placement.

OBJECTIVES: The diagnostic utility of mucosal biopsies taken during colonoscopy-guided colonic manometry catheter placement is unknown. The aims of our study were to determine the frequency and histopathology results of mucosal biopsies during these procedures and to assess whether there were any associations between the histology or gross findings with manometry results. METHODS: We performed a retrospective chart review of children who had a colonic manometry study completed between 2008 and 2020 at a quaternary children's hospital. We captured patient demographics, biopsy locations, histopathology results, gross endoscopy findings, and manometry results. The chi-squared test and when appropriate Fisher exact test was used to evaluate categorical associations. RESULTS: One hundred forty-eight patients were included. One hundred eighteen (80%) had colonic biopsy and 63 (43%) had ileal biopsy. Colonic histology findings, which patients could have multiple, included lymphonodular hyperplasia (34%), normal (27%), chronic inflammation (24%), melanosis coli (21%), colonic eosinophilia (10%), and acute inflammation (8%). Ileal histology findings included increased Peyer patches (44%), normal (44%), acute inflammation (11%), chronic inflammation (3%), eosinophilia (5%), and eosinophilic ileitis (3%). The majority of acute and chronic inflammation was graded as mild. There were no statistically significant associations of histology to gross endoscopy or manometry findings. CONCLUSIONS: Colonic biopsies are obtained in the majority of patients presenting for colonic manometry evaluation with ileal biopsies obtained less frequently. Histopathology findings are noted frequently, but the majority are the result of or did not impact clinical care. There were no associations between abnormal histopathology or abnormal gross endoscopy findings with colonic manometry results.

Gastrointestinal Symptoms Profile in Gastroparesis Compared to Other Functional and Organic Gastrointestinal Diseases.

OBJECTIVES: The primary objective was to compare the patient-reported gastrointestinal symptoms profiles of pediatric patients with gastroparesis to pediatric patients with 1 of 7 other functional gastrointestinal disorders and organic gastrointestinal diseases using the Pediatric Quality of Life Inventory (PedsQL) Gastrointestinal Symptoms Scales. METHODS: The gastrointestinal symptoms profiles of 64 pediatric patients with gastroparesis who manifested abnormal gastric retention based on gastric emptying scintigraphy testing were compared to 582 pediatric patients with 1 of 7 physician-diagnosed gastrointestinal disorders (functional abdominal pain, irritable bowel syndrome, functional dyspepsia, gastroesophageal reflux disease, functional constipation, Crohn disease, ulcerative colitis). The PedsQL Gastrointestinal Symptoms Scales encompass 10 individual multi-item scales which measure stomach pain, stomach discomfort when eating, food and drink limits, trouble swallowing, heartburn and reflux, nausea and vomiting, gas and bloating, constipation, blood in poop, and diarrhea/fecal incontinence, with an overall total gastrointestinal symptoms score. RESULTS: The gastrointestinal symptoms profile analysis identified significantly worse overall total gastrointestinal symptoms scores between pediatric patients with gastroparesis compared to all other gastrointestinal groups except for irritable bowel syndrome (most P s < 0.001), with significant differences for stomach discomfort when eating compared to all 7 other gastrointestinal groups (most P s < 0.001). Nausea and vomiting were significantly worse for gastroparesis compared to all other gastrointestinal groups except for functional dyspepsia (all P s < 0.001). CONCLUSIONS: Pediatric patients with gastroparesis self-reported significantly worse overall total gastrointestinal symptoms compared to all other gastrointestinal diagnostic groups except for irritable bowel syndrome, with stomach discomfort when eating and nausea and vomiting symptoms exhibiting the greatest differences compared to most gastrointestinal diagnostic groups.

Progress in Gastroparesis - A Narrative Review of the Work of the Gastroparesis Clinical Research Consortium.

The Gastroparesis Clinical Research Consortium is a multicenter coalition created and funded by the National Institutes of Diabetes and Digestive and Kidney Disorders, with a mission to advance understanding of the pathophysiology of gastroparesis and develop an effective treatment for patients with symptomatic gastroparesis. In this review, we summarize the results of the published Gastroparesis Clinical Research Consortium studies as a ready and convenient resource for gastroenterologists and others to provide a clear understanding of the consortium's experience and perspective on gastroparesis and related disorders.

Pediatric Aspects of Nutrition Interventions for Disorders of Gut-Brain Interaction.

Dietary factors may play an important role in the generation of symptoms in children with disorders of gut-brain interaction (DGBIs). Although dietary modification may provide successful treatment, there is a relative paucity of controlled trials that have shown the effectiveness of dietary interventions. This study is a narrative review that explores the existing literature on food and pediatric DGBIs. The following have been shown to be beneficial: (i) in infants with colic, removing cow's milk from the infant's diet or from the maternal diet in those who are breastfed; (ii) in infants with regurgitation, adding thickeners to the formula or removing cow's milk protein from the infant's diet or the maternal diet in those who are breastfed; and (iii) in children with pain-predominant DGBIs, using soluble fiber supplementation or a low fermentable oligosaccharides, disaccharides, monosaccharides, and polyols diet. In children with functional constipation, there is no evidence that adding fiber is beneficial. Given that most dietary interventions include restriction of different foods in children, a thoughtful approach and close follow-up are needed.

Randomised trial: Peppermint oil (menthol) pharmacokinetics in children and effects on gut motility in children with functional abdominal pain.

AIMS: Little is known regarding the pharmacokinetics and pharmacodynamics of menthol, the active ingredient in peppermint oil (PMO). Our aim was to investigate the pharmacokinetics of menthol at 3 dose levels in children and determine their effects on gut motility and transit. METHODS: Thirty children ages 7-12 years with functional abdominal pain underwent wireless motility capsule (WMC) testing. Approximately 1 week later they were randomized to 180, 360 or 540 mg of enteric coated PMO (10 participants per dose). Menthol pharmacokinetics were determined via blood sampling over 24 hours. They then took their respective dose of PMO (180 mg once, 180 mg twice or 180 mg thrice daily) for 1 week during which time the WMC test was repeated. RESULTS: Evaluable area under the plasma concentration vs. time curve (AUClast ) data were available in 29 of 30 participants. A direct linear relationship (apparent dose-proportionality for systemic menthol exposure) was observed between PMO dose and menthol systemic exposure with mean elimination half-life 2.1, 3.5 and 4.6 hours for the 180, 360 and 540 mg doses, respectively. WMC technical issues precluded complete motility data in all participants. Colonic transit time was inversely related to AUClast (P = .003); transit time in other regions was not affected. In contrast, stomach, small bowel and whole gut (but not colonic) contractility positively correlated with menthol AUClast (P < .05). CONCLUSION: Pharmacokinetics and pharmacodynamics of menthol derived from PMO demonstrated apparent dose-proportionality. A higher dose of PMO may be needed to achieve maximal gut response. www.clinicaltrials.gov NCT03295747.

Gastrointestinal Symptoms Profile in Pediatric Patients With Gastroparesis Compared to Healthy Controls.

OBJECTIVES: The primary objective was to compare the patient-reported gastrointestinal symptoms profiles of pediatric patients with gastroparesis to matched healthy controls using the Pediatric Quality of Life Inventory™ (PedsQL™) Gastrointestinal Symptoms Scales. The secondary objectives were to compare pediatric patients with gastroparesis to pediatric patients with gastroparesis-like symptoms and normal gastric emptying and to compare pediatric patients with gastroparesis-like symptoms and normal gastric emptying to matched healthy controls. METHODS: The PedsQL™ Gastrointestinal Symptoms Scales were completed by 64 pediatric patients with gastroparesis, 59 pediatric patients with gastroparesis-like symptoms and normal gastric emptying, and 200 age, gender, and race/ethnicity matched healthy controls. The PedsQL™ Gastrointestinal Symptoms Scales encompass 10 individual multi-item scales which measure stomach pain, stomach discomfort when eating, food and drink limits, trouble swallowing, heartburn and reflux, nausea and vomiting, gas and bloating, constipation, blood in poop, and diarrhea/fecal incontinence. Based on gastric emptying scintigraphy testing, those with abnormal gastric retention were classified as having gastroparesis. RESULTS: The gastrointestinal symptoms profile analysis identified large differences between those with gastroparesis compared to healthy controls (most P <0.001), with the largest effect sizes for upper gastrointestinal symptoms including stomach pain, stomach discomfort when eating, food and drink limits, nausea, and vomiting. Those with gastroparesis self-reported similar gastrointestinal symptoms to those with normal gastric emptying, except for increased constipation. CONCLUSIONS: Pediatric patients with gastroparesis self-reported broad multidimensional gastrointestinal symptoms profiles in comparison to healthy controls with large differences, indicating the critical need for more highly efficacious interventions to bring patient functioning within the normal range of healthy functioning.

Multidisciplinary Care for Refractory Pediatric Functional Abdominal Pain Decreases Emergency and Inpatient Utilization.

ABSTRACT: Children with refractory functional abdominal pain (FAP) experience functional disability and may utilize emergency department (ED) and/or inpatient services. Whether multidisciplinary programs which help care for children with refractory FAP affect acute healthcare utilization is unknown. A retrospective chart review of children initially evaluated by the outpatient Multidisciplinary Abdominal Pain Program (MAPP) from October 2016 to May 2019 was completed. Patient characteristics and number of ED visits and hospitalizations for abdominal pain in the year prior to versus year after MAPP evaluation were captured. Thirty-eight children (ages 9-17 years [median 13 years]) were included. The median number of ED visits/patient/year decreased from 1 (range: 0-7) to 0 (range: 0-3) (P < 0.0001). Seven (18%) children had been hospitalized and, in these children, the median number of hospitalizations/patient/year decreased from 1 (range: 1-5) to 0 (range: 0-1) (P < 0.05). These data suggest multidisciplinary outpatient intervention for refractory FAP is associated with significant decreases in acute healthcare utilization.

Meal-Induced Symptoms in Children with Dyspepsia-Relationships to Sex and the Presence of Gastroparesis.

OBJECTIVES: To test the hypothesis that in children with dyspepsia, prospective symptom severity following ingestion of a meal would correlate with percent gastric retention, and those ultimately diagnosed with gastroparesis would report worse symptoms. STUDY DESIGN: Prospective, single-center study with 104 children with dyspepsia completing a prospective dyspepsia symptom questionnaire before and after eating a standardized Tougas meal during gastric emptying scintigraphy. Main outcomes included correlation between gastric retention and symptoms and comparison of symptom severity between those with and without gastroparesis. RESULTS: Fifty-two children (50%) had gastroparesis (gastroparesis: 12.5 ± 2.9 years, 65% female; nongastroparesis: 13.0 ± 2.9 years, 60% female; all P > .05). Bloating was the only symptom significantly worse in youth with gastroparesis. For the entire cohort, bloating and fullness correlated with percent retention. However, in those with gastroparesis, only nausea correlated with retention (4 hours.; rs = 0.275, P < .05). Girls with gastroparesis had significantly worse symptoms (except satiety) when compared with boys with gastroparesis (P < .05). CONCLUSIONS: Overall in children, there is little difference in symptom severity between children with gastroparesis vs normal emptying based on current standards. However, girls with gastroparesis have worse symptoms vs boys with gastroparesis, underscoring a need for further studies into the role of sex in gastroparesis symptoms. In all children, both bloating and fullness correlated modestly with gastric retention, and nausea correlated in those with gastroparesis.

Multisite Pain Is Highly Prevalent in Children with Functional Abdominal Pain Disorders and Is Associated with Increased Morbidity.

OBJECTIVES: To characterize the types of multisite pain experienced by children with functional abdominal pain disorders (FAPDs) and to examine differences in psychosocial distress, functional disability, and health-related quality of life in children with multisite pain vs abdominal pain alone. STUDY DESIGN: Cross-sectional study of children ages 7-17 years (n = 406) with pediatric Rome III FAPDs recruited from both primary and tertiary care between January 2009 and June 2018. Subjects completed 14-day pain and stool diaries, as well as validated questionnaires assessing abdominal and nonabdominal pain symptoms, anxiety, depression, functional disability, and health-related quality of life. RESULTS: In total, 295 (73%) children endorsed at least 1 co-occurring nonabdominal pain, thus, were categorized as having multisite pain with the following symptoms: 172 (42%) headaches, 143 (35%) chest pain, 134 (33%) muscle soreness, 110 (27%) back pain, 94 (23%) joint pain, and 87 (21%) extremity (arms and legs) pain. In addition, 200 children (49%) endorsed 2 or more nonabdominal pain symptoms. Participants with (vs without) multisite pain had significantly higher abdominal pain frequency (P < .001) and severity (P = .03), anxiety (P < .001), and depression (P < .001). Similarly, children with multisite pain (vs without) had significantly worse functional disability (P < .001) and health-related quality of life scores (P < .001). Increasing number of multisite pain sites (P < .001) was associated with increased functional disability when controlling for demographic and other clinical factors. CONCLUSIONS: In children with FAPDs, nonabdominal multisite pain is highly prevalent and is associated with increased psychosocial distress, abdominal pain frequency and severity, functional disability, and lower health-related quality of life.

Development and Assessment of a Pictographic Pediatric Constipation Action Plan.

OBJECTIVE: To assess the Uniformed Services Constipation Action Plan (USCAP) as an evidence-based, personalized, clinical action tool with pictograms to aid clinicians and families in the management of functional constipation. STUDY DESIGN: The USCAP facilitates the management functional constipation by using a health literacy-informed approach to provide instructions for pharmacotherapies and lifestyle modifications. This study included part 1 (pictogram validation) and part 2 (assessment). For part 1, pictogram transparency, translucency, and recall were assessed by parent survey (transparency ≥85%, mean translucency score ≥5, recall ≥85% required for validation). For part 2, the USCAP was assessed by parents, clinical librarians, and clinicians. Parental perceptions (n = 65) were assessed using the Consumer Information Rating Form (17 questions) to gauge comprehensibility, design quality and usefulness. Readability was assessed by 5 formulas and a Readability Composite Score was calculated. Clinical librarians (n = 3) used the Patient Education Materials Assessment Tool to measure understandability (19 questions) and actionability (7 questions) (>80% rating was acceptable). Suitability was assessed by clinicians (n = 34) using Doak's Suitability Assessment of Materials (superior ≥70% rating). RESULTS: All 12 pictograms demonstrated appropriate transparency, translucency, and recall. Parental perceptions reflected appropriate comprehensibility, design quality, and usefulness. The Readability Composite Score was consistent with a fifth-grade level. Clinical librarians reported acceptable understandability and actionability. Clinicians reported superior suitability. CONCLUSIONS: The USCAP met all criteria for clinical implementation and future study of USCAP implementation for treating children with chronic functional constipation.

Clinical Characterization of Pediatric Gastroparesis Using a Four-hour Gastric Emptying Scintigraphy Standard.

OBJECTIVES: Four-hour gastric emptying scintigraphy (GES) is the recommended method to identify both adult and childhood gastroparesis (GP). Previous pediatric studies have, however, not used this standard. We sought to determine the characteristics and outcomes of children versus adolescents with GP using the 4-hour GES evaluation. METHODS: We performed a retrospective chart review of pediatric patients diagnosed with GP by 4-hour GES (>10% retention at 4 hours). Demographics, body mass index, GP-related symptoms, comorbidities, etiologies, therapies (eg, medications), healthcare utilization, and response to therapy were captured systematically. Symptoms were compared from the initial versus last gastroenterology visit. Outcomes were categorized as no improvement; improvement (resolution of at least 1 symptom while remaining on therapy); and complete resolution of symptoms. RESULTS: A total of 239 subjects (12.1 ±â€Š4.1 years [mean ±â€Šstandard deviation], 70% girls) were included. The identified characteristics of childhood GP were broad with idiopathic GP being the most common etiology. Outcomes over a median of 22 months (25%-75%: 9.0-45.5 months) were 34.8% no improvement, 34.8% some improvement, and 30.3% with complete symptom resolution. Compared to younger children, adolescents had a higher female predominance (P < 0.01) and were more likely to have nausea (P = 0.006). Girls were more likely to have abdominal pain (P = 0.001), nausea (P = 0.03), and a documented diagnosis of dysautonomia (P = 0.03). Boys were more likely to have regurgitation (P = 0.006), gastroesophageal reflux disease (P = 0.02), and rumination (P = 0.02). CONCLUSIONS: Using the 4-hour GES standard, childhood GP has broad clinical characteristics and outcomes. There are several significant age- and sex-based differences in childhood GP.

PedsQL™ Gastroparesis Symptoms Module Domain and Item Development: Qualitative Methods.

OBJECTIVES: Like adults, children suffer from gastroparesis; however, there are currently no validated instruments to determine the impact of gastroparesis in pediatric patients. The objective of this study was to develop the items and domains to support the content validity of the new Pediatric Quality of Life Inventory (PedsQL™) Gastroparesis Symptoms Module. METHODS: Patients were recruited from the National Institute of Diabetes and Digestive and Kidney Diseases Pediatric Gastroparesis Registry. The qualitative methods involved an iterative process comprising a literature review of existing questionnaires and gastroparesis clinical research, an expert review panel of pediatric gastroenterologists who provided feedback on the conceptual framework in developing the semi-structured interview, and in-depth focus interviews with six pediatric patients with gastroparesis and five of their parents (one did not participate) in developing relevant domains and item content. In the subsequent cognitive interviews phase, five additional patients with gastroparesis and their parents provided detailed feedback on item content, relevance, importance, and understandability of the domains and items. RESULTS: Ten domains/scales were derived from the qualitative methods, with item content saturation achieved at 67 items, with no further themes or content identified during the final cognitive interviews. The Module is comprised of 10 individual scales measuring nausea, stomach fullness when eating, vomiting, dry heaves, heartburn and reflux, stomach pain and hurt, food and drink limits, bloating, appetite, and worry. CONCLUSIONS: Our study supports the content validity of the new PedsQL Gastroparesis Symptoms Module. The Module field test study will be conducted in a multisite national study.

The gut microbiome as a predictor of low fermentable oligosaccharides disaccharides monosaccharides and polyols diet efficacy in functional bowel disorders.

PURPOSE OF REVIEW: Fermentable oligosaccharides disaccharides monosaccharides and polyols (FODMAP) dietary restriction ameliorates irritable bowel syndrome (IBS) symptoms; however, not all individuals with IBS respond. Given the gut microbiome's role in carbohydrate fermentation, investigators have evaluated whether the gut microbiome may predict low FODMAP diet efficacy. RECENT FINDINGS: Gut microbiome fermentation, even to the same carbohydrate, is not uniform across all individuals with several factors (e.g. composition) playing a role. In both children and adults with IBS, studies are emerging suggesting the gut microbiome may predict low FODMAP diet efficacy. However, there is significant heterogeneity in the approaches (study population, microbiome assessment methods, statistical techniques, etc.) used amongst these studies. SUMMARY: The gut microbiome holds promise as a predictor of low FODMAP diet efficacy. However, further investigation using standardized approaches to evaluate the microbiome while concomitantly assessing other potential predictors are needed to more rigorously evaluate this area.

Routine disaccharidase testing: are we there yet?

PURPOSE OF REVIEW: Disaccharidase testing, as applied to the evaluation of gastrointestinal disturbances is available but it is not routinely considered in the diagnostic work-up. The purpose of this review was to determine if disaccharidase testing is clinically useful and to consider how the results could alter patient management. RECENT FINDINGS: Indicate that carbohydrate maldigestion could contribute functional bowel disorders and negatively impact the fecal microbiome. Diagnostic techniques include enzyme activity assays performed on random endoscopically obtained small intestinal biopsies, immunohistochemistry, stable isotope tracer and nonenriched substrate load breath testing, and genetic testing for mutations. More than 40 sucrase--isomaltase gene variants coding for defective or reduced enzymatic activity have been reported and deficiency conditions are more common than previously thought. SUMMARY: The rationale for disaccharidase activity testing relates to a need to fully assess unexplained recurrent abdominal discomfort and associated symptoms. All disaccharidases share the same basic mechanism of mucosal expression and deficiency has far reaching consequences. Testing for disaccharidase expression appears to have an important role in symptom evaluation, but there are accuracy and logistical issues that should be considered. It is likely that specific recommendations for patient management, dietary modification, and enzyme supplementation would come from better testing methods.

Literature Review: Dietary Intervention Adherence and Adherence Barriers in Functional Gastrointestinal Disorder Studies.

Dietary management is key in controlling functional gastrointestinal disorders (FGIDs) symptoms. However, the degree to which patients with FGIDs in clinical studies adhere to dietary recommendations is unknown. Our aim was to evaluate adherence, dietary education, and adherence-related factors in FGIDs dietary intervention trials. A literature search was performed using MEDLINE, Google Scholar, and SCOPUS for clinical trials evaluating dietary management of FGIDs. Full manuscripts published from January 2000 through August 2018 were reviewed. Data including definitions of adherence and rates, study design, dietary education, use of a dietitian, provision of study foods, potential adherence barriers, and dropouts were captured. A total of 21 publications were included. Fifteen (67%) focused on the low fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAP) diet, 5 (24%) on fiber supplementation, and 1 (9%) on a low carbohydrate diet. Fifteen (71%) were randomized controlled trials. Meals/supplements were provided to the study participants in 27%, 80%, and 100%, of the low FODMAP, fiber, and low carbohydrate studies, respectively, which appeared to significantly enhance adherence (P<0.03). Eighteen studies (81%) provided either written or oral diet information to study participants. Overall, only 10 publications (48%) provided adherence data. Nine (60%) of the low FODMAP studies provided adherence data (range, 30% to 100%); ranges could not be calculated for the other dietary trials. The most common method to measure adherence was via food diaries. Therefore, adherence is not often measured in FGIDs dietary intervention clinical trials; when reported, rates of adherence range widely. Studies providing food had the highest adherence rates.

Proceedings of the 2018 Advances In Motility and In NeuroGastroenterology: AIMING for the Future Single Topic Symposium.

OBJECTIVES: Motility and functional disorders are common in children and often debilitating, yet these disorders remain challenging to treat effectively. At the 2018 Annual North American Society for Pediatric Gastroenterology, Hepatology and Nutrition meeting, the Neurogastroenterology and Motility Committee held a full day symposium entitled, 2018 Advances In Motility and In NeuroGastroenterology - AIMING for the future. The symposium aimed to explore clinical paradigms in pediatric gastrointestinal motility disorders and provided a foundation for advancing new scientific and therapeutic research strategies. METHODS: The symposium brought together leading experts throughout North America to review the state of the art in the diagnosis and management of motility and functional disorders in children. Presentations were divided into esophageal, antral duodenal, and colorectal modules. Each module included oral presentations by experts in the respective fields, leading to thought-provoking discussions. There were 2 breakout sessions with small group discussions on select topics, focusing on defining scientific insights into the diagnosis and management of pediatric functional gastrointestinal and motility disorders in a systematic, segment-based approach. CONCLUSIONS: The field of neurogastroenterology has made remarkable progress in the last decade. The current report summarizes the major learning points from the symposium highlighting the diagnosis and promising therapies on the horizon for pediatric neurogastrointestinal and motility disorders.