Ineffective esophageal motility: Characterization and outcomes across pediatric neurogastroenterology and motility centers in the United States.

OBJECTIVES: Ineffective esophageal motility (IEM) on high-resolution manometry (HRM) is not consistently associated with specific clinical syndromes or outcomes. We evaluated the prevalence, clinical features, management, and outcomes of pediatric IEM patients across the United States. METHODS: Clinical and manometric characteristics of children undergoing esophageal HRM during 2021-2022 were collected from 12 pediatric motility centers. Clinical presentation, test results, management strategies, and outcomes were compared between children with IEM and normal HRM. RESULTS: Of 236 children (median age 15 years, 63.6% female, 79.2% Caucasian), 62 (23.6%) patients had IEM, and 174 (73.7%) patients had normal HRM, with similar demographics, medical history, clinical presentation, and median symptom duration. Reflux monitoring was performed more often for IEM patients (25.8% vs. 8.6%, p = 0.002), but other adjunctive testing was similar. Among 101 patients with follow-up, symptomatic cohorts declined in both groups in relation to the initial presentation (p > 0.107 for each comparison) with management targeting symptoms, particularly acid suppression. Though prokinetics were used more often and behavioral therapy less often in IEM (p ≤ 0.015 for each comparison), symptom outcomes were similar between IEM and normal HRM. Despite a higher proportion with residual dysphagia on follow-up in IEM (64.0% vs. 39.1%, p = 0.043), an alternate mechanism for dysphagia was identified more often in IEM (68.8%) compared to normal HRM (27.8%, p = 0.017). CONCLUSIONS: IEM is a descriptive manometric pattern rather than a clinical diagnosis requiring specific intervention in children. Management based on clinical presentation provides consistent symptom outcomes.

Persistent and newly developed gastrointestinal symptoms after surgery for intestinal malrotation in children: Dysmotility or disorders of gut and brain interaction?

OBJECTIVES: Surgery for intestinal malrotation (IM) aims to correct the defect and improve symptoms; however, many have persistent gastrointestinal (GI) symptoms postoperatively. We evaluated the incidence, clinical presentation, and long-term outcomes of children with surgically repaired IM and its possible association with disorders of gut and brain interaction (DGBI). METHODS: Multicenter retrospective study was conducted in patients from 0 to 21 years old, who had surgery for IM from 2000 to 2021 across three pediatric tertiary care centers. Data analyzed included demographics, time to diagnosis, idiopathic diagnosis, incidental diagnosis, postoperative follow-up, surgical time, and the need for surgery including bowel detorsion. Outcome variables were the presence of postoperative GI symptoms and DGBIs, and overall resolution of symptoms. We also evaluated the potential association of demographics and other included variables with our outcome variables. RESULTS: Ninety-two patients with surgically corrected IM were included, 54% were male, and median age of diagnosis and surgical correction was 4.9 and 7.8 months, respectively. Median follow-up after surgery was 64 months. A total of 77% had postoperative GI symptoms, and notably, 78% of patients without symptoms before surgery (incidental diagnosis) developed GI symptoms postoperatively and 27% of patients met Rome IV criteria for a one or more DGBI. No factors were associated to the presence of postoperative symptoms or DGBIs in multivariate analysis. Female gender was the only factor associated with lack of resolution of symptoms at follow-up. CONCLUSION: Pediatric IM is commonly associated with postoperative GI symptoms and DGBI well beyond surgery. An increased awareness about the prevalence of DGBI in these patients may help reach a prompt and accurate diagnosis, and improve their quality of life.

Vulvovaginal Complaints as the First Manifestation of Crohn's Disease in Children: Case Report.

BACKGROUND: Crohn's disease, a chronic gastrointestinal inflammatory condition, can involve gynecological structures. Rectovaginal or rectovestibular involvement may be the first sign in the pediatric population and may result in delayed diagnosis and treatment. CASE: A 9-year-old premenarchal female with chronic constipation and poor growth presented to the pediatric gynecologist for evaluation of persistent vulvovaginal discharge and vulvar irritation. Examination under anesthesia revealed a rectolabial fistula; colonoscopy was diagnostic of Crohn's disease. Immunotherapy resulted in improvement of symptoms and anatomical changes. SUMMARY AND CONCLUSION: In cases of persistent vulvar complaints in a child without clear diagnosis, a high index of suspicion for a non-gynecologic diagnosis is necessary. Collaboration between pediatric gynecologists, gastroenterologists, and surgeons can result in prompt diagnosis and treatment of genital Crohn's disease.

Significant disparities in risks of diabetes mellitus and metabolic syndrome among chronic hepatitis C virus patients in the U.S.

AIM: Diabetes mellitus (DM) and metabolic syndrome (MetSyn) among chronic hepatitis C virus (HCV) patients increases risks for nonalcoholic fatty liver disease (NAFLD) and more rapid progression to hepatocellular carcinoma (HCC). We aim to evaluate the prevalence of DM and MetSyn among HCV patients, focusing on age-specific and race/ethnicity-specific disparities. METHODS: We retrospectively evaluated 2003-2012 National Health and Nutrition Examination Survey (NHANES) data for age and race disparities in concurrent DM and MetSyn among HCV patients. Multivariate logistic regression models evaluated for independent predictors of DM and MetSyn among HCV patients. RESULTS: Overall U.S. prevalence of HCV was 1.29% from 2003 to 2012 and prevalence of DM and MetSyn among HCV patients was 17.5% and 35.0%, respectively. Higher rates of DM (36.9% vs. 3.3%, p<0.001) and MetSyn (50.5% vs. 11.7%, p<0.001) were seen among HCV patients ≥60 compared to <40years. The highest rates of DM and MetSyn were seen among African Americans (AA) (DM: 39.1%, MetSyn: 29.2%) and the lowest in non-Hispanic whites (DM: 9.4%, MetSyn: 33.0%). On multivariate regression, patients ≥60 were significantly more likely to have DM compared to patients <40 years (OR 11.90, 95% CI 2.73-52.60, p=0.001); AA were significantly more likely to have DM compared to non-Hispanic whites (OR 2.82, 95% CI 1.53-5.20, p=0.001). CONCLUSION: Among chronic HCV patients, the highest risk of DM and MetSyn was seen among older patients, African Americans, and women. These groups are at higher risk of cirrhosis and HCC due to concurrent NAFLD.