Journal of Pediatric Gastroenterology and Nutrition最新文献

Pediatric cholestatic liver diseases are rare conditions that can result from multiple specific underlying etiologies. Among the most common etiologies of pediatric cholestatic liver diseases are biliary atresia, Alagille syndrome (ALGS), and inherited disorders of bile acid transport. These diseases are characterized by episodic or chronic unremitting cholestasis. Due to the chronicity of these conditions, it is imperative to optimize medical management to improve patient quality of life, provide nutritional support, and reduce bile acid toxicity in efforts to slow disease progression. Cholestatic liver diseases remain the leading cause of pediatric liver transplantation, as many underlying disease etiologies have no curative medical therapies. In the present review, we provide an update on the nutritional, medical, and surgical management of pediatric cholestatic liver diseases. As recent advances have occurred in the field with the addition of ileal bile acid transporter (IBAT) inhibitors, we also review the results from prospective clinical trials, including their strengths and limitations. While recent clinical trials have demonstrated improved pruritus using IBAT inhibitors in ALGS and progressive familial intrahepatic cholestasis, establishing medical therapies proven to slow disease progression remains an area of unmet need.

Objectives: Inflammatory bowel disease (IBD) results from genetic susceptibility, gut microbiome, and environmental factors. Diet, one modifiable environmental factor, has been linked to the increased prevalence of IBD. This study aimed to evaluate a potential association between food deserts and disease severity at diagnosis.

Methods: This retrospective study included newly diagnosed IBD patients (ages of 2 and 21 years of age; diagnosed between January 1, 2019, and December 31, 2021). The United States Department of Agriculture (USDA's) Food Access Research Atlas was used to determine if patients resided in a food desert. The Modified Retail Food Environment Index (mRFEI) determined the ratio of healthy to unhealthy food options. The primary endpoint was disease severity at diagnosis based on endoscopy scores. Statistical analyses were applied as appropriate.

Results: Ninety-eight patients were enrolled (75 [77%] Crohn' disease; 23 [23%] ulcerative colitis), 59 (60%) identified as Non-Hispanic White. Fifteen (15%) patients lived in a food desert. Food deserts consisted of more Black patients than White (67%; p = 0.05), more public insurance (12; 80%), and lower median vitamin D (17.6 [interquartile range (IQR): 10.8-24.]). In an adjusted (sex, age, insurance, race) multivariable model mRFEI was associated with reduced odds of a living in a food desert (0.91 [95% confidence interval (CI): 0.83-0.98]). There was no difference between the severity of disease and living in a food desert or food swamp.

Conclusions: Fifteen IBD patients lived in a food desert. Food deserts have less access to healthy food retailers and higher rates of unhealthy food retailers. Further work is needed to better understand spatial disparities related to food accessibility and IBD.

Objectives: Fecal calprotectin (FC) is a marker commonly used in the diagnosis and follow-up of inflammatory bowel diseases (IBD). However, other gastrointestinal conditions, like H. pylori (HP) infection, can result in increased neutrophil activity as well. We set out to assess the impact of HP infection on FC and downstream gastrointestinal care via a retrospective study.

Methods: In this study, we collected data from two institutions in Brooklyn, NY, in a high immigrant density community. We reviewed data from patients who underwent esophagogastroduodenoscopy (EGD) between January 2017 and October 2022. Patients aged 6-18 years old with an FC level 6 months prior to EGD and HP testing were included.

Results: Of 129 patients, 37 (28.7%) tested positive for HP infection. The mean FC level was significantly elevated in HP-positive patients (241.2, confidence interval [CI]: 161.0-321.3) as compared with HP-negative patients (88.1, CI: 59.1-117.0) (p < 0.001). Patients with higher FC levels were also more likely to undergo colonoscopies (p = 0.003).

Discussion: HP infection is associated with increased calprotectin, and calprotectin increases in HP patients are associated with an increased risk of colonoscopy.

Objectives: Supplemental zinc during acute diarrhea reduces illness duration but also increases vomiting. In a recent trial, we found that children receiving lower daily doses of zinc (5 mg or 10 mg vs. 20 mg) had lower rates of vomiting with comparable stool output and duration of diarrhea. We performed a secondary analysis to identify sociodemographic and clinical factors associated with vomiting in children with acute diarrhea.

Methods: We performed a secondary data analysis of 4500 children aged 6-59 months with an acute episode of diarrhea (<72 h before enrollment) in a randomized, double-blind controlled trial in India and Tanzania. To identify clinically important risk factors for overall, regimen-related, and regimen-unrelated vomiting, we created log-binomial models with relative risks (RRs) and 95% confidence intervals (CIs).

Results: The trial enrolled 4500 children, of whom 1203 (26.7%) had any vomiting. After adjusting for multiple demographic and clinical characteristics, the presence of dehydration (RR: 1.45, 95% CI: 1.10-1.92), being underweight (RR: 1.22, 95% CI: 1.05-1.41), receipt of the rotavirus vaccine (RR: 1.89, 95% CI: 1.69-2.12), and household wealth above the median (RR: 1.17, 95% CI: 1.07-1.29) were factors associated with an increased risk of vomiting. Rotavirus vaccine receipt was nearly 100% concordant with the study site of Tanzania. Older age and lower zinc dosing were associated with a lower risk of vomiting.

Conclusions: Young, underweight, or dehydrated children are more likely to have concurrent vomiting with zinc supplementation. Identification of these factors may allow providers to better monitor such children, thus reducing the chances of recurrent dehydration or inadequate dietary intake.

Objectives: The trend of alanine aminotransferase (ALT), a biomarker of metabolic dysfunction-associated steatotic liver disease (MASLD, formerly known as nonalcoholic fatty liver disease), remains poorly described for the pediatric population because no widely accepted cutoffs are available to categorize ALT value. We described the nuanced changes in the distribution of ALT continuous values.

Study design: We analyzed the data from 15,702 adolescents aged 12-19 who participated in the National Health and Nutrition Examination Surveys between 1988 and 2020. The ALT distributions were standardized for age and sex. The prevalence of elevated ALT was also assessed.

Results: The ALT geometric mean increased from 11.82 U/L in 1988-1994 to 17.24 U/L in 1999-2004, stayed above 17 U/L for a decade, and then decreased to 14.04 U/L in 2017-2020 (p for the quadratic trend <0.001). However, the 95^th percentile of the ALT distribution remained above 35 U/L by the end of the study period after jumping from 26.02 U/L in 1988-1994 to 33.83 U/L in 1999-2004. The prevalence of elevated ALT (>42 U/L in boys and 30 U/L in girls), doubled from 1.53% (0.87%-2.19%) in 1988-1994 to 3.49% (2.73%-4.25%) in 1999-2004, and lingered around 4% through 2020.

Conclusions: The ALT mean decreased in recent years, but the prevalence of elevated ALT remained persistently high. Population-wide reductions in fructose consumption may have contributed to the decrease in ALT mean. The stagnant right end of the distribution, manifesting as the high prevalence of elevated ALT, calls for intensified clinical prevention.

Objectives: Rumination syndrome (RS) is diagnosed based on clinical criteria with or without diagnostic testing showing characteristic findings on antroduodenal manometry (ADM), high-resolution esophageal manometry (HREM), and multichannel intraluminal impedance-pH testing (MII-pH). The objective of this study was to evaluate the correlation between diagnostic testing and clinical outcomes.

Methods: We conducted a retrospective review of children with RS evaluated at our institution. Patients were divided into two groups: those with confirmatory diagnostic testing for rumination based on ADM, HREM, and/or MII-pH, and those without. We compared response to treatment based on the proportion no longer having vomiting at follow-up and no longer needing supplemental nutrition.

Results: We included 152 children (60% female, median age of diagnosis 13 years, interquartile range 8-15 years) with RS. 22 patients (14%) had diagnostic testing that confirmed RS. At baseline, there was no statistical difference in the percentage of patients who had overt vomiting (p = 0.311), however, the confirmatory testing group was more likely to need supplemental nutrition (p ≤ 0.001) and to receive intensive treatment (p < 0.001). After treatment, the proportion of patients without vomiting increased in both groups without a statistically significant difference between the two groups. There were also no significant differences in likelihood of reporting improvement in symptoms or needing supplemental nutrition.

Conclusions: Children with RS who had confirmatory diagnostic testing were equally likely to no longer have vomiting after treatment when compared to those without confirmatory testing. Future studies are needed to account for potential differences in baseline severity between groups.

Objectives: Functional defecation disorders (FDDs) are common among children worldwide. The prevalence of these disorders has not been clearly described in Europe. This study performed a systematic review and meta-analysis on the prevalence of FDD in European children and assessed geographical, age, and sex distribution and associated factors.

Methods: PubMed, Embase, Psycinfo, Cochrane Library, and Cinahl were searched from 1999 to July 2023. Included studies were (1) prospective or cross-sectional studies of European population-based samples; (2) reporting the prevalence of infant dyschezia (ID) according to Rome II, III, or IV criteria or functional constipation (FC) or functional non-retentive fecal incontinence (FNRFI) according to Rome III or IV criteria; (3) aged 0-18 years; and (4) published in English, Dutch or Spanish. PRISMA guidelines for extracting data and assessing data quality were followed.

Results: Twenty-eight studies were included. Pooled prevalence was 6.9% (95% confidence interval [CI]: 3.1%-11.9%) for ID in infants 0-12 months (9 studies, n = 5611), 8.17% (95% CI: 6.33%-10.22%) for FC in children <4 years (25 studies, n = 35,189), 11.39% (95% CI: 9.34%-14.11%) for FC in children 4-18 years, and 0.24% (95% CI: 0.07%-0.49%) for FNRFI in children 4-18 years (7 studies, n = 16,873). No sex predominance was found for FC. FC prevalence did not differ significantly when diagnosed according to Rome III versus IV. FC prevalence differed between countries, with greatest rates in Italy, Germany, and Spain. No meta-analysis could be performed on other factors associated with FDD.

Conclusions: FDD is common in European children. Future longitudinal studies are needed to provide better insight into associated factors in pathogenesis.

Mutations in doublecortin domain-containing protein 2 (DCDC2) lead to neonatal sclerosing cholangitis (NSC), and portal hypertension (PHTN). The objective of the study was to systematically evaluate PHTN, variceal bleeding, and outcomes of patients with DCDC2-related NSC. The study included children with homozygous or compound heterozygous variants in DCDC2. All 14 children with DCDC2-related NSC had PHTN. Eight (57.1%) developed variceal bleed at a median age of 3 years (range: 1.9-5 years). Eleven (78.6%) children with high-risk varices underwent endotherapy. Varices were completely eradicated in three, downstaged to low-risk in five, and there was no response with endotherapy in three. All three children with failure to eradicate/downstage varices had rebleed, and required listing for liver transplantation (LT). The study shows that children with variants in DCDC2 have a high incidence of variceal bleed at a very young age. Variceal eradication may often be difficult and rebleed rates are high; often necessitating LT.

Portal hypertension, a common sequela of chronic liver disease, is complicated by variceal hemorrhage, one of its most serious complications. Evidence-based approaches to managing variceal hemorrhage are limited by the scarcity of data related to this rare entity. Multicenter international registries are increasingly utilized to garner critical information about rare diseases. The International Multicenter Pediatric Portal Hypertension Registry (IMPPHR) was developed to acquire pediatric data about the mortality of first variceal hemorrhage and approaches to primary and second prophylaxis of variceal hemorrhage with a goal of improving outcomes in children with portal hypertension. IMPPHR evolved from pediatric portal hypertension symposia at the Baveno V and VI meetings in 2010 and 2015, with a formal executive committee initiating the development of IMPPHR in 2019. The registry opened in 2020, with data closure in 2024, including information from 44 centers and >700 subjects. The complexities and approaches to developing IMPPHR are described.

Objective: Glycogen storage disorders (GSD), inherent disorders of carbohydrate metabolism, feature hypoglycemia as a hallmark. Normoglycemia and glucose monitoring are pivotal in disease management. Conventional glucometer-based monitoring may overlook hypoglycemic trends. This study assesses glycemic control in Asian Indian GSD children using continuous glucose monitoring (CGM) and its role in facilitating dietary adjustments.

Methods: A pre-post study enrolled molecularly confirmed GSDI, GSDIII, GSDVI, and GSDIX patients for baseline dietary compliance and CGM-based glycemic status evaluation. Hypoglycemic patients were stratified into diet-compliant and diet-noncompliant groups. Noncompliant patients received dietary reinforcement; compliant individuals underwent dietary adjustments. Repeat CGM (rCGM) was performed 6 weeks to 6 months postadjustments. Clinical and metabolic parameters were re-evaluated at 6 months.

Results: Of the 20 patients assessed at baseline, 11 were diet compliant. Six among these exhibited hypoglycemia, prompting diet adjustments. Among nine noncompliant patients, eight experienced hypoglycemia and received diet reinforcement. rCGM in 10 patients (five GSDI, three GSDIII, and two GSDIXc) showed a significant reduction in hypoglycemia duration in all. An improvement in height and body mass index was observed in all GSDI and GSDIII patients. Triglyceride levels, raised at baseline in two GSDI and one GSDIII, showed a substantial decline in one GSDI patient. Hepatic transaminase levels decreased in both GSDIXc patients. Plasma lactate levels decreased in all GSDI patients.

Conclusion: CGM is an efficacious adjunct in the personalized management of hepatic GSD patients, in the Asian Indian population. The study also underscores the need for long-term follow-up to determine the role of glycemic management in growth, general well-being, and metabolic control in the GSD subtypes.