Journal of Pediatric Gastroenterology and Nutrition最新文献

Objectives: An acceptable alternative to human milk is US Food and Drug Administration (US FDA)-registered infant formula, which must meet the requirements of the US FDA Infant Formula Act. Human milk contains lactose, but some infant formulas may contain alternative carbohydrate sources such as corn syrup solids, maltodextrin, and sucrose. Recent research shows that infant-formula made with corn syrup solids may be associated with increased obesity risk in the first 5 years of life. A previous study found that of all formulas purchased, 59.0% were lactose-reduced. More US infants consume infant formula with nonlactose carbohydrates more frequently than is medically necessary. The purpose of this study is to use National Health and Nutrition Examination Survey (NHANES) data to describe the type and prevalence of nonlactose carbohydrates consumed in infant formula.

Methods: NHANES data from 1999 to 2020 was used to perform cross-sectional analyses and analyses of comparison of prevalence over time on consumption of nonlactose carbohydrate sources in infant formulas.

Results: We identified 3709 unique infant identifiers associated with 36,084 feeding sessions. More than half of the feeding sessions involved a formula with at least one nonlactose carbohydrate. Feeding sessions involving a formula with at least one nonlactose carbohydrate increased by 163% from 1999-2004 to 2017-2020; formulas containing single or multiple nonlactose carbohydrate types account for the increase in prevalence.

Conclusions: This study highlights an increase in the consumption of infant formula containing a nonlactose carbohydrate. More studies are needed to understand the short- and long-term effects of early exposure to these carbohydrates.

Celiac plexus blocks (CPBs) using endoscopic ultrasound (EUS) guidance provide significant pain relief in adults with chronic pancreatitis. We present on EUS-guided CPB for pediatric patients with abdominal pain from chronic pancreatitis or severe functional dyspepsia necessitating clinically assisted nutrition and hydration. Patients who underwent EUS-CPB were included and followed prospectively at 2-, 4-, and 8-weeks postprocedure about pain, enteral tolerance, and school/activity attendance. Thirteen patients underwent EUS-guided CPB with a total of 21 procedures. In the pancreatitis cohort, mean pain relief was 11.7 weeks for those who responded. In the functional dyspepsia cohort, mean improvement (in either pain or enteral tolerance) was 4.8 weeks. Symptom improvement varied between the two cohorts. Acute recurrent/chronic pancreatitis patients demonstrated more sustained relief than the functional dyspepsia cohort. This study adds to the limited data investigating the utility of EUS-CPB as part of a multimodal treatment plan in pediatrics.

Objectives: We report disease outcomes of pediatric Crohn's disease (CD) affecting the proximal small bowel (SB) and detected through video capsule endoscopy (VCE).

Methods: We undertook a retrospective review of CD patients with VCE performed under age 18 between 2003 and 2017 and having received any biologics. We identified patients from our institutional registry.

Results: Eligible patients (n = 118) had their first VCE performed after a median of 0.1 years after diagnostic endoscopies at a median age of 12.2 years. The proximal SB disease group (Paris classification L4b inclusive) comprised 70 patients with extensive SB lesions in 81% and deep ulcers in 79%. Patients with Paris L1-3 disease with no findings in VCE or disease restricted to the terminal ileum comprised the control group. At first VCE, levels of albumin (34 g/L vs. 37 g/L) and hemoglobin (117 g/L vs. 127 g/L) were lower in SB patients (p < 0.02). After the first VCE, 68% were introduced to biologics, while 10% already received them. Follow-up VCE was performed after a median of 2.4 years (SB group n = 42; controls n = 21). Proximal SB findings had disappeared in 40% of SB patients, and extensive lesions and deep ulcers had decreased to 26% and 29%, respectively (p = 0.001). In the control group, one had progressed to proximal disease. During the clinical follow-up of a median of 4.7 years, one patient with SB underwent surgery for a jejunal stricture.

Conclusions: Proximal SB disease detected through capsule endoscopy abated in most patients with biological medication.

Objectives: Metabolic dysfunction-associated steatotic liver disease (MASLD) is common in children. We hypothesized environmental toxins could drive progression to metabolic dysfunction-associated steatohepatitis (MASH), and assayed serum toxins and metabolites in children with histologically characterized MASLD/MASH.

Methods: Environmental chemicals, common in household items, perfluoroalkyl substances (PFAS), polybrominated flame retardants (polybrominated diphenyl ethers [PBDEs]), and metabolic profiles were assayed in children enrolled in the multicenter NASH Clinical Research Network Pediatric Database 2. Mixture models, using repeated holdout weighted quantile sum regression (WQS_rh) were run in addition to single chemical/metabolite logistic regression. For metabolomic analyses, random subset version of WQS_rh was used for the large number of predictors versus participants. Nominal and false discovery rate (FDR) p-values (two-sided) were computed.

Results: Four hundred and thirty-five children distributed across MASH (n = 293) and MASLD (n = 142), with 304 (69.9%) males. Mean (standard deviation) for Nonalcoholic Steatohepatitis Score (NAS) and alanine aminotransferase (ALT) for MASLD were 3.1 (1.0), 67.9 (43.4), and for MASH 4.2 (1.4), 144 (121). There was an inverse association between PFAS/PBDE mixture and MASH versus MASLD, lobular inflammation (p = 0.026), NAS (p = 0.009, FDR p = 0.04), and log-transformed ALT (p = 0.005, FDR p = 0.025) driven by perfluorohexane sulfonate (PFHXS). Metabolites from positive hydrophilic interaction liquid chromatography mode, biliverdin (p = 0.002) and 1-methylhistidine (associated with meat ingestion, p = 0.02) and reverse phase negative mode, hippuric acid (solvent exposure, p = 0.022) significantly associated with MASH.

Conclusions: Significant negative PFAS/PBDE mixture effect and odds of MASH were dominated by PHFXS. Several metabolites are significantly associated with MASH which inform mechanistic pathways and could drive key therapeutic and diagnostic strategies in children.

Objectives: Celiac disease (CD) is an autoimmune gastrointestinal disorder that requires a strict lifelong gluten-free diet (GFD). Gluten-free (GF) foods are more expensive and less readily accessible than gluten-containing foods, contributing to an increased risk for food insecurity (FI). The study aimed to determine associations between GF-FI, sociodemographic risk factors and child dietary adherence and diet quality (DQ).

Methods: A 26-item, cross-country online survey was administered through social media to parents of children with CD on the GFD. The survey elicited household and CD child sociodemographic and clinical characteristics (e.g., duration of CD), measures of household FI, child DQ and GFD adherence, and parents' concerns related to GF food. Household GF-FI was evaluated using the validated Hunger Vital Sign™ and the US Department of Agriculture Six-Item Short Form Household Food Security Survey Module.

Results: GF-FI occurred in 47% of households with children with CD with >30% reporting low to very low food security. Sociodemographic risk factors identified included lower income, renters, rural residency, single-parental households, and having children with additional dietary restrictions (p < 0.001). Regardless of FI status, a majority of households reported experiencing significantly higher GF food expenditure. GF-FI was associated with reduced adherence to the GFD, increased consumption of processed GF food, and lower intakes of fresh fruits and vegetables and GF grains among children with CD (p < 0.05).

Conclusions: GF-FI is prevalent in this multiethnic cohort of households with CD children and is associated with worsening DQ and GFD adherence. Policy interventions are urgently needed to address GF-FI.

Objective: We conducted a nationwide survey using a validated Gastrointestinal Health Questionnaire (GHQ) for Rett syndrome (RTT) to provide an updated and accurate baseline assessment of the prevalence of common gastrointestinal (GI) issues in RTT, based on parental reporting.

Methods: Parents and caregivers of females with RTT or normally developing, unaffected, age-matched controls completed the GHQ survey. The prevalence of GI symptoms and personality and mood symptoms due to stomach or intestinal problems, as well as GI medication usage and surgical interventions, were assessed in females with RTT and unaffected controls. The relation between GI symptoms and medication usage, surgical status, age, and genetic mutation were analyzed.

Results: Parents of 118 females with RTT and 27 unaffected females completed the GHQ. GI symptoms were common in females with RTT, including constipation (81%), gas and bloating (70%), issues with eating, chewing and swallowing (73%), and irritability because of stomach or intestinal problems (53%). Females with RTT commonly used proton pump inhibitors (52%) and laxatives (64%). Medication usage was associated with significantly higher GHQ symptom scores. Parents of individuals with RTT reported a significantly higher prevalence of GI symptoms affecting their daughters in all symptom categories compared with unaffected females.

Conclusions: GI problems are common in RTT and pose a significant medical burden to caregivers. The GHQ is a useful tool to assess GI issues in individuals with RTT. Improved recognition of these issues may allow for improved treatment and enhanced quality of life for girls and women affected by RTT.

Objective: Acid blocker therapy (ABT) has become common in cystic fibrosis (CF), despite insufficient evidence for benefits and studies showing potentially negative effects. We examined associations between ABT usage and growth, gut microbiome (GM), and early-onset lung disease in young children with CF.

Methods: One hundred forty-five infants with CF born during 2012-2017, diagnosed through newborn screening by age 3 months and followed to 36 months of age at six CF centers were evaluated. Longitudinal data on growth, pancreatic functional status, pulmonary symptoms, and acid blocker medications were prospectively collected. Early-onset lung disease severity was evaluated by a clinical scoring system. GM composition was assessed by 16S rRNA methodology.

Results: ABT use before age 3 years was frequent, with 81 (56%) of patients on H2 receptor antagonist (H2RA) or proton pump inhibitor (PPI), and higher among pancreatic insufficient (60%) versus pancreatic sufficient (26%) children. H2RA was commonly prescribed in infancy before transitioning to PPI. Growth improvements were not significantly greater, while GM α-diversity at 3 years of age was significantly lower and early-onset lung disease more severe, in persistent ABT users compared to nonusers of ABT.

Conclusion: In our cohort of young children with CF, early and persistent ABT use was not associated with significant growth benefits and instead showed associations with reduced GM diversity and negative effects on early-onset lung disease. Consequentially, there is a critical need for systematic evaluation and comprehensive risk-benefit analysis of ABT to ensure proper guidelines for children with CF.

Ubiquitin-specific protease 53 (USP53) is essential for formation of cellular tight junctions and variations in this gene disrupt the tight junctions, resulting in cholestasis. We describe the clinical manifestations and outcomes of patients with USP53 mutations from the Indian progressive familial intrahepatic cholestasis registry. All 29 patients who harbored mutations in the USP53 gene either in the homozygous, compound heterozygous, or heterozygous state and presented with cholestasis were included. USP53 variants related to cholestasis had good outcomes, with native liver survival in 82.7%, whereas 17.3% required liver transplantation. Jaundice developed in 93% and within 3 months of age in 48.8%. Jaundice resolved in 21 (72.4%). Pruritus 76% at a median age of 7 months (severe in 10/22, 45% and refractory to medical therapy in 4, 18.1%). Majority of them (82.7%) had biallelic mutations. Protein-truncating mutations were present in 20 (69%) and missense mutations in 9 (31%). No correlation was found between the genotype and the outcome.

Objectives: The role of nutrition in the recovery of critically ill children has not been investigated and current nutrition provision in the post-pediatric intensive care unit (PICU) period is unknown. The primary objective of this study was to describe ward nutrition support in children following PICU discharge.

Methods: Children up to 18 years admitted to one of nine PICUs over a 2-week period with a length of stay >48 h were enrolled. Data were collected on the first full ward day following PICU discharge and on Days 7, 14, 21, and 28 following PICU admission. Data points included oral intake, enteral (EN) and parenteral nutrition (PN) support, and oral and EN energy and protein provision.

Results: Among the 108 children, on the first full ward day 75/108 (69%) children received EN, 54/108 (50%) oral intake, and 8/108 (7%) PN. Of those receiving oral nutrition only on the first full ward day (25/108; 23%), 9/25 (36%) received <50% of their estimated energy and protein requirements. Of those provided EN only, and where nutrition targets were known, on the first full ward day 8/46 (17%) and 7/46 (15%) met <75% of their estimated energy and protein requirements, respectively. On Day 28, this increased to 4/12 (33%) and 5/12 (42%).

Conclusions: In this study of ward-based nutrition support, key findings included consistent use of EN and PN up to at least 28 days following PICU admission, and a high proportion of children receiving EN or oral intake only not meeting their estimated energy and protein requirements.