Journal of Pediatric Gastroenterology and Nutrition最新文献

Objective: This study examines the association between metabolic-dysfunction-associated steatotic liver disease (MASLD) and cardiovascular risk, focusing on the predictive value of anthropometric, metabolic, and hepatic markers.

Methods: A predictive cross-sectional study was conducted in children aged 5-18 years with overweight, obesity, or metabolic alterations suggestive of hepatic steatosis, with and without MASLD, at a Mexican pediatric endocrine clinic. MASLD was diagnosed via ultrasound, while cardiovascular risk was assessed using carotid intima-media thickness (CIMT) and metabolic markers. Multiple regression models were developed to evaluate the contribution of vascular (CIMT), anthropometric, metabolic, and hepatic variables to cardiovascular risk.

Results: Children with MASLD had significantly higher body mass index (BMI) percentiles (97.56 ± 2.93 vs. 68.91 ± 33.89, p < 0.01), systolic blood pressure (SBP): 113.93 ± 9.38 mmHg versus 101.41 ± 11.45 mmHg, p < 0.01), and Homeostasis Model Assessment of Insulin Resistance (HOMA-IR): 7.55 ± 2.23 versus 3.62 ± 1.76, p < 0.01) compared to controls. CIMT was greater in MASLD patients (0.738 mm vs. 0.56 mm, p < 0.01), suggesting early vascular remodeling. Model 4, which incorporated CIMT, metabolic markers, and liver enzymes, demonstrated the highest predictive value (R² = 0.935, p < 0.001), reinforcing the multifactorial nature of MASLD-related cardiovascular risk.

Conclusions: MASLD is strongly associated with subclinical atherosclerosis and cardiometabolic dysregulation in children. The progressive increase in predictive accuracy across models underscores the importance of a multifactorial approach to cardiovascular risk assessment. Early subclinical cardiovascular findings are detectable in children with MASLD, supporting the rationale for early risk stratification and further research in this population.

Objectives: Dolichocolon (DC), classified under International Classification of Diseases, Tenth Revision (ICD-10) code Q43.8 ("Other specified congenital malformations of intestine"), refers to an elongated or redundant large intestine. Recent studies in adults indicate a role for dolichocolon in constipation and disorders of gut-brain interaction. Contrast enema (CE) imaging is commonly used to identify DC, typically defined as a sigmoid colon extending above, or a transverse colon descending below, the iliac crest line. Despite its potential relevance, the prevalence and clinical significance of DC in pediatric gastroenterology remain poorly understood.

Methods: This retrospective study assessed the prevalence of DC in pediatric patients presenting with constipation and related gastrointestinal symptoms. A total of 155 contrast enemas from pediatric gastroenterology patients meeting predefined inclusion criteria were reviewed. Three blinded pediatric gastroenterologists independently evaluated each CE for features of colonic redundancy consistent with dolichocolon, based on a priori imaging criteria.

Results: Consensus-based identification (i.e., independent agreement among all three reviewers) of DC was observed in 74.1% of children under 2 years old and 88.6% of those aged 2-4 years presenting with constipation. The prevalence significantly decreased with age, with 68.8% in children aged 5-10 years and 47.6% in adolescents aged 11-17 years.

Conclusions: These findings suggest that dolichocolon is common in pediatric patients with constipation and relatable symptoms, particularly in early childhood. Further research is warranted to clarify its clinical implications and potential role in pediatric gastrointestinal disorders.

Objectives: Hepatitis-associated aplastic anemia (HAAA) is described as acute severe hepatitis of unknown origin followed by bone marrow failure (BMF). We aimed to provide a comprehensive picture of pediatric HAAA.

Methods: Two-center retrospective analysis was performed using data from children diagnosed with acquired BMF, including severe aplastic anemia (SAA) and myelodysplastic syndrome type refractory cytopenia of childhood (RCC). The assessment of the subcohort of HAAA included clinical features indicative of diagnosis and disease progression, with additional data from previously published case series.

Results: Cohort comprised 62 children with acquired BMF and 22 children with HAAA. Median age of HAAA patients was 13.5 years. Potentially triggering viral infections were detected in 45%. The median interval from hepatitis onset to cytopenia was 3 weeks. All cases presented with severe hepatitis (median alanine transaminase 2127 U/L) and all but one with hyperbilirubinemia (median bilirubin 15.3 mg/dL). Coagulopathy was variable (median international normalized ratio 1.5). Four patients (18%) developed acute liver failure, two (9%) required liver transplantation. Hepatic parameters normalized within a median of 8.5 weeks. There was no statistically significant difference in the course of hepatitis between patients with SAA and RCC. Early lymphopenia was a key finding in patients with HAAA, progressing from a median of 905/µL at hepatitis onset to 530/µL within 4 weeks.

Conclusions: HAAA occurs in both SAA and RCC. Most cases present with severe acute cholestatic hepatitis and variable coagulopathy. Hepatic recovery is common. Lymphopenia at disease onset is frequent and may serve as a diagnostic marker.

Objectives: People with cystic fibrosis (CF) are at risk of iron deficiency (ID), and although well established in adults, data in children are limited. We assessed iron status in a cohort of children with CF and evaluated the effectiveness of ID management.

Methods: We retrospectively analysed iron status and treatment data from medical records of 190 children with CF (aged 1-16 years) assessed routinely in 2021 and 2022 at the Royal Brompton Hospital (RBH), London. Ferritin, mean corpuscular volume (MCV), and haemoglobin were used to determine the prevalence of ID, ID erythropoiesis (IDE) and ID anaemia (IDA) using RBH definitions.

Results: The proportion of children with abnormal iron status indices decreased between the assessments (2021 - 63% vs. 2022 - 54%, p = 0.03). Prevalence of ID without anaemia (51% vs. 44%, p = 0.12), IDE (8% vs. 6%, p = 0.63), and IDA (4% vs. 3%, p = 1.00) did not differ between assessments. Sixty children received dietary advice for ID without anaemia, and iron supplements were prescribed for six and seven children with IDE and IDA, respectively. Change in iron status indices between assessments did not differ between treated and untreated children, although ferritin increased in the seven children treated for IDA (p = 0.04). There was a positive association between highly effective modulator therapy and change in MCV.

Conclusions: ID remains an issue in children with CF, warranting annual monitoring of iron status. Management with dietary advice and/or iron supplementation showed limited effectiveness using our criteria for abnormal iron status.

Objective: The latest European Society of Gastroenterology Hepatology and Nutrition (ESPGHAN) criteria for celiac disease (CD) diagnosis reduced the requirement for a small intestinal biopsy but still, for most of the cases a small intestinal biopsy is required for a safe diagnosis: hence the attempt to identify serum biomarkers that could replace, in most of these latter cases, the requirement of the biopsy (what is called a "liquid biopsy"). The aim of this study is to identify a set of serum biomarkers able to differentiate celiac patients from age and human leukocyte antigen (HLA)-matched healthy controls.

Methods: Relative concentration of 92 inflammation-linked proteins was examined in the sera of 50 children with CD compared with 50 HLA DQ2/8 and age-matched healthy controls born in genetically at-risk families, using proximity extension immunoassay technology (Olink Proteomics®) with the ProSeek Multiplex Inflammation panel.

Results: Three different multivariate analysis (Univariate and multivariate distribution analysis, random forest classification and linear discriminant analysis) localized a cluster of seven molecules (CASP8, CXCL9, NT-3, SIRT2, STAMBP, ST1A1, and TNFSF14) with a remarkable diagnostic potential, able to differentiate around 90% (95% confidence interval [CI]; 0.7-0.99) of CD patients from controls.

Conclusion: Patients with CD, compared to age- and HLA-matched healthy controls, show in their sera an increased expression of inflammatory molecules, involved in NF-κB cytokine signaling, cell apoptosis, and crypt proliferation pathways. A set of seven of these proteins can differentiate cases from controls with an accuracy higher than 90%. The implementation of this approach in clinical setting could in future facilitate a noninvasive and individualized approach for CD diagnosis.

Objectives: To assess whether low-volume transanal irrigation (L-TAI) is effective as add-on to oral laxative therapy for children with functional constipation and retentive fecal incontinence.

Methods: Two-arm randomized controlled trial, including children aged 4-14 suffering from retentive fecal incontinence. All included children were refractory to at least 2 months treatment with stool softening oral laxatives. Children were included across three pediatric departments in Denmark and randomized into two treatment groups. Treatment duration was 6 weeks. The control group continued oral laxative therapy. The intervention group received L-TAI as add-on. The primary objective was evaluating reduction in fecal incontinence episodes. Secondary objectives included assessment of constipation symptoms, rectal diameter, and well-being based on the WHO-5 questionnaire. Participants were classified as nonresponders (0%-49% reduction) or responders (partial response = 50%-99% reduction, or full response = 100% reduction).

Results: Fifty children were included. The respective median ages were 7 (interquartile range [IQR] = 3) in the intervention group and 6 years (IQR = 1) in the control group. In the intervention group, 75% were responders with 35% experiencing full response, while 33% in the control group were responders, with 4.8% experiencing full response (p = 0.007 and p = 0.020 for response and full response respectively). At follow-up, 55% of the intervention group and 90.5% of the control group still met ROME-IV criteria for constipation (p = 0.010).

Conclusions: L-TAI is effective as add-on to oral laxatives in treating fecal incontinence and constipation. Further studies with longer follow-up periods are needed to assess long-term effects. Clinical Trial identification number: NCT05570318 (https://clinicaltrials.gov/study/NCT05570318).

Extraintestinal manifestations (EIMs) manifest in 6%-47% of patients with inflammatory bowel disease (IBD). Here, we characterize the course of EIMs in pediatric patients receiving vedolizumab included in the VedoKids cohort study. This was a subgroup analysis of the pediatric VedoKids cohort, a multicenter, prospective study of children (aged 0-18 years) with IBD treated with vedolizumab and followed through 54 weeks. EIMs were identified in 18/142 (12.6%) children at baseline; 56% of these cases were articular EIMs; the EIMs resolved in 89% within 18 months. Concomitant medications were administered in 72% of EIM cases, most of which were ongoing at the time of vedolizumab initiation. Of the 124 children without EIMs, five (4%) developed EIMs during follow up: three arthritis, two cutaneous manifestations. The presence of EIMs did not affect the durability of vedolizumab treatment. In conclusion, most EIMs in children with IBD resolved with vedolizumab treatment, but almost half received concomitant medications. Clinical Trial Registration: ClinicalTrials.gov Identifier: NCT02862132. https://clinicaltrials.gov/study/NCT02862132?term=NCT02862132&rank=1.