Journal of Pediatric Gastroenterology and Nutrition最新文献

Hepatoxicity associated with recombinant adeno-associated virus gene therapy is being increasingly encountered by hepatologists in tertiary and quaternary referral units due to the recent increase of these therapies for neuromuscular and haematological disorders. The challenges in managing the condition stem from a lack of good-quality evidence on the appropriate protocols for immunosuppressants due to lack of representative animal models. There is a need for protocols for diagnosing and treating hepatotoxicity and this possible with further research to understand the problem and its management. The review also highlights the importance of a multidisciplinary team in managing hepatotoxicity and recommends further research to better identify at-risk individuals, define the extent of the problem and assess the long-term effects of liver injury and immunosuppressants.

Objective: To describe the clinical impact of lowering the peripheral parenteral nutrition (PPN) maximum osmolarity limit from 1000 to 900 mOsm/L in patients in two neonatal intensive care units (NICUs).

Methods: This was a retrospective cohort study including inborn neonates that received PPN for at least 3 consecutive days within the first 14 days of life. Data were evaluated to compare the ability of PPN with a maximum osmolarity limit of 1000 to 900 mOsm/L to provide daily recommended macronutrient doses, and daily recommended goal calories, as well as to compare the incidence of significant peripheral intravenous (PIV) infiltrates.

Results: A total of 200 PPN orders representing 57 patients were included for analysis, with 100 PPN orders in each osmolarity cohort. Baseline characteristics were similar between the two cohorts. Significantly more PPN orders met goal amino acid doses (45% vs. 24%, p = 0.003) and goal intravenous fat emulsion (IVFE) doses (61% vs. 37%, p = 0.001) in the 1000 mOsm/L osmolarity limit cohort compared to the 900 mOsm/L osmolarity limit cohort. A total of three patients received hyaluronidase for PN infiltration, two in the 1000 mOsm/L osmolarity limit and one in the 900 mOsm/L osmolarity limit cohort (p = 0.6).

Conclusion: A lower PPN osmolarity limit of 900 mOsm/L significantly limited the ability to provide goal amino acid and IVFE doses to NICU patients compared to the previous osmolarity limit of 1000 mOsm/L without reducing the incidence of PIV infiltration or extravasation.

Objectives: We aimed to appraise the real-life efficacy of Crohn's disease exclusion diet (CDED) coupled with partial enteral nutrition (PEN) in inducing clinical and biochemical remission at disease onset and in patients with loss of response to biologics and immunomodulators.

Methods: We retrospectively gathered data of patients aged less than 18 years of age with a diagnosis of Crohn's disease (CD), who received CDED coupled with PEN at a tertiary level pediatric inflammatory bowel disease center.

Results: Sixty-six patients were identified. Forty (60.6%) started CDED plus PEN at disease onset and 26 (39.4%) received CDED with PEN as add-on therapy. Forty-six (69.7%) patients achieved clinical remission (weighted Pediatric Crohn's Disease Activity Index < 12.5) at the end of phase 1, 44 (66.7%) normalized c-reactive protein levels (<0.5 mg/dL) and 18 (27.2%) patients normalized calprotectin levels (<150 µg/g). Nine of 19 (47.3%) of patients with clinically severe disease (defined by Physician Global Assessment) achieved clinical remission at the end of phase I. Patients with extraintestinal manifestations had statistically lower clinical response rates to the dietary regimen (p = 0.018). Among patients who received CDED + PEN as add-on treatment, a previous successful course of Exclusive Enteral Nutrition was associated with statistically higher clinical remission rates at Week 8 (p = 0.026). Clinical response at Week 4 was an independent predictor of clinical remission and fecal calprotectin normalization at Week 8 (p = 0.002).

Conclusion: CDED with PEN confirmed its efficacy in a real-life setting, proving to be effective also in refractory patients and those with severe disease. Early clinical response predicts clinical remission at the end of phase 1.

Significance of autoantibodies in pediatric metabolic dysfunction-associated steatotic liver disease (MASLD) is unknown. Our aim was to determine the prevalence and significance of autoantibodies in MASLD. PubMed and Scopus were searched and six articles (689 [487 males] MASLD patients) were identified. Antinuclear antibodies (ANA) was positive in 28% (95% confidence interval [CI]: 17%-39%, n = 6 studies), antismooth muscle antibodies (ASMA) in 28% (95% CI: 8%-50%, n = 5 studies), actin-positive in 15% (95% CI: 10%-20%, n = 2 studies) and elevated immunoglobulin G in 17% (95% CI: 1%-39%, n = 4 studies). Anti-liver-kidney-microsomal antibody was not present in any patient. There was no significant association of ANA positivity with degree of liver steatosis, liver fibrosis or nonalcoholic fatty liver disease activity score (NAS) but patients with ASMA positivity had advanced fibrosis (pooled risk ratio [RR] 1.77; 95% CI 1.16-2.71) and higher risk of NAS ≥5 (pooled RR 1.21; 95% CI: 1.01-1.44, n = 2 studies, 243 patients). To conclude, non-organ specific autoantibodies are present in over one-fourth of children with MASLD and the presence of ASMA maybe associated with increased disease severity.

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.

Objective: To determine the impact of infant recipient body weight at primary liver transplantation (LT) on both recipient and graft survival rates in complete national data from Poland.

Methods: We conducted a single-center, retrospective cohort study including 142 LT recipients below 1 year of age with body weights below 10 kg who received primary and isolated LT between 2001 and 2017. Patients were divided into two study groups according to body weight at the time of LT: (1) Group I (≤6.0 kg, 32 patients) and (2) Group II (6.1-9.9 kg, 110 patients). Independent impact of body weight on patient and graft survival were assessed using survival curves and a multivariable Cox regression analysis. The univariate predictors of mortality or retransplantation at 1 year post-LT were recipient body weight of ≤6 kg at transplantation, pediatric end-stage liver disease score, urgent LT, graft from deceased donor, cold ischemia time, post-LT hepatic artery thrombosis, and post-LT dialysis.

Results: No statistically significant impact of body weight ≤6 kg on 1-year failure-free survival was found based on the multivariable analysis (p = 0.063). Body weight ≤6 kg was associated with longer post-LT intensive care unit and post-LT hospital stays (p = 0.013 and 0.025, respectively).

Conclusions: Since no evidence of independent negative impact of recipient body weight ≤6 kg on failure-free survival 1 year post-LT was found, LT in infants with end-stage liver disease in Poland should be performed according to medical indications and urgency when an appropriate donor is available.

Objective: Genetic and environmental factors influence pathogenesis and rising incidence of paediatric inflammatory bowel disease (PIBD). The aim was to meta-analyse evidence of diet and environmental factors in PIBD.

Methods: A systematic search was conducted to identify diet and environmental factors with comparable risk outcome measures and had been reported in two or more PIBD studies for inclusion in meta-analyses. Those with ≥2 PIBD risk estimates were combined to provide pooled risk estimates.

Results: Of 4763 studies identified, 36 studies were included. PIBD was associated with higher risk with exposure to ≥/=4 antibiotic courses (includes prescriptions/purchases/courses), passive smoking, not being breastfed, sugary drink intake, being a non-Caucasian child living in a high-income country and infection history (odds ratio [OR] range: 2-3.8). Paediatric Crohn's disease (CD) was associated with higher risk with exposure to antibiotics during early childhood, ≥/=4 antibiotic courses, high socioeconomic status (SES), maternal smoking, history of atopic conditions and infection history (OR range: 1.6-4.4). A history of infection was also associated with higher risk of paediatric ulcerative colitis (UC) (OR: 3.73). Having a higher number of siblings (≥2) was associated with lower risk of paediatric CD (OR: 0.6) and paediatric UC (OR: 0.7). Pet exposure was associated with lower risk of paediatric UC (OR: 0.5).

Conclusion: Several factors associated with PIBD risk were identified that could potentially be used to develop a disease screening tool. Future research is needed to address risk reduction in PIBD.