Journal of Pediatric Gastroenterology and Nutrition最新文献

Objectives: Postoperative steroids after Kasai portoenterostomy (KPE) for biliary atresia (BA) patients remains controversial. We established a postoperative protocol with selective corticosteroid usage depending on postoperative stool color assessment. Herein, we report outcomes in KPE before and after implementation of our tailored steroid protocol.

Methods: At our institution, 28 infants underwent KPE between 2015 and 2025. Group A had 16 infants managed without steroids between 2015 and 2021, while Group B included 12 infants managed under the new tailored steroid protocol between 2021 and 2025. Under the new protocol, infants with postoperative stool color ≤3 based on Japanese Tochigi Stool card received corticosteroids and antibiotics for 5 weeks if they were ≤45 days old or >45 days old with acute inflammation on liver biopsy obtained during operation. Postoperative total bilirubin (TB) levels at 3 months, 2-year native liver survival (NLS), length of stay (LOS) of surgical admission, postoperative reoperations, readmissions, and complications were compared between groups.

Results: Preoperative liver function tests were similar between groups. The 3-month post-KPE TB levels were significantly lower in Group B compared to Group A (0.9 [0.3, 1.9] mg/dL vs. 6.5 [0.6, 10.4] mg/dL, p = 0.036). The 2-year NLS was also significantly higher in Group B (72.9% vs. 37.5%, p = 0.046). LOS, readmissions, reoperations, and complications in the 90-day postoperative period were not different between both groups.

Conclusions: Infants with BA managed with a tailored steroid protocol based on postoperative stool colors and histologic evidence of inflammation had significantly lower 3-month TB levels and better 2-year NLS.

Objectives: After hepatoportoenterostomy (HPE), a minority of biliary atresia (BA) patients reach adolescence without liver transplantation. Several serum markers have been suggested to better predict post-HPE outcomes in BA patients. We aimed to identify serum predictors of native liver survival (NLS) in post-HPE BA patients.

Methods: We searched PubMed, MEDLINE, SCOPUS, and EMBASE databases to identify publications from 1946 through December 2023. Studies included reported serum values as prognostic factors for BA after HPE, specifically total bilirubin (TB), alanine transaminase (ALT), gamma-glutamyl transferase (GGT), matrix metalloproteinase 7 (MMP-7), and total bile acids (TBA). We defined nonfunctioning HPE as persistent jaundice, cirrhosis, LT, or death. We calculated pooled serum variables, including hazard and odds ratios, for NLS using inverse variance weighting.

Results: Thirty studies were included in the meta-analysis including a total of 4399 BA patients, 2073 and 1793 had successful versus nonfunctioning HPE, respectively. The mean HPE age for the successful group was significantly less (63.3 vs. 69.5 days, p < 0.001). TB was significantly elevated in the nonfunctioning group (p < 0.001). Pooled ALT and GGT were significantly lower in the successful group 1-3 months and >5 years after HPE (p < 0.001). The successful group had significantly lower serum TBA (27.55 vs. 69 μmol/L, p < 0.001) > 5 years after HPE.

Conclusion: Established serum values ALT, GGT, and TB are useful for prognostic assessment post-HPE. MMP-7 and TBA require additional evaluation to determine their prognostic relevance for NLS in BA.

Objectives: Progressive familial intrahepatic cholestasis (PFIC) is an overarching term for rare monogenic defects that result in cholestatic liver disease. Larger consortia-based registries have begun to address the challenges of PFIC as a rare disease with variable phenotype, but patient-reported outcomes (PROs) have not been extensively described. Here, we report baseline analysis from the PFIC network patient registry (PNPR), an effort to report outcome measures identified as meaningful and impactful to those living with the disease.

Methods: The PNPR is a prospective, international, voluntary patient registry collecting longitudinal PROs relevant to PFIC and its complications, including diagnosis, symptoms, surgeries, medications as well as validated measures related to itch, sleep, and general health (patient-reported outcomes measurement information system [PROMIS] measures), disease impact on family quality of life (QoL), and financial burden of the disease.

Results: Baseline data from 161 international patients were included. Registrants included patients affected by several subtypes and benign recurrent intrahepatic cholestasis, and 19 participants with an unknown or missing diagnosis. Pruritus is an important contributor to morbidity with severity of itch positively correlating with sleep disturbance and sleep impairment and negatively correlating with family QoL and overall health. The financial burden of disease was reflected by higher out-of-pocket medical costs and more reported challenges arranging medical care compared to the general US population.

Conclusion: The PNPR fills a previously identified gap in PFIC research-the lack of PROs-and reveals the negative impact of disease and pruritus on patient and family function, QoL indicators, finances, and measures of general health.

Objectives: Sleep disturbance, pain, anxiety, depression, and fatigue are prevalent in adolescents and young adults with inflammatory bowel disease (IBD). These symptoms often present in co-occurring sets, known as symptom clusters. We aimed to identify distinct symptom clusters and factors associated with symptom profiles in adolescents with IBD.

Methods: We recruited 105 adolescents with IBD seen at ImproveCareNow clinics. Data were collected from the ImproveCareNow clinical data registry (years since diagnosis, medications, and physician global assessment of disease activity) and an online survey (demographics, diagnosis, comorbidities, symptoms, self-efficacy, self-management, medication adherence, and sleep hygiene). Latent class analysis was used to classify adolescents into subgroups with distinct symptom profiles.

Results: Adolescents were 51.4% female, 83.8% white, a mean age of 14.9 years; 77.1% had Crohn's disease and 62.9% were on biologic therapy. Mean comorbidities were 0.6 and 70.5% had mild or quiescent disease activity. Three symptom profiles emerged: (1) Low Symptom Burden, characterized by a low probability of endorsing any symptoms; (2) High Symptom Burden, characterized by a high probability of endorsing sleep disturbance, pain, anxiety, depression, fatigue; and (3) Impaired Energy, characterized by a high probability of endorsing sleep disturbance and fatigue. Older age, more comorbidities, and lower IBD self-efficacy were associated with the High Symptom Burden profile. Older age and lower IBD self-efficacy were associated with the Impaired Energy profile.

Conclusions: Distinct symptom profiles highlight the unique symptom management needs of adolescents with IBD. Future research should explore biopsychosocial contributors and longitudinal trajectories of symptoms.

We retrospectively evaluated the effectiveness of budesonide orodispersible tablets (BOT) in children with eosinophilic esophagitis (EoE) followed up at our center. A total of 17 children were treated with BOT 0.5 mg twice daily after a median time from diagnosis of 16 months (interquartile range [IQR]: 11-28.5). Previous treatments included proton-pump inhibitors, food elimination diets, and topical swallowed corticosteroids (TSC). After 3 months, 11/17 (65%) patients were in clinical remission. 8/17 (47%) patients repeated an endoscopy after a median time of 11.5 months (6.75-16.75 IQR); 6/8 (75%) were in endoscopic remission and 7/8 (87.5%) achieved histological remission. Eosinophils count at histology significantly decreased from baseline (30 [15-50 IQR] to 6 [6-9 IQR], p = 0.03]. 3/4 (75%) patients who previously failed TSC achieved complete remission on BOT. After a median follow-up of 12.5 months (6.25-21 IQR), 11/17 (65%) patients were still on BOT treatment, and 9/11 (82%) were in clinical remission. No adverse events were reported.

Objectives: Emergency department (ED) visits represent a significant cost in the care of children with inflammatory bowel disease (IBD). Many of these visits are potentially preventable. We aimed to evaluate factors associated with ED use in pediatric patients with IBD.

Methods: Patients with IBD aged 0-21 years at a single tertiary center were identified via electronic medical record query. Demographics, disease characteristics, and recent laboratory results were extracted. ED visits in the 6 months following data extraction were tallied. Multivariable logistic regression was performed to evaluate risk factors for ED use. Kaplan-Meier curve analysis was used to understand the risk of IBD-related ED use over time following initial diagnosis.

Results: Of the 531 patients identified, 49 (9%) visited the ED during the study period and 22 (4.1%) had an IBD-related visit. Number of recent ED visits (odds ratio [OR] 3.168, 95% confidence interval [CI] 1.925-5.466), public insurance (OR 2.232, 95% CI 1.021-4.665), and IBD diagnosis within the last 6 months (OR 2.964, 95% CI 1.039-7.918) were risk factors for all-cause ED use, though only number of recent ED visits (OR 2.105, 95% CI 1.110-3.668) was associated with IBD-related ED use. For the 391 patients included in the analysis of IBD-related ED use over time, the rate of ED use was highest the first month after diagnosis (slope 0.268, 95% CI 0.258-0.279) and plateaued over the following year.

Conclusions: Recent ED use, regardless of cause, is an important risk factor for IBD-related ED use in pediatric patients. The first several months after diagnosis constitute a particularly high-risk time for ED use.

Objectives: Avoidant/restrictive food intake disorder (ARFID) is a relatively new diagnosis in the DSM-5, since 2013. The restrictive and/or selective eating-driven by a lack of interest, sensory sensitivity, and/or concern over aversive consequences-is associated with significant medical and/or psychosocial problems. However, little is known about risk factors of this feeding/eating disorder. Our objective was to investigate which factors predispose to ARFID in children.

Methods: Databases CINAHL, Embase, PsycINFO, PubMed, and Web of Science were searched to identify peer-reviewed articles published from inception to August 27, 2024. Articles that included children with ARFID, a control group, and a potential risk factor were selected.

Results: A total of six studies were included in this review. In these studies, data regarding potential risk factors and their risk of bias were evaluated. All studies were of cross-sectional design. Despite an extensive search strategy, only a small number of studies could be included for analysis. A total of 18 factors were reported, of which 10 factors were significantly associated with ARFID. These factors include physical factors, psychiatric comorbidities, and maternal psychopathological risk.

Conclusions: This systematic review shows which factors are associated with ARFID in children. To better clarify the direction of associations between potential risk factors and ARFID, longitudinal and interventional studies are needed. This knowledge may support early recognition and timely intervention in ARFID.