Journal of Pediatric Gastroenterology and Nutrition最新文献

Objectives: Historically, in children with intestinal failure associated liver disease (IFALD), the presence of splenomegaly and moderate bridging fibrosis would be considered as evidence of advanced liver disease and portal hypertension and be recommended for liver-inclusive intestinal transplant graft. In our experience, the assessment of portal hypertension based on conventional investigations, which are well established in other chronic liver diseases, could be misleading in some children with IFALD, and further investigations could help in assessing the severity of liver disease. Hepatic venous wedge pressure gradient (HVWPG) is used in chronic liver diseases for objectively assessing the severity of portal hypertension. We postulated that HVWPG may be useful to assess the severity of portal hypertension in children with IFALD and, therefore, help in the decision-making process for the need for a liver-inclusive intestinal graft.

Methods: Retrospective analysis of children with IFALD who had HVWPG measured between 2005 and 2020. Demographic details, laboratory parameters, liver biopsy, HVWPG and clinical outcomes were reviewed. Children were grouped into two categories based on HVWPG gradient: HVWPG ≥ 10 mmHg (significant portal hypertension) and HVWPG < 10 mmHg.

Results: Between 2005 and 2020, 23 children (median age: 33 months, interquartile range: 11-54) had 27 HVWPG measurements (4 children had repeat measurements). No procedural complications were documented. 16/23 children had HVWPG < 10 mmHg, 7/23 children had HVWPG ≥ 10 mmHg. Of the 16 children with HVWPG < 10 mmHg, 10 children were referred to the local team for intestinal rehabilitation, while 6 children were recommended for transplantation (4 for isolated intestinal transplant and 2 for liver-inclusive intestinal transplant) as they fulfilled other indications for intestinal transplantation (impaired venous access, etc.). Of the seven children who had significant portal hypertension (HVWPG ≥ 10 mmHg), six were recommended for liver-inclusive intestinal transplant. There was a cohort of four children with at least bridging fibrosis and HVWPG < 10 mmHg who had repeat measurements due to failed intestinal rehabilitation strategies to wean from parenteral nutrition and worsening clinical signs (increasing splenomegaly, etc.). Two children were recommended for liver-inclusive intestinal transplant in view of increase in HVWPG to ≥10 mmHg.

Conclusions: HVWPG measurements can guide in the decision-making process in children with IFALD, especially those with bridging fibrosis without significant clinical evidence of portal hypertension for deciding on the need for liver-inclusive intestinal transplantation.

Introduction: Previously, the parent-reported Childhood Bladder and Bowel Dysfunction Questionnaire (CBBDQ) for children aged 5-12 years was found valid, reliable and responsive. The purpose of this study was to adapt the CBBDQ to construct the adolescent-reported CBBDQ_12-18, translate it into English according to guidelines, and determine its measurement properties, as defined by the Consensus-based Standards for the selection of health Measurement Instruments (COSMIN).

Methods: Delphi panels, surveys on feasibility and acceptability, English translation and cross-cultural adaptation, and a prospective cohort study were conducted. Participants were adolescents, 12-18 years with and without bladder or bowel dysfunctions, and healthcare professionals who were experts in the field. The Medical Ethics Committee of Maastricht University approved the study (MEC 2018-0321).

Results: A total of 107 adolescents and 15 professionals participated from February 2017 to August 2023. The CBBDQ_12-18 was feasible and acceptable, translated into English and cross-culturally adapted. Content validity, moderate construct and fair criterion validity and excellent test-retest reliability with acceptable internal consistencies were demonstrated. Responsiveness at 6 months could not be demonstrated.

Discussion: The CBBDQ_12-18 is a symptom questionnaire on bladder and bowel problems in adolescents 12-18 years. It was developed in close cooperation with adolescents (some of them bilingual) and experienced healthcare workers. The outcomes regarding the measurement properties of the CBBDQ_12-18 were lower than those for the CBBDQ, but remained satisfactory, except responsiveness. A possible limitation of the present cohort study may be its sample sizes, although the total sample sizes of this study were sufficient in line with COSMIN guidelines.

Objectives: Gastroscopy is used to examine the upper gastrointestinal (GI) tract, but no validated method yet exists to assess the quality of mucosal visualization in children. Utilizing validated endoscopic scales can enhance study quality and standardization across centers. This study aimed to validate the existing Toronto Upper Gastrointestinal Cleaning Score (TUGCS) in pediatric patients.

Methods: This was a multicenter, prospective, single-masked study conducted in 10 European pediatric gastroenterology centers. Endoscopists with varying degrees of experience assessed the quality of mucosal visualization in prerecorded gastroscopies using the TUGCS. Each endoscopist assessed the studies two times in random order, with an interval of at least 2 weeks. The correlations of individual and total scores were statistically compared between themselves, between assessors, and between assessment attempts. Internal consistency was also checked with Cronbach's α.

Results: Seventeen endoscopists participated in the study. The TUGCS demonstrated high consistency within raters, with a score of 0.64 (95% confidence interval [CI]: 0.34-0.84), and an excellent test-retest reliability of 0.97 (95% CI: 0.94-0.99). The scale also showed high internal consistency, with a Cronbach's α of 0.95. The correlation between different items ranged from 0.60 to 0.77, and the correlation between individual items and the total score ranged from 0.66 to 0.88. No significant differences in the assessment were found based on the raters' experience performing endoscopy, specialization, age, or gender. The endoscopists found TUGCS easy to learn and potentially useful, especially in clinical trials.

Conclusions: The TUGCS was demonstrated as a reliable and validated method for assessing the visualization quality of the upper GI mucosa in pediatric patients.

Objectives: Esophageal atresia (EA) is a significant congenital anomaly, with most survivors experiencing esophageal dysmotility. Currently, there is no reliable way to predict which patients will develop significant, life-threatening dysmotility. Using high-resolution impedance manometry (HRIM), this study aimed to characterize the common pressure topography patterns in children with repaired EA.

Methods: This prospective longitudinal cohort study focused on children (<18 years) with repaired EA. Utilizing HRIM, esophageal motility patterns were studied. Repeat manometric assessments were performed in a selected group.

Results: Seventy-five patients with EA (M:F = 43:32, median age 15 months [3 months to 17 years]) completed 133 HRIM studies. The majority (54 out of 75, 85.3%) had EA with distal tracheo-esophageal fistula. Thirty-five out of 75 (46.7%) underwent one study, 24 out of 75 (32.0%) two studies, 14 out of 75 (18.7%) three studies, and 2 out of 75 (2.7%) four studies. Seventy-two patients had analyzable studies. Three common motility patterns were demonstrated: (1) aperistalsis (26 out of 72, 36.1%); (2) distal esophageal contraction (25 out of 72, 34.7%); and (3) pressurization (6 out of 72, 8.3%). A minority demonstrated combination patterns, including aperistalsis with weak distal contraction (10 out of 72, 13.9%) and aperistalsis with pressurization (2 out of 72, 2.8%). Contraction was normal in 3 out of 72 (4.2%). At repeat assessment, the dominant motility pattern persisted in 26 out of 38 (68.4%) of the second studies and 9 out of 15 (60.0%) of the third studies.

Conclusion: Utilizing HRIM in children with repaired EA, we have demonstrated objective, distinct, and reproducible motility patterns. In this cohort, the majority of motility patterns were maintained longitudinally, and dysphagia scores remained unchanged, even after dilatation.

Objectives: Inflammatory bowel disease (IBD) is an immune-mediated, chronic, remitting, and relapsing disease. Calprotectin, used in monitoring the disease activity, is expressed from neutrophilic granulocytes during inflammation. Neutrophil gelatinase-associated lipocalin (NGAL) is strongly expressed in both granulocytes and the intestinal epithelial cell layer. The aim of the study was to compare fecal NGAL (FNGAL) with fecal calprotectin (FCAL) in children with IBD.

Methods: Forty-four children with IBD and 22 healthy children were included in the study. The patients were divided into two groups, patients with active disease and remission group. Clinical and demographic characteristics, disease activity scores, and serum and fecal markers of the patients were recorded.

Results: The mean age of the patients was 13.2 ± 3.4 years (range 6-17 years) and male/female: 0.62. FNGAL levels of patients with active disease were higher than those in the remission group (p < 0.001). A statistically significant positive correlation was observed between Pediatric Ulcerative Colitis Activity Index scores and white blood cell count, platelets, neutrophil-to-albumin ratio (NAR), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and FNGAL. There was a positive correlation between Pediatric Crohn's Disease Activity Index scores and platelets, NAR, ESR, CRP, and FNGAL, whereas there was a statistically significantly negative correlation with activity scores and albumin. While FNGAL had 95.5% sensitivity and 81.8% specificity, FCAL had 86.7% sensitivity and 85.7% specificity.

Conclusions: FNGAL levels were found to be high and sensitive in determining disease activity in our patients with IBD, suggesting that it may be a valuable biomarker.

Objectives: To assess the impact of magnetic endoscopic imaging (MEI) on pediatric colonoscopy.

Methods: We analyzed demographics, procedure completion, procedure times, complications, and whether or not MEI was used for all colonoscopies between April 27, 2023, and January 18, 2024. MEI was available for every case but used at the endoscopist's discretion. Attendings were surveyed on the frequency and duration of interventions during fellow-performed colonoscopies.

Results: We analyzed 310 colonoscopies, 113 (36%) of which used MEI. The average patient age was 13.8 years (range 5 months to 23 years). For the aggregate sample and attending-performed cases, there were fewer males in the groups that used MEI (p < 0.01). There were no other statistically significant differences in demographics or procedure indication when MEI was used. Terminal ileum (TI) intubation rate was higher when MEI was used in the aggregate sample (p = 0.02) and for fellow-performed cases (p = 0.04). TI intubation times and total procedure times were quicker when MEI was used in the aggregate sample and in both strata of attending-performed cases and fellow-performed cases (p < 0.001). One complication was reported in an attending-performed case that did not use MEI. Of the 145 fellow-performed procedures, 98 (68%) had completed surveys, 36 (36%) of which used MEI. There was no statistically significant difference in the number (p = 0.89) or duration (p = 0.96) of attending interventions when MEI was used.

Conclusion: MEI use was associated with higher TI intubation rates, faster TI intubation, and shorter total procedure times. MEI may be a valuable adjunctive tool for pediatric endoscopists.

Objective: This study aimed to assess the evolution of nutritional status, parenteral nutrition (PN) composition, and PN dependence index (PNDI) in children under 2 years old with intestinal failure (IF) and malnutrition.

Methods: This retrospective cohort included patients admitted between November 1, 2016, and August 31, 2021, with IF, age ≤24 months, and weight-for-age (Wt/A) <-2 standard deviation with a follow-up of 12 months. Data collected included demographic, anthropometric, biochemical, and nutritional information. The PNDI was compared between enteral autonomy (EA) and nonenteral autonomy (non-EA) groups.

Results: Thirty patients (16 males, 53.33%) were included, and short bowel syndrome (SBS) was the leading cause of IF (n = 27, 90%). The mean age at admission was 7.23 ± 3.90 months, and 36.66% of the patients had a conjugated bilirubin (CB) level >2 mg/dL; 87% had a Wt/A z-score <-3 and 90% had a height-for-age (Ht/A) z-score <-3. At 5 months, the mean Wt/A z-score improved to -1.82. At admission, median protein, lipid, and glucose intake on PN were 3, 1.6, and 17.27 g/kg/day, respectively. In the PNDI analysis, the EA group had a mean index of 141% versus 158% for the non-EA group at admission, with no statistical differences. From the sixth month onward, the EA group showed a significantly lower PNDI (50% vs. 108% for non-EA patients; p = 0.032). Both groups demonstrated adequate nutritional recovery.

Conclusion: Adequate multidisciplinary care can reverse severe malnutrition, and the PNDI may be a predictor of EA.

Background and aims: The Eosinophilic Esophagitis Histology Scoring System (EoEHSS) is useful for diagnosing and characterizing eosinophilic esophagitis (EoE). A limitation of the EoEHSS is that lamina propria fibrosis scores are infrequently determined due to challenges in sampling lamina propria. Low distensibility index (DI) measured by endoluminal functional lumen imaging probe (EndoFLIP) is associated with fibrostenotic severity in pediatric patients with EoE. We investigated the correlation between DI and the EoEHSS to understand whether EndoFLIP could be a useful complementary tool for evaluating EoE-associated remodeling in children.

Methods: We reviewed the medical records of patients <21 years of age who underwent an esophagogastroduodenoscopy (EGD) with biopsy and EndoFLIP between October 2017 and July 2023 with histologic diagnoses of normal/reactive, reflux, or EoE. EoEHSS scores and luminal parameters were compared between groups. DI measured at 30 mL inflation was compared with EoEHSS scores.

Results: One hundred twenty-six EGDs with biopsy and EndoFLIP were performed on 112 patients. There were 80 normal/reactive, 32 reflux, and 14 EoE biopsies. At 30 mL inflation, DI was lowest in the EoE group (p = 0.03). DI at 30 mL inflation negatively correlated with the EoEHSS overall grade score, as well as grade and stage scores for eosinophil abscesses, eosinophil surface layering, dilated intercellular spaces, and basal zone hyperplasia (all p < 0.05). DI at 30 mL inflation also negatively correlated with the eosinophilic inflammation stage score (p < 0.05).

Conclusion: DI measured by EndoFLIP at 30 mL inflation shows a negative correlation with composite EoEHSS scores and subscores, suggestive of remodeling. EndoFLIP may complement the EoEHSS in evaluating EoE-associated esophageal remodeling.