Journal of Pediatric Gastroenterology and Nutrition最新文献

Objectives: Both superior mesenteric artery syndrome (SMAS) and cannabinoid hyperemesis syndrome (CHS) may present with abdominal pain, nausea, and emesis - making them difficult to differentiate or determine when they co-occur in adolescents who regularly use cannabis. Here, we present nine cases of CHS co-occurring with SMAS and characterize their clinical courses.

Methods: Patients admitted at Children's Hospital of Colorado between January 1, 2015, and March 23, 2023, who had both cannabis use (F12.1-F12.99) and chronic vascular disorders of the intestine (K55.1) on their problem lists were identified from the electronic medical record using ICD 10 codes. Nine met criteria for SMAS and chronic cannabis use.

Results: Six of nine patients were female. The most common presenting symptoms were nausea (9), vomiting (9), and weight loss (9). Four patients received cannabis cessation support. Patients lost a mean of 6.0 kg, had an average body mass index (BMI) of 15.61 percentile (17.7 kg/m²) and BMI Z-scores of -1.5. Symptoms were present for a mean of 19.6 weeks before diagnosis.

Conclusions: Adolescents who experience nausea, vomiting, abdominal pain, and weight loss in association with chronic cannabis use and/or SMAS can present with confusing and overlapping symptoms. Our cohort was described as having CHS with co-occurring SMAS on imaging. Although the etiology of weight loss cannot be definitively ascertained, we postulate that the recurring emetic attacks from CHS led to weight loss resulting in SMAS. Improvement in diagnostic criteria for this population as well as cannabis cessation programming may aid in deceasing morbidity from these co-occurring conditions.

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: To investigate associations between the rectoanal inhibitory reflex (RAIR), type of congenital anorectal malformations (ARMs), type of operation that patients with ARM had undergone, and objectively measured fecal incontinence and defecation problems.

Methods: We retrospectively included 69 pediatric patients with ARM. All underwent anorectal function tests at the University Medical Center of Groningen during the last 10 years. We assessed anorectal physiology using the Rome IV criteria and anorectal function tests.

Results: We found the reflex in 67% of patients and all types of ARMs. All patients who had not been operated on, and those who had undergone less extensive surgery possessed the reflex. In contrast, patients who underwent posterior sagittal anorectoplasty, 44% possessed it. We found no difference between mean rectal volumes in patients with and without the reflex (251  vs. 325 mL, respectively, p = 0.266). We found that over time, patients without the reflex seemed to develop significantly higher rectal volumes than patients who had it. We did not find a significant difference between the reflex and fecal incontinence; however, it seems that the absence of the reflex, resting anal sphincter pressure, and fecal incontinence are related.

Conclusion: The RAIR seems present in patients with ARM irrespective of their malformation type. Corrective surgery, however, may impair this reflex. Seemingly, its absence results in constipation with enlarged rectal volumes and fecal incontinence. Every effort should be made to preserve this reflex during surgery and to use extensive surgical procedures as sparingly as possible.

Background: Prevalence of metabolic dysfunction-associated steatotic liver disease (MASLD), previously known as nonalcoholic fatty liver disease (NAFLD), and its sequelae of more severe forms such as metabolic dysfunction-associated steatohepatitis (MASH) is rapidly increasing in children with the rise in obesity. Successful and sustainable treatments for MASLD are lacking in children. We determined the therapeutic effect of N-acetyl cysteine (NAC) on biomarkers of oxidative stress, inflammation and insulin resistance (IR), liver enzymes, liver fat fraction (LFF) and liver stiffness (LS) in children with obesity and biopsy-confirmed MASLD.

Methods: Thirteen children (n = 13; age: 13.6 ± 2.8 years; NAS score >2) underwent a double-blind, placebo-controlled trial of NAC (either 600 or 1200 mg NAC/day) or placebo for 16 weeks. Measurements included LFF (magnetic resonance imaging), LS (ultrasound elastography), and body composition. Erythrocyte glutathione (GSH), liver enzymes, insulin, glucose, adiponectin, high-sensitivity c-reactive protein (hs-CRP), and interleukin-6 (IL-6) were also measured. homeostasis model assessment for insulin resistance (HOMA-IR) was calculated.

Results: Sixteen-week NAC treatment improved (baseline adjusted between-group p < .05 for all) markers of inflammation (IL-6 and hs-CRP), oxidative stress (GSH), and IR (HOMA-IR) and reduced liver enzymes, LFF and LS. Body weight and body composition did not show beneficial changes.

Conclusions: Sixteen-week NAC treatment was well tolerated in children with obesity and MASLD and led to improvements in oxidative stress, inflammation and IR and liver outcomes. The results from this pilot study support further investigation of NAC as a therapeutic agent in children with MASLD.

Objectives: Autoimmune gastritis (AIG) is a rare chronic inflammatory disorder with potential long-term sequelae including gastric neoplasia. There is limited data on the natural history of pediatric AIG. We aimed to characterize the clinical course and outcomes of children with AIG.

Methods: This was a multicenter retrospective study that included pediatric patients diagnosed with AIG between January 1, 2000 and December 31, 2021. Diagnosis of AIG was based on the demonstration of histological corpus-predominant atrophic gastritis, with or without positive antiparietal cell (APCA) or anti-intrinsic factor (IF) antibodies. Demographic, clinical, laboratory, endoscopic, and histologic data were retrieved, along with follow-up data.

Results: Thirty-three patients, (23 females [69.7%], median age 12.0 [interquartile range 7.0-15.0] years at diagnosis) were identified. Twenty-two patients (66.7%) had positive APCA and/or anti-IF serology. The most common presenting manifestation was iron deficiency anemia (75%), and accompanying autoimmune disorders were significantly more common in patients with positive serology (62% vs. 18%, p < 0.05). Pseudo-pyloric or intestinal-type metaplasia was present at diagnosis in eight patients (24%), and 11 additional patients (33%) developed metaplasia during a median follow-up time of 27 (17.5-48.3) months. One patient developed a type 1 gastric neuroendocrine tumor. Helicobacter pylori was identified in only one patient, while two patients had prior eradication. Endoscopic and histologic improvements weren't identified in any patients.

Conclusions: AIG should be considered in patients with autoimmunity and resistant iron-deficiency anemia. H. pylori infection may not be associated with pediatric AIG. The development of neuroendocrine tumor in one patient, and the high rates of metaplasia, highlight the importance of surveillance.

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.