A pilot feasibility study of an ultrasound-based tool to assess muscle mass in children with liver disease.

BACKGROUND: Sarcopenia is associated with adverse outcomes following liver transplantation, and at-risk children must be identified and prehabilitated. The gold standard for assessing sarcopenia in end-stage liver disease (ESLD) is CT assessment of the total Psoas Muscle Area (tPMA). However, radiation exposure and sedation requirements make this approach impractical for children. The bilateral anterior thigh thickness (BATT) is the cumulative measurement of the rectus femoris and vastus intermedius muscles by ultrasound and has been used to identify sarcopenia in adults. There are no studies assessing muscle mass in children using ultrasound. We hypothesized that measuring BATT with ultrasound in children with ESLD is feasible and is associated with sarcopenia. METHODS: A prospective pilot feasibility study of patients with ESLD on the liver transplantation waitlist and age-matched healthy controls. BATT was measured by a single operator using ultrasound. tPMA indices were determined by CT imaging, along with clinical and anthropometric data. RESULTS: Thirty children were studied between September 2021 and December 2022, 15 listed patients aged 4-30 months, and 15 controls aged 4-32 months. No major technical challenges or complications were encountered while performing the ultrasounds. Median BATTs of 30.8 mm (interquartile range: 27.9-32.8 mm) versus 32.7 mm (interquartile range: 31.8-36.9 mm) were demonstrated in the ESLD and control groups, respectively, and p = 0.01. A positive correlation (R = 0.603) was demonstrated between BATT and tPMA at the L4-5 level among patients with ESLD. No correlation was observed between BATT and anthropometrics. CONCLUSIONS: This study yields novel data on the feasibility of ultrasound to measure mid-thigh thickness in children with ESLD and suggests a correlation between BATT and tPMA, the gold standard for diagnosing sarcopenia. It sets the stage for ultrasound as a simple, noninvasive, and easily repeatable tool for assessing sarcopenia in children.

Unmasking a New Type of Gastrointestinal Foreign Body in the COVID-19 Era.

The incidence of foreign body ingestion has increased during the coronavirus disease 2019 pandemic. As face masks became increasingly available, we report a case of accidental ingestion of the metal strip insert of a surgical mask. After initially progressing, its progress halted after 24 hours. This case highlights the challenges of timing the endoscopic removal of long objects, especially considering the reduced endoscopic availability during the pandemic. Despite only causing local trauma, the strip was impacted at the duodenojejunal flexure with the potential to cause obstruction. Limiting morbidity relies on urgent removal and prevention of similar ingestions by emphasizing the safe use and storage of masks.

Portal plate bile duct diameter in biliary atresia is associated with long-term outcome.

PURPOSE: Kasai portoenterostomy (KPE) is the only treatment currently available for biliary atresia (BA). Age at KPE and surgical experience are prognostic factors for a successful KPE. Here, we aimed to assess whether the size of bile ductules at the porta hepatis during KPE correlates with KPE success and transplant-free survival (TFS). METHODS: A retrospective analysis of patients diagnosed with BA during 2000-2019. Porta hepatis biopsies were reviewed for diameters of five representative ducts, and a mean ductal diameter (MDD) was calculated. Laboratory values including pre- and postoperative bilirubin levels were analyzed. RESULTS: The cohort included 77 patients; for 33, ductal plate biopsy was available. KPE was successful in six of eight patients with MDD ≥ 50 µm, and in five of 25 with MDD < 50 µm, p = 0.008, OR = 12.0 (95% CI 1.83-78.3). Ten-year survival with native liver was higher in patients with MDD ≥ 50 µm than in patients with MDD < 50 µm, p < 0.001, HR 0.038 (95% CI 0.007-0.207). Direct bilirubin < 1 mg/dl 3 months post-KPE was associated with improved 2-year post-KPE TFS (27.7% vs. 13.9%, p < 0.0001). CONCLUSIONS: MDD ≥ 50 µm correlates with KPE success and a higher rate of TFS. Direct bilirubin < 1 mg/dl 3 months post-operation may serve as a marker of successful biliary excretion, and a predictor of 2-year TFS.

Antibodies Against Glycoprotein 2 Are Specific Biomarkers for Pediatric Crohn's Disease.

BACKGROUND: Serological markers can assist in accurate differentiation between Crohn's disease (CD) and ulcerative colitis (UC). One such marker is anti-glycoprotein 2 (anti-GP2) which was shown to be a specific marker for CD in adult patients. The aim of our study was to assess the utility of anti-GP2 and GP2 as biomarkers for pediatric CD, and determine whether they correlate with disease activity. METHODS: Serum samples were tested by ELISA for anti-GP2 isoform 4 IgG and IgA, and also for GP2. Results were correlated with demographic and clinical data. RESULTS: The cohort consisted of 53 pediatric patients with CD, 42 with UC, and 53 controls. Levels of anti-GP2 were significantly increased in pediatric patients with CD in comparison with patients with UC, and control subjects, with high positive predictive value for both IgG and IgA (97.9% and 82.6%, respectively). While specificity of anti-GP2 IgG and IgA was very high (98.7% and 90.0%, respectively), sensitivity was low (42.0% and 35.5% for IgG and IgA, respectively). In CD, anti-GP2 correlated with disease activity, and decreased in treatment-naïve patients following successful induction therapy. A higher IgA anti-GP2 was also demonstrated in patients with ileo-colonic involvement, and was associated with a younger age. Finally, positive GP2 level was identified in only 1/211 serum samples. CONCLUSIONS: A positive anti-GP2 level is highly associated with CD, while a negative result does not exclude CD. Additional studies are required to determine whether these markers can be used in pediatric patients with CD for risk stratification.

[APPROACH TO VERY EARLY ONSET INFLAMMATORY BOWEL DISEASE].

INTRODUCTION: Inflammatory bowel diseases (IBD) are chronic inflammatory disorders that develop in genetically susceptible subjects as a result of a dysregulated immune response to environmental triggers and microbial dysbiosis. Most cases manifest in adulthood or in adolescence. In a minority of patients, the disease manifests in the first 5-6 years of life, defined as very early-onset IBD (VEO-IBD). In some of these patients, a monogenic disorder resulting from deleterious mutations in immune-mediated or epithelial genes can be identified. Atypical manifestations, including young age at diagnosis, recurrent infections, accompanying autoimmune disorders, severe medical-refractory disease, malignancy, consanguinity and other features should prompt a detailed genetic and immune work-up. In this review, we will discuss the approach to patients with VEO-IBD and those with atypical features and review the main groups of monogenic IBD. We will also discuss the emerging use of genetic testing to facilitate these diagnoses and provide personalized care for selected patients.