Macroenzymes as a reason for aminotransferases flare in cystic fibrosis patients on CFTR modulators therapy - Report of three cases.

It has been shown that macro-ALT/macro-AST cause false increase of ALT/AST activity in standard laboratory testing. This short communication presents a group of cystic fibrosis subjects who developed aminotranferases flare a few months after initiation of CFTR modulators therapy. Patients did not present any clinical signs or symptoms of liver failure and differential examination did not show any underlying liver disease.

Increased aminotransferases do not necessarily mean hepatotoxicity of CFTR modulator therapy.

Aim Of The Study: The presence of macroenzymes may mimic treatment related hepatotoxicity.

Material And Methods: We present a female subject who developed high alanine aminotransferase (ALT)/aspartate aminotransferase (AST) activity during cystic fibrosis transmembrane regulator (CFTR) modulator therapy.

Results: The differential work-up did not show any underlying liver disease.

Identification of Iatrogenic Perforation in Pediatric Gastrointestinal Endoscopy.

Objectives: Iatrogenic viscus perforation in pediatric gastrointestinal endoscopy (GIE) is a very rare, yet potentially life-threatening event. There are no evidence-based recommendations relating to immediate post-procedure follow-up to identify perforations and allow for timely management. This study aims to characterize the presentation of children with post-GIE perforation to better rationalize post-procedure recommendations.

Improving nutrition in cystic fibrosis: A systematic literature review.

With increasing life expectancy of patients with cystic fibrosis (CF), gastrointestinal manifestations of the disease have been increasingly brought into focus. This was a systematic review of the PubMed database and ongoing phase III clinical trials that aimed to summarize recent (published after June 1 2016) studies reporting the effects of nutritional interventions on anthropometric measures (weight, height, and body mass index) in patients with CF. Two ongoing trials and 40 published studies (18 interventional and 22 observational) were identified.

Recurrence of Primary Sclerosing Cholangitis After Liver Transplant in Children: An International Observational Study.

Background And Aims: Recurrent primary sclerosing cholangitis (rPSC) following liver transplant (LT) has a negative impact on graft and patient survival; little is known about risk factors for rPSC or disease course in children.

Approach And Results: We retrospectively evaluated risk factors for rPSC in 140 children from the Pediatric PSC Consortium, a multicenter international registry. Recipients underwent LT for PSC and had >90 days of follow-up.

The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children.

Background And Aims: Disease progression in children with primary sclerosing cholangitis (PSC) is variable. Prognostic and risk-stratification tools exist for adult-onset PSC, but not for children. We aimed to create a tool that accounts for the biochemical and phenotypic features and early disease stage of pediatric PSC.

Assessing the Validity of Adult-derived Prognostic Models for Primary Sclerosing Cholangitis Outcomes in Children.

Background: Natural history models for primary sclerosing cholangitis (PSC) are derived from adult patient data, but have never been validated in children. It is unclear how accurate such models are for children with PSC.

Methods: We utilized the pediatric PSC consortium database to assess the Revised Mayo Clinic, Amsterdam-Oxford, and Boberg models.

New colon anatomy-related ratios used to predict the course of colonoscopy in children.

Background: In children, colonoscopy is a safe procedure, although it is more difficult to perform in patients whose body mass index (BMI) is under 25.

Objectives: The aim of the study was to establish the relationship between children's age, body mass and height and incomplete colonoscopies due to colon anatomy.

Material And Methods: A retrospective evaluation of diagnostic endoscopies in 403 children aged 3-18 years (192 girls and 211 boys) was performed.

Tryptophan-kynurenine profile in pediatric autoimmune hepatitis.

The impairment of regulatory T cells (Tregs) is a characteristic feature of autoimmune hepatitis (AIH), and the degradation of tryptophan (Trp) to kynurenine (Kyn), by gamma interferon-induced indoleamine-2,3-dioxygenase-1 (IDO-1), is a central metabolomics check point in the differentiation of Tregs. For this reason, we investigate whether or not Kyn and IDO activity is potentially useful biomarkers in pediatric AIH.Between January 2016 and January 2017, children of AIH type-1 (AIH-1, n = 37), AIH type-2 with liver kidney microsome-1 autoantibodies (AIH-2-LKM-1, n = 8), and autoantibody-negative Wilsons Disease (WD, n = 8) and alpha-1 anti-trypsin deficiency (AATD, n = 10), were enrolled in a cross-sectional survey of Kyn and Trp levels and Kyn/Trp ratios (IDO activity) by HPLC, and neopterin levels by ELISA.

Gamma Glutamyltransferase Reduction Is Associated With Favorable Outcomes in Pediatric Primary Sclerosing Cholangitis.

Adverse clinical events in primary sclerosing cholangitis (PSC) happen too slowly to capture during clinical trials. Surrogate endpoints are needed, but no such validated endpoints exist for children with PSC. We evaluated the association between gamma glutamyltransferase (GGT) reduction and long-term outcomes in pediatric PSC patients.

Efficacy and Safety of Lactobacillus plantarum DSM 9843 (LP299V) in the Prevention of Antibiotic-Associated Gastrointestinal Symptoms in Children-Randomized, Double-Blind, Placebo-Controlled Study.

Objectives: To determine if Lactobacillus plantarum DSM9843 (LP299V) reduces the frequency of antibiotic-associated loose/watery stools and gastrointestinal symptoms, and can be administered safely to children who are prescribed antibiotics.

Study Design: We performed a prospective, double-blind, randomized, placebo-controlled, multicenter, parallel-group study in children receiving outpatient antibiotic therapy in primary healthcare settings. The children were given LP299V/placebo during the antibiotic therapy and for 1 week after the end of treatment.

Double Lumen Polyamide Tube-stent for the Treatment of Recurrent Postcorrosive Esophageal Stenosis.

This article presents the results of endoscopic treatment for recurrent postcorrosive esophageal stenosis with a tube-stent developed at our institution. The tube-stent was implanted in 5 children with corrosive esophageal injury at the age of 2 to 8.5 years after 7 to 64 dilatation sessions during 5 to 118 months.

The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration.

Unlabelled: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death.

Solitary Rectal Ulcer Syndrome in Children: A Case Series Study.

Information on solitary rectal ulcer syndrome (SRUS) in children is limited and based on case reports only. This study was undertaken with the objective of describing the clinical history, symptoms, diagnostic work-up, and treatment of a large case series of pediatric patients with SRUS. The study was multi-center and retrospective.

Endoscopic therapy of oesophageal strictures in children - a multicentre study.

Introduction: Oesophageal strictures are rare in children but may require endoscopic dilation.

Aim: To gather information on centres performing endoscopic oesophageal dilation in Poland.

Material And Methods: The data were obtained from questionnaires concerning the relevant data mailed to 22 paediatric endoscopy centres.

The clinical course of hereditary pancreatitis in children - A comprehensive analysis of 41 cases.

Background: Available data from adult patients do not reflect natural course of hereditary pancreatitis (HP) in children. To date, no study has assessed the clinical course of HP in children.

Objective: To investigate the clinical course of HP in children and compare it to non-HP group with chronic pancreatitis (CP).

MRCP Versus ERCP in the Evaluation of Chronic Pancreatitis in Children: Which Is the Better Choice?

Objectives: To evaluate the diagnostic accuracy of magnetic resonance cholangiopancreatography (MRCP) in the detection of chronic pancreatitis (CP)-specific changes in the pediatric population.

Methods: The study included 48 children with pancreatic disorders subjected to both endoscopic retrograde cholangiopancreatography (ERCP) and MRCP within a 1- to 4-month interval. The sensitivity, specificity, positive predictive value, and negative predictive value of MRCP in the detection of CP-specific changes were determined using ERCP as a diagnostic standard.

Autoantibody Profile of Adult Patients With Childhood Onset Type 2 Autoimmune Hepatitis.

Background: Anti-liver kidney microsome (anti-LKM) autoantibodies are a distinguishing feature of type II autoimmune hepatitis (AIH-2). However, the levels of anti-LKM-1 in adult AIH-2 patients and their role in liver immunopathology remain equivocal. The aim of the study was to survey the autoantibody profile and the activity of liver disease in adult patients diagnosed with AIH-2 at childhood.

Interobserver Agreement on Endoscopic Classification of Oesophageal Varices in Children.

Objectives: Data regarding agreement on endoscopic features of oesophageal varices in children with portal hypertension (PH) are scant. The aim of this study was to evaluate endoscopic visualisation and classification of oesophageal varices in children by several European clinicians, to build a rational basis for future multicentre trials.

Methods: Endoscopic pictures of the distal oesophagus of 100 children with a clinical diagnosis of PH were distributed to 10 endoscopists.

Nasogastric tube as protection for recurrent oesophageal stricture: a case report.

This report presents the case of an 8.5-year-old boy with Down syndrome after experiencing extensive caustic injury to the oesophagus and stomach resulting from the accidental ingestion of concentrated sulphuric acid. The patient had undergone 32 unsuccessful endoscopic oesophageal stricture dilatations and stenting procedures performed over a period of 15 mo following the accident.

Comparative safety and efficacy of proton pump inhibitors in paediatric gastroesophageal reflux disease.

Gastroesophageal reflux is one of the most common reasons for referrals to paediatricians or paediatric gastroenterologists. Gastric acid-buffering agents, mucosal surface barriers and gastric anti-secretory agents are the main groups of medications currently used for treating gastroesophageal reflux disease (GERD) in children. Recently, the use of proton pump inhibitors (PPIs) for the treatment of GERD in children has increased considerably.