Giant cell hepatitis associated with autoimmune hemolytic anemia (GCH-AIHA) is a rare disorder with unfavorable prognosis, affecting infants and young children. The mortality rate is high, complications of acute liver failure, sepsis, or liver transplantation can be responsible for fatal outcomes. An 18-month-old child who was diagnosed previously with autoimmune hemolytic anemia, developed acute hepatitis and acute liver failure concomitant to the relapse of the disease.
View Article and Find Full Text PDFIn the TRANS-IBD clinical trial, the outcomes are measured with selected validated questionnaires. Cross-cultural and age adaptations of the Self-Efficacy Scale for adolescents and young adults (IBD-SES), the Transition Readiness Assessment Questionnaire (TRAQ), and the Self-Management and Transition Readiness Questionnaire (STARx) were performed. Linguistic and cultural adaptation was carried out with the usage of reliability coefficients (Cronbach's α coefficients, Spearman's rank correlation), and with confirmatory factor analysis (CFA; root Mean Square Error of Approximation [RMSEA], Comparative Fit Index [CFI], and Tucker-Lewis Index [TLI]).
View Article and Find Full Text PDFInt J Environ Res Public Health
December 2022
Malnutrition and inflammatory bowel disease (IBD) are interrelated conditions. Our aim was to assess the prevalence of malnutrition, to compare anthropometric parameters in the evaluation of nutritional status in pediatric IBD, and to investigate the association between anthropometric parameters and disease activity indices (AI). Pediatric patients with newly diagnosed IBD recorded between 2010 and 2016 in the Hungarian Pediatric IBD Registry were included in this cross-sectional study.
View Article and Find Full Text PDFAccording to the Porto criteria, upper endoscopy and ileocolonoscopy with histology for patients with pediatric inflammatory bowel disease (pIBD) are recommended with small bowel imaging (SBI). We aimed to evaluate the adherence to the Porto criteria and biopsy sampling practice and to evaluate the diagnostic yield of magnetic resonance enterography (MRE) first time in a nationwide pIBD inception cohort. Newly diagnosed pIBD cases (ages 0-18 years) are registered in the prospective, nationwide Hungarian Paediatric IBD Registry (HUPIR).
View Article and Find Full Text PDFPurpose: Since little is known about transitional care practices of adolescents with inflammatory bowel diseases (IBD) in Central-Eastern Europe, we aimed to investigate the currently applied transition practices in Hungary.
Design And Methods: A nationwide, multicentre survey was conducted with the invitation of 41 pediatric and adult IBD centres in February 2019.We developed a 34-item questionnaire, which included single- and multiple-choice questions related to the current clinical practice of IBD transition.
Purpose: To evaluate the effect of our current transition process on clinical outcomes in adolescent patients with inflammatory bowel diseases (IBD).
Design And Methods: Two groups of patients with IBD diagnosed in pediatric care were compared retrospectively: Group A patients did not attend the transition process, while Group B patients entered the planned transition service. Outcomes at 1-year after transfer to adult care were evaluated.
Introduction: Genetic polymorphism of thiopurine S-methyltransferase, the key enzyme in metabolism of thiopurines (azathioprine and 6-mercaptopurine) used in the treatment of inflammatory bowel disease, results in different enzyme activities. Decreased enzyme activity causes myelosuppression whereas abnormally high activity results in hepatotoxicity at standard thiopurine doses. Four allele variants (TMPT*2, TMPT*3A, TPMT*3B and TPMT*3C) account for decreased activity in more than 95% of cases.
View Article and Find Full Text PDFExpert Opin Biol Ther
March 2019
Introduction: At present, there is a significant amount of data related to biologics used in pediatric patients with Crohn's disease. This review characterizes the different biological drugs administered in this population.
Areas Covered: Biological therapy of CD, focusing on children, is summarized in this review.
Background: Laboratory markers are essential tools in the follow-up of patients with Crohn's disease (CD). Our aim was to investigate urinary concentrations of orosomucoid in relation to the inflammatory activity of CD and to compare it with clinical indices and conventional laboratory parameters.
Materials And Methods: Blood and urine samples of 86 patients (55 adults and 31 children) with CD and 68 healthy individuals (38 adults and 30 children) as controls were analysed.
J Pediatr Gastroenterol Nutr
January 2016
Objectives: Predicting short-term relapses and long-term prognosis is of utmost importance in paediatric inflammatory bowel disease (IBD). Our aim was to investigate the short-term disease outcome and medication during the first year in a paediatric incident cohort from Hungary. In addition, association laboratory markers and disease activity indices with short-term disease outcome and medication were analysed.
View Article and Find Full Text PDFObjective: Fat malabsorption can occur in celiac disease (CD) owing to villus atrophy and inflammation of small intestinal mucosa. Abnormal fatty acid (FA) status of intestinal mucosa in children with CD was reported earlier. Previously we found significantly reduced availability of n-3 long-chain polyunsaturated fatty acids (n-3 LCPUFA) in children and young adults with type 1 diabetes mellitus (DM).
View Article and Find Full Text PDFObjectives: The aim of the study was to evaluate the incidence, baseline disease characteristics, and disease location based on the Paris classification in pediatric inflammatory bowel disease (IBD) in the Hungarian nationwide inception cohort. In addition, 1-year follow-up with therapy was analyzed.
Methods: From January 1, 2007 to December 31, 2009, newly diagnosed pediatric patients with IBD were prospectively registered.
J Pediatr Gastroenterol Nutr
April 2002
Background: In contrast with other malformations, congenital anomalies of the gastrointestinal tract have been scarcely investigated.
Methods: The prevalence of gastrointestinal malformations with special reference to associated disorders and intrauterine growth was retrospectively analyzed in the newborn infants admitted to the Neonatal Intensive Care Unit of the Department of Pediatrics, University of Pécs, Hungary, in the 14-year period between 1987 and 2000.
Results: Of 4,241 neonates with gastrointestinal malformations, 241 (5.