Judith Pichler

• MD
• University of Vienna

Background/objective: The high incidence of liver disease associated with intravenous soybean lipid has led to development and use of alternative intravenous lipid emulsions (ILEs). The aim of this study was to compare two new/mixed ILEs: a medium-chain triglyceride (MCT) combined with soybean (i.e., Lipofundin) and a combination of both these lip...

Exome sequencing (ES) has identified biallelic kinesin family member 12 (KIF12) mutations as underlying neonatal cholestatic liver disease. We collected information on onset and progression of this entity. Among consecutively referred pediatric patients at our centers, diagnostic ES identified 4 patients with novel, biallelic KIF12 variants using t...

(1) Background: Progressive familial intrahepatic cholestasis (PFIC) is a rare cause of liver failure. Surgical biliary diversion (SBD) and ileal bile salt inhibitors (IBAT) can delay or prevent liver transplantation (LTX). A comparison of the two methodologies in the literature is lacking. The combination has not been investigated. (2) Methods: We...

Aim Anti tumor necrosis factor (TNF)‐α drugs are effective treatments for the management of moderate/severe Crohn's disease (CD) but treatment failure is common. In the treatment of paediatric CD, there are no data about the use of a third introduced subcutaneous TNF‐antibody golimumab. Methods We evaluated the efficacy of golimumab for adolescent...

Background Inducing and maintaining clinical remission in children with Crohn’s disease (CD) is associated with treatment with antibody to tumor necrosis factor (TNF)-α such as infliximab or adalimumab. In the treatment of paediatric CD, there are no data about the use of a third introduced subcutaneous TNF-antibody, golimumab, Methods We evaluate...

Background: Vedolizumab is safe and effective in adult patients with Crohn's disease (CD) and ulcerative colitis (UC); however, data in children with inflammatory bowel disease (IBD) are scarce. Therefore, we evaluated vedolizumab use in a cohort of Austrian paediatric patients with IBD. Methods: Twelve patients (7 female; 7 CD; 5 UC), aged 8-17...

Background & aims: Pulmonary embolism (PE) is a complication of parenteral nutrition (PN) with a prevalence of 35% in children. In 2003 new intravenous lipid emulsions (ILEs) with MCT, olive and/or fish oil in addition to soybean oil were introduced. The aim was to compare the incidence of PE before and after introduction. Methods: 327 surveilla...

Background: The association between inflammatory bowel disease and joint involvement is well established. There is a paucity of data describing histopathological features of the gut in relation to juvenile idiopathic arthritis (JIA). Methods: We retrospectively identified 33 (21 male) children aged from 3-16 years with JIA (11 oligoarthritis, 5...

In Celiac disease (CD), cytoskeletal integrity of intestinal cells is disrupted by gliadin exposure. This study investigates the role of heat shock protein Hsp70 during cytoskeletal recovery in CD by assessing its induction and effects on junctional proteins. Using an in-vitro model of CD, cytoskeletal injury and recovery was assessed in gliadin ex...

To study whether adalimumab (ADA) was associated with improvement in growth, bone mineral density (BMD) and bone metabolism. In children with Crohn's disease (CD) there is a high prevalence of growth failure and reduced BMD. Treatment with infliximab is associated with an improvement in growth. Anthropometry, paediatric CD activity index (PCDAI), b...

In Shwachman-Diamond syndrome (SDS), pancreatic insufficiency can lead to malabsorption of fat-soluble vitamins and trace elements. The aim of this study was to assess the serum concentrations of vitamins A and E, zinc, copper, and selenium and their deficiencies. This retrospective review was performed in 21 children (12 were male; median age, 7.8...

The aetiology of biliary liver disease in children with intestinal failure (IF) treated with long-term parenteral nutrition (PN) is multifactorial. Risks include the lipid component of PN. Our aim was to compare prevalence and outcome of gallstones with different types of intravenous lipids. Liver and biliary tract imaging and relevant clinical det...

Background/objective: The aim of the study was to compare the prevalence of undernutrition in children on presentation to hospital and on discharge. Methods: On a screening week, 141 children aged from birth to 17 years who were hospitalised for ⩾72 h were reviewed on presentation and discharge or after 3 months (if still in hospital) by auditin...

Children with inflammatory bowel disease (IBD) have a high prevalence of growth retardation and low bone mineral density (BMD). This retrospective study investigated whether the start of infliximab treatment (IFX) was associated with improvement of growth and bone health. Anthropometry, BMD and bone markers were measured 1 year before and after the...

Outcome of children with intestinal failure (IF) has improved on treatment with parenteral nutrition (PN). The effects of PN and IF on body composition (BC) are unknown. The aim was to review BC in PN-treated children and those weaned off and to compare with reference data. Children on long-term/home PN underwent measurement of regional fat mass (F...

Background: Children with chronic intestinal failure (IF) treated with long-term parenteral nutrition (PN) may present with low bone mineral density (BMD). The cause may reflect small body size or suboptimal bone mineralization. Objective: We assessed growth and bone health in children with severe IF. Design: Height, weight, and fracture histo...

Background & aims: Catheter-related-blood-stream-infection (CRBSI) might be prevented by optimal catheter connector antisepsis in children with intestinal failure on parenteral nutrition (PN). We changed the disinfectant used from isopropanol 70% to chlorhexidine 2% in 70% isopropanol, which leaves a residue of chlorhexidine on the connector. Met...

OBJECTIVE AND AIM: Liver disease is a potentially life-threatening complication of intravenous/parenteral nutrition (PN). Our aim was to determine the incidence, aetiology and outcome of intestinal failure-associated liver disease (IFALD) in hospitalised children treated with long-term PN (>27 days). Over 4 years all long-term intestinal failure (I...

To assess feasibility of a finger prick-based kit as method for self-testing of first and second-degree relatives of coeliac disease (CD) patients. A total number of 379 subjects were invited to participate in this study, consisting of 197 first-degree and 182 second-degree relatives of CD patients. The self-testing kit (Biocard™) was sent out with...

During the last 3 decades the use of parenteral nutrition (PN) and the aggressive introduction of enteral feeding in daily practice have transformed the outcome for even the sickest of these infants. More than 90% of infants and children now survive after extensive small bowel resection in the neonatal period. During the last 3 decades the use of p...

Infections accompany intestinal failure (IF) more commonly in children than in adults, with reported incidences of 2% to 29%. Appropriate care of the central venous catheter is the most important factor preventing infections; but in addition, bacteria translocate from the dysmotile gut as a possible source of septicemia. The aim of this retrospecti...

Cardiovascular events are among the most frequent causes for long-term morbidity and mortality in children after renal transplantation. The aim of this study was to analyze the effects of post-transplant changes in arterial hypertension, as assessed by 24-h ambulatory blood pressure measurement (ABPM), on myocardial architecture, as assessed by ech...