Björn Fischler
A5020830252- Pediatric Hepatobiliary Diseases and Treatments
- Drug Transport and Resistance Mechanisms
- Neonatal Health and Biochemistry
- Gallbladder and Bile Duct Disorders
- Liver Disease and Transplantation
- Liver Disease Diagnosis and Treatment
- Organ Transplantation Techniques and Outcomes
- Cytomegalovirus and herpesvirus research
- Liver Diseases and Immunity
- Congenital Anomalies and Fetal Surgery
- Hepatitis B Virus Studies
- Intestinal Malrotation and Obstruction Disorders
- Metabolism and Genetic Disorders
- Pharmacological Effects and Toxicity Studies
- Hepatitis C virus research
- Childhood Cancer Survivors' Quality of Life
- Pancreatic function and diabetes
- Transplantation: Methods and Outcomes
- Infant Nutrition and Health
- Renal Transplantation Outcomes and Treatments
- Clinical Nutrition and Gastroenterology
- Parvovirus B19 Infection Studies
- Digestive system and related health
- Folate and B Vitamins Research
- Gastrointestinal motility and disorders
Karolinska University Hospital
2015-2024
Karolinska Institutet
2015-2024
ERN RARE-LIVER
2020-2023
ERN GUARD-Heart
2020-2023
Children's Hospital Agia Sophia
2021
National and Kapodistrian University of Athens
2021
Wilhelmina Children's Hospital
2020
Kone (Switzerland)
2012
Stockholm University College of Music Education
2002-2005
Pediatrics and Genetics
2002
Cholestatic jaundice in infancy affects approximately 1 every 2500 term infants and is infrequently recognized by primary providers the setting of physiologic jaundice. always pathologic indicates hepatobiliary dysfunction. Early detection care physician timely referrals to pediatric gastroenterologist/hepatologist are important contributors optimal treatment prognosis. The most common causes cholestatic first months life biliary atresia (25%-40%) followed an expanding list monogenic...
Hepatocellular carcinoma (HCC) is rare in young children. We attempted to see if immunohistochemical and mutational-analysis studies could demonstrate that deficiency of the canalicular bile acid transporter salt export pump (BSEP) mutation ABCB11 , encoding BSEP, underlay progressive familial intrahepatic cholestasis (PFIC)—or “neonatal hepatitis” suggesting PFIC—that was associated with HCC studied 11 cases pediatric setting PFIC or PFIC. Archival liver were retrieved immunostained for...
Progressive familial intrahepatic cholestasis (PFIC) is a group of inherited paediatric liver diseases resulting from mutations in genes that impact bile secretion. We aimed to evaluate the effects odevixibat, an ileal acid transporter inhibitor, versus placebo children with PFIC.Patients eligible for this 24-week, randomised, double-blind, completed, phase 3 study were outpatients diagnosed PFIC1 or PFIC2 who had pruritus and elevated serum acids at screening. Patients randomly assigned...
Background: In addition to earlier reports on the association between viral infections and intrahepatic neonatal cholestasis, in recent studies, investigators have suggested a similar link extrahepatic biliary atresia. Methods: Fifty-nine cholestatic infants (mean age 8 weeks) were investigated for signs of infection with large spectrum viruses. Twenty-one had atresia, 38 cholestasis. The virologic methods included serologic investigation 59 54 mothers, virus isolation from stools (49...
Clinical hepatocyte transplantation is hampered by low engraftment rates and gradual loss of function resulting in incomplete correction the underlying disease. Preconditioning with partial hepatectomy improves animal studies. Our aim was to study safety efficacy preconditioning clinical transplantation. Two patients Crigler-Najjar syndrome type I underwent liver resection followed A transient increase growth factor seen, suggesting that this procedure provides a regenerative stimulus. Serum...
Background and Aims: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, approved pharmacologic therapy for cholestatic in ALGS. Since long-term placebo-controlled studies are not feasible or ethical children rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials aligned harmonized natural history cohort the G lobal AL agille A lliance...
Mutations in ATPase phospholipid transporting 8B1 (ATP8B1) can lead to familial intrahepatic cholestasis type 1 (FIC1) deficiency, or progressive 1. The rarity of FIC1 deficiency has largely prevented a detailed analysis its natural history, effects predicted protein truncating mutations (PPTMs), and possible associations serum bile acid (sBA) concentrations surgical biliary diversion (SBD) with long-term outcome. We aimed provide insights by using the largest genetically defined cohort...
Primary sclerosing cholangitis (PSC) is the classical hepatobiliary manifestation of inflammatory bowel disease (IBD) and a lead indication for liver transplantation (LT) in western world. In this article, we present Consensus Statement on LT practice, developed by dedicated Guidelines' Taskforce European Society Organ Transplantation (ESOT). The overarching goal to provide practical guidance commonly debated topics, including indications timing LT, management bile duct stenosis patients...
The aim of the study was to evaluate cognitive and emotional development after pediatric liver transplantation. A total 21 patients, aged 4-16.9 yr (median 9.6 yr) were tested 1-9 4.2 pretransplant diagnoses included biliary atresia (eight patients), various metabolic diseases (n = 6), acute failure 3), miscellaneous 4). functions with Wechsler preschool primary scale intelligence (WPPSI)-R or for children (WISC)-III according age. Piers-Harris self-concept evaluation human figure drawings...
<h3>AIM</h3> To time the onset of cytomegalovirus (CMV) infection in patients (n=39) with CMV associated neonatal cholestasis by analysing DNA on Guthrie cards sampled at 3 days age. <h3>METHODS</h3> was diagnosed serology/urine isolation or detection (polymerase chain reaction) liver biopsy specimens. In order to dry blood filter paper discs were punched out from stored cards. After phenol–chloroform extraction detected nested polymerase reaction. <h3>RESULTS</h3> All control children (n=8)...
ABSTRACT Objectives: The aim of this study was to examine whether reversion histological fibrosis followed partial external biliary diversion (PEBD) in patients with progressive familial intrahepatic cholestasis (PFIC); the duration cholestatic episodes after PEBD influenced evolution fibrosis; and genotyping helpful predicting outcome PEBD. Patients Methods: Children PFIC who underwent were investigated genetic, biochemical, anthropometric standard methods. Serial liver specimens assessed...
To investigate tacrolimus (Tac)-based treatment in juvenile autoimmune hepatitis (AIH). Twenty patients (13 girls; age, 8-17 years; median, 13.25 years) with AIH were treated two daily oral doses of Tac. Six them had advanced liver disease and/or cirrhosis.Drug concentrations blood measured regularly, and the target trough levels 2.5-5 ng/mL. The followed up for 1 year. Their clinical, biochemical, immunological histological status was obtained at baseline after year.In three cases, Tac...
Hepatitis E virus (HEV) is endemic in large parts of the developing world. Waterborne transmission genotypes 1 or 2 commonly causes acute hepatitis, which usually self-limited healthy individuals. In addition, HEV infections also occur outside areas, mostly related to foodborne genotype 3. A growing number publications last decade have reported chronic infection progressing cirrhosis immunosuppressed patients. It has been suggested that may via contaminated blood products. This publication...