Publications by authors named "Runar Almaas"
Background: Cholestasis causes accumulation of bile acids (BAs) and changes the circulating bile acid profile. Quantification of circulating BAs in dried bloodspots (DBS) may demonstrate obstruction of bile flow and altered bile acid metabolism in the liver. High sample throughput enables rapid screening of cholestatic diseases.
View Article and Find Full Text PDFArticle Synopsis
- * Key findings reveal that expression of aquaporins (AQP8 and AQP9) increases during cell differentiation, but AQP8 protein levels decrease post-hypoxia despite increased mRNA levels after reoxygenation.
- * The research indicates that post-transcriptional mechanisms, rather than transcription changes, lead to reduced AQP8 protein concentration after hypoxia and reoxygenation events in liver cells.
Article Synopsis
- The study focuses on using parenteral nutrition (PN) for premature newborns, who are at risk for liver disease associated with PN, and aims to develop predictive models for this condition.
- Researchers utilized liver organoids derived from induced pluripotent stem cells to simulate the liver environment and assess the effects of different lipid treatments used in PN.
- Results indicated that both lipid treatments led to lipid accumulation in liver cells and decreased liver function markers, suggesting that liver organoids could serve as a useful platform for testing new, less harmful PN solutions.
The prevailing concept is that gestational alloimmune liver disease (GALD) is caused by maternal antibodies targeting a currently unknown antigen on the liver of the fetus. This leads to deposition of complement on the fetal hepatocytes and death of the fetal hepatocytes and extensive liver injury. In many cases, the newborn dies.
View Article and Find Full Text PDFArticle Synopsis
- The OXR1 gene plays a crucial role in various biological processes, and mutations in this gene have been linked to conditions like cerebellar atrophy and epilepsy in affected patients.
- A novel mutation in OXR1 has been identified, leading to severe developmental issues, including cognitive disabilities and sensitivity to oxidative stress, which can be partially rescued by replacing the affected domain.
- The study highlights the importance of OXR1 in regulating gene expression during neurodevelopment and its potential role in spatial-temporal histone arginine methylation in the brain.
Article Synopsis
- The development of organoid models, especially for the liver, addresses the limitations of traditional 2D cell culture by creating more physiologically relevant systems that better mimic native tissue.
- The new approach eliminates the need for 2D patterning and extracellular matrices, using small molecules to replicate embryonic liver development, resulting in liver-like organoids with complex cellular structures.
- These liver organoids demonstrate critical functions such as drug metabolism and protein production, and can be transplanted into mice, maintaining their functionality and offering potential for applications in therapy, drug testing, and disease modeling.
Article Synopsis
- Aagenaes syndrome is an autosomal recessive condition that includes symptoms like neonatal cholestasis, lymphedema, and giant cell hepatitis, with its genetic cause previously unknown.
- Researchers studied 26 patients and their parents using techniques like whole-genome sequencing and CRISPR to pinpoint genetic variants affecting the disease.
- The study identified a specific variant (c.-98G>T) in the UNC45A gene present in all patients, indicating it as the genetic cause of Aagenaes syndrome, and showed reduced expression of the UNC45A protein in affected individuals.
Objectives: The incidence of food allergy in children following liver transplantation is high and the pathogenesis is still not known. We aimed to identify risk factors for development of food allergies in liver transplant children.
Methods: 107 children and adolescents who underwent liver transplantation from 1999 to 2019 were included.
Objectives: To assess longitudinal neurocognitive development after liver transplantation and evaluate factors associated with neurocognitive performance.
Study Design: Data from neurocognitive testing of 65 children (aged <18 years) who underwent liver transplantation at Oslo University Hospital between 1995 and 2018 were collected from the testing program after transplantation. The parent-reported version of the Behavior Rating Inventory of Executive Function was used to assess executive function.
Purpose: Patients with Fontan circulation are at risk of developing hepatic fibrosis/cirrhosis. The mechanisms and disease development are unclear and early secondary liver cancer is a concern. This study will describe hepatic imaging findings in a national cohort of adolescents with Fontan circulation.
View Article and Find Full Text PDFArticle Synopsis
- The study examined noninvasive methods for detecting hepatic fibrosis in adolescents with Fontan circulation, comparing magnetic resonance (MR) T1 mapping and extracellular volume measurements to traditional shear-wave elastography.
- Results showed that patients with Fontan circulation had significantly higher hepatic T1 times and extracellular volume fractions compared to healthy controls, indicating potential liver issues.
- The findings emphasized that while both MR and elastography techniques provide insights into liver health, they should be used in conjunction rather than interchangeably, as they measure different aspects of liver condition.
Background: Circulatory miRNAs are promising biomarkers. The feasibility of using miRNA from dried blood spots (DBS) was investigated using newborn screening cards from patients with cholestasis-lymphedema syndrome (Aagenaes syndrome) and controls.
Methods: Total amount of miRNA and specific miRNAs from DBS were analyzed.
Introduction: Rare cholestatic liver diseases may cause debilitating pruritus in children. Partial biliary diversion (PBD) may relieve pruritus and postpone liver transplantation which is the only other alternative when conservative treatment fails. The aim was to report long-term outcome after PBD in a population of 26 million people during a 25-year period.
View Article and Find Full Text PDFGrowth differentiation factor 15 (GDF-15) is strongly associated with cardiovascular disease (CVD). The aim of our study was to evaluate plasma and urinary levels of GDF-15 after pediatric renal transplantation (Rtx) and in children with chronic kidney disease (CKD) and its associations to cardiovascular risk factors. In this cross-sectional study, GDF-15 was measured in plasma and urine from 53 children with a renal transplant and 83 children with CKD and related to cardiovascular risk factors (hypertension, obesity, and cholesterol) and kidney function.
View Article and Find Full Text PDFBackground: LTX in children is associated with increased risk of food allergy, and the mechanisms underlying this are unknown. We wanted to study whether plasma cytokine profile differed in liver transplanted children, with and without food allergy, and whether it differed from untransplanted children with CLD.
Methods: Plasma cytokines, total and specific IgE in nine patients with food allergy were compared with 13 patients without food allergy following LTX, and also with seven untransplanted patients with CLD.
Introduction: There is an increasing demand for accurately measured glomerular filtration rate (GFR). Iohexol serum clearance has become a new gold standard, but it is challenging when GFR is low and 24-hour sampling is required for accurate results. The primary aim of this study was to develop an iohexol pharmacokinetic population model for accurate determination of individual GFR using limited sampling for up to 5 hours also when renal function is <40 ml/min.
View Article and Find Full Text PDFObjective: We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables.
Methods: A cross-sectional sample of 64 patients was examined with dual-energy X-ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI).
Objectives: To describe the prevalence and the relationship between asthma, eczema, food allergy, and rhinitis in children after liver transplantation.
Study Design: Children who were liver transplant recipients were investigated to assess whether the high prevalence of food allergies was accompanied by eczema, rhinitis, and asthma. Furthermore, we included 56 children with chronic liver disease to explore the risk of allergy, eczema, and asthma in this group.