Pediatric Liver Kidney Alimentary Nutrition and Transplantation (PELIKANTRA)

Group:

Runar Almaas, group leader, Associate Professor

Anna Kristina Bjerre, Professor

Gareth Sullivan, Senior Researcher

Sean Harrison, Post doc

Ann Christin Gjerstad, PhD

Embjørg Wollen, PhD

Saphira Baumgarten, PhD

Barbora Smolkova, PhD

Anniken Østensen, PhD student

Anders Ziegler, PhD Student

Hanna Elmi, PhD Student

Ingvild Kindem, PhD student

Marie Kildahl, PhD student

Funding:

Eckbos legater
HSØ
Gidske Sørensens legater
Barnestiftelsen

Ongoing Projects:

Complications and long-term follow-up after organ transplantation in children (several projects on patients transplanted with kidney or liver) including:

- metabolic risk factors and inflammation after kidney transplantation in children

- allergies and lung function in children with liver transplants

- motor skills and cognitive function after transplantation

- Transition: Adherence and graft loss in young people with kidney transplants

Induced pluripotent stem cells and development of organoids. Several projects developing models for the investigation of fat metabolism, cholestasis development (PNALD) and living organoids.
Cholestatic diseases.
Studies on cholestasis disease with genetic investigations and cell models
Early diagnosis of cholestasis and bile duct atresia
Pharmacokinetics in organ transplant children
Project investigating pharmacokinetics in children who have had a liver or kidney transplant, collaboration with Professor Anders Aasberg.
MitraChild: "Tacrolimus measured in capillary volumetric microsamples in pediatric patients - a cross-validation study".
Yotac study:

- Part 1: A feasibility study for improved adherence in adolescents by combining at-home blood microsampling for tacrolimus measurements and the medication management smart phone application.

- Part 2: The effect of self-management on adherence and other clinical outcomes in kidney- and livertransplanted adolescents.

Prospective review of data from the Norwegian Kidney Biopsy Register combined with data from the Norwegian Nephrology Register.
ABO incompatible living donor kidney transplantations in pediatric patients: a collaborative study from the Nordic Pediatric Renal Transplantation Study Group (NPRTSG)
"Genetic factors in children with congenital malformations in the kidneys and urinary tract" - multicenter study from Hannover

National and international multicenter studies

Autoimmune hepatitis in the Nordic region (Collaboration Norway, Denmark, Sweden, Finland, Estonia).
Acute liver failure in neonates (Collaboration Denmark, Norway, Sweden, USA)
Genetic cholestasis disease (Collaboration Norway, Czech Republic)
EVITA study - Epstein-Barr Virus Infection monitoring in kidney transplant recipients - Early identification of increased risk of infection and cancer for individualized immunosuppression. Cooperation with Odense and Aarhus, DK. Prospective multicenter study.

Cooperation

National:

OUS: Newborn Screening, Department of Pediatric Surgery, Department of Radiology, Department of Gastromedicine, Dept. of Medical Biochemistry, Dept. of Medical Genetics, Dept. of Transplantation Medicine, Norwegian Nephrology Register, Benedicte Stavik, Per Morten Sandset, Espen Melum
Haukeland: Norwegian Kidney Biopsy Register, Department for Children and Young People

International:

ESPN/ERA-EDTA Registry
The Nordic Liver Transplant Registry
Nordic AILD (Nordic autoimmune liver disease); Marianne Hørby (Copenhagen), Bjørn Fischler (Stockholm), Laura Merras-Salmio (Helsinki), Audur Gudjonsdottir (Gothenburg)
Nordic Pediatric Surgery Study Consortium
NPRSTG (Nordic Pediatric Renal Study Transplant Group)
Professor Jaroslav Mokry, Charles University, Czech Republic

Recent defenses:

Svanhildur Haflidadottir: "Food allergy and asthma after liver transplantation in children." 9^th

October 2023

Saphira Felicitas Baumgarten: "iPSC derived building blocks for the fat liver axis." 13^th October 2023

Selected publications:

Almaas R, Atneosen-Åsegg M, Ytre-Arne ME, Melheim M, Sorte HS, Cízková D, Reims HM, Bezrouk A, Harrison SP, Strand J, Hermansen JU, Andersen SS, Eiklid KL, Mokrý J, Sullivan GJ, Stray-Pedersen A. Aagenaes syndrome/lymphedema cholestasis syndrome 1 is caused by a founder variant in the 5'-untranslated region of UNC45A. J Hepatol. 2023 Oct;79(4):945-954.
Sharma K, Asp NT, Harrison S, Siller R, Baumgarten SF, Gupta S, Chollet ME, Andersen E, Sullivan GJ, Simonsen A. Autophagy modulates cell fate decisions during lineage commitment. Autophagy. 2022 Aug;18(8):1915-1931.
Chen A, Kristiansen CK, Høyland LE, Ziegler M, Wang J, Sullivan GJ, Li X, Bindoff LA, Liang KX. POLG mutations lead to abnormal mitochondrial remodeling during neural differentiation of human pluripotent stem cells via SIRT3/AMPK pathway inhibition. Cell Cycle. 2022 Jun;21(11):1178-1193.
Drovandi S, Lipska-Ziętkiewicz BS, Ozaltin F, Emma F, Gulhan B, Boyer O, Trautmann A, Ziętkiewicz S, Xu H, Shen Q, Rao J, Riedhammer KM, Heemann U, Hoefele J, Stenton SL, Tsygin AN, Ng KH, Fomina S, Benetti E, Aurelle M, Prikhodina L, Schijvens AM, Tabatabaeifar M, Jankowski M, Baiko S, Mao J, Feng C, Deng F, Rousset-Rouviere C, Stańczyk M, Bałasz-Chmielewska I, Fila M, Durkan AM, Levart TK, Dursun I, Esfandiar N, Haas D, Bjerre A, Anarat A, Benz MR, Talebi S, Hooman N, Ariceta G; PodoNet Consortium; mitoNET Consortium; CCGKDD Consortium; Schaefer F. Variation of the clinical spectrum and genotype-phenotype associations in Coenzyme Q10 deficiency associated glomerulopathy. Kidney Int. 2022 Sep;102(3):592-603.
Harrison SP, Siller R, Tanaka Y, Chollet ME, de la Morena-Barrio ME, Xiang Y, Patterson B, Andersen E, Bravo-Pérez C, Kempf H, Åsrud KS, Lunov O, Dejneka A, Mowinckel MC, Stavik B, Sandset PM, Melum E, Baumgarten S, Bonanini F, Kurek D, Mathapati S, Almaas R, Sharma K, Wilson SR, Skottvoll FS, Boger IC, Bogen IL, Nyman TA, Wu JJ, Bezrouk A, Cizkova D, Corral J, Mokry J, Zweigerdt R, Park IH, Sullivan GJ. Scalable production of tissue-like vascularized liver organoids from human PSCs. Exp Mol Med. 2023 Sep;55(9):2005-2024. doi: 10.1038/s12276-023-01074-1.
Østensen AB, Skarbø AB, Sanengen T, Line PD, Almaas R Impaired Neurocognitive Performance in Children after Liver Transplantation. J Pediatr. 2022 Apr;243:135-141.e2.