Browsing Bergen Open Research Archive by Author "Tøndel, Camilla"

Now showing items 1-20 of 36

    • Accumulation of Globotriaosylceramide in Podocytes in Fabry Nephropathy Is Associated with Progressive Podocyte Loss 

      Najafian, Behzad; Tøndel, Camilla; Svarstad, Einar; Gubler, Marie-Claire; Oliveira, Joao-Paulo; Mauer, Michael (Journal article; Peer reviewed, 2020)
      Background In males with classic Fabry disease, the processes leading to the frequent outcome of ESKD are poorly understood. Defects in the gene encoding α-galactosidase A lead to accumulation of globotriaosylceramide (GL3) ...

    • Accumulation of α-synuclein mediates podocyte injury in Fabry nephropathy 

      Braun, Fabian; Abed, Ahmed; Sellung, Dominik; Rogg, Manuel; Woidy, Mathias; Eikrem, Øystein Solberg; Wanner, Nicola; Gambardella, Jessica; Laufer, Sandra; Haas, Fabian; Wong, Milagros; Dumoulin, Bernhard; Rischke, Paula; Mühlig, Anne; Sachs, Wiebke; von Cossel, Katharina M; Schulz, Kristina; Muschol, Nicole; Gersting, Sören; Muntau, Ania C.; Kretz, Oliver; Hahn, Oliver; Rinschen, Markus; Mauer, Michael; Bork, Tillmann; Grahammer, Florian; Liang, Wei; Eierhoff, Thorsten; Römer, Winfried; Hansen, Arne; Meyer-Schwesinger, Catherine; Iaccarino, Guido; Tøndel, Camilla; Marti, Hans Peter; Najafian, Behzad; Puelles, Victor G; Schell, Christoph; Huber, Tobias B (Journal article; Peer reviewed, 2023)
      Current therapies for Fabry disease are based on reversing intracellular accumulation of globotriaosylceramide (Gb3) by enzyme replacement therapy (ERT) or chaperone-mediated stabilization of the defective enzyme, thereby ...

    • Accuracy of single intravenous access iohexol GFR in children is hampered by marker contamination 

      Eide, Thea Tislevoll; Hufthammer, Karl Ove; Brun, Atle; Brackman, Damien; Svarstad, Einar; Tøndel, Camilla (Journal article; Peer reviewed, 2021)
      Measurement of glomerular filtration rate (GFR) in children by iohexol injection and blood sampling from the contralateral arm is widely used. A single intravenous access for iohexol injection and subsequent blood sampling ...

    • Attack rates amongst household members of outpatients with confirmed COVID-19 in Bergen, Norway: A case-ascertained study 

      Kuwelker, Kanika; Zhou, Fan; Blomberg, Bjørn; Jalloh, Sarah Larteley Lartey; Brokstad, Karl Albert; Trieu, Mai Chi; Bansal, Amit; Madsen, Anders; Krammer, Florian; Mohn, Kristin Greve-Isdahl; Tøndel, Camilla; Linchausen, Dagrunn Waag; Cox, Rebecca Jane; Langeland, Nina (Journal article; Peer reviewed, 2021)
      Background Households studies reflect the natural spread of SARS-CoV-2 in immunologically naive populations with limited preventive measures to control transmission. We hypothesise that seropositivity provides more ...

    • Cardiovascular changes in young renal failure patients 

      Tøndel, Camilla; Marti, Hans Peter (Journal article; Peer reviewed, 2021)
      Progresses in medical care of severe kidney disease and congenital anomalies of kidney and urinary tract make it possible for a higher percentage of young renal failure patients to survive and enter adulthood. There is ...

    • Clinical outcomes among young patients with Fabry disease who initiated agalsidase beta treatment before 30 years of age: An analysis from the Fabry registry 

      Hopkin, Robert J.; Cabrera, Gustavo; Jefferies, John; Yang, Meng; Ponce, Elvira; Brand, Eva; Feldt-Rasmussen, Ulla; Germain, Dominique P.; Guffon, Nathalie; Jovanovic, Ana; Kantola, Ilkka; Karaa, Amel; Martins, Ana; Tøndel, Camilla; Wilcox, William R; Yoo, Han-Wook; Burlina, Alessandro; Mauer, Michael (Journal article; Peer reviewed, 2023)
      Background Clinical manifestations of classic Fabry disease (α-galactosidase A deficiency) usually occur in childhood, while complications involving major organs typically develop in adulthood. Outcomes of Fabry-specific ...

    • Clinical relevance of globotriaosylceramide accumulation in Fabry disease and the effect of agalsidase beta in affected tissues 

      Tøndel, Camilla; Thurberg, Beth L.; DasMahapatra, Pronabesh; Lyn, Nicole; Maski, Manish; Batista, Julie L.; George, Kelly; Patel, Hiren; Hariri, Ali (Journal article; Peer reviewed, 2022)
      Fabry disease (FD) is a rare lysosomal storage disorder, characterized by a reduction in α-galactosidase A enzyme activity and the progressive accumulation of globotriaosylceramide (GL3) and its metabolites in the cells ...

    • Consensus recommendations for the treatment and management of patients with Fabry disease on migalastat: a modified Delphi study 

      Bichet, Daniel G; Hopkin, Robert J.; Aguiar, Patricio; Allam, Sridhar R; Chien, Yin-Hsiu; Giugliani, Roberto; Kallish, Staci; Kineen, Sabina; Lidove, Olivier; Niu, Dau-Ming; Olivotto, Iacopo; Politei, Juan Manuel; Rakoski, Paul; Torra, Roser; Tøndel, Camilla; Hughes, Derralynn A. (Journal article; Peer reviewed, 2023-09-01)
      Objective: Fabry disease is a progressive disorder caused by deficiency of the α-galactosidase A enzyme (α-Gal A), leading to multisystemic organ damage with heterogenous clinical presentation. The addition of the oral ...

    • Durable immune responses after BNT162b2 vaccination in home-dwelling old adults 

      Hansen, Lena; Brokstad, Karl Albert; Bansal, Amit; Zhou, Fan; Bredholt, Geir; Onyango, Therese Bredholt; Sandnes, Helene Heitmann; Elyanow, Rebecca; Madsen, Anders; Trieu, Mai Chi; Sævik, Marianne; Søyland, Hanne; Olofsson, Jan Stefan; Vahokoski, Juha; Ertesvåg, Nina Urke; Fjelltveit, Elisabeth Berg; Shafiani, Shahin; Tøndel, Camilla; Chapman, Heidi; Kaplan, Ian; Mohn, Kristin Greve-Isdahl; Langeland, Nina; Cox, Rebecca Jane (Journal article; Peer reviewed, 2023)
      Objectives Elderly are an understudied, high-risk group vulnerable to severe COVID-19. We comprehensively analyzed the durability of humoral and cellular immune responses after BNT162b2 vaccination and SARS-CoV-2 infection ...

    • Early Induction of Cross-Reactive CD8+ T-Cell Responses in Tonsils After Live-Attenuated Influenza Vaccination in Children 

      Mohn, Kristin Greve-Isdahl; Brokstad, Karl Albert; Islam, Shahinul; Oftung, Fredrik; Tøndel, Camilla; Aarstad, Hans Jørgen; Cox, Rebecca Jane (Journal article; Peer reviewed, 2020)
      Background Live-attenuated influenza vaccine (LAIV) was licensed for prophylaxis of children 2–17 years old in Europe in 2012 and is administered as a nasal spray. Live-attenuated influenza vaccine induces both mucosal ...

    • Early start of enzyme replacement therapy in pediatric male patients with classical Fabry disease is associated with attenuated disease progression 

      van der Veen, Sanne; Körver, Simon; Hirsch, A; Hollak, Carla E.M.; Wijburg, Frits A; Brands, M; Tøndel, Camilla; Van Kuilenburg, André B.P.; Langeveld, Miriam (Journal article; Peer reviewed, 2022)
      Background Enzyme replacement therapy (ERT) slows disease progression of Fabry disease (FD), especially when initiated before the onset of irreversible organ damage. However, with the clinically asymptomatic progression ...

    • An expert consensus on the recommendations for the use of biomarkers in Fabry disease 

      Burlina, Allesandro; Brand, Eva; Hughes, Derralynn A.; Kantola, Ilkka; Krämer, Johannes; Nowak, Albina; Tøndel, Camilla; Wanner, Christoph; Spada, Marco (Journal article; Peer reviewed, 2023)
      Fabry disease is an X-linked lysosomal storage disorder caused by the accumulation of glycosphingolipids in various tissues and body fluids, leading to progressive organ damage and life-threatening complications. Phenotypic ...

    • Gene Expression Analysis in gla-Mutant Zebrafish Reveals Enhanced Ca2+ Signaling Similar to Fabry Disease 

      Elsaid, Hassan Osman Alhassan; Tjeldnes, Håkon; Rivedal, Mariell Lossius; Serre, Camille Julia; Eikrem, Øystein Solberg; Svarstad, Einar; Tøndel, Camilla; Marti, Hans Peter; Furriol, Jessica; Babickova, Janka (Journal article; Peer reviewed, 2022)
      Fabry disease (FD) is an X-linked inborn metabolic disorder due to partial or complete lysosomal α-galactosidase A deficiency. FD is characterized by progressive renal insufficiency and cardio- and cerebrovascular involvement. ...

    • Growth Differentiation Factor 15 in Children with Chronic Kidney Disease and after Renal Transplantation 

      Thorsteinsdottir, Hjørdis; Salvador, Cathrin Lytomt; Mjøen, Geir; Lie, Anine; Sugulle, Meryam; Tøndel, Camilla; Brun, Atle; Almaas, Runar; Bjerre, Anna Kristina (Journal article; Peer reviewed, 2020)
      Growth 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 ...

    • Kidney biopsy diagnosis in childhood in the Norwegian Kidney Biopsy Registry and the long-term risk of kidney replacement therapy: a 25-year follow-up 

      Gjerstad, Ann Christin; Skrunes, Rannveig; Tøndel, Camilla; Åsberg, Anders; Leh, Sabine; Klingenberg, Claus Andreas; Døllner, Henrik; Hammarström, Clara Louise; Bjerre, Anna Kristina (Journal article; Peer reviewed, 2022)
      Background: There is scarce information on biopsy-verified kidney disease in childhood and its progression to chronic kidney disease stage 5 (CKD 5). This study aims to review biopsy findings in children, and to investigate ...

    • Live attenuated influenza vaccine in children induces b-cell responses in tonsils 

      Mohn, Kristin Greve-Isdahl; Brokstad, Karl Albert; Pathirana, Rishi; Bredholt, Geir; Jul-Larsen, Åsne; Trieu, Mai Chi; Jalloh, Sarah Larteley Lartey; Montemoli, Emanuele; Tøndel, Camilla; Aarstad, Hans Jørgen; Cox, Rebecca Jane (Peer reviewed; Journal article, 2016)
      Background. Tonsils play a key role in eliciting immune responses against respiratory pathogens. Little is known about how tonsils contribute to the local immune response after intranasal vaccination. Here, we uniquely ...

    • Long COVID in a prospective cohort of home-isolated patients 

      Blomberg, Bjørn; Mohn, Kristin Greve-Isdahl; Brokstad, Karl Albert; Zhou, Fan; Linchausen, Dagrunn Waag; Hansen, Bernt-Are; Jalloh, Sarah Larteley Lartey; Onyango, Therese Bredholt; Kuwelker, Kanika; Sævik, Marianne; Bartsch, Hauke; Tøndel, Camilla; Kittang, Bård Reiakvam; Cox, Rebecca Jane; Langeland, Nina (Journal article; Peer reviewed, 2021)
      Long-term complications after coronavirus disease 2019 (COVID-19) are common in hospitalized patients, but the spectrum of symptoms in milder cases needs further investigation. We conducted a long-term follow-up in a ...

    • Longevity of B-cell and T-cell responses after live attenuated influenza vaccination in children 

      Mohn, Kristin Greve-Isdahl; Bredholt, Geir; Brokstad, Karl Albert; Pathirana, Rishi; Aarstad, Hans Jørgen; Tøndel, Camilla; Cox, Rebecca Jane (Peer reviewed; Journal article, 2015-05-15)
      Background. The live attenuated influenza vaccine (LAIV) is the preferred vaccine for children, but the mechanisms behind protective immune responses are unclear, and the duration of immunity remains to be elucidated. This ...

    • Low birthweight is associated with lower glomerular filtration rate in middle-aged mainly healthy women 

      Lillås, Bjørn Steinar; Tøndel, Camilla; Assmus, Jörg; Vikse, Bjørn Egil (Journal article; Peer reviewed, 2020)
      Background Low birthweight (LBW) has been shown to increase the risk of severe kidney disease. Studies have also shown associations between LBW and lower estimated glomerular filtration rate (GFR) in young adults. In this ...

    • Markers of nephropathy in young Fabry disease patients; role of kidney biopsies and functional measurements 

      Tøndel, Camilla (Doctoral thesis, 2013-04-05)
      Backgound: Fabry disease is an X-linked disease affecting glycosphingolipid metabolism due to deficiency of the lysosomal enzyme α-galactosidase. The natural course of the disease is related to progressive accumulation of ...