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Article: Cardiovascular changes in young renal failure patients.

Tøndel, Camilla / Marti, Hans-Peter

2021 Volume 15, Issue 2, Page(s) 183–185

Abstract: 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 thus an increasing need to focus on ...

Abstract	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 thus an increasing need to focus on the long-term effects of severely reduced kidney function early in life. Cardiovascular changes are known to contribute considerably in adulthood to the severe complications of renal failure. In young chronic kidney disease patients, there is limited knowledge of subclinical cardiovascular disease. In this issue of
Language	English
Publishing date	2021-11-11
Publishing country	England
Document type	Editorial
ZDB-ID	2655800-2
ISSN	2048-8513 ; 2048-8505
ISSN (online)	2048-8513
ISSN	2048-8505
DOI	10.1093/ckj/sfab223
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Zs.A 6587: Show issues

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Article ; Online: Renal Functional Response-Association With Birth Weight and Kidney Volume.

Lillås, Bjørn Steinar / Tøndel, Camilla / Melsom, Toralf / Eriksen, Bjørn Odvar / Marti, Hans-Peter / Vikse, Bjørn Egil

Kidney international reports

2023 Volume 8, Issue 5, Page(s) 1034–1042

Abstract: Introduction: Renal functional response (RFR) is the acute increase in glomerular filtration rate (GFR) after a protein load. Low RFR is a marker of single nephron hyperfiltration. Low birth weight (LBW) is associated with reduced number of nephrons, ... ...

Abstract	Introduction: Renal functional response (RFR) is the acute increase in glomerular filtration rate (GFR) after a protein load. Low RFR is a marker of single nephron hyperfiltration. Low birth weight (LBW) is associated with reduced number of nephrons, lower kidney function, and smaller kidneys in adults. In the present study, we investigate the associations among LBW, kidney volume, and RFR. Methods: We studied adults aged 41 to 52 years born with either LBW (≤2300 g) or normal birth weight (NBW; 3500-4000 g). GFR was measured using plasma clearance of iohexol. A stimulated GFR (sGFR) was measured on a separate day after a protein load of 100 g using a commercially available protein powder, and RFR was calculated as delta GFR. Kidney volume was estimated from magnetic resonance imaging (MRI) images using the ellipsoid formula. Results: A total of 57 women and 48 men participated. The baseline mean ± SD GFR was 118 ± 17 ml/min for men and 98 ± 19 ml/min for women. The overall mean RFR was 8.2 ± 7.4 ml/min, with mean RFR of 8.3 ± 8.0 ml/min and 8.1 ± 6.9 ml/min in men and women, respectively ( Conclusion: Larger kidney size and lower GFR per kidney volume were associated with higher RFR. Birth weight was not shown to associate with RFR in mainly healthy middle-aged men and women.
Language	English
Publishing date	2023-02-20
Publishing country	United States
Document type	Journal Article
ISSN	2468-0249
ISSN (online)	2468-0249
DOI	10.1016/j.ekir.2023.02.1079
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Article ; Online: Response to Birth Weight and Renal Functional Reserve in Adults.

Lillås, Bjørn Steinar / Tøndel, Camilla / Melsom, Toralf / Eriksen, Bjørn Odvar / Marti, Hans-Peter / Vikse, Bjørn Egil

Kidney international reports

2023 Volume 8, Issue 8, Page(s) 1700–1701

Language	English
Publishing date	2023-06-17
Publishing country	United States
Document type	Journal Article
ISSN	2468-0249
ISSN (online)	2468-0249
DOI	10.1016/j.ekir.2023.06.012
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Article ; Online: Development of an automated estimation of foot process width using deep learning in kidney biopsies from patients with Fabry, minimal change, and diabetic kidney diseases.

Smerkous, David / Mauer, Michael / Tøndel, Camilla / Svarstad, Einar / Gubler, Marie-Claire / Nelson, Robert G / Oliveira, João-Paulo / Sargolzaeiaval, Forough / Najafian, Behzad

Kidney international

2023 Volume 105, Issue 1, Page(s) 165–176

Abstract: Podocyte injury plays a key role in pathogenesis of many kidney diseases with increased podocyte foot process width (FPW), an important measure of podocyte injury. Unfortunately, there is no consensus on the best way to estimate FPW and unbiased ... ...

Abstract	Podocyte injury plays a key role in pathogenesis of many kidney diseases with increased podocyte foot process width (FPW), an important measure of podocyte injury. Unfortunately, there is no consensus on the best way to estimate FPW and unbiased stereology, the current gold standard, is time consuming and not widely available. To address this, we developed an automated FPW estimation technique using deep learning. A U-Net architecture variant model was trained to semantically segment the podocyte-glomerular basement membrane interface and filtration slits. Additionally, we employed a post-processing computer vision approach to accurately estimate FPW. A custom segmentation utility was also created to manually classify these structures on digital electron microscopy (EM) images and to prepare a training dataset. The model was applied to EM images of kidney biopsies from 56 patients with Fabry disease, 15 with type 2 diabetes, 10 with minimal change disease, and 17 normal individuals. The results were compared with unbiased stereology measurements performed by expert technicians unaware of the clinical information. FPW measured by deep learning and by the expert technicians were highly correlated and not statistically different in any of the studied groups. A Bland-Altman plot confirmed interchangeability of the methods. FPW measurement time per biopsy was substantially reduced by deep learning. Thus, we have developed a novel validated deep learning model for FPW measurement on EM images. The model is accessible through a cloud-based application making calculation of this important biomarker more widely accessible for research and clinical applications.
MeSH term(s)	Humans ; Diabetic Nephropathies/diagnosis ; Diabetic Nephropathies/pathology ; Diabetes Mellitus, Type 2 ; Deep Learning ; Glomerular Basement Membrane/pathology ; Biopsy
Language	English
Publishing date	2023-09-27
Publishing country	United States
Document type	Journal Article
ZDB-ID	120573-0
ISSN	1523-1755 ; 0085-2538
ISSN (online)	1523-1755
ISSN	0085-2538
DOI	10.1016/j.kint.2023.09.011
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Zs.A 797: Show issues

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Article ; Online: An expert consensus on the recommendations for the use of biomarkers in Fabry disease.

Burlina, Alessandro / Brand, Eva / Hughes, Derralynn / Kantola, Ilkka / Krӓmer, Johannes / Nowak, Albina / Tøndel, Camilla / Wanner, Christoph / Spada, Marco

Molecular genetics and metabolism

2023 Volume 139, Issue 2, Page(s) 107585

Abstract: 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 classification is based on ... ...

Abstract	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 classification is based on disease progression and severity and can be used to predict outcomes. Patients with a classic Fabry phenotype have little to no residual α-Gal A activity and have widespread organ involvement, whereas patients with a later-onset phenotype have residual α-Gal A activity and disease progression can be limited to a single organ, often the heart. Diagnosis and monitoring of patients with Fabry disease should therefore be individualized, and biomarkers are available to support with this. Disease-specific biomarkers are useful in the diagnosis of Fabry disease; non-disease-specific biomarkers may be useful to assess organ damage. For most biomarkers it can be challenging to prove they translate to differences in the risk of clinical events associated with Fabry disease. Therefore, careful monitoring of treatment outcomes and collection of prospective data in patients are needed. As we deepen our understanding of Fabry disease, it is important to regularly re-evaluate and appraise published evidence relating to biomarkers. In this article, we present the results of a literature review of evidence published between February 2017 and July 2020 on the impact of disease-specific treatment on biomarkers and provide an expert consensus on clinical recommendations for the use of those biomarkers.
MeSH term(s)	Humans ; Fabry Disease/diagnosis ; Fabry Disease/genetics ; Fabry Disease/complications ; alpha-Galactosidase/genetics ; Consensus ; Prospective Studies ; Enzyme Replacement Therapy/adverse effects ; Biomarkers ; Disease Progression
Chemical Substances	alpha-Galactosidase (EC 3.2.1.22) ; Biomarkers
Language	English
Publishing date	2023-04-17
Publishing country	United States
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	1418518-0
ISSN	1096-7206 ; 1096-7192
ISSN (online)	1096-7206
ISSN	1096-7192
DOI	10.1016/j.ymgme.2023.107585
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Article ; Online: Renal function, sex and age influence purines and pyrimidines in urine and could lead to diagnostic misinterpretation.

Salvador, Cathrin Lytomt / Flemmen, Per Trygge Kjelland / Tøndel, Camilla / Bliksrud, Yngve Thomas / Tsui, Ellen Fun Fong / Brun, Atle / Bjerre, Anna / Mørkrid, Lars

Molecular genetics and metabolism

2023 Volume 140, Issue 3, Page(s) 107649

Abstract: Glomerular filtration rate (GFR) is commonly used in clinical practice for the diagnosis and follow-up of chronic kidney disease. Screening for inborn errors of metabolism (IEM) is based on analysis of biomarkers in urine, reported by their ratio to ... ...

Abstract	Glomerular filtration rate (GFR) is commonly used in clinical practice for the diagnosis and follow-up of chronic kidney disease. Screening for inborn errors of metabolism (IEM) is based on analysis of biomarkers in urine, reported by their ratio to urinary creatinine (crn). Impaired renal function may complicate the interpretation of several biomarkers used for screening of IEM. Our goal was to investigate the influence of kidney function, in terms of measured GFR (mGFR) on purines and pyrimidines in urine, in addition to the relationship to sex, age, pH and ketosis. Children (n = 96) with chronic kidney disease (CKD), in different CKD stages, were included. Urine samples were obtained prior to the injection of iohexol. Serum samples at 7 time-points were used to calculate mGFR based on iohexol plasma clearance. The association with sex, age, ketosis and pH was examined in samples of the laboratory production from 2015 to 2021 (n = 8192). Age was a highly significant covariate for all markers. GFR correlated positively to several purines and pyrimidines; the ratios hypoxanthine/crn, xanthine/crn and urate/crn (p = 2.0 × 10
MeSH term(s)	Child ; Female ; Humans ; beta-Alanine ; Biomarkers ; Creatinine/metabolism ; Iohexol/metabolism ; Ketosis ; Kidney/metabolism ; Purines ; Pyrimidines ; Renal Insufficiency, Chronic/diagnosis ; Uracil ; Uric Acid ; Male ; Adolescent
Chemical Substances	beta-Alanine (11P2JDE17B) ; Biomarkers ; Creatinine (AYI8EX34EU) ; Iohexol (4419T9MX03) ; Purines ; Pyrimidines ; Uracil (56HH86ZVCT) ; Uric Acid (268B43MJ25)
Language	English
Publishing date	2023-07-05
Publishing country	United States
Document type	Clinical Study ; Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1418518-0
ISSN	1096-7206 ; 1096-7192
ISSN (online)	1096-7206
ISSN	1096-7192
DOI	10.1016/j.ymgme.2023.107649
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Article ; Online: 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

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association

2020 Volume 37, Issue 1, Page(s) 92–99

Abstract: 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 study we investigated whether ... ...

Abstract	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 study we investigated whether LBW associates with measured GFR (mGFR) in middle-aged mainly healthy adults. Methods: We invited individuals with LBW (1100-2300 g) and individuals with normal BW (NBW; 3500-4000 g) ages 41-52 years. GFR was measured using plasma clearance of iohexol. BW and BW for gestational age (BWGA) were obtained from the Medical Birth Registry of Norway and tested as main predictors. GFR was the main outcome. Results: We included 105 individuals (57 LBW and 48 NBW). The mean GFR was 95 ± 14 mL/min/1.73 m2 in the LBW group and 100 ± 13 mL/min/1.73 m2 in the NBW group (P = 0.04). There was a significant sex difference: in women the mean GFR was 90 ± 12 versus 101 ± 14 mL/min/1.73 m2 in the LBW and NBW groups, respectively (P = 0.006), whereas corresponding values for men were 101 ± 15 versus 100 ± 11 mL/min/1.73 m2 (P = 0.7). Using linear regression, we found the GFR was 4.5 mL/min/1.73 m2 higher per 1 kg higher BW for women (P = 0.02), with a non-significant 1.2 mL/min/1.73 m2 lower GFR for men (P = 0.6). In analyses of BWGA, there was also a significant association for women, but not for men. Conclusions: Middle-aged mainly healthy women with LBW had lower mGFR as compared with women with NBW. No such difference was found for men.
MeSH term(s)	Adult ; Birth Weight ; Female ; Glomerular Filtration Rate ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Iohexol ; Kidney ; Male ; Middle Aged ; Young Adult
Chemical Substances	Iohexol (4419T9MX03)
Language	English
Publishing date	2020-11-23
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	90594-x
ISSN	1460-2385 ; 0931-0509
ISSN (online)	1460-2385
ISSN	0931-0509
DOI	10.1093/ndt/gfaa306
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Zs.A 2198: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: 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

Scientific reports

2021 Volume 11, Issue 1, Page(s) 23224

Abstract: 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 has the obvious advantages of ... ...

Abstract	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 has the obvious advantages of being less painful and easier to perform. The purpose of our study was to determine if blood samples drawn from the injection access are feasible and accurate for iohexol GFR (iGFR) measurements. Thirty-one children, median age 10.5 (range 6-17) years, with chronic kidney disease were given a bolus of iohexol followed by extended saline flushing and subsequent venous blood samples collected from the injection access as well as from a cannula in the contralateral arm, the latter serving as the reference method. Paired venous blood samples were collected at four time points (2, 3, 3.5 and 4 h) after the iohexol bolus. Blood sample discarding preceded and saline flushing followed each blood sampling to avoid marker contamination. iGFR based on samples drawn from the injection access at 2 and 3 h showed significantly lower iGFR than measurement from the contralateral arm (p < 0.01). Singlepoint iGFR did not differ significantly after 3-4 repeated procedures of blood discarding and saline flusing (3.5 and 4 h). Despite thorough saline flushing there is still a relatively high risk of falsely low iGFR due to marker contamination in blood samples from the injection site. Hence, blood sampling from a second intravenous access is recommended for routine iohexol GFR measurements in children.Clinical trial registration: ClinicalTrials.gov, Identifier NCT01092260, https://clinicaltrials.gov/ct2/show/NCT01092260?term=tondel&rank=2 .
MeSH term(s)	Administration, Intravenous ; Adolescent ; Blood Specimen Collection/methods ; Child ; Glomerular Filtration Rate ; Humans ; Iohexol/administration & dosage ; Iohexol/pharmacokinetics ; Metabolic Clearance Rate ; Renal Insufficiency, Chronic/blood ; Renal Insufficiency, Chronic/diagnosis
Chemical Substances	Iohexol (4419T9MX03)
Language	English
Publishing date	2021-12-01
Publishing country	England
Document type	Clinical Trial ; Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2615211-3
ISSN	2045-2322 ; 2045-2322
ISSN (online)	2045-2322
ISSN	2045-2322
DOI	10.1038/s41598-021-02759-1
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Article ; Online: 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 / Døllner, Henrik / Hammarstrøm, Clara / Bjerre, Anna Kristina

Pediatric nephrology (Berlin, Germany)

2022 Volume 38, Issue 4, Page(s) 1249–1256

Abstract: 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 risk of kidney replacement ... ...

Abstract	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 risk of kidney replacement therapy (KRT). Methods: We conducted a retrospective long-term follow-up study of children included in the Norwegian Kidney Biopsy Registry (NKBR) and in the Norwegian Renal Registry (NRR) from 1988 to 2021. Results: In total, 575 children with a median (interquartile range, IQR) age of 10.7 (6.1 to 14.1) years were included, and median follow-up time (IQR) after kidney biopsy was 14.3 (range 8.9 to 21.6) years. The most common biopsy diagnoses were minimal change disease (MCD; n = 92), IgA vasculitis nephritis (IgAVN; n = 76), IgA nephropathy (n = 63), and focal and segmental glomerulosclerosis (FSGS; n = 47). In total, 118 (20.5%) of the biopsied children reached CKD 5, median (IQR) time to KRT 2.3 years (7 months to 8.4 years). Most frequently, nephronophthisis (NPHP; n = 16), FSGS (n = 30), IgA nephropathy (n = 9), and membranoproliferative glomerulonephritis (MPGN; n = 9) led to KRT. Conclusions: The risk of KRT after a kidney biopsy diagnosis is highly dependent on the diagnosis. None of the children with MCD commenced KRT, while 63.8% with FSGS and 100% with NPHP reached KRT. Combining data from kidney biopsy registries with registries on KRT allows for detailed information concerning the risk for later CKD 5 after biopsy-verified kidney disease in childhood. A higher resolution version of the Graphical abstract is available as Supplementary information.
MeSH term(s)	Child ; Humans ; Adolescent ; Young Adult ; Adult ; Glomerulosclerosis, Focal Segmental/pathology ; Follow-Up Studies ; Retrospective Studies ; Glomerulonephritis, IGA/pathology ; Kidney/pathology ; Glomerulonephritis, Membranoproliferative/pathology ; Renal Replacement Therapy ; Kidney Failure, Chronic/pathology ; Registries ; Biopsy/adverse effects
Language	English
Publishing date	2022-08-22
Publishing country	Germany
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-022-05706-y
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Article ; Online: 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

Molecular genetics and metabolism

2022 Volume 137, Issue 4, Page(s) 328–341

Abstract: 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 of various organs. Agalsidase beta, ...

Abstract	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 of various organs. Agalsidase beta, an enzyme replacement therapy (ERT), is approved for use in patients with FD in Europe, Canada, Australia, South America, and Asia, and is the only ERT approved for use in the United States. In this review, we discuss the clinical relevance of GL3 accumulation, the effect of agalsidase beta on GL3 in target tissues, and the association between treatment-related tissue GL3 clearance and long-term structure, function, or clinical outcomes. Accumulation of GL3 in the kidney, heart, vasculature, neurons, skin, gastrointestinal tract and auditory system correlates to cellular damage and irreversible organ damage, as a result of sclerosis, fibrosis, apoptosis, inflammation, and endothelial dysfunction. Damage leads to renal dysfunction and end-stage renal disease; myocardial hypertrophy with heart failure and arrhythmias; ischemic stroke; neuropathic pain; skin lesions; intestinal ischemia and dysmotility; and hearing loss. Treatment with agalsidase beta is effective in substantially clearing GL3 in a range of cells from the tissues affected by FD. Agalsidase beta has also been shown to slow renal decline and lower the overall risk of clinical progression, demonstrating an indirect link between treatment-related GL3 clearance and stabilization of FD.
Language	English
Publishing date	2022-10-29
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	1418518-0
ISSN	1096-7206 ; 1096-7192
ISSN (online)	1096-7206
ISSN	1096-7192
DOI	10.1016/j.ymgme.2022.10.005
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