111 results on '"Schaefer, Franz"'
Search Results
2. Investigating the use of finerenone in children with chronic kidney disease and proteinuria: design of the FIONA and open-label extension studies
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Schaefer, Franz, Montini, Giovanni, Kang, Hee Gyung, Walle, Johan Vande, Zaritsky, Joshua, Schreuder, Michiel F., Litwin, Mieczyslaw, Scalise, Andrea, Scott, Helen, Potts, James, Iveli, Pablo, Breitenstein, Stefanie, and Warady, Bradley A.
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- 2024
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3. Real-world evidence on the dosing and safety of C.E.R.A. in pediatric dialysis patients: findings from the International Pediatric Dialysis Network registries
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Kohlhas, Laura, Studer, Milena, Rutten-Jacobs, Loes, Reigner, Sylvie Meyer, Sander, Anja, Yap, Hui-Kim, Vondrak, Karel, Coccia, Paula A., Cano, Francisco, Schmitt, Claus Peter, Warady, Bradley A., and Schaefer, Franz
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- 2024
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4. Progression of Chronic Kidney Disease and Nephroprotective Therapy
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Wühl, Elke, Schaefer, Franz, Schaefer, Franz, editor, and Greenbaum, Larry A., editor
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- 2023
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5. Renal Hypertension: Etiology and Management
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Wühl, Elke, Schaefer, Franz, Schaefer, Franz, editor, and Greenbaum, Larry A., editor
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- 2023
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6. Pathophysiology and consequences of arterial stiffness in children with chronic kidney disease
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Azukaitis, Karolis, Jankauskiene, Augustina, Schaefer, Franz, and Shroff, Rukshana
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- 2021
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7. Differential assessment of fluid compartments by bioimpedance in pediatric patients with kidney diseases
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Frey, Sandra M., Vogt, Bruno, Simonetti, Giacomo D., Büscher, Rainer, Habbig, Sandra, and Schaefer, Franz
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- 2021
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8. Treatment of Hypertension in Chronic Kidney Disease
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Wühl, Elke, Schaefer, Franz, Mancia, Giuseppe, Series Editor, Agabiti Rosei, Enrico, Series Editor, Lurbe, Empar, editor, and Wühl, Elke, editor
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- 2019
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9. Averting the legacy of kidney disease: focus on childhood.
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Ingelfinger, Julie, Schaefer, Franz, and Kalantar-Zadeh, Kamyar
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chronic kidney disease ,congenital anomalies of the kidney and urinary tract (CAKUT) ,developmental origins of health and disease (DoHAD) ,pediatrics - Abstract
World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD) in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for date newborns have relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, while only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood.
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- 2016
10. Averting the legacy of kidney disease – focus on childhood
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Ingelfinger, Julie R, Kalantar-Zadeh, Kamyar, and Schaefer, Franz
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chronic kidney disease ,paediatric nephrology ,paediatrics ,congenital anomalies of the kidney and urinary tract ,developmental origins of health and disease - Abstract
World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and chronic kidney disease in later childhood or in adult life. Children born early or who are small-for date newborns have relatively increased risk for the development of chronic kidney disease later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced chronic kidney disease in childhood; there is evidence that children fare better than adults, if they receive kidney replacement therapy including dialysis and transplantation, while only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers and caregivers about the needs and possibilities surrounding kidney disease in childhood.
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- 2016
11. World Kidney Day 2016: Averting the legacy of kidney disease—focus on childhood
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Ingelfinger, Julie R, Kalantar-Zadeh, Kamyar, Schaefer, Franz, and on behalf of the World Kidney Day Steering Committee
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Biomedical and Clinical Sciences ,Clinical Sciences ,Pediatric ,Clinical Research ,Kidney Disease ,Prevention ,Renal and urogenital ,Quality Education ,Age of Onset ,Disease Progression ,Global Health ,Health Promotion ,Health Services Accessibility ,Healthcare Disparities ,Humans ,Prognosis ,Renal Insufficiency ,Chronic ,Risk Factors ,Transition to Adult Care ,Chronic kidney disease ,Pediatrics ,Congenital anomalies of the kidney and urinary tract ,Developmental origins of health and disease ,World Kidney Day Steering Committee ,Paediatrics and Reproductive Medicine ,Urology & Nephrology ,Clinical sciences ,Paediatrics - Abstract
World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD) in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early, or who are small-for-date newborns, have a relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely in order to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults if they receive kidney replacement therapy including dialysis and transplantation, while only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy-makers, and caregivers about the needs and possibilities surrounding kidney disease in childhood.
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- 2016
12. Editorial: World Kidney Day 2016: Averting the Legacy of Kidney Disease--Focus on Childhood
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Ingelfinger, Julie R, Kalantar-Zadeh, Kamyar, Schaefer, Franz, and Committee, on behalf of the World Kidney Day Steering
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Biomedical and Clinical Sciences ,Clinical Sciences ,Pediatric ,Clinical Research ,Kidney Disease ,Prevention ,Renal and urogenital ,Quality Education ,Adolescent ,Age of Onset ,Child ,Disease Progression ,Global Health ,Health Promotion ,Health Services Accessibility ,Humans ,Morbidity ,Prognosis ,Renal Insufficiency ,Chronic ,Risk Factors ,Transition to Adult Care ,Pediatric nephrology ,chronic kidney disease ,pediatrics ,congenital anomalies of the kidney and urinary tract ,developmental origins of health and disease ,World Kidney Day Steering Committee ,developmental origins of health and disease (DoHAD). ,Urology & Nephrology ,Clinical sciences - Abstract
World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD) in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life. Children born early or who are small-for-date newborns have a relatively increased risk for the development of CKD later in life. Persons with a high-risk birth and early childhood history should be watched closely to help detect early signs of kidney disease in time to provide effective prevention or treatment. Successful therapy is feasible for advanced CKD in childhood; there is evidence that children fare better than adults if they receive kidney replacement therapy including dialysis and transplantation, although only a minority of children may require this ultimate intervention. Because there are disparities in access to care, effort is needed so that those children with kidney disease, wherever they live, may be treated effectively, irrespective of their geographic or economic circumstances. Our hope is that World Kidney Day will inform the general public, policy makers, and caregivers about the needs and possibilities surrounding kidney disease in childhood.
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- 2016
13. Indoxyl sulfate associates with cardiovascular phenotype in children with chronic kidney disease
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Holle, Johannes, Querfeld, Uwe, Kirchner, Marietta, Anninos, Alexandros, Okun, Jürgen, Thurn-Valsassina, Daniela, Bayazit, Aysun, Niemirska, Ana, Canpolat, Nur, Bulut, Ipek Kaplan, Duzova, Ali, Anarat, Ali, Shroff, Rukshana, Bilginer, Yelda, Caliskan, Salim, Candan, Cengiz, Harambat, Jerome, Özcakar, Zeynep Birsin, Soylemezoglu, Oguz, Tschumi, Sibylle, Habbig, Sandra, Yilmaz, Ebru, Balat, Ayse, Zurowska, Aleksandra, Cakar, Nilgun, Kranz, Birgitta, Ertan, Pelin, Melk, Anette, Azukaitis, Karolis, and Schaefer, Franz
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- 2019
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14. Progression of Chronic Kidney Disease and Nephroprotective Therapy
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Wühl, Elke, Schaefer, Franz, Geary, Denis F., editor, and Schaefer, Franz, editor
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- 2016
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15. A randomized, double-blind, placebo-controlled study to assess the efficacy and safety of cinacalcet in pediatric patients with chronic kidney disease and secondary hyperparathyroidism receiving dialysis
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Warady, Bradley A., Iles, Janet N., Ariceta, Gema, Dehmel, Bastian, Hidalgo, Guillermo, Jiang, Xun, Laskin, Benjamin, Shahinfar, Shahnaz, Vande Walle, Johan, and Schaefer, Franz
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- 2019
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16. Validating the use of bioimpedance spectroscopy for assessment of fluid status in children
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Dasgupta, Indranil, Keane, David, Lindley, Elizabeth, Shaheen, Ihab, Tyerman, Kay, Schaefer, Franz, Wühl, Elke, Müller, Manfred J., Bosy-Westphal, Anja, Fors, Hans, Dahlgren, Jovanna, Chamney, Paul, Wabel, Peter, and Moissl, Ulrich
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- 2018
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17. Hypertension in Chronic Kidney Disease
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Schaefer, Franz, Wühl, Elke, Flynn, Joseph T., editor, Ingelfinger, Julie R., editor, and Portman, Ronald J., editor
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- 2013
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18. Endocrine and Growth Disorders in Chronic Kidney Disease
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Schaefer, Franz, Avner, Ellis, editor, Harmon, William, editor, Niaudet, Patrick, editor, and Yoshikawa, Norishige, editor
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- 2009
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19. Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD
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Nalcacioglu, Hulya, Dunand, Oliver, Mastrangelo, Antonio, Murer, Luisa, Emma, Francesco, Ruzgiene, Dovile, Taranta-Janusz, Katarzyna, Balasz-Chmielewska, Irena, Miklaszewska, Monika, Stanczyk, Malgorzata, Sikora, Przemyslaw, Kowalewska, Claudia, Szczepanska, Maria, Teixeira, Ana, Rachisan, Andreea, Papachristou, Fotios, Paripović, Dušan, Prikhodina, Larisa, Jilani, Houweyda, Bayazit, Aysun Karabay, Soylu, Alper, Candan, Cengiz, Sever, Lale, Emre, Sevinc, Cicek, Neslihan, Akinci, Nurver, Mir, Sevgi, Poyrazoğlu, Hakan M., Tabel, Yilmaz, Mencarelli, Francesca, Burgmaier, Kathrin, Kilian, Samuel, Arbeiter, Klaus, Atmis, Bahriye, Büscher, Anja, Derichs, Ute, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Galiano, Matthias, Gessner, Michaela, Gokce, Ibrahim, Haeffner, Karsten, Hooman, Nakysa, Jankauskiene, Augustina, Körber, Friederike, Longo, Germana, Massella, Laura, Mekahli, Djalila, Miloševski-Lomić, Gordana, Rus, Rina, Shroff, Rukshana, Stabouli, Stella, Weber, Lutz T., Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Dötsch, Jörg, Schaefer, Franz, Liebau, Max Christoph, Potemkina, Alexandra, Ranguelov, Nadejda, Collard, Laure, De Mul, Aurélie, Feldkoetter, Markus, Seeman, Tomas, Zieg, Jakub, Thumfart, Julia, Grundmann, Franziska, Buchholz, Björn, Pape, Lars, Gross, Oliver, Patzer, Ludwig, Schild, Raphael, Haffner, Dieter, Bernhardt, Wanja, Wuehl, Elke, Henn, Michael, Halbritter, Jan, Klaus, Günter, Lechner, Felix, Lange-Sperandio, Bärbel, Uetz, Barbara, Benz, Marcus, König, Jens, Staude, Hagen, Wurm, Donald, Bald, Martin, Soliman, Neveen A., Ariceta, Gema, Rodriguez, Juan David Gonzalez, de la Cerda Ojeda, Francisco, Harambat, Jerome, Ranchin, Bruno, Fila, Marc, Dossier, Claire, Boyer, Olivia, Marlais, Matko, UCL - (SLuc) Département de pédiatrie, Ruzgienė, Dovilė, Burgmaier, Kathrin, Kilian, Samuel, Arbeiter, Klaus, Atmis, Bahriye, Buescher, Anja, Derichs, Ute, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Galiano, Matthias, Gessner, Michaela, Gokce, Ibrahim, Haeffner, Karsten, Hooman, Nakysa, Jankauskiene, Augustina, Koerber, Friederike, Longo, Germana, Massella, Laura, Mekahli, Djalila, Milosevski-Lomic, Gordana, Nalcacioglu, Hulya, Rus, Rina, Shroff, Rukshana, Stabouli, Stella, Weber, Lutz T., Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Doetsch, Joerg, Schaefer, Franz, and Liebau, Max Christoph
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Liver Cirrhosis ,Male ,glomerulus filtration rate ,Medizin ,cell surface receptor ,preschool child ,DISEASE ,Cohort Studies ,Chronic kidney disease ,Polycystic kidney disease ,Medicine ,genetics ,Longitudinal Studies ,Child ,pathophysiology ,Ultrasonography ,Kidney ,Multidisciplinary ,longitudinal study ,organ size ,chronic kidney failure ,biological marker ,cohort analysis ,Prognosis ,Autosomal Recessive Polycystic Kidney Disease ,PKHD1 protein, human ,Multidisciplinary Sciences ,medicine.anatomical_structure ,female ,Quartile ,Child, Preschool ,Cohort ,Disease Progression ,Science & Technology - Other Topics ,Glomerular Filtration Rate ,medicine.medical_specialty ,kidney ,Adolescent ,Science ,Urology ,Renal function ,Kidney Volume ,Receptors, Cell Surface ,Article ,Humans ,ddc:610 ,human ,Renal Insufficiency, Chronic ,Polycystic Kidney, Autosomal Recessive ,Paediatric kidney disease ,Science & Technology ,business.industry ,echography ,Infant ,medicine.disease ,mortality ,kidney polycystic disease ,disease exacerbation ,physiology ,business ,metabolism ,Biomarkers ,Kidney disease - Abstract
Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational study we analyzed the longitudinal development of sonographic kidney measurements in a cohort of 456 ARPKD patients from the international registry study ARegPKD. We furthermore evaluated correlations of sonomorphometric findings and functional kidney disease with the aim to describe the natural disease course and to identify potential prognostic markers. Kidney pole-to-pole (PTP) length and estimated total kidney volume (eTKV) increase with growth throughout childhood and adolescence despite individual variability. Height-adjusted PTP length decreases over time, but such a trend cannot be seen for height-adjusted eTKV (haeTKV) where we even observed a slight mean linear increase of 4.5 ml/m per year during childhood and adolescence for the overall cohort. Patients with two null PKHD1 variants had larger first documented haeTKV values than children with missense variants (median (IQR) haeTKV 793 (450–1098) ml/m in Null/null, 403 (260–538) ml/m in Null/mis, 230 (169–357) ml/m in Mis/mis). In the overall cohort, estimated glomerular filtration rate decreases with increasing haeTKV (median (IQR) haeTKV 210 (150–267) ml/m in CKD stage 1, 472 (266–880) ml/m in stage 5 without kidney replacement therapy). Strikingly, there is a clear correlation between haeTKV in the first eighteen months of life and kidney survival in childhood and adolescence with ten-year kidney survival rates ranging from 20% in patients of the highest to 94% in the lowest quartile. Early childhood haeTKV may become an easily obtainable prognostic marker of kidney disease in ARPKD, e.g. for the identification of patients for clinical studies. © 2021, The Author(s)., ESPN 2014.2; PKD Foundation, PKDF; Bundesministerium für Bildung und Forschung, BMBF: 01GM1515, 01GM1903; Universität zu Köln, UoC; Marga und Walter Boll-Stiftung; Universitätsklinikum Köln, We thank the German Society for Pediatric Nephrology (GPN), the ESCAPE Network, and the European Society for Paediatric Nephrology (ESPN; Working Groups CAKUT and Inherited Renal Diseases) for their support. ML was supported by grants of the GPN, ESPN (Grant ESPN 2014.2), and the German PKD foundation. KB and ML were supported by the Medical Faculty of the University of Cologne (Koeln Fortune program), and the Marga and Walter Boll-Foundation. FS and ML are supported by the German Federal Ministry of Research and Education (BMBF grant 01GM1515 and 01GM1903). This work was generated within the European Reference Network for Rare Kidney Disorders (ERKNet).
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- 2021
20. Genome-wide association studies in pediatric chronic kidney disease
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Gupta, Jayanta, Kanetsky, Peter A., Wuttke, Matthias, Köttgen, Anna, Schaefer, Franz, and Wong, Craig S.
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- 2016
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21. Safety and usage of darbepoetin alfa in children with chronic kidney disease: prospective registry study
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Schaefer, Franz, Hoppe, Bernd, Jungraithmayr, Therese, Klaus, Günter, Pape, Lars, Farouk, Mourad, Addison, Janet, Manamley, Nick, and Vondrak, Karel
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- 2016
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22. Long-term growth hormone treatment in short children with CKD does not accelerate decline of renal function: results from the KIGS registry and ESCAPE trial
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Mehls, Otto, Lindberg, Anders, Haffner, Dieter, Schaefer, Franz, Wühl, Elke, for members of the German KIGS Board, and ESCAPE Trial Group
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- 2015
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23. Treatment and long-term outcome in primary distal renal tubular acidosis
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Lopez-Garcia, Sergio Camilo, Emma, Francesco, Walsh, Stephen B, Fila, Marc, Hooman, Nakysa, Zaniew, Marcin, Bertholet-Thomas, Aurelia, Colussi, Giacomo, Burgmaier, Kathrin, Levtchenko, Elena, Sharma, Jyoti, Singhal, Jyoti, Soliman, Neveen A, Ariceta, Gema, Basu, Biswanath, Murer, Luisa, Tasic, Velibor, Tsygin, Alexey, Decramer, Stephane, Gil-Pena, Helena, Koster-Kamphuis, Linda, La Scola, Claudio, Gellermann, Jutta, Konrad, Martin, Lilien, Marc, Francisco, Telma, Tramma, Despoina, Trnka, Peter, Yuksel, Selcuk, Caruso, Maria Rosa, Chromek, Milan, Ekinci, Zelal, Gambaro, Giovanni, Kari, Jameela A, Koenig, Jens, Taroni, Francesca, Thumfart, Julia, Trepiccione, Francesco, Winding, Louise, Wuehl, Elke, Agbas, Ayse, Belkevich, Anna, Vargas-Poussou, Rosa, Blanchard, Anne, Conti, Giovanni, Boyer, Olivia, Dursun, Ismail, Pinarbasi, Ayse Seda, Melek, Engin, Miglinas, Marius, Novo, Robert, Mallett, Andrew, Milosevic, Danko, Szczepanska, Maria, Wente, Sarah, Cheong, Hae Il, Sinha, Rajiv, Gucev, Zoran, Dufek, Stephanie, Iancu, Daniela, Kleta, Robert, Schaefer, Franz, Bockenhauer, Detlef, Peco-Antic, Amira, Kaur, Amrit, Paglialunga, Antonino, Servais, Aude, Lutovac, Branko, Hoorn, Ewout J, Shasha-Lavsky, Hadas, Harambat, Jerome, Godron-Dubrasquet, Astrid, Buder, Kathrin, Allard, Lise, Patzer, Ludwig, Shumikhina, Marina, Hansen, Matthias, Printza, Nikoleta, Kucuk, Nuran, Beringer, Ortraud, Bhimma, Rajendra, Cerkauskiene, Rimante, Klinikos, Santaros, Neuhaus, Thomas J, Stavileci, Valbona, Ulinski, Tim, Dincel, Nida Temizkan, Mohebbi, Nilufar, Çukurova Üniversitesi, Lopez-Garcia, Sergio Camilo, Emma, Francesco, Walsh, Stephen B, Fila, Marc, Hooman, Nakysa, Zaniew, Marcin, Bertholet-Thomas, Aurélia, Colussi, Giacomo, Burgmaier, Kathrin, Levtchenko, Elena, Sharma, Jyoti, Singhal, Jyoti, Soliman, Neveen A, Ariceta, Gema, Basu, Biswanath, Murer, Luisa, Tasic, Velibor, Tsygin, Alexey, Decramer, Stéphane, Gil-Peña, Helena, Koster-Kamphuis, Linda, La Scola, Claudio, Gellermann, Jutta, Konrad, Martin, Lilien, Marc, Francisco, Telma, Tramma, Despoina, Trnka, Peter, Yüksel, Selçuk, Caruso, Maria Rosa, Chromek, Milan, Ekinci, Zelal, Gambaro, Giovanni, Kari, Jameela A, König, Jen, Taroni, Francesca, Thumfart, Julia, Trepiccione, Francesco, Winding, Louise, Wühl, Elke, Ağbaş, Ayşe, Belkevich, Anna, Vargas-Poussou, Rosa, Blanchard, Anne, Conti, Giovanni, Boyer, Olivia, Dursun, Ismail, Pınarbaşı, Ayşe Seda, Melek, Engin, Miglinas, Mariu, Novo, Robert, Mallett, Andrew, Milosevic, Danko, Szczepanska, Maria, Wente, Sarah, Cheong, Hae Il, Sinha, Rajiv, Gucev, Zoran, Dufek, Stephanie, Iancu, Daniela, Kleta, Robert, Schaefer, Franz, Bockenhauer, Detlef, and Internal Medicine
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Male ,glomerulus filtration rate ,ATP6V1B1 protein, human ,DNA Mutational Analysis ,kidney calcification ,distal renal tubular acidosis ,Sensorineural ,nephrocalcinosi ,ATP6V1B1 gene ,Renal tubular acidosis ,0302 clinical medicine ,newborn ,Chronic kidney disease ,middle aged ,Medicine ,genetics ,Young adult ,Child ,adult ,cohort analysis ,perception deafness ,Sensorineural hearing loss ,aged ,Nephrocalcinosis ,priority journal ,Nephrology ,Child, Preschool ,Cohort ,Acidosis ,mutational analysis ,Vacuolar Proton-Translocating ATPases ,medicine.medical_specialty ,Hearing Loss, Sensorineural ,rare disease ,Renal function ,bicarbonate ,complication ,Article ,03 medical and health sciences ,nephrocalcinosis ,Humans ,human ,gross national product ,Hearing Loss ,Aged ,Infant ,economic aspect ,Distal renal tubular acidosis ,medicine.disease ,major clinical study ,proton transporting adenosine triphosphate synthase ,Mutation ,nephrolithiasis ,estimated glomerular filtration rate ,chronic kidney disease ,SLC4A1 gene ,030232 urology & nephrology ,Deafness ,030204 cardiovascular system & hematology ,preschool child ,sensorineural hearing loss ,nephrolithiasi ,Cohort Studies ,distal renal tubular acidosi ,Interquartile range ,kidney tubule acidosis ,gene mutation ,kidney function ,Acidosis, Renal Tubular ,chronic kidney failure ,Middle Aged ,urine ,female ,genetic association study ,medical care ,body height ,young adult ,Female ,Renal Tubular ,Glomerular Filtration Rate ,onset age ,Adult ,Adolescent ,prevalence ,Nephrolithiasis ,Young Adult ,Rare Diseases ,primary distal renal tubular acidosis ,blood ,Internal medicine ,follow up ,gene ,Preschool ,outcome assessment ,Genetic Association Studies ,Transplantation ,calcium ,business.industry ,Infant, Newborn ,hearing impairment ,Newborn ,Bicarbonates ,Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11] ,Calcium ,metabolic regulation ,business ,Kidney disease - Abstract
PubMedID: 30773598 Background. Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-Termoutcome. Methods. We contacted paediatric and adult nephrologists through European professional organizations. Responding clinicians entered demographic, biochemical, genetic and clinical data in an online form. Results. Adequate data were collected on 340 patients (29 countries, female 52%). Mutation testing had been performed on 206 patients (61%); pathogenic mutations were identified in 170 patients (83%). The median (range) presentation age was 0.5 (0-54) years and age at last follow-up was 11.0 (0-70.0) years. Adult height was slightly below average with a mean (SD score) of -0.57 (61.16). There was an increased prevalence of chronic kidney disease (CKD) Stage -2 in children (35%) and adults (82%). Nephrocalcinosis was reported in 88%. Nephrolithiasis was more common with SLC4A1 mutations (42% versus 21%). Thirty-six percent had hearing loss, particularly in ATP6V1B1 (88%). The median (interquartile range) prescribed dose of alkali (mEq/kg/day) was 1.9 (1.2-3.3). Adequate metabolic control (normal plasma bicarbonate and normocalciuria) was achieved in 158 patients (51%), more commonly in countries with higher gross domestic product (67% versus 23%), and was associated with higher height and estimated glomerular filtration rate. Conclusion. Long-Term follow-up from this large dRTA cohort shows an overall favourable outcome with normal adult height for most and no patient with CKD Stage 5. However, 82% of adult patients have CKD Stages 2-4. Importance of adequate metabolic control was highlighted by better growth and renal function but was achieved in only half of patients. © The Author(s) 2018. Erciyes Üniversitesi Iran University of Medical Sciences Università degli Studi di Padova Chung Hua University American Ornithologists' Union University of Queensland 7Centre University College London Aristotle University of Thessaloniki Lunds Universitet Cairo University National Rosacea Society Heart of England NHS Foundation Trust Centre hospitalier universitaire Sainte-Justine Erasmus Universiteit Rotterdam Aristotle University of Thessaloniki 1Department of Paediatric Nephrology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK, 2Centre for Nephrology, University College London, London, UK, 3Division of Nephrology, Bambino Gesù Children’s Hospital—IRCCS, Rome, Italy, 4Pediatric Nephrology—CHU Arnaud de Villeneuve, Montpellier University Hospital, Montpellier, France, 5Ali-Asghar Clinical Research Development Center, Iran University of Medical Sciences, Tehran, Iran, 6Department of Pediatrics, University of Zielona Góra, Zielona Góra, Poland, 7Centre de référence Maladies rénales rares, Bron, France, 8ASST Niguarda, Milan, Italy, 9Department of Pediatrics, University Hospital of Cologne, Cologne, Germany, 10University Hospital Leuven, Leuven, Belgium, 11King Edward Memorial Hospital, Pune, India, 12Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt, 13Hospital Universitario Vall d’Hebron, Barcelona, Spain, 14Division of Pediatric Nephrology, NRS Medical College, Kolkata, India, 15Pediatric Nephrology, Dialysis and Transplant Unit, Azienda Ospedaliera & University of Padova, Padova, Italy, 16University Children’s Hospital, Medical School, Skopje, Macedonia, 17National Medical and Research Centre for Children’s Health, Moscow, Russia, 18Centre Hospitalier Universitaire de Toulouse, Service de Nephrologie Pediatrique, Hopital des Enfants, Centre De Reference des Maladies Rénales Rares du Sud Ouest, Toulouse, France, 19Hospital Universitario Central de Asturias, Oviedo, Spain, 20Radboud University Medical Centre, Nijmegen, The Netherlands, 21Nephrology and Dialysis Unit, Department of Woman, Child and Urological Diseases, Azienda Ospedaliero—Universitaria Sant’Orsola-Malpighi, Bologna, Italy, 22Charité Universitätsmedizin Berlin, Berlin, Germany, 23University Children’s Hospital, Münster, Germany, 24Wilhelmina Children’s Hospital, University Medical Center, Utrecht, The Netherlands, 25Centro Hospitalar de Lisboa Central, Lisbon, Portugal, 26Fourth Pediatric Department, Aristotle University, Thessaloniki, Greece, 27Lady Cilento Children’s Hospital, Brisbane, Australia, 28School of Medicine, the University of Queensland, Brisbane, Australia, 29Department of Pediatric Nephrology, Pamukkale University School of Medicine, Denizli, Turkey, 30Nephrology Unit Azienda Ospedaliera, Papa Giovani XXIII, Bergamo, Italy, 31Karolinska Institutet, Lund University,Sweden,GroupFlorenceNightingaleHospitals,Ist32· anbul, Turkey,Fondazione Policlinico A. Gemelli, Universita` Cattolica del33 Sacro Cuore, Rome, Italy, 34Pediatric Nephrology Center of Excellence and Pediatric Department, Faculty of Medicine, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia, 35Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca’ Granda—Ospedale Maggiore Policlinico, Milan, Italy, 36Department of Translational Medical Sciences, University of Campania “L. Vanvitelli”, Naples, Italy, 37Pediatric Department, Lillebaelt Hospital Kolding, Kolding, Denmark, 38Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, University Hospital of Heidelberg, Heidelberg, Germany, 39Haseki Education and Research Hospital, Istanbul, Turkey, 40Belarusian State Medical University, Minsk, Belarus, 41Department of Genetics, Assistance Publique Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France, 42Pediatric Nephrology Unit, AOU Policlinic G Martino, Messina, Italy, 43Necker Hospital, Paris, France, 44Faculty of Medicine, Department of Pediatric Nephrology, Erciyes University, Kayseri, Turkey, 45Cukurova University, Adana, Turkey, 46Nephrology Centre, Santaros Klinikos, Vilnius University, Vilnius, Lithuania, 47University Hospital of Lille, France, 48Department of Renal Medicine, Royal Brisbane and Women’s Hospital, Brisbane, Australia, 49University Hospital Centre Zagreb, Zagreb, Croatia, 50Department of Pediatrics, SMDZ in Zabrze, SUM in Katowice, Poland, 51Department of Pediatric Nephrology, Hannover Medical School, Hannover, Germany, The European dRTA Consortium consists of the authors, as well as: Amira Peco-Antic(Department of Nephrology, University Children’s Hospital, Belgrade, Serbia), Amrit Kaur (Department of Paediatric Nephrology, Royal Manchester Children’s Hospital, Manchester, UK), Antonino Paglialunga (ASP de Ragusa, Modica, Italy), Aude Servais (Department of Nephrology, Centre Hospitalier Universitaire Necker, APHP, Paris, France), Branko Lutovac (Clinical Centre of Montenegro, Institute for Children’s Disease, Podgorica, Montenegro), Ewout J. Hoorn (Erasmus Medical Center, Rotterdam, The Netherlands), Hadas Shasha-Lavsky (Galilee Medical Center, Nahariya, Israel), Jerome Harambat (Pediatric Nephrology Unit, Bordeaux University Hospital, Bordeaux, France), Astrid Godron-Dubrasquet (Pediatric Nephrology Unit, Bordeaux University Hospital, Bordeaux, France), Kathrin Buder (Pediatric Department, University Hospital, Carl Gustav Carus Dresden, Dresden, Germany), Lise Allard (Department of Pediatrics, Angers University Hospital, Angers, France), Ludwig Patzer (Children’s Hospital St Elisabeth and St Barbara, Halle, Germany), Marina Shumikhina (Filatov Children’s Clinical Hospital No. 13, Moscow, Russia), Matthias Hansen (KfH Centre of Paediatric Nephrology, Clementine Children’s Hospital, Frankfurt, Germany), Nikoleta Printza (First Pediatric Department, Aristotle University, Thessaloniki, Greece), Nuran Küc¸ük (Kartal Dr. Lütfi Kırdar Training and Research Hospital, İstanbul, Turkey), Ortraud Beringer (University Children’s Hospital, Ulm, Germany), Rajendra Bhimma (Inkosi Albert Luthuli, Central Hospital, Durban, South Africa), Rimante Cerkauskiene (Faculty of Medicine, Children’s Hospital, Vilnius University, Vilnius, Lithuania; Santaros Klinikos, Vilnius University Hospital, Vilnius, Lithuania), Thomas J. Neuhaus (Children’s Hospital of Lucerne, Cantonal Hospital of Lucerne, Lucerne, Switzerland), Valbona Stavileci (Pediatric Clinic, Prishtina, Kosovo), Tim Ulinski (Pediatric Nephrology Department, Armand Trousseau University Hospital, APHP, Paris, France), Nida Temizkan Dincel (Health Sciences University, Izmir Dr Behcet Uz Children’s Hospital, İzmir, Turkey) and Nilufar Mohebbi (Division of Nephrology, University Hospital Zurich, Zurich, Switzerland)
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- 2019
24. IPNA Clinical Practice Recommendations for the Diagnosis and Management of Children with Steroid-resistant Nephrotic Syndrome
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Trautmann, Agnes, Vivarelli, Marina, Samuel, Susan, Gipson, Debbie, Sinha, Aditi, Schaefer, Franz, Hui, Ng Kar, Boyer, Olivia, Saleem, Moin A, Feltran, Luciana, Müller-Deile, Janina, Becker, Jan Ulrich, Cano, Francisco, Xu, Hong, Lim, Yam Ngo, Smoyer, William, Anochie, Ifeoma, Nakanishi, Koichi, Hodson, Elisabeth, Haffner, Dieter, and International Pediatric Nephrology Association
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Male ,Nephrology ,Pediatrics ,medicine.medical_specialty ,Nephrotic Syndrome ,Adolescent ,Population ,Drug Resistance ,Guidelines ,Prednisone ,Internal medicine ,Chronic kidney disease ,Genetics ,medicine ,Humans ,ddc:610 ,Child ,education ,Glucocorticoids ,Congenital nephrotic syndrome ,Children ,Steroid-resistant nephrotic syndrome ,Outcome ,education.field_of_study ,business.industry ,Remission Induction ,Infant, Newborn ,Infant ,medicine.disease ,Immunosuppressive treatment ,Transplantation ,Clinical Practice ,Proteinuria ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Female ,business ,Nephrotic syndrome ,Immunosuppressive Agents ,medicine.drug ,Kidney disease - Abstract
Idiopathic nephrotic syndrome newly affects 1–3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4–6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10–30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given., 論文
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- 2020
25. Treatment and long-term outcome in primary nephrogenic diabetes insipidus
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Lopez-Garcia, Sergio C, Downie, Mallory L, Kim, Ji Soo, Boyer, Olivia, Walsh, Stephen B, Nijenhuis, Tom, Papizh, Svetlana, Yadav, Pallavi, Reynolds, Ben C, Decramer, Stéphane, Besouw, Martine, Perelló Carrascosa, Manel, La Scola, Claudio, Trepiccione, Francesco, Ariceta, Gema, Hummel, Aurélie, Dossier, Claire, Sayer, John A, Konrad, Martin, Keijzer-Veen, Mandy G, Awan, Atif, Basu, Biswanath, Chauveau, Dominique, Madariaga, Leire, Koster-Kamphuis, Linda, Furlano, Mónica, Zacchia, Miriam, Marzuillo, Pierluigi, Tse, Yincent, Dursun, Ismail, Pinarbasi, Ayse Seda, Tramma, Despoina, Hoorn, Ewout J, Gokce, Ibrahim, Nicholls, Kathleen, Eid, Loai A, Sartz, Lisa, Riordan, Michael, Hooman, Nakysa, Printza, Nikoleta, Bonny, Olivier, Arango Sancho, Pedro, Schild, Raphael, Sinha, Rajiv, Guarino, Stefano, Martinez Jimenez, Victor, Rodríguez Peña, Lidia, Belge, Hendrica, Devuyst, Olivier, Wlodkowski, Tanja, Emma, Francesco, Levtchenko, Elena, Knoers, Nine V A M, Bichet, Daniel G, Schaefer, Franz, Kleta, Robert, Wasilewska, Anna, Longo, Germana, Espinosa, Laura, Miglinas, Marius, Stroescu, Ramona, Huseynova, Shafa, Stabouli, Stella, Sathyanarayana, Vijaya, Andronesi, Andreea G, Hahn, Deirdre, Sharma, Deepak, Petrosyan, Edita, Frangou, Eleni, Mohebbi, Nilufar, Dinçel, Nida Temizkan, Braconnier, Philippe, Gilbert, Rodney D, Sambo, Adamu, Tasic, Velibor, Henne, Thomas, Bockenhauer, Detlef, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, Lopez-Garcia, Sergio C, Downie, Mallory L, Kim, Ji Soo, Boyer, Olivia, Walsh, Stephen B, Nijenhuis, Tom, Papizh, Svetlana, Yadav, Pallavi, Reynolds, Ben C, Decramer, Stéphane, Besouw, Martine, Perelló Carrascosa, Manel, La Scola, Claudio, Trepiccione, Francesco, Ariceta, Gema, Hummel, Aurélie, Dossier, Claire, Sayer, John A, Konrad, Martin, Keijzer-Veen, Mandy G, Awan, Atif, Basu, Biswanath, Chauveau, Dominique, Madariaga, Leire, Koster-Kamphuis, Linda, Furlano, Mónica, Zacchia, Miriam, Marzuillo, Pierluigi, Tse, Yincent, Dursun, Ismail, Pinarbasi, Ayse Seda, Tramma, Despoina, Hoorn, Ewout J, Gokce, Ibrahim, Nicholls, Kathleen, Eid, Loai A, Sartz, Lisa, Riordan, Michael, Hooman, Nakysa, Printza, Nikoleta, Bonny, Olivier, Arango Sancho, Pedro, Schild, Raphael, Sinha, Rajiv, Guarino, Stefano, Martinez Jimenez, Victor, Rodríguez Peña, Lidia, Belge, Hendrica, Devuyst, Olivier, Wlodkowski, Tanja, Emma, Francesco, Levtchenko, Elena, Knoers, Nine V A M, Bichet, Daniel G, Schaefer, Franz, Kleta, Robert, and Bockenhauer, Detlef
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Nephrology ,Pediatrics ,medicine.medical_specialty ,endocrine system diseases ,030232 urology & nephrology ,030204 cardiovascular system & hematology ,urologic and male genital diseases ,03 medical and health sciences ,0302 clinical medicine ,nephrogenic diabetes insipidus ,Internal medicine ,medicine ,AVPR2 ,Transplantation ,business.industry ,AQP2 ,medicine.disease ,Nephrogenic diabetes insipidus ,Mental health ,Obesity ,female genital diseases and pregnancy complications ,Cohort ,flow uropathy ,business ,Body mass index ,chronic kidney disease ,Kidney disease - Abstract
BackgroundPrimary nephrogenic diabetes insipidus (NDI) is a rare disorder and little is known about treatment practices and long-term outcome.MethodsPaediatric and adult nephrologists contacted through European professional organizations entered data in an online form.ResultsData were collected on 315 patients (22 countries, male 84%, adults 35%). Mutation testing had been performed in 270 (86%); pathogenic variants were identified in 258 (96%). The median (range) age at diagnosis was 0.6 (0.0–60) years and at last follow-up 14.0 (0.1–70) years. In adults, height was normal with a mean (standard deviation) score of −0.39 (±1.0), yet there was increased prevalence of obesity (body mass index >30 kg/m2; 41% versus 16% European average; P ConclusionThis large NDI cohort shows an overall favourable outcome with normal adult height and only mild to moderate CKD in most. Yet, while full-time employment was similar to the European average, educational achievement was lower, and more than half had urological and/or mental health problems.
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- 2020
26. Effects of Chronic Kidney Disease on Nanomechanics of the Endothelial Glycocalyx Are Mediated by the Mineralocorticoid Receptor.
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Fels, Benedikt, Beyer, Arne, Cazaña-Pérez, Violeta, Giraldez, Teresa, Navarro-González, Juan F., de la Rosa, Diego Alvarez, Schaefer, Franz, Bayazit, Aysun K., Obrycki, Łukasz, Ranchin, Bruno, Holle, Johannes, Querfeld, Uwe, and Kusche-Vihrog, Kristina
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MINERALOCORTICOID receptors ,CHRONIC kidney failure ,NANOMECHANICS ,ATOMIC force microscopy ,PULSE wave analysis ,ENDOTHELIAL cells ,GLYCOCALYX - Abstract
Endothelial mechanics control vascular reactivity and are regulated by the mineralocorticoid receptor (MR) and its downstream target, the epithelial Na
+ channel (ENaC). Endothelial dysfunction is a hallmark of chronic kidney disease (CKD), but its mechanisms are poorly understood. We hypothesized that CKD disrupts endothelial mechanics in an MR/ENaC-dependent process. Methods: Primary human endothelial cells were cultured with uremic serum derived from children with stage 3–5 (predialysis) CKD or adult hemodialysis (HD) patients or healthy controls. The height and stiffness of the endothelial glycocalyx (eGC) and cortex were monitored by atomic force microscopy (AFM) using an ultrasensitive mechanical nanosensor. Results: In a stage-dependent manner, sera from children with CKD induced a significant increase in eGC and cortex stiffness and an incremental reduction of the eGC height. AFM measurements were significantly associated with individual pulse wave velocity and serum concentrations of gut-derived uremic toxins. Serum from HD patients increased MR expression and mechanical stiffness of the endothelial cortex, an effect reversed by MR and ENaC antagonists, decreased eNOS expression and NO bioavailability, and augmented monocyte adhesion. Conclusion: These data indicate progressive structural damage of the endothelial surface with diminishing kidney function and identify the MR as a mediator of CKD-induced endothelial dysfunction. [ABSTRACT FROM AUTHOR]- Published
- 2022
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27. Therapeutic strategies to slow chronic kidney disease progression
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Wühl, Elke and Schaefer, Franz
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- 2008
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28. Hypertension in children with chronic kidney disease: pathophysiology and management
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Hadtstein, Charlotte and Schaefer, Franz
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- 2008
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29. Genetic testing in the diagnosis of chronic kidney disease: recommendations for clinical practice.
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Knoers, Nine, Antignac, Corinne, Bergmann, Carsten, Dahan, Karin, Giglio, Sabrina, Heidet, Laurence, Lipska-Ziętkiewicz, Beata S, Noris, Marina, Remuzzi, Giuseppe, Vargas-Poussou, Rosa, and Schaefer, Franz
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CHRONIC kidney failure ,GENETIC testing ,KIDNEY disease diagnosis ,GENETIC disorder diagnosis ,GENETIC counseling - Abstract
The overall diagnostic yield of massively parallel sequencing–based tests in patients with chronic kidney disease (CKD) is 30% for paediatric cases and 6–30% for adult cases. These figures should encourage nephrologists to frequently use genetic testing as a diagnostic means for their patients. However, in reality, several barriers appear to hinder the implementation of massively parallel sequencing–based diagnostics in routine clinical practice. In this article we aim to support the nephrologist to overcome these barriers. After a detailed discussion of the general items that are important to genetic testing in nephrology, namely genetic testing modalities and their indications, clinical information needed for high-quality interpretation of genetic tests, the clinical benefit of genetic testing and genetic counselling, we describe each of these items more specifically for the different groups of genetic kidney diseases and for CKD of unknown origin. [ABSTRACT FROM AUTHOR]
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- 2022
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30. Effects of nutritional Vitamin D supplementation on markers of bone and mineral metabolism in children with chronic kidney disease
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Cakar, N., Basin, E., Yalcinkaya, F., KARABAY BAYAZIT, AYSUN, Anarat, A., Laube, G., Bucher, B., Peco-Antic, A., Texeira, A., Donmez, O., Balat, A., Szczepanska, M., Niemirska, A., Litwin, M., Urasinski, T., Tkaczyk, M., Kiyak, A., Drodz, D., Zurowska, A., Azukaitis, K., Jankauskiene, A., Picca, S., Matteucci, C., Vidal, E., Testa, S., Lugani, F., Montini, G., Wigger, M., Kranz, B., Jeck, N., Wygoda, S., Pohl, M., Wuehl, E., Doyon, A., Schaefer, F., Thurn, D., Melk, A., Kemper, M., ÇALIŞKAN, Salim, Gimpel, C., Buescher, R., Galiano, M., Habbig, S., Querfeld, U., Zalosczyk, A., Fischbach, M., Ranchin, B., Harambat, J., Canpolat, N., Dusek, J., Arbeiter, K., Cortina, G., Haffner, Dieter, Candan, C., Schaefer, Franz, Sander, Anja, Leifheit-Nestler, Maren, Civilibal, M., Querfeld, Uwe, Melk, Anette, Rosales, Alejandra, Candan, Cengiz, Soylemezoglu, Oguz, Zaloszyc, Ariane, Habbig, Sandra, ALPAY, HARİKA, Emre, S., Alpay, H., Ozcelik, G., Kiyak, Aysel, Harambat, Jerome, Mir, S., Sozeri, B., Yalcinkaya, Fatos, Yilmaz, Ebru, Azukaitis, Karolis, Yavascan, O., DÜZOVA, ALİ, CANPOLAT, Nur, Niemirska, Anna, Tabel, Y., Ertan, P., Thurn, Daniela, KAPLAN BULUT, İPEK, Lerch, Christian, Yilmaz, E., Prytula, A., Bachetta, J., Haffner, D., Klaus, G., Gessner, M., Schmitt, C. P., Stabouli, S., Reusz, G., Verrina, E., Groothoff, J., Tondel, C., Gamero, M. A., Petrosyan, E., Dursun, I., Erdogan, H., Bilginer, Y., Soylemezoglu, O., Shroff, Rukshana, Shroff, R., Wan, Mandy, Bakkaloglu, SEVCAN AZİME, Aitkenhead, Helen, Rees, Lesley, Çukurova Üniversitesi, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, ARD - Amsterdam Reproduction and Development, Paediatric Nephrology, AGEM - Inborn errors of metabolism, APH - Quality of Care, APH - Methodology, Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children's Hospital, Hannover, Germany, Renal Unit, Great Ormond Street Hospital for Children, London, United Kingdom, Department of Chemical Pathology, Great Ormond Street Hospital for Children, London, United Kingdom, Department of Pediatric Nephrology, Ege University, Bornova, Izmir, Turkey, Department of Pediatric Nephrology, Faculty of Medicine, Cukurova University, Adana, Turkey, Department of Nephrology, Kidney Transplantation and Arterial Hypertension, Children's Memorial Health Institute, Warsaw, Poland, Department of Pediatrics, Istanbul University Cerrahpasa, Faculty of Medicine, Istanbul, Turkey, Division of Pediatric Nephrology, Hacettepe University, Faculty of Medicine, Ankara, Turkey, Clinic of Pediatrics, Faculty of Medicine, Vilnius University, Vilnius, Lithuania, Department of Pediatric Nephrology, Sanliurfa Children's Hospital, Sanliurfa, Turkey, Department of Pediatric Nephrology, School of Medicine, Ankara University, Ankara, Turkey, Department of Pediatrics, Bordeaux University Hospital, Bordeaux, France, Department of Pediatric Nephrology, Yenimahalle Egitim ve Arastirma Hastanesi Bakirkoy, Istanbul, Turkey, Department of Pediatric Nephrology, Marmara University School of Medicine, Istanbul, Turkey, Division of Pediatric Nephrology, University Children's, Adolescent's Hospital, Cologne, Germany, Pole Médico-Chirurgical de Pédiatrie, Service de Pédiatrie I, Hôpitaux Universitaires de Strasbourg, Strasbourg, France, Department of Pediatric Nephrology, Gazi University Hospital, Ankara, Turkey, Department of Pediatric Nephrology, Göztepe Egitim ve Arastirma Hastanesi, Cocuk Klinigi, Göztepe, Istanbul, Turkey, Department of Pediatrics, Innsbruck Medical University, Innsbruck, Austria, Clinic of Pediatric Nephrology, Charite Children's Hospital, Berlin, Germany, Institute of Medical Biometry and Informatics, University Heidelberg, Heidelberg, Germany, Division of Pediatric Nephrology, Heidelberg, Germany, Children's Hospital, Innsbruck, Austria, University Children's Hospital, Vienna, Austria, University Hospital Motol, Prague, Czech Republic, Hôpital des Enfants, Bordeaux, France, Hôpital Femme Mère Enfant, Université de Lyon, France, Hôpital de Hautepierre, Strasbourg, France, Charité Children's Hospital, Berlin, Germany, University Children's Hospital, Cologne, Germany, University Children's Hospital, Erlangen, Germany, University Children's Hospital, Essen, Germany, Center for Pediatrics and Adolescent Medicine, Freiburg, Germany, UKE University Children's Hospital, Hamburg, Germany, Hannover Medical School, Hannover, Germany, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany, Center for Pediatrics and Adolescent Medicine, Jena, Germany, City Hospital St. Georg, Leipzig, Germany, KfH Kidney Center for Children, Marburg, Germany, University Children's Hospital, Münster, Germany, Children's Hospital, Rostock, Germany, S. Orsola-Malpighi Hospital, Bologna, Italy, Istituto Giannina Gaslini, Genova, Italy, Fondazione Ospedale Maggiore Policlinico, Milano, Italy, Pediatric Nephrology, Dialysis and Transplant Unit, Padova, Italy, Ospedale Bambino Gesú, Rome, Italy, University Children's Hospital, Vilnius, Lithuania, Pediatric and Adolescent Nephrology, Gdansk, Poland, University Children's Hospital, Krakow, Poland, Polish Mothers Memorial Hospital Research Institute, Lodz, Poland, Clinic of Pediatrics, Szczecin, Poland, Children's Memorial Health Institute, Warsaw, Poland, Zabrze, Poland, Hospital Sao Joao, Porto, Portugal, University Children's Hospital, Belgrade, Serbia, Inselspital, Bern, Switzerland, University Children's Hospital, Zurich, Switzerland, Cukurova University, Adana, Turkey, University Faculty of Medicine, Ankara, Turkey, Baskent University, Faculty of Medicine, Ankara, Turkey, Diskapi Children's Hospital, Ankara, Turkey, Gazi University Hospital, Ankara, Turkey, Hacettepe Medical Faculty, Ankara, Turkey, Dortcelik Children's Hospital, Bursa, Turkey, Uludag University, Bursa, Turkey, University of Gaziantep, Turkey, Bakirkoy Children's Hospital, Istanbul, Turkey, Istanbul University Cerrahpasa, Faculty of Medicine, Istanbul, Turkey, Goztepe Educational and Research Hospital, Istanbul, Turkey, Haseki Educational and Research Hospital, Istanbul, Turkey, Istanbul Medical Faculty, Istanbul, Turkey, Marmara University Medical Faculty, Istanbul, Turkey, Sisli Educational and Research Hospital, Istanbul, Turkey, Ege University Medical Faculty, Izmir, Turkey, Tepecik Training and Research Hospital, Izmir, Turkey, Inonu University, Malatya, Turkey, Celal Bayar University, Manisa, Turkey, Ghent University, Utopaed, Belgium, University Children's Hospital, Lyon, France, University Children's Hospital, Marburg, Germany, University Children's Hospital, Tübingen, Germany, University Children's Hospital, Thessaloniki, Greece, Semmelweis University, Budapest, Hungary, G. Gaslini Institute, Genoa, Italy, Academic Medical Center, Amsterdam, Netherlands, University Children's Hospital, Bergen, Norway, Reina Sofia Universitary Hospital, Cordoba, Spain, Russian National Research Medical University, Moscow, Russian Federation, Erciyes University, Faculty of Medicine, Kayseri, Turkey, Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı., Dönmez, Osman, AAA-8778-2021, Lerch, Christian, Shroff, Rukshana, Wan, Mandy, Rees, Lesley, Aitkenhead, Helen, Bulut, Ipek Kaplan, Thurn, Daniela, Bayazit, Aysun Karabay, Niemirska, Anna, Canpolat, Nur, Duzova, Ali, Azukaitis, Karolis, Yilmaz, Ebru, Yalcinkaya, Fatos, Harambat, Jerome, Kiyak, Aysel, Alpay, Harika, Habbig, Sandra, Zaloszyc, Ariane, Soylemezoglu, Oguz, Candan, Cengiz, Rosales, Alejandra, Melk, Anette, Querfeld, Uwe, Leifheit-Nestler, Maren, Sander, Anja, Schaefer, Franz, Haffner, Dieter, Cortina, G., Arbeiter, K., Dusek, J., Harambat, J., Ranchin, B., Fischbach, M., Zalosczyk, A., Querfeld, U., Habbig, S., Galiano, M., Buescher, R., Gimpel, C., Kemper, M., Melk, A., Thurn, D., Schaefer, F., Doyon, A., Wuehl, E., Pohl, M., Wygoda, S., Jeck, N., Kranz, B., Wigger, M., Montini, G., Lugani, F., Testa, S., Vidal, E., Matteucci, C., Picca, S., Jankauskiene, A., Azukaitis, K., Zurowska, A., Drodz, D., Tkaczyk, M., Urasinski, T., Litwin, M., Niemirska, A., Szczepanska, M., Texeira, A., Peco-Antic, A., Bucher, B., Laube, G., Anarat, A., Bayazit, A. K., Yalcinkaya, F., Basin, E., Cakar, N., Soylemezoglu, O., Duzova, A., Bilginer, Y., Erdogan, H., Donmez, O., Balat, A., Kiyak, A., Caliskan, S., Canpolat, N., Candan, C., Civilibal, M., Emre, S., Alpay, H., Ozcelik, G., Mir, S., Sozeri, B., Yavascan, O., Tabel, Y., Ertan, P., Yilmaz, E., Shroff, R., Prytula, A., Bachetta, J., Haffner, D., Klaus, G., Gessner, M., Schmitt, C. P., Stabouli, S., Reusz, G., Verrina, E., Groothoff, J., Tondel, C., Gamero, M. A., Petrosyan, E., Bakkaloglu, S. A., and Dursun, I.
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Male ,Fibroblast growth factor 23 ,Comorbidity ,urologic and male genital diseases ,Dietary supplement ,0302 clinical medicine ,Chronic kidney disease ,Chronic ,Child ,Klotho ,Children ,Clinical outcome ,Serum sclerostin ,Double blind procedure ,Vitamins ,Multicenter study ,female genital diseases and pregnancy complications ,3. Good health ,Clinical trial ,Bone and mineral metabolism ,Vitamin D deficiency ,Vitamin D supplementation ,Adolescent ,Alkaline Phosphatase ,Biomarkers ,Bone Density ,Double-Blind Method ,Female ,Fibroblast Growth Factors ,Follow-Up Studies ,Glomerular Filtration Rate ,Humans ,Renal Insufficiency, Chronic ,Vitamin D ,Dietary Supplements ,Blood ,Randomized controlled trial ,Nephrology ,Cohort analysis ,Human ,medicine.medical_specialty ,Mineral metabolism ,Sclerostin ,Clinical article ,Diet supplementation ,Article ,vitamin D deficiency ,Ergocalciferol ,03 medical and health sciences ,Cholecalciferol supplementation ,CKD ,Vitamin D and neurology ,Follow up ,medicine.disease ,Renal-failure ,Clinical effectiveness ,Endocrinology ,chemistry ,School child ,Chronic kidney failure ,Physiology ,Beta Glucuronidase ,Klotho Protein ,Chronic Kidney Disease-Mineral and Bone Disorder ,Fibroblast growth factor ,030232 urology & nephrology ,Medizin ,030204 cardiovascular system & hematology ,Growth-factor 23 ,chemistry.chemical_compound ,Randomized controlled trial (topic) ,Urology & nephrology ,Estimated glomerular filtration rate ,Renal Insufficiency ,Alpha-klotho ,Protein expression level ,Vitamin supplementation ,Priority journal ,Double-blind ,Klotho protein ,Glomerulus filtration rate ,medicine.drug ,Vitamin ,Bone metabolism ,Pathophysiology ,Hemodialysis-patients ,Internal medicine ,medicine ,Renal insufficiency, chronic ,FGF-23 ,Transplantation ,business.industry ,Alkaline phosphatase bone isoenzyme ,Fibroblast Growth Factor-23 ,Biological marker ,Metabolism ,business ,Controlled study ,Kidney disease - Abstract
PubMedID: 29481636 Background: We investigated the effects of nutritional vitamin D supplementation on markers of bone and mineral metabolism, i.e. serum levels of fibroblast growth factor 23(FGF23), Klotho, bone alkaline phosphatase (BAP) and sclerostin, in two cohorts with chronic kidney disease (CKD). Methods: In all, 80 vitamin D-deficient children were selected: 40 with mild to moderate CKD from the ERGO study, a randomized trial of ergocalciferol supplementation [estimated glomerular filtration rate (eGFR) 55 mL/min/1.73 m 2 ], and 40 with advanced CKD from the observational Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study (eGFR 24 mL/min/1.73 m 2 ). In each study, vitamin D supplementation was started in 20 children and 20 matched children not receiving vitamin D served as controls. Measures were taken at baseline and after a median period of 8 months. Age- and gender-related standard deviation scores (SDSs) were calculated. Results: Before vitamin D supplementation, children in the ERGO study had normal FGF23 (median 0.31 SDS) and BAP (-0.10 SDS) but decreased Klotho and sclerostin (-0.77 and - 1.04 SDS, respectively), whereas 4C patients had increased FGF23 (3.87 SDS), BAP (0.78 SDS) and sclerostin (0.76 SDS) but normal Klotho (-0.27 SDS) levels. Vitamin D supplementation further increased FGF23 in 4C but not in ERGO patients. Serum Klotho and sclerostin normalized with vitamin D supplementation in ERGO but remained unchanged in 4C patients. BAP levels were unchanged in all patients. In the total cohort, significant effects of vitamin D supplementation were noted for Klotho at eGFR 40-70mL/min/1.73m 2 . Conclusions: Vitamin D supplementation normalized Klotho and sclerostin in children with mild to moderate CKD but further increased FGF23 in advanced CKD. © The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. American Society of Pediatric Nephrology: ESPN 2014.3 This work was supported by the European Society for Pediatric Nephrology (reference number ESPN 2014.3), KfH Foundation for Preventive Medicine and ERA-EDTA (to D.H.).
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- 2018
31. Impaired Systolic and Diastolic Left Ventricular Function in Children with Chronic Kidney Disease - Results from the 4C Study
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Doyon, Anke, Haas, Pascal, Erdem, Sevcan, Ranchin, Bruno, Kassai, Behrouz, Mencarelli, Francesca, Lugani, Francesca, Harambat, Jerome, Matteucci, Maria Chiara, Chinali, Marcello, Habbig, Sandra, Zaloszyc, Ariane, Testa, Sara, Vidal, Enrico, Gimpel, Charlotte, Azukaitis, Karolis, Kovacevic, Alexander, Querfeld, Uwe, Schaefer, Franz, Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche en Informatique et en Automatique (Inria)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Bordeaux population health (BPH), Université de Bordeaux (UB)-Institut de Santé Publique, d'Épidémiologie et de Développement (ISPED)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Bordeaux [Bordeaux], and Hôpital de Hautepierre [Strasbourg]
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Male ,Adolescent ,Systole ,Heart Ventricles ,lcsh:Medicine ,[SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology ,Article ,Ventricular Function, Left ,Ventricular Dysfunction, Left ,[SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system ,Diastole ,Chronic kidney disease ,Humans ,Prospective Studies ,Child ,lcsh:Science ,[SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics ,Carotid artery disease ,lcsh:R ,Atherosclerosis ,Echocardiography, Doppler ,Hypertension ,cardiovascular system ,Kidney Failure, Chronic ,Female ,Hypertrophy, Left Ventricular ,lcsh:Q ,[SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie ,tissue doppler-echocardiography ,European-society ,heart-failure ,dysfunction - Abstract
International audience; Children with chronic kidney disease suffer from excessive cardiovascular mortality and early alterations of the cardiovascular system. Tissue doppler imaging is a validated echocardiographic tool to assess early systolic and diastolic cardiac dysfunction. We hypothesized that tissue Doppler velocities would reveal reduced cardiac function in children with chronic kidney disease compared to healthy children. A standardized echocardiographic exam was performed in 128 patients of the Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) Study aged 6-17 years with an estimated glomerular filtration rate (eGFR) below 60 ml/min/1.73 m(2). Tissue Doppler measurements included early (E') and late (A') diastolic and systolic (S') velocity at the mitral and septal annulus of the left ventricle. Measured values were normalized to z-scores using published reference data. Predictors of E'/A', E/E', S' and left ventricular mass index (LVMI) were assessed by multiple linear regression analyses. Tissue Doppler E' was reduced and tissue Doppler A' increased, resulting in a reduced tissue Doppler E'/A' ratio (z-score -0.14, p < 0.0001) indicating reduced diastolic function compared to healthy children. Reduced tissue Doppler E'/A' Z-Scores were independently associated with lower eGFR (p = 0.002) and increased systolic blood pressure (p = 0.02). While E/E' Z-Scores were increased (Z-score 0.57, p < 0.0001), patients treated with pharmacological RAS blockade but not with other antihypertensive treatments had significantly lower E/E' and higher E'/A' Z-Scores. Systolic tissue Doppler velocities were significantly decreased (Z-score -0.24, p = 0.001) and inversely correlated with E/E' Z-Scores (r = -0.41, p < 0.0001). LVMI was not associated with systolic or diastolic tissue Doppler velocities. Concentric left ventricular hypertrophy showed a tendency to lower S' in multivariate analysis (p = 0.13) but no association to diastolic function. Concentric left ventricular geometry was significantly associated with lower midwall fractional shortening. In summary, systolic and diastolic function assessed by tissue Doppler is impaired. eGFR, systolic blood pressure and the type of antihypertensive medications are significant predictors of diastolic function in children with CKD. Left ventricular morphology is largely independent of tissue Doppler velocities. Tissue Doppler velocities provide sensitive information about early left ventricular dysfunction in this population.
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- 2019
32. Determinants of Statural Growth in European Children With Chronic Kidney Disease: Findings From the Cardiovascular Comorbidity in Children With Chronic Kidney Disease (4C) Study
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Behnisch, Rouven, Kirchner, Marietta, Anarat, Ali, Bacchetta, Justine, Shroff, Rukshana, Bilginer, Yelda, Mir, Sevgi, Caliskan, Salim, Paripovic, Dusan, Harambat, Jerome, Mencarelli, Francesca, Buescher, Rainer, Arbeiter, Klaus, Soylemezoglu, Oguz, Zaloszyc, Ariane, Zurowska, Aleksandra, Melk, Anette, Querfeld, Uwe, Schaefer, Franz, Bordeaux population health (BPH), Université de Bordeaux (UB)-Institut de Santé Publique, d'Épidémiologie et de Développement (ISPED)-Institut National de la Santé et de la Recherche Médicale (INSERM), and Ege Üniversitesi
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Medizin ,Pediatrics ,anemia ,LEHA ,GFR-glomerular filtration rate ,hyperparathyroidism ,children ,statural growth ,Pediatrics, Perinatology and Child Health ,[SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie ,acidosis ,GFR—glomerular filtration rate ,chronic kidney disease ,Original Research ,height - Abstract
WOS: 000474288200002, PubMed ID: 31334210, Failure of statural growth is one of the major long-term sequelae of chronic kidney disease (CKD) in children. In recent years effective therapeutic strategies have become available that lead to evidence based practice recommendations. To assess the current growth performance of European children and adolescents with CKD, we analyzed a cohort of 594 patients from 12 European countries who were followed prospectively for up to 6 years in the 4C Study. While all patients were on conservative treatment with a mean estimated glomerular filtration rate of 28 ml/min/1.73 m(2) at study entry, 130 children commenced dialysis during the observation period. At time of enrolment the mean height standard deviation score (SDS) was -1.57; 36% of patients had a height below the third percentile. The prevalence of growth failure varied between countries from 7 to 44% Whereas patients on conservative treatment showed stable growth, height SDS gradually declined on those on dialysis. Parental height, pubertal status and treatment with recombinant growth hormone (GH) were positively, and the diagnosis of syndromic disease and CKD stage were negatively associated with height SDS during the observation period. Unexpectedly, higher body mass index (BMI) SDS was associated with lower height SDS both at enrolment and during follow up. Renal anemia, metabolic acidosis, and hyperparathyroidism were mostly mild and not predictive of growth rates by multivariable analysis. GH therapy was applied in only 15% of growth retarded patients with large variation between countries. When adjusting for all significant covariates listed above, the country of residence remained a highly significant predictor of overall growth performance. In conclusion, growth failure remains common in European children with CKD, despite improved general management of CKD complications. The widespread underutilization of GH, an approved efficacious therapy for CKD-associated growth failure, deserves further exploration., ERA-EDTA Research Programme; KfH Foundation for Preventive Medicine; German Federal Ministry of Education and ResearchFederal Ministry of Education & Research (BMBF) [01EO0802], Support for the 4C Study was received from the ERA-EDTA Research Programme, the KfH Foundation for Preventive Medicine and the German Federal Ministry of Education and Research (reference number: 01EO0802). The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.
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- 2019
33. Low levels of urinary epidermal growth factor predict chronic kidney disease progression in children
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Mir, Sevgi, Hohbach-Hohenfellner, Katharina, Jeck, Nickola, Smith, Michelle, Klaus, Guenter, Ardissino, Gianluigi, Testa, Sara, Montini, Giovanni, Charbit, Marina, Niaudet, Patrick, Afonso, Alberto Caldas, Fernandes-Teixeira, Ana, Peruzzi, Licia, Arbeiter, Klaus, Jankauskiene, Augustina, Grenda, Ryszard, Litwin, Mieczyslaw, Neuhaus, Thomas J., Fang, Zhiyin, Dusek, Jiri, Matteucci, Chiara, Azukaitis, Karolis, Picca, Stefano, Ju, Wenjun, Thurn-Valsassina, Daniela, Kirchner, Marietta, Wigger, Marianne, Berg, Ulla B., Celsi, Giovanni, Fischbach, Michel, Terzic, Joelle, Vidal, Enrico, TABEL, YILMAZ, Gimpel, Charlotte, ERTAN, PELİN, Yavascan, Onder, Melk, Anette, Querfeld, Uwe, Wuehl, Elke, Kretzler, Matthias, Schaefer, Franz, Rosales, Alejandra, Zaloszyc, Ariane, Gellermann, Jutta, Liebau, Max, Weber, Lutz, Muschiol, Evelin, Buescher, Rainer, Oh, Jun, Thurn-Valassina, Daniela, Haffner, Dieter, John, Ulrike, Wygoda, Simone, Jeck, Nikola, Murer, Luisa, Drozdz, Dorota, Coppo, Rosanna, Lugani, Francesca, Zurowska, Aleksandra, Zaniew, Marcin, Nimierska, Anna, Teixeira, Ana, Peco-Antic, Amira, Laube, Guido, Dali, Cocuk Nefrolojisi Bilim, Anarat, Ali, Bayazit, Aysun, DÜZOVA, ALİ, BİLGİNER, YELDA, ÇALIŞKAN, Salim, CANPOLAT, Nur, Civilibal, Mahmut, Shroff, Rukshana, Soezeri, Betul, Kranz, Brigitta, Mencarelli, Francesca, Dorn, Brigitte, Nair, Viji, Yalcinkaya, Fatos, Baskin, Esra, Cakar, Nilgun, Soylemezoglu, Oguz, Emre, Sevinc, Candan, Cengiz, Kiyak, Aysel, Ozcelik, Gul, Alpay, Harika, Rachin, Bruno, Szczepanska, Maria, Erdogan, Hakan, DÖNMEZ, OSMAN, Balat, Ayse, Aksu, Nejat, Tabel, Yilmaz, Ertan, Pelin, Yilmaz, Ebru, Bakkaloglu, Aysin, ÖZALTIN, FATİH, Sallay, Peter, Bonzel, Klaus-Eugen, Wingen, Anna-Margrete, Urowska, Aleksandra Z., Balasz, Irena, Trivelli, Antonella, Perfumo, Francesco, Mueller-Wiefel, Dirk-Erhard, Moeller, Kerstin, Offner, Gisela, Enke, Barbara, Hadtstein, Charlotte, Mehls, Otto, Fydryk, Janusz, Urasinski, Tomasz, KARABAY BAYAZIT, AYSUN, Niemirska, Anna, KAPLAN BULUT, İPEK, Paripovic, Dusan, Harambat, Jerome, ÇAKAR, NİLGÜN, ALPAY, HARİKA, Çukurova Üniversitesi, Bursa Uludağ Üniversitesi/Tıp Fakültesi/Dahili Tıp Bilimleri/Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı., Dönmez, Osman, AAA-8778-2021, Clinic of Pediatrics, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, Vilnius, Lithuania, Department of Internal Medicine/Nephrology, University of Michigan, Ann Arbor, MI, United States, Department of Computational Medicine and Bioinformatics, University of Michigan, Ann Arbor, MI, United States, Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany, Department of Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany, Department of Pediatric Nephrology, Cukurova University, Adana, Turkey, Department of Nephrology, Kidney Transplantation and Arterial Hypertension, The Children`s Memorial Health Institute, Warsaw, Poland, Department of Pediatric Nephrology, Istanbul University Cerrahpasa Faculty of Medicine, Istanbul, Turkey, Division of Pediatric Nephrology, Department of Pediatrics, Ege University Faculty of Medicine, Izmir, Turkey, Division of Pediatric Nephrology, Department of Pediatrics, School of Medicine, Ankara University, Ankara, Turkey, Department of Pediatric Nephrology, University Children's Hospital, Belgrade, Serbia, Department of Pediatrics, Bordeaux University Hospital, Bordeaux, France, Department of Pediatric Nephrology, Marmara University Faculty of Medicine, Istanbul, Turkey, Division of Nephrology, Dialysis, Transplantation, University of Genoa, G. Gaslini Institute, Genoa, Italy, Pediatric Nephrology Unit, Department of Pediatrics, S.Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy, Department of Pediatric Nephrology, Haseki Educational and Research Hospital, Istanbul, Turkey, Department of Pediatric Nephrology, Bursa Yuksek Ihtisas Teaching and Researching Hospital, Bursa, Turkey, Pediatric Nephrology, Charité Children's Hospital, Berlin, Germany, Pediatric Nephrology, Dialysis and Transplantation Unit, Department of Woman's and Child's Health, University-Hospital of Padova, Padova, Italy, Department of Pediatric Nephrology, Faculty of Medicine, İnönü University, Malatya, Turkey, Department of General Pediatrics, Adolescent Medicine and Neonatology, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany, Department of Pediatric Nephrology, Celal Bayar University, Manisa, Turkey, Department of Pediatric Nephrology, University of Health Sciences, İzmir Tepecik Training and Research Hospital, İzmir, Turkey, Department of Pediatric Nephrology, Charité University, Berlin, Germany, and Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany
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Male ,0301 basic medicine ,Chronic kidney failure ,Physiology ,Protein function ,Medizin ,030232 urology & nephrology ,Comorbidity ,Urine ,urologic and male genital diseases ,Gastroenterology ,0302 clinical medicine ,Pathology ,Urology & nephrology ,Estimated glomerular filtration rate ,Disease course ,pediatric CKD ,Child ,Priority journal ,Kidney ,female genital diseases and pregnancy complications ,CKD progression ,chronic kidney disease ,epidermal growth factor ,3. Good health ,Risk-factors ,Proteinuria ,medicine.anatomical_structure ,Nephrology ,Systolic blood pressure ,Glomerulus filtration rate ,Biomarker (medicine) ,Female ,Glomerular filtration rate ,Cohort analysis ,Age factors ,Human ,medicine.medical_specialty ,Adolescent ,Urinary system ,Excretion ,Predictive value ,Renal function ,Follow-up studies ,Major clinical study ,Urinalysis ,Pathophysiology ,Article ,Roles ,03 medical and health sciences ,Age ,Internal medicine ,Post-hoc analysis ,medicine ,Humans ,Predictive value of tests ,Post hoc analysis ,Renal insufficiency, chronic ,Prospective study ,Disease exacerbation ,Egf ,Disease progression ,Renal replacement therapy ,business.industry ,Follow up ,medicine.disease ,Glomerulopathy ,Chronic Kidney Failure ,Kidney Diseases ,Young-adults ,Biological marker ,030104 developmental biology ,Risk factors ,Risk factor ,Prediction ,business ,Prospective studies ,Controlled study ,Biomarkers ,Kidney disease - Abstract
PubMedID: 31005273 Urinary epidermal growth factor (uEGF) has recently been identified as a promising biomarker of chronic kidney disease (CKD) progression in adults with glomerular disease. Low levels of uEGF predict CKD progression and appear to reflect the extent of tubulointerstitial damage. We investigated the relevance of uEGF in pediatric CKD. We performed a post hoc analysis of the Cardiovascular Comorbidity in Children with CKD (4C) study, which prospectively follows children aged 6–17 years with baseline estimated glomerular filtration rate (eGFR) of 10–60 ml/min/1.73 m2. uEGF levels were measured in archived urine collected within 6 months of enrollment. Congenital abnormalities of the kidney and urinary tract were the most common cause of CKD, with glomerular diseases accounting for
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- 2019
34. Active vitamin D is cardioprotective in experimental uraemia but not in children with CKD Stages 3–5.
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Schön, Anne, Leifheit-Nestler, Maren, Deppe, Jennifer, Fischer, Dagmar-Christiane, Bayazit, Aysun K, Obrycki, Lukasz, Canpolat, Nur, Bulut, Ipek Kaplan, Azukaitis, Karolis, Yilmaz, Alev, Mir, Sevgi, Yalcinkaya, Fatos, Soylemezoglu, Oguz, Melk, Anette, Stangl, Gabriele I, Behnisch, Rouven, Shroff, Rukshana, Bacchetta, Justine, Querfeld, Uwe, and Schaefer, Franz
- Abstract
Background Uraemic cardiac remodelling is associated with vitamin D and Klotho deficiency, elevated fibroblast growth factor 23 (FGF23) and activation of the renin–angiotensin system (RAS). The cardioprotective properties of active vitamin D analogues in this setting are unclear. Methods In rats with 5/6 nephrectomy (5/6Nx) treated with calcitriol, the cardiac phenotype and local RAS activation were investigated compared with controls. A nested case–control study was performed within the Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study, including children with chronic kidney disease (CKD) Stages 3–5 [estimated glomerular filtration rate (eGFR) 25 mL/min/1.73 m
2 ] treated with and without active vitamin D. Echocardiograms, plasma FGF23 and soluble Klotho (sKlotho) were assessed at baseline and after 9 months. Results In rats with 5/6Nx, left ventricular (LV) hypertrophy, LV fibrosis and upregulated cardiac RAS were dose-dependently attenuated by calcitriol. Calcitriol further stimulated FGF23 synthesis in bone but not in the heart, and normalized suppressed renal Klotho expression. In the 4C study cohort, treatment over a mean period of 9 months with active vitamin D was associated with increased FGF23 and phosphate and decreased sKlotho and eGFR compared with vitamin D naïve controls, whereas LV mass index did not differ between groups. Conclusions Active vitamin D ameliorates cardiac remodelling and normalizes renal Klotho expression in 5/6Nx rats but does not improve the cardiac phenotype in children with CKD Stages 3–5. This discrepancy may be due to further enhancement of circulating FGF23 and faster progression of CKD associated with reduced sKlotho and higher serum phosphate in vitamin D-treated patients. [ABSTRACT FROM AUTHOR]- Published
- 2021
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35. Patient- and parent proxy-reported outcome measures for life participation in children with chronic kidney disease: a systematic review.
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Kerklaan, Jasmijn, Hannan, Elyssa, Baumgart, Amanda, Manera, Karine E, Ju, Angela, McCulloch, Mignon, Admani, Bashir, Dominello, Amanda, Esezobor, Christopher, Foster, Bethany, Hamilton, Alexander, Jankauskiene, Augustina, Johnson, Rebecca J, Liu, Isaac, Marks, Stephen D, Neu, Alicia, Schaefer, Franz, Sutton, Shanna, Wolfenden, Sebastian, and Craig, Jonathan C
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CHRONIC kidney failure ,PEDIATRIC nephrology ,PARTICIPATION ,CRITICALLY ill children ,KIDNEY transplantation - Abstract
Background The burden of chronic kidney disease (CKD) and its treatment may severely limit the ability of children with CKD to do daily tasks and participate in family, school, sporting and recreational activities. Life participation is critically important to affected children and their families; however, the appropriateness and validity of available measures used to assess this outcome are uncertain. The aim of this study was to identify the characteristics, content and psychometric properties of existing measures for life participation used in children with CKD. Methods We searched MEDLINE, Embase, PsychINFO, Cumulative Index to Nursing and Allied Health Literature and the Cochrane Kidney and Transplant register to August 2019 for all studies that used a measure to report life participation in children with CKD. For each measure, we extracted and analyzed the characteristics, dimensions of life participation and psychometric properties. Results From 128 studies, we identified 63 different measures used to assess life participation in children with CKD. Twenty-five (40%) of the measures were patient reported, 7 (11%) were parent proxy reported and 31 (49%) had both self and parent proxy reports available. Twenty-two were used in one study only. The Pediatric Quality of Life Inventory version 4.0 generic module was used most frequently in 62 (48%) studies. Seven (11%) were designed to assess ability to participate in life, with 56 (89%) designed to assess other constructs (e.g. quality of life) with a subscale or selected questions on life participation. Across all measures, the three most frequent activities specified were social activities with friends and/or family, leisure activities and self-care activities. Validation data in the pediatric CKD population were available for only 19 (30%) measures. Conclusions Life participation is inconsistently measured in children with CKD and the measures used vary in their characteristics, content and validity. Validation data supporting these measures in this population are often incomplete and are sparse. A meaningful and validated measure for life participation in children with CKD is needed. [ABSTRACT FROM AUTHOR]
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- 2020
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36. Markers of bone metabolism are affected by renal function and growth hormone therapy in children with chronic kidney disease
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Doyon, Anke, Fischer, Christiane Dagmar, Bayazit, Aysun Karabay, Canpolat, Nur, Düzova, Ali, Sözeri, Betül, Bacchetta, Justine, Balat, Ayşe, Büscher, Anja, Candan, Cengiz, Çakar, Nilgün, Dusek, Jiri, Heckel, Martina, Klaus, Günter, Mir, Sevgi, Özçelik, Gül, Sever, Lale, Shroff, Rukshana, Vidal, Enrico, Wühl, Elke, Gondan, Matthias, Melk, Anette, Querfeld, Uwe, Haffner, Dieter, Schaefer, Franz, Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Nefrolojisi Anabilim Dalı., Dönmez, Osman, and AAA-8778-2021
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Kidney function tests ,Male ,Scoring system ,Science & technology - other topics ,SOST protein, human ,Osteoclastogenesis ,Chronic kidney failure ,Fgf23 ,Beta Glucuronidase ,Klotho Protein ,Chronic Kidney Disease-Mineral and Bone Disorder ,Fibroblast growth factor ,Velocity ,Comorbidity ,Ossification ,Parathyroid hormone ,Bone and bones ,Chronic kidney disease ,Disorder ,Bone mineral ,Child ,Recombinant growth hormone ,Parathyroid hormone blood level ,C reactive protein ,Human growth hormone ,Osteoblast ,Osteocyte ,Classification ,Osteodystrophy ,Multicenter study ,Isoenzymes ,Clinical trial ,Blood ,Glomerulus filtration rate ,Female ,Cohort analysis ,Hormonal therapy ,Human ,Adult ,Bone turnover ,Adolescent ,Tartrate-resistant acid phosphatase ,Sclerostin ,Bone metabolism ,Serum-levels ,Major clinical study ,Osteocytes ,Fibroblast growth factor 23 ,Pathophysiology ,Multidisciplinary sciences ,Article ,Bone morphogenetic protein ,Kidney function ,Hemodialysis-patients ,Bone morphogenetic proteins ,Alkaline phosphatase ,Acid phosphatase ,Humans ,Renal insufficiency, chronic ,Bone ,Growth hormone ,Genetic marker ,Consequences ,Alkaline phosphatase bone isoenzyme ,Isoenzyme ,Fibroblast growth factors ,Biological marker ,Kidney function test ,Metabolism ,Preschool child ,Genetic markers ,School child ,Controlled study ,Biomarkers ,Alkaline-phosphatase - Abstract
The extent and relevance of altered bone metabolism for statural growth in children with chronic kidney disease is controversial. We analyzed the impact of renal dysfunction and recombinant growth hormone therapy on a panel of serum markers of bone metabolism in a large pediatric chronic kidney disease cohort. Methods Bone alkaline phosphatase (BAP), tartrate-resistant acid phosphatase 5b (TRAP5b), sclerostin and C-terminal FGF-23 (cFGF23) normalized for age and sex were analyzed in 556 children aged 6-18 years with an estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73m(2). 41 children receiving recombinant growth hormone therapy were compared to an untreated matched control group. Results Standardized levels of BAP, TRAP5b and cFGF-23 were increased whereas sclerostin was reduced. BAP was correlated positively and cFGF-23 inversely with eGFR. Intact serum parathormone was an independent positive predictor of BAP and TRAP5b and negatively associated with sclerostin. BAP and TRAP5B were negatively affected by increased C-reactive protein levels. In children receiving recombinant growth hormone, BAP was higher and TRAP5b lower than in untreated controls. Sclerostin levels were in the normal range and higher than in untreated controls. Serum sclerostin and cFGF-23 independently predicted height standard deviation score, and BAP and TRAP5b the prospective change in height standard deviation score. Conclusion Markers of bone metabolism indicate a high-bone turnover state in children with chronic kidney disease. Growth hormone induces an osteoanabolic pattern and normalizes osteocyte activity. The osteocyte markers cFGF23 and sclerostin are associated with standardized height, and the markers of bone turnover predict height velocity. KFH Foundation for Preventive Medicine European Renal Association-European Dialysis and Transplant Association Federal Ministry of Education & Research (BMBF) (01EO0802) Pfizer Deutschland GmbH Kidney Research UK (KRUK) (RP39/2013)
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- 2015
37. Intimal and medial arterial changes defined by ultra-high-frequency ultrasound: Response to changing risk factors in children with chronic kidney disease.
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Dangardt, Frida, Charakida, Marietta, Chiesa, Scott, Bhowruth, Devina, Rapala, Alicja, Thurn, Daniela, Schaefer, Franz, Deanfield, John, and Shroff, Rukshana
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PEDIATRIC nephrology diagnosis ,CARDIOVASCULAR diseases risk factors ,ULTRASONIC imaging ,HEMODIALYSIS ,HYPERTENSION - Abstract
Background: Patients with chronic kidney disease (CKD) are exposed to both traditional ‘Framingham’ and uremia related cardiovascular risk factors that drive atherosclerotic and arteriosclerotic disease, but these cannot be differentiated using conventional ultrasound. We used ultra-high-frequency ultrasound (UHFUS) to differentiate medial thickness (MT) from intimal thickness (IT) in CKD patients, identify their determinants and monitor their progression. Methods: Fifty-four children and adolescents with CKD and 12 healthy controls underwent UHFUS measurements using 55-70MHz transducers in common carotid and dorsal pedal arteries. Annual follow-up imaging was performed in 31 patients. Results: CKD patients had higher carotid MT and dorsal pedal IT and MT compared to controls. The carotid MT in CKD correlated with serum phosphate (p<0.001, r = 0.42), PTH (p = 0.03, r = 0.36) and mean arterial pressure (p = 0.03, r = 0.34). Following multivariable analysis, being on dialysis, serum phosphate levels and mean arterial pressure remained the only independent predictors of carotid MT (R
2 64%). Transplanted children had lower carotid and dorsal pedal MT compared to CKD and dialysis patients (p = 0.02 and p = 0.01 respectively). At 1-year follow-up, transplanted children had a decrease in carotid MT (p = 0.01), but an increase in dorsal pedal IT (p = 0.04) that independently correlated with annualized change in BMI. Conclusions: Using UHFUS, we have shown that CKD is associated with exclusively medial arterial changes that attenuate when the uremic milieu is ameliorated after transplantation. In contrast, after transplantation intimal disease develops as hypertension and obesity become prevalent, representing rapid vascular remodeling in response to a changing cardiovascular risk factor profile. [ABSTRACT FROM AUTHOR]- Published
- 2018
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38. Averting the legacy of kidney disease–Focus on childhood.
- Author
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Ingelfinger, Julie R., Kalantar-Zadeh, Kamyar, Schaefer, Franz, Kam Tao Li, Philip, Garcia-Garcia, Guillermo, Couser, William G., Erk, Timur, Kernahan, Charles, Osafo, Charlotte, Riella, Miguel C., Segantini, Luca, and Zakharova, Elena
- Published
- 2016
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39. Long-term control of parathyroid hormone and calcium-phosphate metabolism after parathyroidectomy in children with chronic kidney disease.
- Author
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Schaefer, Betti, Schlosser, Katja, Wühl, Elke, Schall, Petra, Klaus, Günter, Schaefer, Franz, and Schmitt, Claus Peter
- Subjects
HYPERPARATHYROIDISM ,PARATHYROID hormone ,CALCIUM phosphate ,PARATHYROIDECTOMY ,KIDNEY diseases ,PEDIATRIC nephrology - Abstract
Background. Hyperparathyroidism (HPT) is an essential contributor to bone disease and cardiovascular calcifications in children with chronic kidney disease (CKD). Pharmacological and dietary interventions are of limited efficacy; calcimimetics are not yet recommended in children. Parathyroidectomy (PTX) is ultimately performed if HPT becomes refractory to conservative measures; the long-term results and the impact of subsequent kidney transplantation (NTX), however, have not yet been evaluated. [ABSTRACT FROM PUBLISHER]
- Published
- 2010
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40. Clinical Interventions and All-Cause Mortality of Patients with Chronic Kidney Disease: An Umbrella Systematic Review of Meta-Analyses.
- Author
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Kim, Jong Yeob, Steingroever, Johanna, Lee, Keum Hwa, Oh, Jun, Choi, Min Jae, Lee, Jiwon, Larkins, Nicholas G., Schaefer, Franz, Hong, Sung Hwi, Jeong, Gwang Hun, Shin, Jae Il, and Kronbichler, Andreas
- Subjects
CHRONIC kidney failure ,CHRONICALLY ill ,META-analysis ,CORONARY artery bypass ,PERCUTANEOUS coronary intervention - Abstract
Patients with chronic kidney disease (CKD) have altered physiologic processes, which result in different treatment outcomes compared with the general population. We aimed to systematically evaluate the efficacy of clinical interventions in reducing mortality of patients with CKD. We searched PubMed, MEDLINE, Embase, and Cochrane Database of Systematic Reviews for meta-analyses of randomized controlled trials (RCT) or observational studies (OS) studying the effect of treatment on all-cause mortality of patients with CKD. The credibility assessment was based on the random-effects summary estimate, heterogeneity, 95% prediction intervals, small study effects, excess significance, and credibility ceilings. Ninety-two articles yielded 130 unique meta-analyses. Convincing evidence from OSs supported mortality reduction with three treatments: angiotensin-converting-enzyme inhibitors or angiotensin II receptor blockers for patients not undergoing dialysis, warfarin for patients with atrial fibrillation not undergoing dialysis, and (at short-term) percutaneous coronary intervention compared to coronary artery bypass grafting for dialysis patients. Two treatment comparisons were supported by highly credible evidence from RCTs in terms of all-cause mortality. These were high-flux hemodialysis (HD) versus low-flux HD as a maintenance HD method and statin versus less statin or placebo for patients not undergoing dialysis. Most significant associations identified in OSs failed to be replicated in RCTs. Associations of high credibility from RCTs were in line with current guidelines. Given the heterogeneity of CKD, it seems hard to assume mortality reductions based on findings from OSs. [ABSTRACT FROM AUTHOR]
- Published
- 2020
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41. The Kidney in Sickle Cell Disease
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Lebensburger, Jeffrey, Kaspar, Cristin, Schaefer, Franz, editor, and Greenbaum, Larry A., editor
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- 2023
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42. Cardiovascular Disease in Pediatric Chronic Kidney Disease
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Doyon, Anke, Mitsnefes, Mark, Schaefer, Franz, editor, and Greenbaum, Larry A., editor
- Published
- 2023
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43. Growth and Puberty in Chronic Kidney Disease
- Author
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Haffner, Dieter, Rees, Lesley, Schaefer, Franz, editor, and Greenbaum, Larry A., editor
- Published
- 2023
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44. Anemia in Chronic Renal Disease
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Greenbaum, Larry A., Schaefer, Franz, editor, and Greenbaum, Larry A., editor
- Published
- 2023
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45. Neurodevelopment in Chronic Kidney Disease
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Johnson, Rebecca J., Harshman, Lyndsay A., Schaefer, Franz, editor, and Greenbaum, Larry A., editor
- Published
- 2023
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46. The prodromal phase of obesity-related chronic kidney disease: early alterations in cardiovascular and renal function in obese children and adolescents.
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Doyon, Anke and Schaefer, Franz
- Subjects
- *
MEDICAL publishing , *NEPHROLOGY , *OBESITY , *KIDNEY diseases , *CARDIOVASCULAR diseases , *METABOLIC syndrome , *HYPERTENSION - Published
- 2013
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47. Immune Function and Immunizations in Dialyzed Children
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Chua, Annabelle N., Bakkaloğlu, Sevcan A., Warady, Bradley A., editor, Alexander, Steven R., editor, and Schaefer, Franz, editor
- Published
- 2021
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48. Transition and Transfer to Adult Care for Adolescents and Young Adults with Advanced Chronic Kidney Disease
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Bell, Lorraine E., Bethe, Dirk, Warady, Bradley A., editor, Alexander, Steven R., editor, and Schaefer, Franz, editor
- Published
- 2021
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49. Health-Related Quality of Life of Children and Adolescents on Dialysis
- Author
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Johnson, Rebecca J., Furth, Susan L., Warady, Bradley A., editor, Alexander, Steven R., editor, and Schaefer, Franz, editor
- Published
- 2021
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- View/download PDF
50. Controlled Enteral and Parenteral Nutrition in Children on Dialysis
- Author
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Foster, Bethany J., Tsampalieros, Anne, Warady, Bradley A., editor, Alexander, Steven R., editor, and Schaefer, Franz, editor
- Published
- 2021
- Full Text
- View/download PDF
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