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1. Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?

Author

Celegen, Kubra, Gulhan, Bora, Fidan, Kibriya, Yuksel, Selcuk, Yilmaz, Neslihan, Yılmaz, Aysun Caltik, Demircioğlu Kılıç, Beltinge, Gokce, Ibrahim, Kavaz Tufan, Aslı, Kalyoncu, Mukaddes, Nalcacıoglu, Hulya, Ozlu, Sare Gulfem, Kurt Sukur, Eda Didem, Canpolat, Nur, K. Bayazit, Aysun, Çomak, Elif, Tabel, Yılmaz, Tulpar, Sebahat, Celakil, Mehtap, Bek, Kenan, Zeybek, Cengiz, Duzova, Ali, Özçakar, Zeynep Birsin, Topaloglu, Rezan, Soylemezoglu, Oguz, and Ozaltin, Fatih

Published
2024
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3. Incidence, risk factors, management strategies, and outcomes of antibody-mediated rejection in pediatric kidney transplant recipients—a multicenter analysis of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN)

Author

Fichtner, Alexander, Gauché, Laura, Süsal, Caner, Tran, Thuong Hien, Waldherr, Rüdiger, Krupka, Kai, Guzzo, Isabella, Carraro, Andrea, Oh, Jun, Zirngibl, Matthias, Weitz, Marcus, König, Jens, Büscher, Anja, Berta, Laszlo, Simon, Thomas, Awan, Atif, Rusai, Krisztina, Topaloglu, Rezan, Peruzzi, Licia, Printza, Nikoleta, Kim, Jon Jin, Weber, Lutz T., Melk, Anette, Pape, Lars, Rieger, Susanne, Patry, Christian, Höcker, Britta, and Tönshoff, Burkhard

Published
2024
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5. Correction to: Adolescence‑onset atypical hemolytic uremic syndrome: is it different from infant‑onset?

Author

Celegen, Kubra, Gulhan, Bora, Fidan, Kibriya, Yuksel, Selcuk, Yilmaz, Neslihan, Yılmaz, Aysun Caltik, Demircioğlu Kılıç, Beltinge, Gokce, Ibrahim, Kavaz Tufan, Aslı, Kalyoncu, Mukaddes, Nalcacıoglu, Hulya, Ozlu, Sare Gulfem, Kurt Sukur, Eda Didem, Canpolat, Nur, K. Bayazit, Aysun, Çomak, Elif, Tabel, Yılmaz, Tulpar, Sebahat, Celakil, Mehtap, Bek, Kenan, Zeybek, Cengiz, Duzova, Ali, Özçakar, Zeynep Birsin, Topaloglu, Rezan, Soylemezoglu, Oguz, and Ozaltin, Fatih

Published
2024
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7. Acute kidney injury in children with moderate-severe COVID-19 and multisystem inflammatory syndrome in children: a referral center experience

Author

Tastemel Ozturk, Tugba, Düzova, Ali, Oygar, Pembe Derin, Baltu, Demet, Ozcilingir Hakverdi, Pelin, Lacinel Gurlevik, Sibel, Kurt-Sukur, Eda Didem, Aykan, Hayrettin Hakan, Ozen, Seza, Ertugrul, Ilker, Kesici, Selman, Gulhan, Bora, Ozaltin, Fatih, Ozsurekci, Yasemin, Cengiz, Ali Bulent, and Topaloglu, Rezan

Published
2024
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8. Urine soluble TLR4 levels may contribute to predict urinary tract infection in children: the UTILISE Study

Author

Aksu, Bagdagul, Afonso, Alberto Caldas, Akil, Ipek, Alpay, Harika, Atmis, Bahriye, Aydog, Ozlem, Bayazıt, Aysun Karabay, Bayram, Meral Torun, Bilge, Ilmay, Bulut, Ipek Kaplan, Buyukkaragoz, Bahar, Comak, Elif, Demir, Belde Kasap, Dincel, Nida, Donmez, Osman, Durmus, Mehmet Akif, Dursun, Hasan, Dusunsel, Ruhan, Duzova, Ali, Ertan, Pelin, Gedikbasi, Asuman, Goknar, Nilufer, Guven, Sercin, Hacihamdioglu, Duygu, Jankauskiene, Augustina, Kalyoncu, Mukaddes, Kavukcu, Salih, Kenan, Bahriye Uzun, Kucuk, Nuran, Kural, Bahar, Litwin, Mieczysław, Montini, Giovanni, Morello, William, Obrycki, Lukasz, Omer, Beyhan, Oner, Huseyin Adil, Ozdemir, Ebru Misirli, Ozkayin, Nese, Paripovic, Dusan, Pehlivanoglu, Cemile, Saygili, Seha, Schaefer, Franz, Schaefer, Susanne, Sonmez, Ferah, Tabel, Yilmaz, Tas, Nesrin, Tasdemir, Mehmet, Teixeira, Ana, Tekcan, Demet, Topaloglu, Rezan, Tulpar, Sebahat, Turkkan, Ozde Nisa, Uysal, Berfin, Uysalol, Metin, Vitkevic, Renata, Yavuz, Sevgi, Yel, Sibel, Yildirim, Tarik, Yildirim, Zeynep Yuruk, Yildiz, Nurdan, Yuksel, Selcuk, Yurtseven, Eray, and Yilmaz, Alev

Published
2024
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9. Application of the updated International IgA Nephropathy Prediction Tool in children one or two years post-biopsy

Author

Zeng, Caihong, Su, Biage, Zhong, Xuhui, Nakanishi, Koichi, Zhai, Yihui, Urushihara, Maki, Hattori, Motoshi, Camassei, Francesca Diomedi, Barreca, Antonella, Robert, Thomas, Prikhodina, Larisa, Berg, Ulla, Topaloglu, Rezan, Mizerska-Wasiak, Malgorzata, Papagianni, Aikaterini, Bellur, Shubha S., Roberts, Ian, Barbour, Sean J., Coppo, Rosanna, Er, Lee, Russo, Maria Luisa, Liu, Zhi-Hong, Ding, Jie, Katafuchi, Ritsuko, Yoshikawa, Norishige, Xu, Hong, Kagami, Shoji, Yuzawa, Yukio, Emma, Francesco, Cambier, Alexandra, Peruzzi, Licia, Wyatt, Robert J., and Cattran, Daniel C.

Published
2024
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10. IPNA-ESPN Junior Master Class-a decade of successful continuing education and training in pediatric nephrology

Author

Teixeira, Ana, Topaloglu, Rezan, Cochat, Pierre, Coppo, Rosanna, Levtchenko, Elena, Haffner, Dieter, and Mahan, John D.

Subjects
Pediatrics -- Educational aspects, Medical personnel -- Training, Nephrology -- Educational aspects, Health
Abstract

Author(s): Ana Teixeira [sup.1] , Rezan Topaloglu [sup.2] , Pierre Cochat [sup.3] , Rosanna Coppo [sup.4] , Elena Levtchenko [sup.5] , Dieter Haffner [sup.6] , John D. Mahan [sup.7] , [...]

Published
2023
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11. Rituximab-associated hypogammaglobulinemia in children with idiopathic nephrotic syndrome: results of an ESPN survey

Author

Zurowska, Aleksandra, Drozynska-Duklas, Magdalena, Topaloglu, Rezan, Bouts, Antonia, Boyer, Olivia, Shenoy, Mohan, and Vivarelli, Marina

Subjects
Children -- Diseases, Agammaglobulinemia -- Surveys -- Risk factors, Nephrotic syndrome -- Surveys -- Drug therapy, Health
Abstract

Background There is paucity of information on rituximab-associated hypogammaglobulinemia (HGG) and its potential infectious consequences in children treated for idiopathic nephrotic syndrome (INS). Methods A survey was distributed by the European Society Pediatric Nephrology to its members. It addressed the screening and management practices of pediatric nephrology units for recognizing and treating RTX-associated HGG and its morbidity and mortality. Eighty-four centers which had treated an overall 1328 INS children with RTX responded. Results The majority of centers administered several courses of RTX and continued concomitant immunosuppressive therapy. Sixty-five percent of centers routinely screened children for HGG prior to RTX infusion, 59% during, and 52% following RTX treatment. Forty-seven percent had observed HGG prior to RTX administration, 61% during and 47% >9 months following treatment in 121, 210, and 128 subjects respectively. Thirty-three severe infections were reported among the cohort of 1328 RTX-treated subjects, of whom 3 children died. HGG had been recognized in 30/33 (80%) of them. Conclusions HGG in steroid-dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS) children is probably multifactorial and can be observed prior to RTX administration in children with SDNS/FRNS. Persistent HGG lasting >9 months from RTX infusion is not uncommon and may increase the risk of severe infections in this cohort. We advocate for the obligatory screening for HGG in children with SDNS/FRNS prior to, during, and following RTX treatment. Further research is necessary to identify risk factors for developing both HGG and severe infections before recommendations are made for its optimal management. Graphical abstract, Author(s): Aleksandra Zurowska [sup.1] [sup.2] , Magdalena Drozynska-Duklas [sup.1] , Rezan Topaloglu [sup.3] , Antonia Bouts [sup.4] , Olivia Boyer [sup.5] [sup.6] , Mohan Shenoy [sup.7] , Marina Vivarelli [sup.8] [...]

Published
2023
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12. Favorable Outcome After Single-kidney Transplantation From Small Donors in Children: A Match-controlled CERTAIN Registry Study

Author

Schild, Raphael, Carvajal Abreu, Karla, Büscher, Anja, Kanzelmeyer, Nele, Lezius, Susanne, Krupka, Kai, Weitz, Marcus, Prytula, Agnieszka, Printza, Nikoleta, Berta, László, Saygılı, Seha Kamil, Sellier-Leclerc, Anne-Laure, Spartà, Giuseppina, Marks, Stephen D., Kemper, Markus J., König, Sabine, Topaloglu, Rezan, Müller, Dominik, Klaus, Günter, Weber, Stefanie, Oh, Jun, Herden, Uta, Carraro, Andrea, Dello Strologo, Luca, Ariceta, Gema, Hoyer, Peter, Tönshoff, Burkhard, and Pape, Lars

Published
2024
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13. Metabolic Acidosis Is Associated With an Accelerated Decline of Allograft Function in Pediatric Kidney Transplantation

Author

Ariceta, Gema, Awan, Atif, Bakkaloğlu, Sevcan, Bonthuis, Marjolein, Robroeks, Charlotte Bootsma, Bouts, Antonia, Christian, Martin, Cornelissen, Marlies, Duzova, Ali, Esfandiar, Nasrin, Ghio, Luciana, Grenda, Ryszard, Guzzo, Isabella, Goni, Maria Herrero, Hogan, Julien, Hongsawong, Nattaphorn, Kanzelmeyer, Nele, Bayazit, Aysun Karabay, Aksoy, Gülşah Kaya, Knops, Noel, Kamphuis, Linda Koster, Erez, Daniella Levy, Lopez-Baez, Victor, Madrid, Alvaro, Marks, Stephen, Melk, Anette, Murer, Luisa, Pape, Lars, Peruzzi, Licia, Petrosyan, Edita, Preka, Evgenia, Printza, Nikoleta, Rachisan, Andreea Liana, Raes, Ann, Shenoy, Mohan, Soylemezoglu, Oguz, Strologo, Luca Dello, Teixeira, Ana, Topaloglu, Rezan, Weitz, Markus, Zieg, Jakub, Zlatanova, Galia, Patry, Christian, Harambat, Jerome, Ağbaş, Ayşe, Askiti, Varvara, Avramescu, Marina, Bacchetta, Justine, Bakkaloglu, Sevcan, Bontuis, Marjolein, Booth, Caroline, Dehoux, Laurene, Dizazzo, Giacomo, Drozdz, Dorota, Dursun, Ismail, Gessner, Michaela, Groothoff, Jaap, Guido, Giuliana, Klaus, Guenter, Koster-Kamphuis, Linda, Lalayiannis, Alexander, Leifheit-Nestler, Maren, Manish, Sinha, Matteucci, Chiara, Oh, Jun, Ozkaya, Ozan, Pietrement, Christine, Prytula, Agnieszka, Reusz, George, Schaefer, Franz, Schmitt, Claus Peter, Schön, Anne, Sever, Fatma Lale, Stabouli, Stella, Döven, Serra Sürmeli, Tondel, Camilla, Verrina, Enrico, Vidal, Enrico, Wallace, Dean, Arslan, Zainab, Bald, M., Fehrenbach, H., Haffner, D., Hansen, M., Hempel, C., John, U., Klaus, G., König, J., Lange-Sperandio, B., Müller, D., Oh, J., Pape, L., Pohl, M., Sauerstein, K., Schalk, G., Staude, H., Strotmann, P., Weber, L.T., Weitz, M., Berta, L., Heindl-Rusai, K., Shroff, Rukshana, van Gremberghe, Ineke, Krupka, Kai, Benetti, Elisa, Büyükkaragöz, Bahar, Kranz, Birgitta, Nalçacıoğlu, Hülya, Sellier-Leclerc, Anne-Laure, and Tönshoff, Burkhard

Published
2024
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15. A rare cause of nephrotic syndrome-sphingosine-1-phosphate lyase (SGPL1) deficiency: 6 cases and a review of the literature

Author

Tastemel Ozturk, Tugba, Canpolat, Nur, Saygili, Seha, Bayrakci, Umut Selda, Soylemezoglu, Oguz, Ozaltin, Fatih, and Topaloglu, Rezan

Subjects
Comorbidity -- Diagnosis -- Care and treatment, Genetic counseling -- Methods, Adrenal insufficiency -- Risk factors -- Prevention, Nephrotic syndrome -- Diagnosis -- Care and treatment -- Genetic aspects, Health
Abstract

Background Recently, recessive mutations in SGPL1 (sphingosine-1-phosphate lyase), which encodes the final enzyme of sphingolipid metabolism, have been reported to cause steroid-resistant nephrotic syndrome, adrenal insufficiency, and many other organ/system involvements. We aimed to determine the clinical and genetic characteristics, and outcomes in patients with SGPL1 mutations. Methods The study included 6 patients with bi-allelic SGPL1 mutation. Clinical, genetic, and laboratory characteristics, and outcomes of the patients were evaluated retrospectively. We also reviewed previously reported patients with SGPL1 mutations and compared them to the presented patients. Results The median age at kidney presentation was 5 months. Four patients (67%) were diagnosed before age 1 year. Kidney biopsy showed focal segmental glomerulosclerosis in 2 patients and diffuse mesangial sclerosis in one patient. Steroids were given to 3 patients, but they did not respond. All 6 patients progressed to chronic kidney disease; 5 required kidney replacement therapy (KRT) at a median age of 6 months. Deceased kidney transplantation was performed in one patient. All 6 patients had adrenal insufficiency, of which 5 were diagnosed at age < 6 months. Three patients had hypothyroidism, 2 had ichthyosis, 4 had immunodeficiency, 5 had neurological findings, and 2 had genitourinary system anomalies. Four patients died at a median age of 30.5 months. Two patients are being followed up with KRT. One patient had a novel mutation. Conclusions Patients with SGPL1 mutations have a poor prognosis, and many types of extrarenal organ/system involvement beyond adrenal insufficiency can be seen. Genetic diagnosis of such patients is important for treatment, genetic counseling, and screening for comorbid conditions. Graphical Abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Tugba Tastemel Ozturk [sup.1] , Nur Canpolat [sup.2] , Seha Saygili [sup.2] , Umut Selda Bayrakci [sup.3] , Oguz Soylemezoglu [sup.4] , Fatih Ozaltin [sup.1] [sup.5] , Rezan Topaloglu [...]

Published
2023
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18. Urinary HSP70 improves diagnostic accuracy for urinary tract infection in children: UTILISE study

Author

Yilmaz, Alev, Afonso, Alberto Caldas, Akil, Ipek, Aksu, Bagdagul, Alpay, Harika, Atmis, Bahriye, Aydog, Ozlem, Bayazıt, Aysun Karabay, Bayram, Meral Torun, Bilge, Ilmay, Bulut, Ipek Kaplan, Buyukkaragoz, Bahar, Comak, Elif, Demir, Belde Kasap, Dincel, Nida, Donmez, Osman, Durmus, Mehmet Akif, Dursun, Hasan, Dusunsel, Ruhan, Duzova, Ali, Ertan, Pelin, Gedikbasi, Asuman, Goknar, Nilufer, Guven, Sercin, Hacihamdioglu, Duygu, Jankauskiene, Augustina, Kalyoncu, Mukaddes, Kavukcu, Salih, Kenan, Bahriye Uzun, Kucuk, Nuran, Kural, Bahar, Litwin, Mieczysław, Montini, Giovanni, Morello, William, Nayir, Ahmet, Obrycki, Lukasz, Omer, Beyhan, Ozdemir, Ebru Misirli, Ozkayin, Nese, Paripovic, Dusan, Pehlivanoglu, Cemile, Saygili, Seha, Schaefer, Susanne, Sonmez, Ferah, Tabel, Yilmaz, Tas, Nesrin, Tasdemir, Mehmet, Teixeira, Ana, Tekcan, Demet, Tulpar, Sebahat, Turkkan, Ozde Nisa, Uysal, Berfin, Uysalol, Metin, Vaiciuniene, Daiva, Yavuz, Sevgi, Yel, Sibel, Yildirim, Tarik, Yildirim, Zeynep Yuruk, Yildiz, Nurdan, Yuksel, Selcuk, Yurtseven, Eray, Schaefer, Franz, and Topaloglu, Rezan

Published
2023
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19. Predictors of kidney complications and analysis of hypertension in children with allogeneic hematopoietic stem cell transplantation

Author

Gurbanov, Anar, Gülhan, Bora, Kuskonmaz, Baris, Okur, Fatma Visal, Ozaltin, Fatih, Düzova, Ali, Çetinkaya, Duygu Uçkan, and Topaloglu, Rezan

Subjects
Hematopoietic stem cells -- Transplantation, Kidney diseases -- Risk factors -- Statistics, Pediatric research, Hypertension -- Risk factors -- Complications and side effects, Health
Abstract

Background This study aimed to determine incidence of kidney complications in pediatric allogeneic hematopoietic stem cell transplantation (HSCT) patients. Methods Pediatric allogeneic HSCT patients were included. Post-transplantation urinary system complications were collected from medical records and glomerular filtration rates at last visit compared with clinical parameters. Additionally, 24-h ambulatory blood pressure monitoring was performed. Results The study included 165 pediatric patients. Acute kidney injury (AKI) developed in 125 (75.8%) patients of whom 54 (43.2%) had stage 1, 36 (28.8%) stage 2, and 35 (28%) stage 3 AKI. Primary malignant disease and viral infection post-HSCT were associated with increased risk of AKI (OR: 4; 95%CI: 1.2-13, p = 0.022 and OR: 2.9; 95%CI: 1.2-6.8, p = 0.014, respectively). Mean duration of post-HSCT follow-up was 4.4 ± 2.5 years, during which time 8 patients had chronic kidney disease (CKD) (stage 1, 4 patients; stage 2, 3 patients; stage 3, 1 patient). CKD incidence was higher in patients in whom stem cell product was bone marrow + cord blood and mobilized peripheral blood, compared to bone marrow alone (40-37.5% versus 5.1%, p = 0.002). Based on 24-h ABPM, 14.7% and 7.4% of patients with normal office blood pressure had pre-hypertension and hypertension, respectively. In patients with albuminuria/severe albuminuria, daytime and nighttime systolic SDS scores were higher than those without albuminuria/severe albuminuria (p = 0.010 and p = 0.004, respectively). Conclusions Incidence of AKI is higher in pediatric HSCT patients with primary malignant disease and those with documented viral infection. Our study highlights the beneficial role of 24-h ABPM as a routine part of standard care of pediatric HSCT recipients., Author(s): Anar Gurbanov [sup.1] , Bora Gülhan [sup.2] , Baris Kuskonmaz [sup.3] , Fatma Visal Okur [sup.3] , Fatih Ozaltin [sup.2] , Ali Düzova [sup.2] , Duygu Uçkan Çetinkaya [sup.3] [...]

Published
2023
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20. Determinants of Kidney Failure in Primary Hyperoxaluria Type 1: Findings of the European Hyperoxaluria Consortium

Author

Metry, Elisabeth L., Garrelfs, Sander F., Deesker, Lisa J., Acquaviva, Cecile, D’Ambrosio, Viola, Bacchetta, Justine, Beck, Bodo B., Cochat, Pierre, Collard, Laure, Hogan, Julien, Ferraro, Pietro Manuel, Franssen, Casper F.M., Harambat, Jérôme, Hulton, Sally-Anne, Lipkin, Graham W., Mandrile, Giorgia, Martin-Higueras, Cristina, Mohebbi, Nilufar, Moochhala, Shabbir H., Neuhaus, Thomas J., Prikhodina, Larisa, Salido, Eduardo, Topaloglu, Rezan, Oosterveld, Michiel J.S., Groothoff, Jaap W., and Peters-Sengers, Hessel

Published
2023
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21. Autoantibodies and Kidney Diseases

Author

Topaloglu, Rezan, Levart, Tanja Kersnik, Nagata, Michio, Avcin, Tadej, Emma, Francesco, editor, Goldstein, Stuart L., editor, Bagga, Arvind, editor, Bates, Carlton M., editor, and Shroff, Rukshana, editor

Published
2022
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27. Incidence, risk factors, management strategies and outcomes of antibody-mediated rejection in pediatric kidney transplant recipients – a multicenter analysis of the Cooperative European Paediatric Renal Transplant Initiative (CERTAIN)

Author

Fichtner, Alexander, primary, Gauché, Laura, additional, Süsal, Caner, additional, Tran, Thuong Hien, additional, Waldherr, Rüdiger, additional, Krupka, Kai, additional, Guzzo, Isabella, additional, Carraro, Andrea, additional, Oh, Jun, additional, Zirngibl, Matthias, additional, Weitz, Marcus, additional, König, Jens, additional, Büscher, Anja, additional, Berta, Laszlo, additional, Simon, Thomas, additional, Awan, Atif, additional, Rusai, Krisztina, additional, Topaloglu, Rezan, additional, Peruzzi, Licia, additional, Printza, Nikoleta, additional, Kim, Jon Jin, additional, Weber, Lutz T., additional, Melk, Anette, additional, Pape, Lars, additional, Rieger, Susanne, additional, Patry, Christian, additional, Höcker, Britta, additional, and Tönshoff, Burkhard, additional

Published
2024
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31. Updating the International IgA Nephropathy Prediction Tool for use in children

Author

Zeng, Caihong, Su, Biage, Zhong, Xuhui, Nakanishi, Koichi, Zhai, Yihui, Urushihara, Maki, Hattori, Motoshi, Camassei, Francesca Diomedi, Barreca, Antonella, Robert, Thomas, Prikhodina, Larisa, Berg, Ulla, Topaloglu, Rezan, Mizerska-Wasiak, Malgorzata, Papagianni, Aikaterini, Bellur, Shubha S., Roberts, Ian, Barbour, Sean J., Coppo, Rosanna, Er, Lee, Russo, Maria Luisa, Liu, Zhi-Hong, Ding, Jie, Katafuchi, Ritsuko, Yoshikawa, Norishige, Xu, Hong, Kagami, Shoji, Yuzawa, Yukio, Emma, Francesco, Cambier, Alexandra, Peruzzi, Licia, Wyatt, Robert J., and Cattran, Daniel C.

Published
2021
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32. Nephropathic cystinosis: an update on genetic conditioning

Author

Topaloglu, Rezan

Subjects
Gene mutations -- Research, Pediatric research, Kidney diseases -- Development and progression -- Complications and side effects -- Care and treatment, Cystinosis -- Complications and side effects -- Care and treatment -- Development and progression, Health
Abstract

Cystinosis is an autosomal recessive lysosomal storage disorder caused by CTNS gene mutations. The CTNS gene encodes the protein cystinosin, which transports free cystine from lysosomes to cytoplasm. In cases of cystinosin deficiency, free cystine accumulates in lysosomes and forms toxic crystals that lead to tissue and organ damage. Since CTNS gene mutations were first described, many variations have been identified that vary according to geographic region, although the phenotype remains the same. Cystinosis is a hereditary disease that can be treated with the cystine-depleting agent cysteamine. Cysteamine slows organ deterioration, but cannot treat renal Fanconi syndrome or prevent eventual kidney failure; therefore, novel treatment modalities for cystinosis are of great interest to researchers. The present review aims to highlight the geographic differences in cystinosis-specifically in terms of its genetic aspects, clinical features, management, and long-term complications., Author(s): Rezan Topaloglu [sup.1] Author Affiliations: (1) grid.14442.37, 0000 0001 2342 7339, Department of Pediatric Nephrology, School of Medicine, Hacettepe University, , Ankara, Turkey Introduction Cystinosis is an autosomal recessive [...]

Published
2021
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33. Application of the updated International IgA Nephropathy Prediction Tool in children one or two years post-biopsy

Author

Barbour, Sean J., Coppo, Rosanna, Er, Lee, Russo, Maria Luisa, Liu, Zhi-Hong, Ding, Jie, Zhong, Xuhui, Katafuchi, Ritsuko, Yoshikawa, Norishige, Xu, Hong, Kagami, Shoji, Yuzawa, Yukio, Emma, Francesco, Cambier, Alexandra, Peruzzi, Licia, Wyatt, Robert J., Cattran, Daniel C., Zeng, Caihong, Su, Biage, Zhong, Xuhui, Nakanishi, Koichi, Zhai, Yihui, Urushihara, Maki, Hattori, Motoshi, Camassei, Francesca Diomedi, Barreca, Antonella, Robert, Thomas, Prikhodina, Larisa, Berg, Ulla, Topaloglu, Rezan, Mizerska-Wasiak, Malgorzata, Papagianni, Aikaterini, Bellur, Shubha S., and Roberts, Ian

Abstract

The pediatric International IgA Nephropathy (IgAN) Prediction Tool comprises two models with and without ethnicity and is the first method to predict the risk of a 30% decline in estimated glomerular filtration rate (eGFR) or kidney failure in children at the time of biopsy using clinical risk factors and Oxford MEST histology scores. However, it is unknown if the Prediction Tool can be applied after a period of observation post-biopsy. Using an international multi-ethnic cohort of 947 children with IgAN, 38% of whom were followed into adulthood, the Prediction Tool was updated for use one year after biopsy. Compared to the original pediatric Prediction Tool, the updated post-biopsy Prediction Tool had a better model fit with higher R2D(51%/50% vs 20%), significant increase in 4-year C-statistics (0.83 vs 0.73/0.69, ΔC 0.09 [95% confidence interval 0.07-0.10] and ΔC 0.14 [0.12-0.15]) and better 4-year calibration with lower integrated calibration indices (0.74/0.54 vs 2.45/1.01). Results were similar after internal validation and when the models were applied two years after biopsy. Trajectories of eGFR after a baseline one year post-biopsy were non-linear and those at higher predicted risk started with a lower eGFR and experienced a more rapid decline over time. In children, eGFR had a variable rate of increase until 15-18 years old and then decreased linearly with a more rapid decline in higher risk groups that was similar to young adults of comparable risk. Thus, the original pediatric Prediction Tool should be used in children at the time of biopsy, and the updated pediatric Prediction Tool should be used to re-evaluate risk one or two years after biopsy.

Published
2024
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34. An international cohort study spanning five decades assessed outcomes of nephropathic cystinosis

Author

Emma, Francesco, Hoff, William van’t, Hohenfellner, Katharina, Topaloglu, Rezan, Greco, Marcella, Ariceta, Gema, Bettini, Chiara, Bockenhauer, Detlef, Veys, Koenraad, Pape, Lars, Hulton, Sally, Collin, Suzanne, Ozaltin, Fatih, Servais, Aude, Deschênes, Georges, Novo, Robert, Bertholet-Thomas, Aurélia, Oh, Jun, Cornelissen, Elisabeth, Janssen, Mirian, Haffner, Dieter, Ravà, Lucilla, Antignac, Corinne, Devuyst, Olivier, Niaudet, Patrick, and Levtchenko, Elena

Published
2021
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36. The relationship between urine heat shock protein 70 and congenital anomalies of the kidney and urinary tract: UTILISE study

Author

Aksu, Bagdagul, primary, Afonso, Alberto Caldas, additional, Akil, Ipek, additional, Alpay, Harika, additional, Atmis, Bahriye, additional, Aydog, Ozlem, additional, Bakkaloglu, Sevcan, additional, Bayazıt, Aysun Karabay, additional, Bayram, Meral Torun, additional, Bilge, Ilmay, additional, Bulut, Ipek Kaplan, additional, Cetinkaya, Ayse Pinar Goksu, additional, Comak, Elif, additional, Demir, Belde Kasap, additional, Dincel, Nida, additional, Donmez, Osman, additional, Durmus, Mehmet Akif, additional, Dursun, Hasan, additional, Dusunsel, Ruhan, additional, Duzova, Ali, additional, Ertan, Pelin, additional, Gedikbasi, Asuman, additional, Goknar, Nilufer, additional, Guven, Sercin, additional, Hacihamdioglu, Duygu, additional, Jankauskiene, Augustina, additional, Kalyoncu, Mukaddes, additional, Kavukcu, Salih, additional, Kenan, Bahriye Uzun, additional, Kucuk, Nuran, additional, Kural, Bahar, additional, Litwin, Mieczysław, additional, Montini, Giovanni, additional, Morello, William, additional, Obrycki, Lukasz, additional, Omer, Beyhan, additional, Misirli Ozdemir, Ebru, additional, Ozkayin, Nese, additional, Paripovic, Dusan, additional, Pehlivanoglu, Cemile, additional, Saygili, Seha, additional, Schaefer, Franz, additional, Schaefer, Susanne, additional, Sonmez, Ferah, additional, Tabel, Yilmaz, additional, Tas, Nesrin, additional, Tasdemir, Mehmet, additional, Teixeira, Ana, additional, Tekcan, Demet, additional, Topaloglu, Rezan, additional, Tulpar, Sebahat, additional, Turkkan, Ozde Nisa, additional, Uysal, Berfin, additional, Uysalol, Metin, additional, Vitkevic, Renata, additional, Yavuz, Sevgi, additional, Yel, Sibel, additional, Yildirim, Tarik, additional, Yildirim, Zeynep Yuruk, additional, Yildiz, Nurdan, additional, Yuksel, Selcuk, additional, Yurtseven, Eray, additional, and Yilmaz, Alev, additional

Published
2024
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37. Diversity of kidney care referral pathways in national child health systems of 48 European countries

Author

Tasic, Velibor, primary, Edvardsson, Vidar O., additional, Preka, Evgenia, additional, Prikhodina, Larisa, additional, Stefanidis, Constantinos J., additional, Topaloglu, Rezan, additional, Shtiza, Diamant, additional, Sarkissian, Ashot, additional, Mueller-Sacherer, Thomas, additional, Fataliyeva, Rena, additional, Kazyra, Ina, additional, Levtchenko, Elena, additional, Pokrajac, Danka, additional, Roussinov, Dimitar, additional, Milošević, Danko, additional, Elia, Avraam, additional, Seeman, Tomas, additional, Faerch, Mia, additional, Vainumae, Inga, additional, Kataja, Janne, additional, Tsimaratos, Michel, additional, Rtskhiladze, Irakli, additional, Hoyer, Peter F., additional, Reusz, George, additional, Awan, Atif, additional, Lotan, Danny, additional, Peruzzi, Licia, additional, Nigmatullina, Nazim, additional, Beishebaeva, Nasira, additional, Jeruma, Edite, additional, Jankauskiene, Augustina, additional, Niel, Olivier, additional, Said-Conti, Valerie, additional, Ciuntu, Angela, additional, Pavićević, Snežana, additional, Oosterveld, Michiel, additional, Bjerre, Anna, additional, Tkaczyk, Marcin, additional, Teixeira, Ana, additional, Lungu, Adrian C., additional, Tsygin, Alexey, additional, Stojanović, Vesna, additional, Podracka, Ludmila, additional, Kersnik Levart, Tanja, additional, Espino-Hernández, Mar, additional, Brandström, Per, additional, Sparta, Giuseppina, additional, Alpay, Harika, additional, Ivanov, Dmytro, additional, Dudley, Jan, additional, Khamzaev, Komiljon, additional, Haffner, Dieter, additional, and Ehrich, Jochen, additional

Published
2024
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40. Sex and age as determinants for high blood pressure in pediatric renal transplant recipients: a longitudinal analysis of the CERTAIN Registry

Author

Sugianto, Rizky I., Schmidt, Bernhard M. W., Memaran, Nima, Duzova, Ali, Topaloglu, Rezan, Seeman, Tomas, and König, Sabine

Subjects
Kidneys -- Transplantation, Cardiovascular diseases -- Risk factors, Pediatric research, Hypertension -- Diagnosis -- Complications and side effects, Health
Abstract

Background High prevalence of arterial hypertension is known in pediatric renal transplant patients, but how blood pressure (BP) distribution and control differ between age groups and whether sex and age interact and potentially impact BP after transplantation have not been investigated. Methods This retrospective analysis included 336 pediatric renal transplant recipients (62% males) from the Cooperative European Pediatric Renal Transplant Initiative Registry (CERTAIN) with complete BP measurement at discharge and 1, 2 and 3 years post-transplant. Results At discharge and 3 years post-transplant, arterial hypertension was highly prevalent (84% and 77%); antihypertensive drugs were used in 73% and 68% of the patients. 27% suffered from uncontrolled and 9% from untreated hypertension at 3 years post-transplant. Children transplanted at age < 5 years showed sustained high systolic BP z-score and received consistently less antihypertensive treatment over time. Younger age, shorter time since transplantation, male sex, higher body mass index (BMI), high cyclosporine A (CSA) trough levels, and a primary renal disease other than congenital anomalies of the kidney and urinary tract (CAKUT) were significantly associated with higher systolic BP z-score. Sex-stratified analysis revealed a significant association between high CSA and higher systolic BP in older girls that likely had started puberty already. An association between BP and estimated glomerular filtration rate was not detected. Conclusions BP control during the first 3 years was poor in this large European cohort. The description of age- and sex-specific risk profiles identified certain recipient groups that may benefit from more frequent BP monitoring (i.e. young children) or different choices of immunosuppression (i.e. older girls)., Author(s): Rizky I. Sugianto [sup.1] , Bernhard M. W. Schmidt [sup.2] , Nima Memaran [sup.1] , Ali Duzova [sup.3] , Rezan Topaloglu [sup.3] , Tomas Seeman [sup.4] , Sabine König [...]

Published
2020
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43. Nephropathic cystinosis: an international consensus document

Author

Emma, Francesco, Nesterova, Galina, Langman, Craig, Labbé, Antoine, Cherqui, Stephanie, Goodyer, Paul, Janssen, Mirian C, Greco, Marcella, Topaloglu, Rezan, Elenberg, Ewa, Dohil, Ranjan, Trauner, Doris, Antignac, Corinne, Cochat, Pierre, Kaskel, Frederick, Servais, Aude, Wühl, Elke, Niaudet, Patrick, Hoff, William Van't, Gahl, William, and Levtchenko, Elena

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Kidney Disease, Rare Diseases, Pediatric, Child, Cystinosis, Fanconi Syndrome, Humans, Practice Guidelines as Topic, Societies, Medical, CTNS gene, cysteamine treatment, cystinosis, extra-renal complications, renal Fanconi syndrome, Urology & Nephrology, Clinical sciences
Abstract

Cystinosis is caused by mutations in the CTNS gene (17p13.2), which encodes for a lysosomal cystine/proton symporter termed cystinosin. It is the most common cause of inherited renal Fanconi syndrome in young children. Because of its rarity, the diagnosis and specific treatment of cystinosis are frequently delayed, which has a significant impact on the overall prognosis. In this document, we have summarized expert opinions on several aspects of the disease to improve knowledge and provide guidance for diagnosis and treatment.

Published
2014

46. Outcomes from the International Society of Nephrology Hemolytic Uremic Syndromes International Forum

Author

Lungu, Adrian Catalin, Żurowska, Aleksandra, Gerogianni, Alexandra, Durkan, Anne, Schijvens, Anne, Lapeyraque, Anne-Laure, Java, Anuja, Awan, Atif, Covella, Bianca, Dixon, Brad, El Sissy, Carine, Duinevel, Caroline, Maville, Christine, Turudic, Daniel, Karpman, Diana, Haffner, Dieter, Trembecka-Dubel, Elżbieta, Ozaltin, Fatih, Emma, Francesco, Schaefer, Franz, Kang, Hee Gyung, Trimarchi, Hernán, Trujillo, Hernando, Ulasi, Ifeoma, Ekwueme, Alex, Menne, Jan, Laurence, Jeffrey, Calado, Joaquim, Hofer, Johannes, Zuber, Julien, Oh, Jun, Bakar, Karmila Abu, Jackson, Kate Smith, Milosevic, Danko, Ariceta, Gema, Claes, Kathleen J., Kaartinen, Kati, Alhasan, Khalid, Wijnsma, Kioa, van den Heuvel, L.P., Alconcher, Laura, Izabel de Holanda, Maria, Szczepańska, Maria, Meuleman, Marie-Sophie, Lemaire, Mathieu, Harris, Meredith, Michalopulos, Michael G., Malina, Michal, Józsi, Mihály, Stajić, Nataša, Isbel, Nicole, Walsh, Patrick, Coccia, Paula A., Ramachandran, Raja, Topaloglu, Rezan, Timmermans, Sjoerd A.M.E.G., Chauvet, Sophie, Levart, Tanja Kersnik, Seeman, Tomas, Tasic, Velibor, Tesař, Vladimír, Song, Wen-Chao, Zhang, Yuzhou, Prohászka, Zoltán, Kavanagh, David, Ardissino, Gianluigi, Brocklebank, Vicky, Bouwmeester, Romy N., Bagga, Arvind, ter Heine, Rob, Johnson, Sally, Licht, Christoph, Ma, Alison L.T., Noris, Marina, Praga, Manuel, Rondeau, Eric, Sinha, Aditi, Smith, Richard J.H., Sheerin, Neil S., Trimarchi, H., Wetzels, Jack F.M., Vivarelli, Marina, Van de Kar, Nicole C.A.J., and Greenbaum, Larry A.

Published
2024
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48. Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

Author

Ranguelov, Nadejda, Godefroid, Nathalie, Collard, Laure, Lombet, Jacques, Maquet, Julie, Schalk, Gesa, Querfeld, Uwe, Beck, Bodo B., Benzing, Thomas, Buettner, Reinhard, Grundmann, Franziska, Kurschat, Christine, Benz, Kerstin, Tzschoppe, Anja, Buchholz, Björn, Buescher, Rainer, Häffner, Karsten, Pohl, Martin, Gross, Oliver, Krügel, Jenny, Stock, Johanna, Patzer, Ludwig, Oh, Jun, Bernhardt, Wanja, Doyon, Anke, Vinke, Tobias, Sander, Anja, Henn, Michael, Derichs, Ute, Beetz, Rolf, Jeck, Nikola, Lange-Sperandio, Bärbel, Ponsel, Sabine, Kusser, Franziska, Uetz, Barbara, Benz, Marcus, Schmidt, Silke, Huppertz-Kessler, Christina, Kranz, Birgitta, Titieni, Andrea, Wurm, Donald, Leichter, Heinz E., Bald, Martin, Billing, Heiko, Nabhan, Marwa M., Lara, Luis Enrique, Papachristou, Fotios, Emma, Francesco, Cerkauskiene, Rimante, Azukaitis, Karolis, Wasilewska, Anna, Balasz-Chmielewska, Irena, Miklaszewska, Monika, Tkaczyk, Marcin, Sikora, Przemyslaw, Zaniew, Marcin, Niemirska, Ania, Antoniewicz, Jolanta, Lesiak, Justyna, Afonso, Alberto Caldas, Teixeira, Ana, Milosevski-Lomic, Gordana, Paripović, Dusan, Peco-Antic, Amira, Papizh, Svetlana, Bayazit, Aysun Karabay, Anarat, Ali, Soylu, Alper, Kavukcu, Salih, Candan, Cengiz, Caliskan, Salim, Canpolat, Nur, Emre, Sevinc, Alpay, Harika, Akinci, Nurver, Conkar, Secil, Poyrazoglu, Hakan M., Dusunsel, Ruhan, Burgmaier, Kathrin, Kunzmann, Kevin, Ariceta, Gema, Bergmann, Carsten, Buescher, Anja Katrin, Burgmaier, Mathias, Dursun, Ismail, Duzova, Ali, Eid, Loai, Erger, Florian, Feldkoetter, Markus, Galiano, Matthias, Geßner, Michaela, Goebel, Heike, Gokce, Ibrahim, Haffner, Dieter, Hooman, Nakysa, Hoppe, Bernd, Jankauskiene, Augustina, Klaus, Guenter, König, Jens, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Mir, Sevgi, Pape, Lars, Prikhodina, Larisa, Ranchin, Bruno, Schild, Raphael, Seeman, Tomas, Sever, Lale, Shroff, Rukshana, Soliman, Neveen A., Stabouli, Stella, Stanczyk, Malgorzata, Tabel, Yilmaz, Taranta-Janusz, Katarzyna, Testa, Sara, Thumfart, Julia, Topaloglu, Rezan, Weber, Lutz Thorsten, Wicher, Dorota, Wühl, Elke, Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Zerres, Klaus, Dötsch, Jörg, Schaefer, Franz, and Liebau, Max Christoph

Published
2018
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49. JC polyomavirus replication and associated disease in pediatric renal transplantation: an international CERTAIN Registry study

Author

Höcker, Britta, Tabatabai, Julia, Schneble, Lukas, Oh, Jun, Thiel, Florian, Pape, Lars, Rusai, Krisztina, Topaloglu, Rezan, Kranz, Birgitta, Klaus, Günter, Printza, Nikoleta, Yavascan, Onder, Fichtner, Alexander, Krupka, Kai, Bruckner, Thomas, Waldherr, Rüdiger, Pawlita, Michael, Schnitzler, Paul, Hirsch, Hans H., and Tönshoff, Burkhard

Published
2018
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