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1. COVID-19 in pediatric nephrology centers in Turkey

Author

Taşdemir, Mehmet (ORCID 0000-0002-5579-6339 & YÖK ID 175867); Bilge, İlmay (ORCID 0000-0002-4852-989X & YÖK ID 198907), Leventoglu, Emre; Ozdemir Atikel, Yesim; Nalcacioglu, Hulya; Dursun, Ismail; Dursun, Hasan; Yuruk Yildirim, Zeynep; Yildiz, Nurdan; Kaya Aksoy, Gulsah; Celakil, Mehtap; Demircioglu Kilic, Beltinge; Zirhli Selcuk, Sena; Canpolat, Nur; Kargin Cakici, Evrim; Ozlu, Sare Gulfem; Tulpar, Sebahat; Yuksel, Selcuk; Atmis, Bahriye; Surmeli Doven, Serra; Taner, Sevgin; Ertan, Pelin; Kavaz, Asli; Torun Bayram, Meral; Kalyoncu, Mukaddes; Gulleroglu, Kaan; Kabasakal, Caner; Kasap Demir, Belde; Cicek, Rumeysa Yasemin; Donmez, Osman; Kara, Aslihan; Yavascan, Onder; Ozcelik, Gul; Gezgin Yildirim, Deniz; Guler, Muhammet Akif; Sonmez, Ferah; Poyrazoglu, Hakan; Akman, Sema; Topaloglu, Rezan; Alpay, Harika; Bakkaloglu, Sevcan A., School of Medicine, Taşdemir, Mehmet (ORCID 0000-0002-5579-6339 & YÖK ID 175867); Bilge, İlmay (ORCID 0000-0002-4852-989X & YÖK ID 198907), Leventoglu, Emre; Ozdemir Atikel, Yesim; Nalcacioglu, Hulya; Dursun, Ismail; Dursun, Hasan; Yuruk Yildirim, Zeynep; Yildiz, Nurdan; Kaya Aksoy, Gulsah; Celakil, Mehtap; Demircioglu Kilic, Beltinge; Zirhli Selcuk, Sena; Canpolat, Nur; Kargin Cakici, Evrim; Ozlu, Sare Gulfem; Tulpar, Sebahat; Yuksel, Selcuk; Atmis, Bahriye; Surmeli Doven, Serra; Taner, Sevgin; Ertan, Pelin; Kavaz, Asli; Torun Bayram, Meral; Kalyoncu, Mukaddes; Gulleroglu, Kaan; Kabasakal, Caner; Kasap Demir, Belde; Cicek, Rumeysa Yasemin; Donmez, Osman; Kara, Aslihan; Yavascan, Onder; Ozcelik, Gul; Gezgin Yildirim, Deniz; Guler, Muhammet Akif; Sonmez, Ferah; Poyrazoglu, Hakan; Akman, Sema; Topaloglu, Rezan; Alpay, Harika; Bakkaloglu, Sevcan A., and School of Medicine

Abstract

Background/aim: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. Materials and methods: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. Results: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10-15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. Conclusion: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients' susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage., NA

Published
2023

2. Phenotypic Variability in Siblings With Autosomal Recessive Polycystic Kidney Disease.

Author

UCL - (SLuc) Département de pédiatrie, Ajiri, Ramona, Burgmaier, Kathrin, Akinci, Nurver, Broekaert, Ilse, Büscher, Anja, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Fila, Marc, Gessner, Michaela, Gokce, Ibrahim, Massella, Laura, Mastrangelo, Antonio, Miklaszewska, Monika, Prikhodina, Larisa, Ranchin, Bruno, Ranguelov, Nadejda, Rus, Rina, Sever, Lale, Thumfart, Julia, Weber, Lutz Thorsten, Wühl, Elke, Yilmaz, Alev, Dötsch, Jörg, Schaefer, Franz, Liebau, Max Christoph, UCL - (SLuc) Département de pédiatrie, Ajiri, Ramona, Burgmaier, Kathrin, Akinci, Nurver, Broekaert, Ilse, Büscher, Anja, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Fila, Marc, Gessner, Michaela, Gokce, Ibrahim, Massella, Laura, Mastrangelo, Antonio, Miklaszewska, Monika, Prikhodina, Larisa, Ranchin, Bruno, Ranguelov, Nadejda, Rus, Rina, Sever, Lale, Thumfart, Julia, Weber, Lutz Thorsten, Wühl, Elke, Yilmaz, Alev, Dötsch, Jörg, Schaefer, Franz, and Liebau, Max Christoph

Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disorder characterized by early onset fibrocystic hepatorenal changes. Previous reports have documented pronounced phenotypic variability even among siblings in terms of patient survival. The underlying causes for this clinical variability are incompletely understood. We present the longitudinal clinical courses of 35 sibling pairs included in the ARPKD registry study ARegPKD, encompassing data on primary manifestation, prenatal and perinatal findings, genetic testing, and family history, including kidney function, liver involvement, and radiological findings. We identified 70 siblings from 35 families with a median age of 0.7 (interquartile range 0.1-6.0) years at initial diagnosis and a median follow-up time of 3.5 (0.2-6.2) years. Data on variants were available for 37 patients from 21 families. There were 8 patients from 7 families who required kidney replacement therapy (KRT) during follow-up. For 44 patients from 26 families, antihypertensive therapy was documented. Furthermore, 37 patients from 24 families had signs of portal hypertension with 9 patients from 6 families having substantial hepatic complications. Interestingly, pronounced variability in the clinical course of functional kidney disease was documented in only 3 sibling pairs. In 17 of 20 families of our cohort of neonatal survivors, siblings had only minor differences of kidney function at a comparable age. In patients surviving the neonatal period, our longitudinal follow-up of 70 ARPKD siblings from 35 families revealed comparable clinical courses of kidney and liver diseases in most families. The data suggest a strong impact of the underlying genotype.

Published
2022

3. Phenotypic Variability in Siblings With Autosomal Recessive Polycystic Kidney Disease.

Author

UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de pédiatrie générale, Ajiri, Ramona, Burgmaier, Kathrin, Akinci, Nurver, Broekaert, Ilse, Büscher, Anja, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Fila, Marc, Gessner, Michaela, Gokce, Ibrahim, Massella, Laura, Mastrangelo, Antonio, Miklaszewska, Monika, Prikhodina, Larisa, Ranchin, Bruno, Ranguelov, Nadejda, Rus, Rina, Sever, Lale, Thumfart, Julia, Weber, Lutz Thorsten, Wühl, Elke, Yilmaz, Alev, Dötsch, Jörg, Schaefer, Franz, Liebau, Max Christoph, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de pédiatrie générale, Ajiri, Ramona, Burgmaier, Kathrin, Akinci, Nurver, Broekaert, Ilse, Büscher, Anja, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Fila, Marc, Gessner, Michaela, Gokce, Ibrahim, Massella, Laura, Mastrangelo, Antonio, Miklaszewska, Monika, Prikhodina, Larisa, Ranchin, Bruno, Ranguelov, Nadejda, Rus, Rina, Sever, Lale, Thumfart, Julia, Weber, Lutz Thorsten, Wühl, Elke, Yilmaz, Alev, Dötsch, Jörg, Schaefer, Franz, and Liebau, Max Christoph

Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disorder characterized by early onset fibrocystic hepatorenal changes. Previous reports have documented pronounced phenotypic variability even among siblings in terms of patient survival. The underlying causes for this clinical variability are incompletely understood. We present the longitudinal clinical courses of 35 sibling pairs included in the ARPKD registry study ARegPKD, encompassing data on primary manifestation, prenatal and perinatal findings, genetic testing, and family history, including kidney function, liver involvement, and radiological findings. We identified 70 siblings from 35 families with a median age of 0.7 (interquartile range 0.1-6.0) years at initial diagnosis and a median follow-up time of 3.5 (0.2-6.2) years. Data on variants were available for 37 patients from 21 families. There were 8 patients from 7 families who required kidney replacement therapy (KRT) during follow-up. For 44 patients from 26 families, antihypertensive therapy was documented. Furthermore, 37 patients from 24 families had signs of portal hypertension with 9 patients from 6 families having substantial hepatic complications. Interestingly, pronounced variability in the clinical course of functional kidney disease was documented in only 3 sibling pairs. In 17 of 20 families of our cohort of neonatal survivors, siblings had only minor differences of kidney function at a comparable age. In patients surviving the neonatal period, our longitudinal follow-up of 70 ARPKD siblings from 35 families revealed comparable clinical courses of kidney and liver diseases in most families. The data suggest a strong impact of the underlying genotype.

Published
2022

4. Phenotypic Variability in Siblings With Autosomal Recessive Polycystic Kidney Disease

Author

Ajiri, Ramona, Burgmaier, Kathrin, Akinci, Nurver, Broekaert, Ilse, Buescher, Anja, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Fila, Marc, Gessner, Michaela, Gokce, Ibrahim, Massella, Laura, Mastrangelo, Antonio, Miklaszewska, Monika, Prikhodina, Larisa, Ranchin, Bruno, Ranguelov, Nadejda, Rus, Rina, Sever, Lale, Thumfart, Julia, Weber, Lutz Thorsten, Wuehl, Elke, Yilmaz, Alev, Doetsch, Joerg, Schaefer, Franz, Liebau, Max Christoph, Ajiri, Ramona, Burgmaier, Kathrin, Akinci, Nurver, Broekaert, Ilse, Buescher, Anja, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Fila, Marc, Gessner, Michaela, Gokce, Ibrahim, Massella, Laura, Mastrangelo, Antonio, Miklaszewska, Monika, Prikhodina, Larisa, Ranchin, Bruno, Ranguelov, Nadejda, Rus, Rina, Sever, Lale, Thumfart, Julia, Weber, Lutz Thorsten, Wuehl, Elke, Yilmaz, Alev, Doetsch, Joerg, Schaefer, Franz, and Liebau, Max Christoph

Abstract

Introduction: Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disorder characterized by early onset fibrocystic hepatorenal changes. Previous reports have documented pronounced phenotypic variability even among siblings in terms of patient survival. The underlying causes for this clinical variability are incompletely understood. Methods: We present the longitudinal clinical courses of 35 sibling pairs included in the ARPKD registry study ARegPKD, encompassing data on primary manifestation, prenatal and perinatal findings, genetic testing, and family history, including kidney function, liver involvement, and radiological findings. Results: We identified 70 siblings from 35 families with a median age of 0.7 (interquartile range 0.1-6.0) years at initial diagnosis and a median follow-up time of 3.5 (0.2-6.2) years. Data on PKHD1 variants were available for 37 patients from 21 families. There were 8 patients from 7 families who required kidney replacement therapy (KRT) during follow-up. For 44 patients from 26 families, antihypertensive therapy was documented. Furthermore, 37 patients from 24 families had signs of portal hypertension with 9 patients from 6 families having substantial hepatic complications. Interestingly, pronounced variability in the clinical course of functional kidney disease was documented in only 3 sibling pairs. In 17 of 20 families of our cohort of neonatal survivors, siblings had only minor differences of kidney function at a comparable age. Conclusion: In patients surviving the neonatal period, our longitudinal follow-up of 70 ARPKD siblings from 35 families revealed comparable clinical courses of kidney and liver diseases in most families. The data suggest a strong impact of the underlying genotype.

Published
2022

5. Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD

Author

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, Liebau, Max Christoph, 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

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.

Published
2021

6. Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD

Author

UCL - (SLuc) Département de pédiatrie, 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, 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, and Marlais, Matko

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.

Published
2021

7. Niveles de magnesio en niños con enfermedad renal crónica estadios 1-3

Author

Yilmaz, Kenan, Dursun, Ismail, Dusunsel, Ruhan, Gunduz, Zubeyde, Yel, Sibel, Yilmaz, Kenan, Dursun, Ismail, Dusunsel, Ruhan, Gunduz, Zubeyde, and Yel, Sibel

Abstract

Objective: The objective of this study was to assess serum and urinary magnesium levels in children who have chronic kidney disease stages 1-3. Methods: Eighty-seven patients who were followed at pediatric nephrology department for chronic kidney disease were included in the study. Age, gender, magnesium, dietary magnesium, and creatinine levels, and fractionated magnesium excretion for all cases were recorded. Patients with chronic kidney disease and control groups were compared in terms of these data. Results: Thirty-nine cases with chronic kidney disease were stage 1, 26 were stage 2, and 22 were stage 3. Average age was 9.9 ± 2.8 years in the control group and 10.2 ± 2.6 years in the chronic kidney disease group. The serum magnesium levels were significantly higher in the stage 3 group than in the control group (P<0.001). Also, in stage 3, fractionated magnesium excretion levels were higher compared to the control group (P<0.001). Conclusion: In chronic kidney disease with advancing renal failure, hypermagnesemia is frequently seen. Serum magnesium levels should be measured periodically in all the children with chronic kidney disease stage 3 to investigate magnesium abnormalities and assess clinical results., Objetivo: El objetivo de este estudio fue evaluar los niveles de magnesio sérico y urinario en niños con enfermedad renal crónica en estadios 1-3. Material y métodos: Se incluyeron en el estudio 87 pacientes que tuvieron seguimiento en el servicio de nefrología pediátrica por enfermedad renal crónica. Se registraron los siguientes datos: edad, sexo, niveles de magnesio, ingesta de alimentos con magnesio, y creatinina, así como también la excreción fraccionada de magnesio para todos estos casos. Sobre la base de dichos datos, se compararon los pacientes con enfermedad renal crónica y los grupos de control. Resultados: De los 87 casos de enfermedad renal crónica, 39 se hallaban en estadio 1; 26, en estadio 2, y 22, en estadio 3. La edad promedio fue de 9,9 ± 2,8 años en el grupo control y de 10,2 ± 2,6 años en el grupo de enfermedad renal crónica. Los niveles de magnesio en suero fueron significativamente más altos en el grupo del estadio 3 que en el grupo control (p <0,001). Además, en el estadio 3, los niveles de excreción fraccionada de magnesio fueron más altos en comparación con el grupo control (p <0,001). Conclusión: En la enfermedad renal crónica con insuficiencia renal avanzada, se observa con frecuencia una hipermagnesemia. Los niveles séricos de magnesio deben medirse periódicamente en todos los niños con enfermedad renal crónica en estadio 3 para investigar las anomalías del magnesio y evaluar los resultados clínicos.

Published
2021

8. Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD

Author

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, Liebau, Max Christoph, 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

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.

Published
2021

9. Treatment and long-term outcome in primary nephrogenic diabetes insipidus

Author

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, 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, 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, and Bockenhauer, Detlef

Abstract

BACKGROUND: Primary nephrogenic diabetes insipidus (NDI) is a rare disorder and little is known about treatment practices and long-term outcome. METHODS: Paediatric and adult nephrologists contacted through European professional organizations entered data in an online form. RESULTS: Data 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 < 0.001). There was also increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (32%) and adults (48%). Evidence of flow uropathy was present in 38%. A higher proportion of children than adults (85% versus 54%; P < 0.001) received medications to reduce urine output. Patients ≥25 years were less likely to have a university degree than the European average (21% versus 35%; P = 0.003) but full-time employment was similar. Mental health problems, predominantly attention-deficit hyperactivity disorder (16%), were reported in 36% of patients. CONCLUSION: This 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.

Published
2020

10. Treatment and long-term outcome in primary distal renal tubular acidosis

Author

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, 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, and Mohebbi, Nilufar

Abstract

Background Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome. 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 (1.16). 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.

Published
2019

11. Treatment and long-term outcome in primary distal renal tubular acidosis

Author

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, 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, and Mohebbi, Nilufar

Abstract

Background Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome. 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 (1.16). 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.

Published
2019

12. Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease.

Author

UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de pédiatrie générale, 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, ESCAPE Study Group, GPN Study Group, Dötsch, Jörg, Schaefer, Franz, Liebau, Max Christoph, ARegPKD consortium, Ranguelov, Nadejda, Godefroid, Nathalie, UCL - SSS/IREC/PEDI - Pôle de Pédiatrie, UCL - (SLuc) Service de pédiatrie générale, 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, ESCAPE Study Group, GPN Study Group, Dötsch, Jörg, Schaefer, Franz, Liebau, Max Christoph, ARegPKD consortium, Ranguelov, Nadejda, and Godefroid, Nathalie

Abstract

OBJECTIVE: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. STUDY DESIGN: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. RESULTS: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. CONCLUSIONS: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.

Published
2018

13. Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

Author

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, Koenig, Jens, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Mir, Sevgi, Pape, Lars, Prikhodina, Larisa, Ranchin, Bruno, Schild, Raphael, Seeman, Tomas, Sever, Late, 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, Wuehl, Elke, Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz, Liebau, Max Christoph, 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, Koenig, Jens, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Mir, Sevgi, Pape, Lars, Prikhodina, Larisa, Ranchin, Bruno, Schild, Raphael, Seeman, Tomas, Sever, Late, 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, Wuehl, Elke, Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz, and Liebau, Max Christoph

Abstract

Objective To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. Study design A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. Results Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. Conclusions This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life. may be helpful in prenatal parental counseling in cases of suspected ARPKD.

Published
2018

14. LONG-TERM OUTCOME AND TREATMENT PRACTICES IN DISTAL RENALTUBULAR ACIDOSIS

Author

Garcia, Sergio Camilo Lopez, Emma, Francesco, Walsh, Stephen, Fila, Marc, Hooman, Nakysa, Marcin, Zaniew, Bertholet-thomas, Aurelia, Colussi, Giacomo, Ebner, Kathrin, Levtchenko, Elena, Sharma, Jyoti, Singhal, Jyoti, Soliman, Nevem A., Ariceta, Ciema, Basu, Biswanath, Murer, Luisa, Tasic, Velibor, Tsygin, Alexey, Demmer, Stephan, Gil-pena, Helena, Koster-Kamphuis, Linda, La Scola, Claudio, Gellermann, Jutta, Konrad, Martin, Lilien, Marc, Francisco, Telma, Tramma, Despoina, Tmka, Peter, Yuksel, Selcuk, Caruso, Maria Rosa, Chromek, Milan, Ekinci, Zelal, Gambaro, Giovanni, Kari, Jameela, Konig, Jens, Taroni, Francesca, Thumfart, Julia, Trepiccione, Francesco, Winding, Louise, Wuhl, Elke, Agbas, Ayse, Belkevich, Anna, Vargas-poussou, Rosa, Blanchard, Anne, Conti, Giovanni, Boyer, Olivia, Dursun, Ismail, Coskun, Ayse Seda, Melek, Engin, Miglinas, Marius, Novo, Robert, Mallen, Andrew, Milosevic, Danko, Szczepanska, Maria, Wente, Sarah, Consortium, Drta, Kleta, Robert, Schaefer, Franz, Bockenhauer, Detlef, Garcia, Sergio Camilo Lopez, Emma, Francesco, Walsh, Stephen, Fila, Marc, Hooman, Nakysa, Marcin, Zaniew, Bertholet-thomas, Aurelia, Colussi, Giacomo, Ebner, Kathrin, Levtchenko, Elena, Sharma, Jyoti, Singhal, Jyoti, Soliman, Nevem A., Ariceta, Ciema, Basu, Biswanath, Murer, Luisa, Tasic, Velibor, Tsygin, Alexey, Demmer, Stephan, Gil-pena, Helena, Koster-Kamphuis, Linda, La Scola, Claudio, Gellermann, Jutta, Konrad, Martin, Lilien, Marc, Francisco, Telma, Tramma, Despoina, Tmka, Peter, Yuksel, Selcuk, Caruso, Maria Rosa, Chromek, Milan, Ekinci, Zelal, Gambaro, Giovanni, Kari, Jameela, Konig, Jens, Taroni, Francesca, Thumfart, Julia, Trepiccione, Francesco, Winding, Louise, Wuhl, Elke, Agbas, Ayse, Belkevich, Anna, Vargas-poussou, Rosa, Blanchard, Anne, Conti, Giovanni, Boyer, Olivia, Dursun, Ismail, Coskun, Ayse Seda, Melek, Engin, Miglinas, Marius, Novo, Robert, Mallen, Andrew, Milosevic, Danko, Szczepanska, Maria, Wente, Sarah, Consortium, Drta, Kleta, Robert, Schaefer, Franz, and Bockenhauer, Detlef

Published
2018

15. Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

Author

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, Koenig, Jens, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Mir, Sevgi, Pape, Lars, Prikhodina, Larisa, Ranchin, Bruno, Schild, Raphael, Seeman, Tomas, Sever, Late, 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, Wuehl, Elke, Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz, Liebau, Max Christoph, 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, Koenig, Jens, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Mir, Sevgi, Pape, Lars, Prikhodina, Larisa, Ranchin, Bruno, Schild, Raphael, Seeman, Tomas, Sever, Late, 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, Wuehl, Elke, Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz, and Liebau, Max Christoph

Abstract

Objective To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. Study design A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. Results Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. Conclusions This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life. may be helpful in prenatal parental counseling in cases of suspected ARPKD.

Published
2018

16. LONG-TERM OUTCOME AND TREATMENT PRACTICES IN DISTAL RENALTUBULAR ACIDOSIS

Author

Garcia, Sergio Camilo Lopez, Emma, Francesco, Walsh, Stephen, Fila, Marc, Hooman, Nakysa, Marcin, Zaniew, Bertholet-thomas, Aurelia, Colussi, Giacomo, Ebner, Kathrin, Levtchenko, Elena, Sharma, Jyoti, Singhal, Jyoti, Soliman, Nevem A., Ariceta, Ciema, Basu, Biswanath, Murer, Luisa, Tasic, Velibor, Tsygin, Alexey, Demmer, Stephan, Gil-pena, Helena, Koster-Kamphuis, Linda, La Scola, Claudio, Gellermann, Jutta, Konrad, Martin, Lilien, Marc, Francisco, Telma, Tramma, Despoina, Tmka, Peter, Yuksel, Selcuk, Caruso, Maria Rosa, Chromek, Milan, Ekinci, Zelal, Gambaro, Giovanni, Kari, Jameela, Konig, Jens, Taroni, Francesca, Thumfart, Julia, Trepiccione, Francesco, Winding, Louise, Wuhl, Elke, Agbas, Ayse, Belkevich, Anna, Vargas-poussou, Rosa, Blanchard, Anne, Conti, Giovanni, Boyer, Olivia, Dursun, Ismail, Coskun, Ayse Seda, Melek, Engin, Miglinas, Marius, Novo, Robert, Mallen, Andrew, Milosevic, Danko, Szczepanska, Maria, Wente, Sarah, Consortium, Drta, Kleta, Robert, Schaefer, Franz, Bockenhauer, Detlef, Garcia, Sergio Camilo Lopez, Emma, Francesco, Walsh, Stephen, Fila, Marc, Hooman, Nakysa, Marcin, Zaniew, Bertholet-thomas, Aurelia, Colussi, Giacomo, Ebner, Kathrin, Levtchenko, Elena, Sharma, Jyoti, Singhal, Jyoti, Soliman, Nevem A., Ariceta, Ciema, Basu, Biswanath, Murer, Luisa, Tasic, Velibor, Tsygin, Alexey, Demmer, Stephan, Gil-pena, Helena, Koster-Kamphuis, Linda, La Scola, Claudio, Gellermann, Jutta, Konrad, Martin, Lilien, Marc, Francisco, Telma, Tramma, Despoina, Tmka, Peter, Yuksel, Selcuk, Caruso, Maria Rosa, Chromek, Milan, Ekinci, Zelal, Gambaro, Giovanni, Kari, Jameela, Konig, Jens, Taroni, Francesca, Thumfart, Julia, Trepiccione, Francesco, Winding, Louise, Wuhl, Elke, Agbas, Ayse, Belkevich, Anna, Vargas-poussou, Rosa, Blanchard, Anne, Conti, Giovanni, Boyer, Olivia, Dursun, Ismail, Coskun, Ayse Seda, Melek, Engin, Miglinas, Marius, Novo, Robert, Mallen, Andrew, Milosevic, Danko, Szczepanska, Maria, Wente, Sarah, Consortium, Drta, Kleta, Robert, Schaefer, Franz, and Bockenhauer, Detlef

Published
2018

17. CHARACTERIZATION OF POTENTIAL RISK MARKERS OF THE RENAL ARPKD PHENOTYPE

Author

Ebner, Kathrin, Feldkoetter, Markus, Ariceta, Gema, Bergmann, Carsten, Buettner, Reinhard, Doyon, Anke, Dursun, Ismail, Duzova, Ali, Galiano, Matthias, Gessner, Michaela, Goebel, Heike, Goekce, Ibrahim, Haffner, Dieter, Hero, Barbara, Hoppe, Bernd, Illig, Thomas, Jankauskiene, Augustina, Klopp, Norman, Koenig, Jens, Kunzmann, Kevin, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Miklaszewska, Monika, Ranchin, Bruno, Sander, Anja, Shroff, Rukshana, Testa, Sara, Weber, Lutz Thorsten, Wicher, Dorota, Yuzbasioglu, Ayse, Zachwieja, Katarzyna, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz, Liebau, Max Christoph, Ebner, Kathrin, Feldkoetter, Markus, Ariceta, Gema, Bergmann, Carsten, Buettner, Reinhard, Doyon, Anke, Dursun, Ismail, Duzova, Ali, Galiano, Matthias, Gessner, Michaela, Goebel, Heike, Goekce, Ibrahim, Haffner, Dieter, Hero, Barbara, Hoppe, Bernd, Illig, Thomas, Jankauskiene, Augustina, Klopp, Norman, Koenig, Jens, Kunzmann, Kevin, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Miklaszewska, Monika, Ranchin, Bruno, Sander, Anja, Shroff, Rukshana, Testa, Sara, Weber, Lutz Thorsten, Wicher, Dorota, Yuzbasioglu, Ayse, Zachwieja, Katarzyna, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz, and Liebau, Max Christoph

Published
2017

18. CHARACTERIZATION OF POTENTIAL RISK MARKERS OF THE RENAL ARPKD PHENOTYPE

Author

Ebner, Kathrin, Feldkoetter, Markus, Ariceta, Gema, Bergmann, Carsten, Buettner, Reinhard, Doyon, Anke, Dursun, Ismail, Duzova, Ali, Galiano, Matthias, Gessner, Michaela, Goebel, Heike, Goekce, Ibrahim, Haffner, Dieter, Hero, Barbara, Hoppe, Bernd, Illig, Thomas, Jankauskiene, Augustina, Klopp, Norman, Koenig, Jens, Kunzmann, Kevin, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Miklaszewska, Monika, Ranchin, Bruno, Sander, Anja, Shroff, Rukshana, Testa, Sara, Weber, Lutz Thorsten, Wicher, Dorota, Yuzbasioglu, Ayse, Zachwieja, Katarzyna, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz, Liebau, Max Christoph, Ebner, Kathrin, Feldkoetter, Markus, Ariceta, Gema, Bergmann, Carsten, Buettner, Reinhard, Doyon, Anke, Dursun, Ismail, Duzova, Ali, Galiano, Matthias, Gessner, Michaela, Goebel, Heike, Goekce, Ibrahim, Haffner, Dieter, Hero, Barbara, Hoppe, Bernd, Illig, Thomas, Jankauskiene, Augustina, Klopp, Norman, Koenig, Jens, Kunzmann, Kevin, Litwin, Mieczyslaw, Massella, Laura, Mekahli, Djalila, Melek, Engin, Miklaszewska, Monika, Ranchin, Bruno, Sander, Anja, Shroff, Rukshana, Testa, Sara, Weber, Lutz Thorsten, Wicher, Dorota, Yuzbasioglu, Ayse, Zachwieja, Katarzyna, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz, and Liebau, Max Christoph

Published
2017

19. Defects of CRB2 Cause Steroid-Resistant Nephrotic Syndrome

Author

Ebarasi, Lwaki, Ashraf, Shazia, Bierzynska, Agnieszka, Gee, Heon Yung, McCarthy, Hugh J., Lovric, Svjetlana, Sadowski, Carolin E., Pabst, Werner, Vega-Warner, Virginia, Fang, Humphrey, Koziell, Ania, Simpson, Michael A., Dursun, Ismail, Serdaroglu, Erkin, Levy, Shawn, Saleem, Moin A., Hildebrandt, Friedhelm, Majumdar, Arindam, Ebarasi, Lwaki, Ashraf, Shazia, Bierzynska, Agnieszka, Gee, Heon Yung, McCarthy, Hugh J., Lovric, Svjetlana, Sadowski, Carolin E., Pabst, Werner, Vega-Warner, Virginia, Fang, Humphrey, Koziell, Ania, Simpson, Michael A., Dursun, Ismail, Serdaroglu, Erkin, Levy, Shawn, Saleem, Moin A., Hildebrandt, Friedhelm, and Majumdar, Arindam

Abstract

Nephrotic syndrome (NS), the association of gross proteinuria, hypoalbuminaemia, edema, and hyperlipidemia, can be clinically divided into steroid-sensitive (SSNS) and steroid-resistant (SRNS) forms. SRNS regularly progresses to end-stage renal failure. By homozygosity mapping and whole exome sequencing, we here identify recessive mutations in Crumbs homolog 2 (CRB2) in four different families affected by SRNS. Previously, we established a requirement for zebrafish crb2b, a conserved regulator of epithelial polarity, in podocyte morphogenesis. By characterization of a loss-of-function mutation in zebrafish crb2b, we now show that zebrafish crb2b is required for podocyte foot process arborization, slit diaphragm formation, and proper nephrin trafficking. Furthermore, by complementation experiments in zebrafish, we demonstrate that CRB2 mutations result in loss of function and therefore constitute causative mutations leading to NS in humans. These results implicate defects in podocyte apico-basal polarity in the pathogenesis of NS.

Published
2015
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20. Effect of elamipretide (SS-31) on inflammasom formation in mice model with oxalate nephropathy

Author

Dursun, Ismail, Hamurcu, Zuhal, Taheri, Serpil, Tufan, Esra, Mehmetbeyoglu, Ecmel, Delibas, Nesrin, Doganyigit, Zuleyha, Alan, Aydin, Kuyucu, Yurdun, Polat, Sait, Zararsiz, Gokmen, Poyrazoglu, Hakan, Dursun, Ismail, Hamurcu, Zuhal, Taheri, Serpil, Tufan, Esra, Mehmetbeyoglu, Ecmel, Delibas, Nesrin, Doganyigit, Zuleyha, Alan, Aydin, Kuyucu, Yurdun, Polat, Sait, Zararsiz, Gokmen, and Poyrazoglu, Hakan

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