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1. Timing and Modality of Kidney Replacement Therapy in Children and Adolescents

Author

Cortina, G., Arbeiter, K., Dusek, J., Harambat, J., Ranchin, B., Fischbach, M., Querfeld, U., Habbig, S., Galiano, M., Büscher, R., Gimpel, C., Kemper, M., Melk, A., Thurn, D., Schaefer, F., Doyon, A., Wühl, E., Pohl, M., Wygoda, S., Jeck, N., Kranz, B., Wigger, M., Montini, G., Lugani, F., Testa, S., Vidal, E., Matteucci, C., Picca, S., Jankauskiene, A., Azukaitis, K., Zurowska, A., Drodz, D., Tkaczyk, M., Urasinski, T., Litwin, M., Szczepanska, M., Texeira, A., Peco-Antic, A., Bucher, B., Laube, G., Anarat, A., Bayazit, A.K., Yalcinkaya, F., Basin, E., Cakar, N., Soylemezoglu, O., Duzova, A., Bilginer, Y., Erdogan, H., Donmez, O., Balat, A., Kiyak, A., Caliskan, S., Canpolat, N., Candan, C., Civilibal, M., Emre, S., Ozcelik, G., Mir, S., Sözeri, B., Yavascan, O., Tabel, Y., Ertan, P., Yilmaz, E., Shroff, R., Thumfart, Julia, Wagner, Steffen, Kirchner, Marietta, Azukaitis, Karolis, Bayazit, Aysun K., Obrycki, Lukasz, Canpolat, Nur, Bulut, Ipek Kaplan, Duzova, Ali, Anarat, Ali, Bessenay, Lucie, Shroff, Rukshana, Paripovic, Dusan, Sever, Lale, Candan, Cengiz, Lugani, Francesca, Yilmaz, Alev, Yalcinkaya, Fatos, Arbeiter, Klaus, Kiyak, Aysel, Zurowska, Aleksandra, Galiano, Matthias, Querfeld, Uwe, Melk, Anette, and Schaefer, Franz

Published
2024
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2. Long-term Effects of Kidney Transplantation Compared With Dialysis on Intima–media Thickness in Children—Results From the 4C-T Study

Author

Grabitz, Carl, Sugianto, Rizky I., Doyon, Anke, Azukaitis, Karolis, Anarat, Ali, Bacchetta, Justine, Bayazit, Aysun K., Bulut, Ipek K., Caliskan, Salim, Canpolat, Nur, Duzova, Ali, Habbig, Sandra, Harambat, Jerome, Kiyak, Aysel, Longo, Germana, Obrycki, Lukasz, Paripovic, Dusan, Söylemezoğlu, Oğuz, Thurn-Valsassina, Daniela, Yilmaz, Alev, Shroff, Rukshana, Schaefer, Franz, Schmidt, Bernhard M.W., Melk, Anette, Cortina, Gerard, Arbeiter, Klaus, Dusek, Jiri, Harambat, Jerome, Ranchin, Bruno, Fischbach, Michel, Zaloszyc, Ariane, Querfeld, Uwe, Gellermann, Jutta, Habbig, Sandra, Liebau, Max, Galiano, Matthias, Büscher, Rainer, Gimpel, Charlotte, Kemper, Matthias, Oh, Jun, Melk, Anette, Thurn-Valassina, Daniela, Doyon, Anke, Wühl, Elke, Schaefer, Franz, John, Ulrike, Wygoda, Simone, Jeck, Nicola, Kranz, Birgitta, Wigger, Marianne, Mencarelli, Francesca, Lugani, Francesca, Testa, Sara, Montini, Giovanni, Morello, William, Vidal, Enrico, Benetti, Elisa, Murer, Luisa, Matteucci, Ciara, Picca, Stefano, Gesù, Ospedale Bambino, Jankauskiene, Augustina, Azukaitis, Karolis, Zurowska, Aleksandra, Zagozozon, Ilona, Drodz, Dorota, Urasinski, Tomasz, Litwin, Mieczyslaw, Niemirska, Anna, Obrycki, Lukasz, Szczepanska, Maria, Texeira, Ana, Peco-Antic, Amira, Paripovic, Dusan, Simonetti, Giacomo, Laube, Guido, Anarat, Ali, Bayazit, Aysun K., Yalcinkaya, Fatos, Baskin, Esra, Cakar, Nilgun, Soylemezoglu, Oguz, Duzova, Ali, Bilginer, Yelda, Erdogan, Hakan, Donmez, Osman, Balat, Ayse, Kiyak, Aysel, Caliskan, Salim, Canpolat, Nur, Civilibal, Mahmut, Candan, Cengiz, Emre, Sevinc, Yilmaz, Alev, Alpay, Harika, Ozcelik, Gul, Mir, Sevgi, Sözeri, Betul, Bulut, Ipek K., Aksu, Nejat, Yavascan, Onder, Tabel, Yilmaz, Ertan, Pelin, Yilmaz, Ebru, and Shroff, Rukshana

Published
2024
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3. Timing and modality of kidney replacement therapy in children and adolescents

Author

Thumfart, Julia, primary, Wagner, Steffen, additional, Kirchner, Marietta, additional, Azukaitis, Karolis, additional, Bayazit, Aysun K., additional, Obrycki, Lukasz, additional, Canpolat, Nur, additional, Bulut, Ipek Kaplan, additional, Duzova, Ali, additional, Anarat, Ali, additional, Bessenay, Lucie, additional, Shroff, Rukshana, additional, Paripovic, Dusan, additional, Sever, Lale, additional, Candan, Cengiz, additional, Lugani, Francesca, additional, Yilmaz, Alev, additional, Yalcinkaya, Fatos, additional, Arbeiter, Klaus, additional, Kiyak, Aysel, additional, Zurowska, Aleksandra, additional, Galiano, Matthias, additional, Querfeld, Uwe, additional, Melk, Anette, additional, Schaefer, Franz, additional, Cortina, G., additional, Arbeiter, K., additional, Dusek, J., additional, Harambat, J., additional, Ranchin, B., additional, Fischbach, M., additional, Zalosczyk, A., additional, Querfeld, U., additional, Habbig, S., additional, Galiano, M., additional, Büscher, R., additional, Gimpel, C., additional, Kemper, M., additional, Melk, A., additional, Thurn, D., additional, Schaefer, F., additional, Doyon, A., additional, Wühl, E., additional, Pohl, M., additional, Wygoda, S., additional, Jeck, N., additional, Kranz, B., additional, Wigger, M., additional, Montini, G., additional, Lugani, F., additional, Testa, S., additional, Vidal, E., additional, Matteucci, C., additional, Picca, S., additional, Jankauskiene, A., additional, Azukaitis, K., additional, Zurowska, A., additional, Drodz, D., additional, Tkaczyk, M., additional, Urasinski, T., additional, Litwin, M., additional, Niemirska, A., additional, Szczepanska, M., additional, Texeira, A., additional, Peco-Antic, A., additional, Bucher, B., additional, Laube, G., additional, Anarat, A., additional, Bayazit, A.K., additional, Yalcinkaya, F., additional, Basin, E., additional, Cakar, N., additional, Soylemezoglu, O., additional, Duzova, A., additional, Bilginer, Y., additional, Erdogan, H., additional, Donmez, O., additional, Balat, A., additional, Kiyak, A., additional, Caliskan, S., additional, Canpolat, N., additional, Candan, C., additional, Civilibal, M., additional, Emre, S., additional, Alpay, H., additional, Ozcelik, G., additional, Mir, S., additional, Sözeri, B., additional, Yavascan, O., additional, Tabel, Y., additional, Ertan, P., additional, Yilmaz, E., additional, and Shroff, R., additional

Published
2024
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5. Progressive Kidney Failure by Angiotensinogen Inactivation in the Germline.

Author

Wopperer, Florian J., Olinger, Eric, Wiesener, Antje, Broeker, Katharina A. E., Knaup, Karl X., Schaefer, Jan T., Galiano, Matthias, Schneider, Karen, Schiffer, Mario, Büttner-Herold, Maike, Reis, André, Schmieder, Roland, Pasutto, Francesca, Hilgers, Karl F., Poglitsch, Marko, Ziegler, Christine, Shoemaker, Robin, Sayer, John A., and Wiesener, Michael S.

Abstract

BACKGROUND: Autosomal recessive renal tubular dysgenesis is a rare, usually fatal inherited disorder of the renin-angiotensis system (RAS). Herein, we report an adolescent individual experiencing an unknown chronic kidney disease and aim to provide novel insights into disease mechanisms. METHODS: Exome sequencing for a gene panel associated with renal disease was performed. The RAS was assessed by comprehensive biochemical analysis in blood. Renin expression was determined in primary tubular cells by quantitative polymerase chain reaction and in situ hybridization on kidney biopsy samples. Allele frequencies of heterozygous and biallelic deleterious variants were determined by analysis of the Genomics England 100,000 Genomes Project. RESULTS: The patient was delivered prematurely after oligohydramnios was detected during pregnancy. Postnatally, he recovered from third-degree acute kidney injury but developed chronic kidney disease stage G3b over time. Exome sequencing revealed a previously reported pathogenic homozygous missense variant, p.(Arg375Gln), in the AGT (angiotensinogen) gene. Blood AGT concentrations were low, but plasma renin concentration and gene expression in kidney biopsy, vascular, and tubular cells revealed strong upregulation of renin. Angiotensin II and aldosterone in blood were not abnormally elevated. CONCLUSIONS: Renal tubular dysgenesis may present as chronic kidney disease with a variable phenotype, necessitating broad genetic analysis for diagnosis. Functional analysis of the RAS in a patient with AGT mutation revealed novel insights regarding compensatory upregulation of renin in vascular and tubular cells of the kidney and in plasma in response to depletion of AGT substrate as a source of Ang II (similarly observed with hepatic AGT silencing for the treatment of hypertension). [ABSTRACT FROM AUTHOR]

Published
2024
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6. Refining genotype–phenotype correlations in 304 patients with autosomal recessive polycystic kidney disease and PKHD1 gene variants

Author

Eid, Loai Akram, Arbeiter, Klaus, Godefroid, Nathalie, Lombet, Jacques, De Mul, Aurélie, Feldkoetter, Markus, Zieg, Jakub, Grundmann, Franziska, Galiano, Matthias, Buchholz, Björn, Buescher, Anja, Häffner, Karsten, Gross, Oliver, Patzer, Ludwig, Oh, Jun, Haffner, Dieter, Bernhardt, Wanja, Schaefer, Susanne, Wygoda, Simone, Halbritter, Jan, Derichs, Ute, Klaus, Günter, Lechner, Felix, Ponsel, Sabine, König, Jens, Staude, Hagen, Wurm, Donald, Bald, Martin, Gessner, Michaela, Soliman, Neveen A., Ariceta, Gema, Gonzalez Rodriguez, Juan David, Ojeda, Francisco de la Cerda, Harambat, Jerome, Morin, Denis, Dossier, Claire, Dorval, Guillaume, Shroff, Rukshana, Stabouli, Stella, Hooman, Nakysa, Mencarelli, Francesca, Morello, William, Longo, Germana, Emma, Francesco, Jankauskiene, Augustina, Taranta-Janusz, Katarzyna, Zagozdzon, Ilona, Zachwieja, Katarzyna, Stanczyk, Malgorzata, Bienias, Beata, Litwin, Mieczyslaw, Morawiec-Knysak, Aurelia, Afonso, Alberto Caldas, Dunand, Oliver, Rachisan, Andreea, Miloševski-Lomić, Gordana, Papizh, Svetlana, Rus, Rina, Jilani, Houweyda, Atmis, Bahriye, Duzova, Ali, Soylu, Alper, Candan, Cengiz, Caliskan, Salim, Yilmaz, Alev, Gökce, İbrahim, Akinci, Nurver, Mir, Sevgi, Dursun, Ismail, Tabel, Yilmaz, Nalcacioglu, Hulya, Burgmaier, Kathrin, Brinker, Leonie, Erger, Florian, Beck, Bodo B., Benz, Marcus R., Bergmann, Carsten, Boyer, Olivia, Collard, Laure, Dafinger, Claudia, Fila, Marc, Kowalewska, Claudia, Lange-Sperandio, Bärbel, Massella, Laura, Mastrangelo, Antonio, Mekahli, Djalila, Miklaszewska, Monika, Ortiz-Bruechle, Nadina, Prikhodina, Larisa, Ranchin, Bruno, Ranguelov, Nadejda, Schild, Raphael, Seeman, Tomas, Sever, Lale, Sikora, Przemyslaw, Szczepanska, Maria, Teixeira, Ana, Thumfart, Julia, Uetz, Barbara, Weber, Lutz Thorsten, Wühl, Elke, Zerres, Klaus, Dötsch, Jörg, Schaefer, Franz, and Liebau, Max Christoph

Published
2021
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7. Cardiovascular Outcome of Pediatric Patients With Bi-Allelic (Homozygous) Familial Hypercholesterolemia Before and After Initiation of Multimodal Lipid Lowering Therapy Including Lipoprotein Apheresis

Author

Taylan, Christina, Driemeyer, Joenna, Schmitt, Claus P., Pape, Lars, Büscher, Rainer, Galiano, Matthias, König, Jens, Schürfeld, Carsten, Spitthöver, Ralf, Versen, Axel, Koziolek, Michael, Marsen, Tobias A., Stein, Holger, Schaefer, Juergen R., Heibges, Andreas, Klingel, Reinhard, Oh, Jun, Weber, Lutz T., and Klaus, Günter

Published
2020
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8. A multicenter, randomized, placebo-controlled, double-blind phase 3 trial with open-arm comparison indicates safety and efficacy of nephroprotective therapy with ramipril in children with Alport’s syndrome

Author

Koziolek, Michael, Bramlage, Carsten Paul, Weber, Frauke, Albrecht-Nock, Tanja, Sonntag, Joseph, Frese, Jenny, Kettwig, Matthias, Hilgers, Reinhard, Hansen, Matthias, Wedekin, Mirja, Meyer, Nicole, Klaiber, Susanne, Gessner, Michaela, Liebau, Max, Vogt-Weigeldt, Anne-Kristin, Jungraithmayr, Therese, Ponsel, Sabine, Jacoby, Ulrike, Konrad, Martin, Kranz, Brigitta, Koenig, Jens, Loechtermann, Lisa, Pohl, Michael, Husain, Ralf, Mueller, Katrin, Thumfart, Julia, Schalk, Gesa, Feldkoetter, Markus, Schmidt, Sabine, Sauerstein, Katja, Muschiol, Evelin, Billing, Heiko, Wilkening, Frauke, Gross, Oliver, Tönshoff, Burkhard, Weber, Lutz T., Pape, Lars, Latta, Kay, Fehrenbach, Henry, Lange-Sperandio, Baerbel, Zappel, Hildegard, Hoyer, Peter, Staude, Hagen, König, Sabine, John, Ulrike, Gellermann, Jutta, Hoppe, Bernd, Galiano, Matthias, Hoecker, Britta, Ehren, Rasmus, Lerch, Christian, Kashtan, Clifford E., Harden, Markus, Boeckhaus, Jan, and Friede, Tim

Published
2020
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9. “Multisystem Inflammatory Syndrome in Children”-Like Disease after COVID-19 Vaccination (MIS-V) with Potential Significance of Functional Active Autoantibodies Targeting G-Protein-Coupled Receptors (GPCR-fAAb) for Pathophysiology and Therapy

Author

Schmidt, Marius, primary, Hébert, Steven, additional, Wallukat, Gerd, additional, Ponader, Rolf, additional, Krickau, Tobias, additional, Galiano, Matthias, additional, Reutter, Heiko, additional, Woelfle, Joachim, additional, Agaimy, Abbas, additional, Mardin, Christian, additional, Hoerning, André, additional, and Hohberger, Bettina, additional

Published
2023
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11. Clean Intermittent Catheterization in Children under 12 Years Does Not Have a Negative Impact on Long-Term Graft Survival following Pediatric Kidney Transplantation.

Author

Marcou, Marios, Galiano, Matthias, Tzschoppe, Anja, Sauerstein, Katja, Wach, Sven, Taubert, Helge, Wullich, Bernd, Hirsch-Koch, Karin, and Apel, Hendrik

Subjects
*KIDNEY transplantation, *URINARY tract infections, *INTERMITTENT urinary catheterization, *GRAFT survival, *CHRONIC kidney failure, *URINARY organs, *MEDICAL care
Abstract

Background: Congenital anomalies of the kidneys and urinary tract (CAKUTs) are one of the most prevalent primary causes of end-stage renal disease (ESRD) in young children, and approximately one-third of these children present with lower urinary tract dysfunction (LUTD). Many children with LUTD require therapy with clean intermittent catheterization (CIC). CIC commonly leads to bacteriuria, and considerations have arisen regarding whether CIC in immunosuppressed children is safe or whether repeated febrile urinary tract infections (UTIs) may lead to the deterioration of kidney graft function. Material and Methods: We retrospectively reviewed all cases of primary kidney transplantation performed in our center between 2001 and 2020 in recipients aged less than twelve years. The number of episodes of febrile UTIs as well as the long-term kidney graft survival of children undergoing CIC were compared to those of children with urological causes of ESRD not undergoing CIC, as well as to those of children with nonurological causes of ESRD. Results: Following successful kidney transplantation in 41 children, CIC was needed in 8 of these patients. These 8 children undergoing CIC had significantly more episodes of febrile UTIs than did the 18 children with a nonurological cause of ESRD (p = 0.04) but not the 15 children with a urological cause of ESRD who did not need to undergo CIC (p = 0.19). Despite being associated with a higher rate of febrile UTIs, CIC was not identified as a risk factor for long-term kidney graft survival, and long-term graft survival did not significantly differ between the three groups at a median follow-up of 124 months. Conclusions: Our study demonstrates that, under regular medical care, CIC following pediatric transplantation is safe and is not associated with a higher rate of long-term graft loss. [ABSTRACT FROM AUTHOR]

Published
2024
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13. Clinical Characteristics and Courses of Patients With Autosomal Recessive Polycystic Kidney Disease-Mimicking Phenocopies

Author

Halawi, Abdul A., primary, Burgmaier, Kathrin, additional, Buescher, Anja K., additional, Dursun, Ismail, additional, Erger, Florian, additional, Galiano, Matthias, additional, Gessner, Michaela, additional, Gökce, Ibrahim, additional, Mekahli, Djalila, additional, Mir, Sevgi, additional, Obrycki, Lukasz, additional, Shroff, Rukshana, additional, Stabouli, Stella, additional, Szczepanska, Maria, additional, Teixeira, Ana, additional, Weber, Lutz T., additional, Wenzel, Andrea, additional, Wühl, Elke, additional, Zachwieja, Katarzyna, additional, Dötsch, Jörg, additional, Schaefer, Franz, additional, and Liebau, Max C., additional

Published
2023
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14. 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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15. Ratio of Urinary Proteins to Albumin Excretion Shifts Substantially during Progression of the Podocytopathy Alport Syndrome, and Spot Urine Is a Reliable Method to Detect These Pathologic Changes

Author

Boeckhaus, Jan, primary, Mohr, Lea, additional, Dihazi, Hassan, additional, Tönshoff, Burkhard, additional, Weber, Lutz T., additional, Pape, Lars, additional, Latta, Kay, additional, Fehrenbach, Henry, additional, Lange-Sperandio, Baerbel, additional, Kettwig, Matthias, additional, Staude, Hagen, additional, König, Sabine, additional, John-Kroegel, Ulrike, additional, Gellermann, Jutta, additional, Hoppe, Bernd, additional, Galiano, Matthias, additional, Haffner, Dieter, additional, Rhode, Heidrun, additional, and Gross, Oliver, additional

Published
2023
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16. Isolated nocturnal and isolated daytime hypertension associate with altered cardiovascular morphology and function in children with chronic kidney disease: findings from the Cardiovascular Comorbidity in Children with Chronic Kidney Disease study

Author

Düzova, Ali, Karabay Bayazit, Aysun, Canpolat, Nur, Niemirska, Anna, Kaplan Bulut, Ipek, Azukaitis, Karolis, Karagoz, Tevfik, Oguz, Berna, Erdem, Sevcan, Anarat, Ali, Ranchin, Bruno, Shroff, Rukshana, Djukic, Milan, Harambat, Jerome, Yilmaz, Alev, Yildiz, Nurdan, Ozcakar, Birsin, Büscher, Anja, Lugani, Francesca, Wygoda, Simone, Tschumi, Sibylle, Zaloszyc, Ariane, Jankauskiene, Augustina, Laube, Guido, Galiano, Matthias, Kirchner, Marietta, Querfeld, Uwe, Melk, Anette, Schaefer, Franz, and Wühl, Elke

Published
2019
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18. Clinical Characteristics and Courses of Patients With Autosomal Recessive Polycystic Kidney Disease-Mimicking Phenocopies

Author

Halawi, Abdul A., Burgmaier, Kathrin, Buescher, Anja K., Dursun, Ismail, Erger, Florian, Galiano, Matthias, Gessner, Michaela, Gokce, Ibrahim, Mekahli, Djalila, Mir, Sevgi, Obrycki, Lukasz, Shroff, Rukshana, Stabouli, Stella, Szczepańska, Maria, Teixeira, Ana, Weber, Lutz T., Wenzel, Andrea, Wuhl, Elke, Zachwieja, Katarzyna, Dotsch, Jorg, Schaefer, Franz, Liebau, Max C., and Halawi A. A., Burgmaier K., Buescher A. K., DURSUN İ., Erger F., Galiano M., Gessner M., GÖKCE İ., Mekahli D., Mir S., et al.

Subjects
Internal Diseases, TMEM67, Internal Medicine Sciences, polycystic kidney disease, Klinik Tıp, PKD2, PKD1, Medizin, PKHD1, Dahili Tıp Bilimleri, CLINICAL MEDICINE, Sağlık Bilimleri, İç Hastalıkları, Clinical Medicine (MED), Tıp, Nefroloji, Nephrology, UROLOGY & NEPHROLOGY, Health Sciences, Medicine, Klinik Tıp (MED), ciliopathies, ÜROLOJİ VE NEFROLOJİ
Abstract

in press, CA extern

Published
2023

19. Clinical courses and complications of young adults with Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Author

Burgmaier, Kathrin, Kilian, Samuel, Bammens, Bert, Benzing, Thomas, Billing, Heiko, Büscher, Anja, Galiano, Matthias, Grundmann, Franziska, Klaus, Günter, Mekahli, Djalila, Michel-Calemard, Laurence, Milosevski-Lomic, Gordana, Ranchin, Bruno, Sauerstein, Katja, Schaefer, Susanne, Shroff, Rukshana, Sterenborg, Rosalie, Verbeeck, Sarah, Weber, Lutz T., Wicher, Dorota, Wühl, Elke, Dötsch, Jörg, Schaefer, Franz, and Liebau, Max C.

Published
2019
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20. Timing of first arteriovenous fistula cannulation in children on hemodialysis

Author

Almasi-Sperling, Veronika, Galiano, Matthias, Lang, Werner, Rother, Ulrich, Rascher, Wolfgang, and Regus, Susanne

Subjects
Hemodialysis -- Physiological aspects -- Usage -- Research, Arteriovenous fistula -- Physiological aspects -- Research, Chronic kidney failure -- Care and treatment -- Patient outcomes -- Research, Health
Abstract

Background Due to lower complication rates in comparison to central venous catheter (CVC) arteriovenous fistulas (AVFs) are now the preferred hemodialysis access. Recommendations for the first access cannulation range from 6 to 12 weeks, which could lead to temporary or even permanent preference for CVC while awaiting the maturation of the newly created AVF. The aim of this study was to evaluate the influence of first cannulation of AVFs on primary (PP) and secondary (SP) patency rates in children on hemodialysis (HD). Methods This was a retrospective cohort study of 42 pediatric patients with a median age of 14 (range 7-17) years. At the time of surgical AVF creation 21 patients (end-stage renal disease) were still on HD via CVC or peritoneal catheter, while 21 were pre-emptive with initiation of HD expected within a few weeks. All patients received an AVF by the same experienced surgeon between February 1993 and May 2014. Primary failure (PF) was defined as the inability to use the AVF even once due to absent maturation or occlusion within 4 weeks after creation. PP was defined as the interval from time of access placement to any intervention designed to maintain or reestablish patency, to access thrombosis or the time of measurement of patency, while SP was defined as the total lifespan from creation to access abandonment, end of follow-up or loss. Results Primary failure was observed in six (14.3 %) of 42 AVFs (all radiocephalic fistulas) within the first 10 days after cannulation. Excluding PF, the PP/SP rates at 1, 3, 6, 12, 18 and 24 months were 100/100, 91/99, 86/98, 76/95, 55/85 and 44/77 %, respectively. There was a significant decrease in PP when first cannulation was performed within the first 30 days after creation compared to first cannulation performed after 30 days (p = 0.004). In terms of PP/SP outcome and timing of the first cannulation, there was no significant difference in thee outcome of PP/SP between first cannulation within the first 45 days after creation and that after 45 days (p = 0.091/0.883). Conclusions The findings suggest that cannulation of AVF within 30 days after surgical creation reduces PP, while SP may be influenced less by time until cannulation. We also found no significant differences in PP after maturing periods of >45 days., Author(s): Veronika Almasi-Sperling[sup.1] , Matthias Galiano[sup.2] , Werner Lang[sup.1] , Ulrich Rother[sup.1] , Wolfgang Rascher[sup.2] , Susanne Regus[sup.1] Author Affiliations: (1) Department of Vascular Surgery, Hospital of the Friedrich-Alexander-University Erlangen-Nürnberg [...]

Published
2016
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21. Diagnostic and therapeutic management of vesico-ureteral reflux in pediatric kidney transplantation-Results of an online survey on behalf of the European Society for Paediatric Nephrology

Author

Murer, Luisa, Knops, Noël, de Jong, Huib, Konrad, Martin, Levtchenko, Elena, Jankauskiene, Augustina, Madrid-Aris, Alvaro, Marks, Stephen D., Mattoo, Tej K., Maxted, Andrew, Jahnukainen, Timo, Hooman, Nakisa, Higueras, Walter, Goñi, Maria Herrero, Herthelius, Maria, Henne, Thomas, Grenda, Ryszard, Gessner, Michaela, Gander, Romy, Zirngibl, Matthias, Galiano, Matthias, Fila, Marc, Espinosa-Román, Laura, Esfandiar, Nasrin, Dinçel, Nida Temizkan, Kılıç, Beltinge Demircioğlu, Buder, Kathrin, Dehoux, Laurène, Cornelissen, Marlies, Clothier, Joanna, Cicek, Neslihan, Christian, Martin, Bulum, Burcu, Büscher, Anja, Melgosa-Hijosa, Marta, Luithle, Tobias, Tönshoff, Burkhard, Weitz, Marcus, Zieg, Jakub, Yüksel, Selçuk, Yıldız, Nurdan, Yap, Yok-Chin, Yalçınkaya, Fatma Fatoş, Weber, Lutz T., Mincham, Christine Marie, Alonso-Melgar, Ángel, Ariceta, Gema, Mitsioni, Andromachi, Montini, Giovanni, Awan, Atif, Morgan, Henry, Bakkaloglu, SEVCAN AZİME, Baskin, Esra, Vidal, Enrico, Bekassy, Zivile, Bhimma, Rajendra, Verrina, Enrico Eugenio, Bitzan, Martin, Aki, Fazil Tuncay, Tse, Yincent, Tschumi, Sibylle, Trnka, Peter, Bjerre, Anna Kristina, Torres, Diletta, Topaloğlu, Rezan, Bootsma-Robroeks, Charlotte M., Thumfart, Julia, Teixeira, Ana, Bouts, Antonia, Tasic, Velibor, Taşdemir, Mehmet, Stabouli, Stella, Sinha, Rajiv, Silva, Ana Cristina Simões E., Shenoy, Mohan, Seeman, Tomas, Rus, Rina, Rumyantsev, Alexander, Rubik, Jacek, Roussinov, Dimitar, Reynolds, Ben, Prytula, Agnieszka, Printza, Nikoleta, Parvex, Paloma, Pape, Lars, Özçakar, Z Birsin, Müller-Sacherer, Thomas, Pediatrics, Paediatric Nephrology, and ARD - Amsterdam Reproduction and Development

Subjects
Transplantation, therapy, vesico-ureteral reflux, Pediatrics, Perinatology and Child Health, online survey, febrile urinary tract infection, pediatric kidney transplantation, ureteral implantation
Abstract

© 2022 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.Background: Vesico-ureteral reflux (VUR) is considered to be a risk factor for recurrent febrile urinary tract infections and impaired renal transplant survival. Methods: An online survey supported by the European Society for Paediatric Nephrology was designed to evaluate current management strategies of VUR in native and transplanted kidneys of recipients aged

Published
2022

22. Relationship between age at initiation of cysteamine treatment, adherence with therapy, and glomerular kidney function in infantile nephropathic cystinosis

Author

Nießl, Christina, primary, Boulesteix, Anne-Laure, additional, Oh, Jun, additional, Palm, Katja, additional, Schlingmann, Peter, additional, Wygoda, Simone, additional, Haffner, Dieter, additional, Wühl, Elke, additional, Tönshoff, Burkhard, additional, Buescher, Anja, additional, Billing, Heiko, additional, Hoppe, Bernd, additional, Zirngibl, Matthias, additional, Kettwig, Matthias, additional, Moeller, Kristina, additional, Acham-Roschitz, Birgit, additional, Arbeiter, Klaus, additional, Bald, Martin, additional, Benz, Marcus, additional, Galiano, Matthias, additional, John-Kroegel, Ulrike, additional, Klaus, Guenter, additional, Marx-Berger, Daniela, additional, Moser, Katja, additional, Mueller, Dirk, additional, Patzer, Ludwig, additional, Pohl, Martin, additional, Seitz, Barbara, additional, Treikauskas, Ulrike, additional, von Vigier, Rodo O., additional, Gahl, William Allen, additional, and Hohenfellner, Katharina, additional

Published
2022
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23. Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD

Author

Nalcacioglu, Hulya, Dunand, Oliver, Mastrangelo, Antonio, Murer, Luisa, Emma, Francesco, Ruzgiene, Dovile, Taranta-Janusz, Katarzyna, Balasz-Chmielewska, Irena, Miklaszewska, Monika, Stanczyk, Malgorzata, Sikora, Przemyslaw, Kowalewska, Claudia, Szczepanska, Maria, Teixeira, Ana, Rachisan, Andreea, Papachristou, Fotios, Paripović, Dušan, Prikhodina, Larisa, Jilani, Houweyda, Bayazit, Aysun Karabay, Soylu, Alper, Candan, Cengiz, Sever, Lale, Emre, Sevinc, Cicek, Neslihan, Akinci, Nurver, Mir, Sevgi, Poyrazoğlu, Hakan M., Tabel, Yilmaz, Mencarelli, Francesca, Burgmaier, Kathrin, Kilian, Samuel, Arbeiter, Klaus, Atmis, Bahriye, Büscher, Anja, Derichs, Ute, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Galiano, Matthias, Gessner, Michaela, Gokce, Ibrahim, Haeffner, Karsten, Hooman, Nakysa, Jankauskiene, Augustina, Körber, Friederike, Longo, Germana, Massella, Laura, Mekahli, Djalila, Miloševski-Lomić, Gordana, Rus, Rina, Shroff, Rukshana, Stabouli, Stella, Weber, Lutz T., Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Dötsch, Jörg, Schaefer, Franz, Liebau, Max Christoph, Potemkina, Alexandra, Ranguelov, Nadejda, Collard, Laure, De Mul, Aurélie, Feldkoetter, Markus, Seeman, Tomas, Zieg, Jakub, Thumfart, Julia, Grundmann, Franziska, Buchholz, Björn, Pape, Lars, Gross, Oliver, Patzer, Ludwig, Schild, Raphael, Haffner, Dieter, Bernhardt, Wanja, Wuehl, Elke, Henn, Michael, Halbritter, Jan, Klaus, Günter, Lechner, Felix, Lange-Sperandio, Bärbel, Uetz, Barbara, Benz, Marcus, König, Jens, Staude, Hagen, Wurm, Donald, Bald, Martin, Soliman, Neveen A., Ariceta, Gema, Rodriguez, Juan David Gonzalez, de la Cerda Ojeda, Francisco, Harambat, Jerome, Ranchin, Bruno, Fila, Marc, Dossier, Claire, Boyer, Olivia, Marlais, Matko, UCL - (SLuc) Département de pédiatrie, Ruzgienė, Dovilė, Burgmaier, Kathrin, Kilian, Samuel, Arbeiter, Klaus, Atmis, Bahriye, Buescher, Anja, Derichs, Ute, Dursun, Ismail, Duzova, Ali, Eid, Loai Akram, Galiano, Matthias, Gessner, Michaela, Gokce, Ibrahim, Haeffner, Karsten, Hooman, Nakysa, Jankauskiene, Augustina, Koerber, Friederike, Longo, Germana, Massella, Laura, Mekahli, Djalila, Milosevski-Lomic, Gordana, Nalcacioglu, Hulya, Rus, Rina, Shroff, Rukshana, Stabouli, Stella, Weber, Lutz T., Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Doetsch, Joerg, Schaefer, Franz, and Liebau, Max Christoph

Subjects
Liver Cirrhosis, Male, glomerulus filtration rate, Medizin, cell surface receptor, preschool child, DISEASE, Cohort Studies, Chronic kidney disease, Polycystic kidney disease, Medicine, genetics, Longitudinal Studies, Child, pathophysiology, Ultrasonography, Kidney, Multidisciplinary, longitudinal study, organ size, chronic kidney failure, biological marker, cohort analysis, Prognosis, Autosomal Recessive Polycystic Kidney Disease, PKHD1 protein, human, Multidisciplinary Sciences, medicine.anatomical_structure, female, Quartile, Child, Preschool, Cohort, Disease Progression, Science & Technology - Other Topics, Glomerular Filtration Rate, medicine.medical_specialty, kidney, Adolescent, Science, Urology, Renal function, Kidney Volume, Receptors, Cell Surface, Article, Humans, ddc:610, human, Renal Insufficiency, Chronic, Polycystic Kidney, Autosomal Recessive, Paediatric kidney disease, Science & Technology, business.industry, echography, Infant, medicine.disease, mortality, kidney polycystic disease, disease exacerbation, physiology, business, metabolism, Biomarkers, Kidney disease
Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational study we analyzed the longitudinal development of sonographic kidney measurements in a cohort of 456 ARPKD patients from the international registry study ARegPKD. We furthermore evaluated correlations of sonomorphometric findings and functional kidney disease with the aim to describe the natural disease course and to identify potential prognostic markers. Kidney pole-to-pole (PTP) length and estimated total kidney volume (eTKV) increase with growth throughout childhood and adolescence despite individual variability. Height-adjusted PTP length decreases over time, but such a trend cannot be seen for height-adjusted eTKV (haeTKV) where we even observed a slight mean linear increase of 4.5 ml/m per year during childhood and adolescence for the overall cohort. Patients with two null PKHD1 variants had larger first documented haeTKV values than children with missense variants (median (IQR) haeTKV 793 (450–1098) ml/m in Null/null, 403 (260–538) ml/m in Null/mis, 230 (169–357) ml/m in Mis/mis). In the overall cohort, estimated glomerular filtration rate decreases with increasing haeTKV (median (IQR) haeTKV 210 (150–267) ml/m in CKD stage 1, 472 (266–880) ml/m in stage 5 without kidney replacement therapy). Strikingly, there is a clear correlation between haeTKV in the first eighteen months of life and kidney survival in childhood and adolescence with ten-year kidney survival rates ranging from 20% in patients of the highest to 94% in the lowest quartile. Early childhood haeTKV may become an easily obtainable prognostic marker of kidney disease in ARPKD, e.g. for the identification of patients for clinical studies. © 2021, The Author(s)., ESPN 2014.2; PKD Foundation, PKDF; Bundesministerium für Bildung und Forschung, BMBF: 01GM1515, 01GM1903; Universität zu Köln, UoC; Marga und Walter Boll-Stiftung; Universitätsklinikum Köln, We thank the German Society for Pediatric Nephrology (GPN), the ESCAPE Network, and the European Society for Paediatric Nephrology (ESPN; Working Groups CAKUT and Inherited Renal Diseases) for their support. ML was supported by grants of the GPN, ESPN (Grant ESPN 2014.2), and the German PKD foundation. KB and ML were supported by the Medical Faculty of the University of Cologne (Koeln Fortune program), and the Marga and Walter Boll-Foundation. FS and ML are supported by the German Federal Ministry of Research and Education (BMBF grant 01GM1515 and 01GM1903). This work was generated within the European Reference Network for Rare Kidney Disorders (ERKNet).

Published
2021

24. The Phenotypic Spectrum of Nephropathies Associated with Mutations in Diacylglycerol Kinase ε

Author

Azukaitis, Karolis, Simkova, Eva, Majid, Mohammad Abdul, Galiano, Matthias, Benz, Kerstin, Amann, Kerstin, Bockmeyer, Clemens, Gajjar, Radha, Meyers, Kevin E., Cheong, Hae Il, Lange-Sperandio, Bärbel, Jungraithmayr, Therese, Frémeaux-Bacchi, Véronique, Bergmann, Carsten, Bereczki, Csaba, Miklaszewska, Monika, Csuka, Dorottya, Prohászka, Zoltán, Gipson, Patrick, Sampson, Matthew G., Lemaire, Mathieu, and Schaefer, Franz

Published
2017
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25. Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention

Author

Bausch, Birke, Schiavi, Francesca, Ni, Ying, Welander, Jenny, Patocs, Attila, Ngeow, Joanne, Wellner, Ulrich, Malinoc, Angelica, Taschin, Elisa, Barbon, Giovanni, Lanza, Virginia, Söderkvist, Peter, Stenman, Adam, Larsson, Catharina, Svahn, Fredrika, Chen, Jin-Lian, Marquard, Jessica, Fraenkel, Merav, Walter, Martin A., Peczkowska, Mariola, Prejbisz, Aleksander, Jarzab, Barbara, Hasse-Lazar, Kornelia, Petersenn, Stephan, Moeller, Lars C., Meyer, Almuth, Reisch, Nicole, Trupka, Arnold, Brase, Christoph, Galiano, Matthias, Preuss, Simon F., Kwok, Pingling, Lendvai, Nikoletta, Berisha, Gani, Makay, Özer, Boedeker, Carsten C., Weryha, Georges, Racz, Karoly, Januszewicz, Andrzej, Walz, Martin K., Gimm, Oliver, Opocher, Giuseppe, Eng, Charis, and Neumann, Hartmut P. H.

Published
2017
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26. PHENOCOPIESOF CLINICALLY DIAGNOSED ARPKD REVEALED IN CHILDREN

Author

DURSUN, İSMAİL, Schaefer, Franz, Liebau, Max, Halawi, Abdul, GÖKCE, İBRAHİM, Kowalewska, Claudia, Mekahli, Djalila, Mir, Sevgi, Shroff, Rukshana, Stabouli, Stella, Szczepanska, Maria, Teixeira, Ana, Weber, Lutz, WUehl, Elke, Wygoda, Simone, Zachwieja, Katarzyna, Doetsch, Joerg, Burgmaier, Kathrin, Buescher, Anja, Gessner, Michaela, and Galiano, Matthias

Published
2021

27. EARLY CHILDHOOD HEIGHT-ADJUSTED TOTAL KIDNEY VOLUME AS A RISK MARKER OF KIDNEY SURVIVAL IN AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE (ARPKD)

Author

Burgmaier, Kathrin, Kilian, Samuel, Arbeiter, Klaus, Atmis, Bahriye, Buescher, Anja, Derichs, Ute, Dursun, Ismail, Duzova, Ali, Eid, Loai, Galiano, Matthias, Gessner, Michaela, Gokce, Ibrahim, Haeffner, Karsten, Hooman, Nakysa, Jankauskiene, Augustina, Koerber, Friederike, Longo, Germana, Massella, Laura, Mekahli, Djalila, Milosevski-Lomic, Gordana, Hulya Nalcacioglu, Rus, Rina, Shroff, Rukshana, Stabouli, Stella, Weber, Lutz, Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Doetsch, Joerg, Schaefer, Franz, and Liebau, Max Christoph

Published
2021

29. Refining genotype–phenotype correlations in 304 patients with autosomal recessive polycystic kidney disease and PKHD1 gene variants

Author

Burgmaier, Kathrin, primary, Brinker, Leonie, additional, Erger, Florian, additional, Beck, Bodo B., additional, Benz, Marcus R., additional, Bergmann, Carsten, additional, Boyer, Olivia, additional, Collard, Laure, additional, Dafinger, Claudia, additional, Fila, Marc, additional, Kowalewska, Claudia, additional, Lange-Sperandio, Bärbel, additional, Massella, Laura, additional, Mastrangelo, Antonio, additional, Mekahli, Djalila, additional, Miklaszewska, Monika, additional, Ortiz-Bruechle, Nadina, additional, Patzer, Ludwig, additional, Prikhodina, Larisa, additional, Ranchin, Bruno, additional, Ranguelov, Nadejda, additional, Schild, Raphael, additional, Seeman, Tomas, additional, Sever, Lale, additional, Sikora, Przemyslaw, additional, Szczepanska, Maria, additional, Teixeira, Ana, additional, Thumfart, Julia, additional, Uetz, Barbara, additional, Weber, Lutz Thorsten, additional, Wühl, Elke, additional, Zerres, Klaus, additional, Dötsch, Jörg, additional, Schaefer, Franz, additional, Liebau, Max Christoph, additional, Eid, Loai Akram, additional, Arbeiter, Klaus, additional, Godefroid, Nathalie, additional, Lombet, Jacques, additional, De Mul, Aurélie, additional, Feldkoetter, Markus, additional, Zieg, Jakub, additional, Grundmann, Franziska, additional, Galiano, Matthias, additional, Buchholz, Björn, additional, Buescher, Anja, additional, Häffner, Karsten, additional, Gross, Oliver, additional, Oh, Jun, additional, Haffner, Dieter, additional, Bernhardt, Wanja, additional, Schaefer, Susanne, additional, Wygoda, Simone, additional, Halbritter, Jan, additional, Derichs, Ute, additional, Klaus, Günter, additional, Lechner, Felix, additional, Ponsel, Sabine, additional, König, Jens, additional, Staude, Hagen, additional, Wurm, Donald, additional, Bald, Martin, additional, Gessner, Michaela, additional, Soliman, Neveen A., additional, Ariceta, Gema, additional, Gonzalez Rodriguez, Juan David, additional, Ojeda, Francisco de la Cerda, additional, Harambat, Jerome, additional, Morin, Denis, additional, Dossier, Claire, additional, Dorval, Guillaume, additional, Shroff, Rukshana, additional, Stabouli, Stella, additional, Hooman, Nakysa, additional, Mencarelli, Francesca, additional, Morello, William, additional, Longo, Germana, additional, Emma, Francesco, additional, Jankauskiene, Augustina, additional, Taranta-Janusz, Katarzyna, additional, Zagozdzon, Ilona, additional, Zachwieja, Katarzyna, additional, Stanczyk, Malgorzata, additional, Bienias, Beata, additional, Litwin, Mieczyslaw, additional, Morawiec-Knysak, Aurelia, additional, Afonso, Alberto Caldas, additional, Dunand, Oliver, additional, Rachisan, Andreea, additional, Miloševski-Lomić, Gordana, additional, Papizh, Svetlana, additional, Rus, Rina, additional, Jilani, Houweyda, additional, Atmis, Bahriye, additional, Duzova, Ali, additional, Soylu, Alper, additional, Candan, Cengiz, additional, Caliskan, Salim, additional, Yilmaz, Alev, additional, Gökce, İbrahim, additional, Akinci, Nurver, additional, Mir, Sevgi, additional, Dursun, Ismail, additional, Tabel, Yilmaz, additional, and Nalcacioglu, Hulya, additional

Published
2021
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31. Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)

Author

Häffner, Karsten, Gross, Oliver, Bernhardt, Wanja, Doyon, Anke, Henn, Michael, Halbritter, Jan, Derichs, Ute, Klaus, Günter, Lange-Sperandio, Bärbel, Uetz, Barbara, Benz, Marcus, Titieni, Andrea, Staude, Hagen, Leichter, Heinz E., Soliman, Neveen A., Lara, Luis Enrique, de la Cerda Ojeda, Francisco, Harambat, Jerome, Ranchin, Bruno, Fila, Marc, Dossier, Claire, Boyer, Olivia, Stabouli, Stella, Hooman, Nakysa, Mencarelli, Francesca, Morello, William, Longo, Germana, Emma, Francesco, Ruzgiene, Dovile, Wasilewska, Anna, Balasz-Chmielewska, Irena, Miklaszewska, Monika, Stanczyk, Malgorzata, Sikora, Przemyslaw, Litwin, Mieczyslaw, Morawiec-Knysak, Aurelia, Teixeira, Ana, Milosevski-Lomic, Gordana, Prikhodina, Larisa, Rus, Rina, Jilani, Houweyda, Melek, Engin, Duzova, Ali, Candan, Cengiz, Sever, Lale, Ranguelov, Nadejda, Yilmaz, Alev, Cicek, Neslihan, Akinci, Nurver, Mir, Sevgi, Dursun, Ismail, Tabel, Yilmaz, Nalcacioglu, Hulya, Shroff, Rukshana, Marlais, Matko, Soylu, Alper, Arbeiter, Klaus, Eid, Loai Akram, Liebau, Max Christoph, Schaefer, Franz, Oh, Jun, Dötsch, Jörg, Zagozdzon, Ilona, Wygoda, Simone, Wurm, Donald, Wühl, Elke, Weber, Lutz Thorsten, Tkaczyk, Marcin, Burgmaier, Kathrin, Ariceta, Gema, Bald, Martin, Buescher, Anja Katrin, Burgmaier, Mathias, Erger, Florian, Gessner, Michaela, GÖKCE, İBRAHİM, König, Jens, Kowalewska, Claudia, Massella, Laura, Mastrangelo, Antonio, Mekahli, Djalila, Pape, Lars, Patzer, Ludwig, Potemkina, Alexandra, Schalk, Gesa, Schild, Raphael, Szczepanska, Maria, Taranta-Janusz, Katarzyna, Collard, Laure, De Mul, Aurélie, Feldkoetter, Markus, Seeman, Tomas, Thumfart, Julia, Grundmann, Franziska, Galiano, Matthias, Buchholz, Björn, Buescher, Rainer, UCL - (SLuc) Service de pédiatrie générale, De Mul, Aurélie, Burgmaier, Kathrin, Ariceta, Gema, Bald, Martin, Buescher, Anja Katrin, Burgmaier, Mathias, Erger, Florian, Gessner, Michaela, Gokce, Ibrahim, Koenig, Jens, Kowalewska, Claudia, Massella, Laura, Mastrangelo, Antonio, Mekahli, Djalila, Pape, Lars, Patzer, Ludwig, Potemkina, Alexandra, Schalk, Gesa, Schild, Raphael, Shroff, Rukshana, Szczepanska, Maria, Taranta-Janusz, Katarzyna, Tkaczyk, Marcin, Weber, Lutz Thorsten, Wuehl, Elke, Wurm, Donald, Wygoda, Simone, Zagozdzon, Ilona, Doetsch, Joerg, Oh, Jun, Schaefer, Franz, Liebau, Max Christoph, and Ege Üniversitesi

Subjects
Male, Pediatrics, medicine.medical_treatment, Medizin, 030232 urology & nephrology, INFANTS, lcsh:Medicine, Nephrectomy, RECOMMENDATIONS, Cohort Studies, 0302 clinical medicine, Postoperative Complications, Risk Factors, HYPOTENSION, Polycystic Kidney, Medicine, UNILATERAL NEPHRECTOMY, ENCODES, lcsh:Science, Renal Dialysis/statistics & numerical data, PERITONEAL-DIALYSIS, Kidney, ddc:618, Multidisciplinary, Autosomal recessive polycystic kidney disease (ARPKD), Autosomal Recessive Polycystic Kidney Disease, medicine.anatomical_structure, Cohort, ARegPKD consortium, Disease Progression, Female, Cohort study, Nephrectomy/adverse effects, medicine.medical_specialty, Renal function, MATURATION, Article, 03 medical and health sciences, Polycystic kidney disease, Renal Dialysis, 030225 pediatrics, MANAGEMENT, Humans, In patient, Dialysis, Polycystic Kidney, Autosomal Recessive, Nervous System Diseases/epidemiology/etiology, Autosomal Recessive/surgery, Paediatric kidney disease, business.industry, lcsh:R, 1ST YEAR, NEUROPATHY, Infant, Newborn, Infant, Newborn, medicine.disease, Postoperative Complications/epidemiology/etiology, lcsh:Q, Nervous System Diseases, business
Abstract

To test the association between bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD) and long-term clinical outcome and to identify risk factors for severe outcomes, a dataset comprising 504 patients from the international registry study ARegPKD was analyzed for characteristics and complications of patients with very early (? 3 months; VEBNE) and early (4–15 months; EBNE) bilateral nephrectomies. Patients with very early dialysis (VED, onset ? 3 months) without bilateral nephrectomies and patients with total kidney volumes (TKV) comparable to VEBNE infants served as additional control groups. We identified 19 children with VEBNE, 9 with EBNE, 12 with VED and 11 in the TKV control group. VEBNE patients suffered more frequently from severe neurological complications in comparison to all control patients. Very early bilateral nephrectomies and documentation of severe hypotensive episodes were independent risk factors for severe neurological complications. Bilateral nephrectomies within the first 3 months of life are associated with a risk of severe neurological complications later in life. Our data support a very cautious indication of very early bilateral nephrectomies in ARPKD, especially in patients with residual kidney function, and emphasize the importance of avoiding severe hypotensive episodes in this at-risk cohort. © 2020, The Author(s)., Marga und Walter Boll-Stiftung Universität zu Köln, UoC Bundesministerium für Bildung und Forschung, BMBF: 01GM1903, 01GM1515 PKD Foundation, PKDF European Paediatric Neurology Society, EPNS, We thank the German Society for Pediatric Nephrology (GPN) and the ESCAPE Network for their support. ML was supported by grants of the GPN, the European Society for Paediatric Nephrology (ESPN), the German PKD foundation, the Koeln Fortune program, the GEROK program of the Medical Faculty of University of Cologne, and the Marga and Walter Boll-Foundation. FS and ML are supported by the the German Federal Ministry of Research and Education (BMBF grant 01GM1515 and 01GM1903). KB was supported by the Koeln Fortune program of the Medical Faculty of University of Cologne and the Marga and Walter Boll-Foundation. This work was generated within the European Reference Network for Rare Kidney Disorders (ERKNet). Open Access funding provided by Projekt DEAL.

Published
2020

35. A multicenter, randomized, placebo-controlled, double-blind phase 3 trial with open-arm comparison indicates safety and efficacy of nephroprotective therapy with ramipril in children with Alport’s syndrome

Author

Gross, Oliver, primary, Tönshoff, Burkhard, additional, Weber, Lutz T., additional, Pape, Lars, additional, Latta, Kay, additional, Fehrenbach, Henry, additional, Lange-Sperandio, Baerbel, additional, Zappel, Hildegard, additional, Hoyer, Peter, additional, Staude, Hagen, additional, König, Sabine, additional, John, Ulrike, additional, Gellermann, Jutta, additional, Hoppe, Bernd, additional, Galiano, Matthias, additional, Hoecker, Britta, additional, Ehren, Rasmus, additional, Lerch, Christian, additional, Kashtan, Clifford E., additional, Harden, Markus, additional, Boeckhaus, Jan, additional, Friede, Tim, additional, Koziolek, Michael, additional, Bramlage, Carsten Paul, additional, Weber, Frauke, additional, Albrecht-Nock, Tanja, additional, Sonntag, Joseph, additional, Frese, Jenny, additional, Kettwig, Matthias, additional, Hilgers, Reinhard, additional, Hansen, Matthias, additional, Wedekin, Mirja, additional, Meyer, Nicole, additional, Klaiber, Susanne, additional, Gessner, Michaela, additional, Liebau, Max, additional, Vogt-Weigeldt, Anne-Kristin, additional, Jungraithmayr, Therese, additional, Ponsel, Sabine, additional, Jacoby, Ulrike, additional, Konrad, Martin, additional, Kranz, Brigitta, additional, Koenig, Jens, additional, Loechtermann, Lisa, additional, Pohl, Michael, additional, Husain, Ralf, additional, Mueller, Katrin, additional, Thumfart, Julia, additional, Schalk, Gesa, additional, Feldkoetter, Markus, additional, Schmidt, Sabine, additional, Sauerstein, Katja, additional, Muschiol, Evelin, additional, Billing, Heiko, additional, and Wilkening, Frauke, additional

Published
2020
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36. Homozygous familial hypercholesterolemia with severe involvement of the aortic valve—A sibling‐controlled case study on the efficacy of lipoprotein apheresis

Author

Galiano, Matthias, primary, Hammersen, Johanna, additional, Sauerstein, Katja, additional, Blessing, Holger, additional, Rümmele, Petra, additional, Purbojo, Ariawan, additional, Schöber, Martin, additional, Moosmann, Julia, additional, Raffelsbauer, Gunter, additional, Heibges, Andreas, additional, and Klingel, Reinhard, additional

Published
2020
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37. 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, 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, and Miloševski-Lomić, Gordana

Subjects
AUTOSOMAL recessive polycystic kidney, TUMOR markers, CHILD patients, SURVIVAL rate, MISSENSE mutation, KIDNEYS, PROGNOSIS
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. [ABSTRACT FROM AUTHOR]

Published
2021
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39. Relevance of glomerular C4d deposition in pediatric patients with Henoch-Schönlein Purpura compared to IgA nephritis.

Author

Benz, Kerstin, Ferrazzi, Fulvia, Galiano, Matthias, Sauerstein, Katja, Vonbrunn, Eva, Daniel, Christoph, Büttner-Herold, Maike, and Amann, Kerstin

Subjects
CHILD patients, SCHOENLEIN-Henoch purpura, IGA glomerulonephritis, NEPHRITIS, IMMUNOGLOBULIN A, KIDNEY failure, RENAL biopsy
Abstract

Introduction: IgA nephropathy (IgAN) is the most common primary glomerulonephritis (GN) in western countries and Henoch-Schönlein purpura nephritis (HSPN) is the most common form of vasculitis in childhood. Renal biopsy findings in both nephropathies are often similar and are characterized by mesangioproliferative GN with mesangial or mesangiocapillary IgA and C3c deposits. Objectives: The aim of this study was to investigate the significance of glomerular C4d-deposition as a discriminating factor between pediatric HSPN and IgAN. Patients and Methods: We retrospectively analyzed patient records and renal biopsies from 53 pediatric patients from one single center with a median age of 10.5 years (range 2.3-18 years). Twenty-two patients suffered from IgAN and 31 from HSPN. Work-up of all renal biopsies was performed using standard protocols including immunohistochemistry for C4d. Results: Pediatric IgAN patients presented significantly more often with gross hematuria, higher serum creatinine, lower glomerular filtration rate, lower serum C3 and proteinuria and on histology less endocapillary hypercellularity compared to HSPN patients. However, the rate of glomerular C4d-positivity was not different between IgAN (36%) and HSPN (42%). Comparing all cases with positive versus negative glomerular C4d-staining, pediatric patients with glomerular C4d-positivity showed significantly lesser gross hematuria and received significantly more often cyclophosphamide. This was in line with a tendency towards more proteinuria, hypertension and renal insufficiency at last follow-up in C4d-positive compared to C4d-negative patients. Conclusion: In conclusion, in our monocentric study glomerular C4d does not differ between pediatric HSPN and IgAN, but was associated with a tendency to a more severe course of the disease that needs to be confirmed in larger multicentric studies. [ABSTRACT FROM AUTHOR]

Published
2021
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41. Precise variant interpretation, phenotype ascertainment, and genotype–phenotype correlation of children in the EARLY PRO‐TECT Alport trial.

Author

Boeckhaus, Jan, Hoefele, Julia, Riedhammer, Korbinian M., Tönshoff, Burkhard, Ehren, Rasmus, Pape, Lars, Latta, Kay, Fehrenbach, Henry, Lange‐Sperandio, Baerbel, Kettwig, Matthias, Hoyer, Peter, Staude, Hagen, Konrad, Martin, John, Ulrike, Gellermann, Jutta, Hoppe, Bernd, Galiano, Matthias, Gessner, Michaela, Pohl, Michael, and Bergmann, Carsten

Subjects
PHENOTYPES, KIDNEY failure, GENETIC testing, EXOMES, GUIDELINES, SAMPLE size (Statistics)
Abstract

Early initiation of therapy in patients with Alport syndrome (AS) slows down renal failure by many years. Genotype–phenotype correlations propose that the location and character of the individual's variant correlate with the renal outcome and any extra renal manifestations. In‐depth clinical and genetic data of 60/62 children who participated in the EARLY PRO‐TECT Alport trial were analyzed. Genetic variants were interpreted according to current guidelines and criteria. Genetically solved patients with X‐linked inheritance were then classified according to the severity of their COL4A5 variant into less‐severe, intermediate, and severe groups and disease progress was compared. Almost 90% of patients were found to carry (likely) pathogenic variants and classified as genetically solved cases. Patients in the less‐severe group demonstrated a borderline significant difference in disease progress compared to those in the severe group (p = 0.05). While having only limited power according to its sample size, an obvious strength is the precise clinical and genetic data of this well ascertained cohort. As in published data differences in clinical progress were shown between patients with COL4A5 less‐severe and severe variants. Therefore, clinical and segregational data are important for variant (re)classification. Genetic testing should be mandatory allowing early diagnosis and therapy of AS. [ABSTRACT FROM AUTHOR]

Published
2021
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42. Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

Author

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

Published
2018
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45. Transvascular kidney biopsy in adolescent patients—safe alternative to open procedures.

Author

Utsch, Boris, Hoppe, Hanno, Dittrich, Katalin, Amann, Kerstin, Gugger, Mathias, Tschumi, Sibylle, Galiano, Matthias, Plank, Christian, Rascher, Wolfgang, Schmid, Axel, Uder, Michael, and Dötsch, Jörg

Subjects
RENAL biopsy, TEENAGERS
Abstract

Transvascular kidney biopsy in adolescent patients - safe alternative to open procedures. [Extracted from the article]

Published
2021
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46. Autosomal-Recessive Mutations in SLC34A1 Encoding Sodium-Phosphate Cotransporter 2A Cause Idiopathic Infantile Hypercalcemia

Author

Schlingmann, Karl P., primary, Ruminska, Justyna, additional, Kaufmann, Martin, additional, Dursun, Ismail, additional, Patti, Monica, additional, Kranz, Birgitta, additional, Pronicka, Ewa, additional, Ciara, Elzbieta, additional, Akcay, Teoman, additional, Bulus, Derya, additional, Cornelissen, Elisabeth A.M., additional, Gawlik, Aneta, additional, Sikora, Przemysław, additional, Patzer, Ludwig, additional, Galiano, Matthias, additional, Boyadzhiev, Veselin, additional, Dumic, Miroslav, additional, Vivante, Asaf, additional, Kleta, Robert, additional, Dekel, Benjamin, additional, Levtchenko, Elena, additional, Bindels, René J., additional, Rust, Stephan, additional, Forster, Ian C., additional, Hernando, Nati, additional, Jones, Glenville, additional, Wagner, Carsten A., additional, and Konrad, Martin, additional

Published
2016
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47. Sustained Need for High-Dose Zinc Supplementation in Children With Acrodermatitis Enteropathica.

Author

Hammersen, Johanna, Has, Cristina, Galiano, Matthias, Lindner, Martin, Rossi, Rainer, Kohlhase, Jürgen, and Schneider, Holm

Subjects
DIETARY supplements, ACRODERMATITIS, BIOPSY, HISTOLOGY, ZINC
Abstract

The article presents cases of five children with acrodermatitis enteropathica (AE). First patient showed epidermal blistering and loose stools. Second patient showed perioral and acral skin blistering combined with diarrhea. Third patient showed perioral and acral blisters. Fourth and Fifth patients developed diarrhea, enoral, and perianal skin erosions. All patients showed symptoms of defect in intestinal zinc absorption and the symptoms disappeared after resuming zinc supplement.

Published
2018
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48. Estimating true incidence of O157 and non-O157 Shiga toxin-producing Escherichia coli illness in Germany based on notification data of haemolytic uraemic syndrome

Author

Kuehne, A., Bouwknegt, M., Havelaar, A., Gilsdorf, A., Hoyer, Peter Friedrich, Stark, K., Werber, D., Amon, Oliver, Büscher (R.), Rainer, Hampel, Tobias, Fehrenbach, Henry, Habbig, Sandra, Pohl, Martin, Häffner, Karsten, Hoppe, Bernd, Klaus, Günter, Konrad, Martin, Latta, Kay, Leichter, Heinz, Loos, Sebastian, Montoya, Carmen, Müller, Dominik, Galiano, Matthias, Muschiol, Evelin, Pape, Lars, Staude, Hagen, Wühl, Elke, Henn, Michael, Pohl, Michael, and Wygoda, Simone

Subjects
0301 basic medicine, Pediatrics, Epidemiology, enterohaemorrhagic Escherichia coli, animal diseases, Medizin, Non o157, 0302 clinical medicine, fluids and secretions, Germany, 030212 general & internal medicine, Burden of illness, Child, Escherichia coli Infections, Aged, 80 and over, education.field_of_study, Shiga-Toxigenic Escherichia coli, Incidence (epidemiology), Middle Aged, Original Papers, Bloody, Monte Carlo method, Infectious Diseases, Child, Preschool, Disease Notification, Adult, medicine.medical_specialty, Adolescent, 030106 microbiology, Population, Biology, Escherichia coli O157, Microbiology, 03 medical and health sciences, Young Adult, medicine, Humans, education, Shiga toxin-producing Escherichia coli, Aged, Infant, Newborn, Infant, biochemical phenomena, metabolism, and nutrition, bacterial infections and mycoses, Hemolytic-Uremic Syndrome, Etiology, incidence, bacteria, Haemolytic-uraemic syndrome
Abstract

SUMMARYShiga toxin-producingEscherichia coli(STEC) is an important cause of gastroenteritis (GE) and haemolytic uraemic syndrome (HUS). Incidence of STEC illness is largely underestimated in notification data, particularly of serogroups other than O157 (‘non-O157’). Using HUS national notification data (2008–2012, excluding 2011), we modelled true annual incidence of STEC illness in Germany separately for O157 and non-O157 STEC, taking into account the groups’ different probabilities of causing bloody diarrhoea and HUS, and the resulting difference in their under-ascertainment. Uncertainty of input parameters was evaluated by stochastic Monte Carlo simulations. Median annual incidence (per 100 000 population) of STEC-associated HUS and STEC-GE was estimated at 0·11 [95% credible interval (CrI) 0·08-0·20], and 35 (95% CrI 12-145), respectively. German notification data underestimated STEC-associated HUS and STEC-GE incidences by factors of 1·8 and 32·3, respectively. Non-O157 STEC accounted for 81% of all STEC-GE, 51% of all bloody STEC-GE and 32% of all STEC-associated HUS cases. Non-O157 serogroups dominate incidence of STEC-GE and contribute significantly to STEC-associated HUS in Germany. This might apply to many other countries considering European surveillance data on HUS. Non-O157 STEC should be considered in parallel with STEC O157 when searching aetiology in patients with GE or HUS, and accounted for in modern surveillance systems.

Published
2016

49. Die Rolle der beiden Neuropeptide Galanin und Calcitonin gene related protein (CGRP), der endothelialen und neuronalen Stickoxidsynthetase (e-, nNOS) und von Interleukin 6 (IL6) bei der Regeneration zentral im Fazialiskern nach Fazialisaxotomie und peripher nach Fazialisquetschung

Author

Galiano, Matthias Luigi, Kreutzberg, Georg W. (Prof. Dr. Dr. h.c.), and Konnerth, Arthur (Prof. Dr.)

Subjects
Fazialiskern, Fazialisnerv, Fazialisaxotomie, Regenerationsmechanismen, Interleukin 6, Galanin, CGRP, eNOS, nNOS, Medizin und Gesundheit, nervous system, ddc:610
Abstract

Wir untersuchten Mäuse mit verschiedenen Gendeletionen für Substanzen, die einen möglichen Einfluss auf die Regeneration von Motoneuronen haben. Nach Fazialisaxotomie wurde die frühe Phase am Tag 1 bis 4, die mittlere bis späte Phase am Tag 7 bis 14 untersucht. Wir zeigten, dass IL6-Defizienz nach Axotomie zu einer verminderten Einwanderung von Lymphozyten in den axotomierten Fazialiskern in der frühen und späten Phase führt. Wir sahen weniger aktivierte Mikroglia in der frühen und mittleren Phase und weniger reaktive Astrozyten. Auch konnten mehr Galanin-positive axonale Wachstumskegel nach 2 Wochen und eine mäßig verminderte Regenerationsgeschwindigkeit der Galanin- und CGRP-positiven Axone am Tag 4 gezeigt werden. CGRP-Defizienz steigerte die Einwanderung von Lymphozyten in den Fazialiskern. Galanin-Defizienz zeigte keinen Einfluss auf die Regeneration. eNOS-Defizienz führte zu mehr Galanin-positiven Axone und Neuronen in der späten Phase. n-NOS Defizienz verminderte die lymphozytäre Einwanderung in der frühen und späten Phase. Zusammenfassend zeigte sich überraschenderweise kein Einfluss auf das Regenerationsverhalten bei Galanin-Defizienz, ein mäßiger Einfluss auf die interzellulären Kommunikationskaskaden von CGRP, eNOS und nNOS und ein beachtlicher des Akutphaseproteins Interleukin 6. We investigated the possible role of different substances that could play a role in the regeneration of motor neurons. We used the animal model of a group of gene-deficient mice in comparison to there wild type. Each group was missing one of the substances. After facial axatomy we investigated the cellular changes of regeneration of the early phase on day 1 to 4, the middle and the late phase on day 7 up to day 14 using different techniques of immunohistochemistry. Interleukin 6 deficient mice showed a clearly reduced migration of lymphocytes into the facial nucleus in the early and in the late phase of regeneration. We could observe less activated microglia and astroglia in the early and in the late phase. There was to be seen an increase in the intensity of Galanin in the sprouting growth cones and a higher number of those. Interleukin 6 deficiency led to a slightly slower regeneration of the Galanin- and the CGRP positive axons. CGRP deficiency led to more migrating lymphocytes into the facial nucleus. eNOS deficiency showed more Galanin positive sprouting growth cones on day 4 and an increased intensity of the Galanin positive neurons. nNOS deficiency led to a reduced migration of lymphocytes in the early and in the late phase. However Galanin deficiency, which is highly expressed after facial axatomy in the facial nucleus in the wild type mice, did not show any effect after trauma. Conclusion: Surprisingly, after facial axatomy there was no effect on the changes of regeneration in Galanin deficient mice. There was a mild effect of CGRP, eNOS and nNOS deficiency on the intercellular changes and their underlying molecular communication and a strong effect of Interleukin 6 deficiency.

Published
2007

50. The AP-1 Transcription Factor c-Jun Is Required for Efficient Axonal Regeneration

Author

Raivich, Gennadij, primary, Bohatschek, Marion, additional, Da Costa, Clive, additional, Iwata, Osuke, additional, Galiano, Matthias, additional, Hristova, Maria, additional, Nateri, Abdolrahman S, additional, Makwana, Milan, additional, Riera-Sans, Lluı́s, additional, Wolfer, David P, additional, Lipp, Hans-Peter, additional, Aguzzi, Adriano, additional, Wagner, Erwin F, additional, and Behrens, Axel, additional

Published
2004
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