Kathrin Ebner, Franz Schaefer, Max Christoph Liebau, The ARegPKD Consortium, L. A. Eid, N. Ranguelov, B. Adams, K. van Hoeck, A. Raes, D. Mekahli, L. Collard, J. Lombet, J. Maquet, F. Cachat, G. Schalk, T. Seeman, N. Ortiz Bruechle, K. Zerres, J. Thumfart, S. Briese, U. Querfeld, B. Hoppe, M. Feldkoetter, M. Kirschstein, G. Gruening, B. B. Beck, T. Benzing, R. Buettner, J. Dötsch, H. Goebel, F. Grundmann, B. Hero, C. Kurschat, L. T. Weber, B. Mayer, J. Weber, B. Ritter, K. Benz, M. Galiano, A. Tzschoppe, B. Buchholz, R. Buescher, A. Buescher, K. Latta, K. Häffner, M. Pohl, O. Gross, J. Krügel, J. Stock, L. Patzer, H. Teichler, J. Oh, R. Schild, T. Illig, N. Klopp, L. Pape, S. Wahrendorf, W. Bernhardt, A. Doyon, E. Wuehl, T. Vinke, A. Sander, K. Kunzmann, C. Bergmann, S. Wygoda, M. Henn, D. Wiemann, K. Blaschke, U. Derichs, R. Beetz, N. Jeck, G. Klaus, H. Fehrenbach, T. Hampel, S. Zoetler, M. Wallot, H. Kyrieleis, B. Lange-Sperandio, S. Ponsel, F. Kusser, J. Hoefele, B. Uetz, M. Benz, S. Schmidt, C. Huppertz-Kessler, B. Kranz, J. Koenig, A. Titieni, M. Boeswald, H. Staude, U. Jacoby, D. Wurm, H. E. Leichter, M. Bald, H. Billing, M. Gessner, O. Beringer, M.-L. Ilmoja, N. A. Soliman, M. M. Nabhan, G. Ariceta, L. E. Lara, M. A. Garcia-Gonzalez, C. Diaz-Rodriguez, M. Garcia-Vidal, B. Ranchin, R. Shroff, R. Sterenborg, T. Davitala, F. Papachristou, S. Stabouli, P. Sallay, N. Hooman, G. Ardissino, S. Testa, L. Massella, F. Emma, A. Jankauskiene, R. Cerkauskiene, K. Azukaitis, A. Bokenkamp, J. van Wijk, K. Taranta-Janusz, A. Wasilewska, I. Zagozdzon, I. Balasz-Chmielewska, M. Miklaszewska, K. Zachwieja, D. Drozdz, M. Tkaczyk, M. Stanczyk, P. Sikora, M. Zaniew, M. Litwin, A. Niemirska, D. Wicher, I. Jankowska, J. Antoniewicz, J. Lesiak, P. Lipinski, M. Szczepanska, P. Adamczyk, A. Morawiec-Knysak, A. Caldas Afonso, A. Teixeira, G. Milosevski-Lomic, D. Paripović, A. Peco-Antic, L. Prikhodina, S. Papizh, A. K. Bayazit, A. Anarat, E. Melek, U. S. Bayrakci, A. Kantar, S. Cayci, U. E. Baskin, A. Duzova, A. Yuzbasioglu, A. Soylu, S. Kavukcu, S. Kalman, H. Evrengül, S. Yüksel, A. Kara, M. K. Gurgoze, C. Candan, L. Sever, S. Caliskan, N. Canpolat, S. Emre, A. Yilmaz, I. Gökce, H. Alpay, N. Akinci, S. Mir, B. Sozeri, I. Dursun, H. M. Poyrazoglu, R. Dusunsel, H. Nalcacioglu, Z. Ekinci, Y. Tabel, A. Delibas, D. Övünc Hacihamdioglu, L. Guay-Woodford, ESCAPE Study, GPN Study
Abstract
Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disease with a severe phenotype often presenting prenatally or in early childhood. With its obligate renal and hepatic involvement, ARPKD is one of the most important indications for liver and/or kidney transplantation in childhood. Marked phenotypic variability is observed, the genetic basis of which is largely unknown. Treatment is symptomatic and largely empiric as evidence-based guidelines are lacking. Therapeutic initiatives for ARPKD face the problem of highly variable cohorts and lack of clinical or biochemical risk markers without clear-cut clinical end points. ARegPKD is an international, multicenter, retro- and prospective, observational study to deeply phenotype patients with the clinical diagnosis of ARPKD. Initiated in 2013 as a web-based registry (www.aregpkd.org), ARegPKD enrolls patients across large parts of Europe and neighboring countries. By January 2017, more than 400 patients from 17 mostly European countries have been registered in the ARPKD registry study with significant follow-up data. Due to comprehensive retro- and prospective data collection and associated biobanking, ARegPKD will generate a unique ARPKD cohort with detailed longitudinal clinical characterization providing a basis for future clinical trials as well as translational research. Hence, ARegPKD is hoped to contribute to the pathophysiological understanding of the disease and to the improvement of clinical management.
This research output has an Altmetric Attention Score of 5. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 25 May 2017.
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Altmetric has tracked 22,947,506 research outputs across all sources so far. This one has received more attention than most of these and is in the 71st percentile.
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