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

2018; Elsevier BV; Volume: 199; Linguagem: Inglês

10.1016/j.jpeds.2018.03.052

1097-6833

Kathrin Burgmaier, Kevin Kunzmann, Gema Ariceta, Carsten Bergmann, Anja Buescher, Mathias Burgmaier, İsmail Dursun, Ali Düzova, Loai Eid, Florian Erger, Markus Feldkoetter, Matthias Galiano, Michaela Geßner, Heike Goebel, İbrahim Gökçe, Dieter Haffner, Nakysa Hooman, Bernd Höppe, Augustina Jankauskienė, Günter Klaus, Jens König, Mieczysław Litwin, Laura Massella, Djalila Mekahli, Engin Melek, Sevgı Mır, Lars Pape, Larisa Prikhodina, Bruno Ranchin, Raphael Schild, Tomáš Seeman, Lale Sever, Rukshana Shroff, Neveen A. Soliman, Stella Stabouli, Małgorzata Stańczyk, Yılmaz Tabel, Katarzyna Taranta‐Janusz, Sara Testa, Julia Thumfart, Rezan Topaloğlu, Lutz T. Weber, Dorota Wicher, Elke Wühl, Simone Wygoda, Alev Yılmaz, Katarzyna Zachwieja, Ilona Zagożdżon, Klaus Zerres, Jörg Dötsch, Franz Schaefer, Max C. Liebau, Nadejda Ranguelov, Nathalie Godefroid, Laure Collard, Jacques Lombet, Julie Maquet, Gesa Schalk, Uwe Querfeld, Bodo B. Beck, Thomas Benzing, Reinhard Buettner, Franziska Grundmann, Christine Kurschat, Kerstin Benz, Anja Tzschoppe, Bjoern Buchholz, Rainer Buescher, Karsten Häffner, Martin Pöhl, Oliver Groß, Jenny Krügel, Johanna Stock, Ludwig Patzer, Jun Oh, Wanja M. Bernhardt, Anke Doyon, Tobias Vinke, Anja Sander, Michael Henn, Ute Derichs, Rolf Beetz, Nikola Jeck, Bärbel Lange-Sperandio, Sabine Ponsel, Franziska Kusser, Barbara Uetz, Marcus R. Benz, Silke Schmidt, C. J. Huppertz-Kessler, Birgitta Kranz, Andrea Titieni, Donald Wurm, Heinz E. Leichter, Martin Bald, Heiko Billing, Marwa M. Nabhan, Luis Enrique Lara, Fotiοs Papachristou, Francesco Emma, Rimantė Čerkauskienė, Karolis Ažukaitis, Anna Wasilewska, Irena Bałasz–Chmielewska, Monika Miklaszewska, Marcin Tkaczyk, Przemysław Sikora, Marcin Zaniew, Anna Niemirska, Jolanta Antoniewicz, Justyna Lesiak, Alberto Caldas Afonso, Ana Teixeira, Gordana Miloševski‐Lomić, Dušan Paripović, Amira Peco‐Antić, Svetlana Papizh, Aysun Karabay Bayazıt, Ali Anarat, Alper Soylu, Salìh Kavukçu, Cengiz Candan, Salim Çalışkan, Nur Canpolat, Sevinç Emre, Harika Alpay, Nurver Akıncı, Seçil Çonkar, Hakan M. Poyrazoğlu, Ruhan Düşünsel,

Renal and related cancers

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.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.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.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.