Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)
Kathrin Burgmaier, Gema Ariceta, Martin Bald, Anja Buescher, Mathias Burgmaier, Florian Erger, Michaela Geßner, İbrahim Gökçe, Jens König, Claudia Kowalewska, Laura Massella, Antonio Mastrangelo, Djalila Mekahli, Lars Pape, Ludwig Patzer, Alexandra Potemkina, Gesa Schalk, Raphael Schild, Rukshana Shroff, Maria Szczepańska, Katarzyna Taranta‐Janusz, Marcin Tkaczyk, Lutz T. Weber, Elke Wühl, Donald Wurm, Simone Wygoda, Ilona Zagożdżon, Jörg Dötsch, Jun Oh, Franz Schaefer, Max C. Liebau, Loai Eid, Klaus Arbeiter, Nadejda Ranguelov, Laure Collard, Aurélie De Mul, Markus Feldkoetter, Tomáš Seeman, Julia Thumfart, Franziska Grundmann, Matthias Galiano, Bjoern Buchholz, Rainer Buescher, Karsten Häffner, Oliver Groß, Wanja M. Bernhardt, Anke Doyon, Michael Henn, Jan Halbritter, Ute Derichs, Günter Klaus, Bärbel Lange-Sperandio, Barbara Uetz, Marcus R. Benz, Andrea Titieni, Hagen Staude, Heinz E. Leichter, Neveen A. Soliman, Luis Enrique Lara, Francisco de la Cerda Ojeda, Jérôme Harambat, Bruno Ranchin, Marc Fila, Claire Dossier, Olivia Boyer, Matko Marlais, Stella Stabouli, Nakysa Hooman, Francesca Mencarelli, William Morello, Germana Longo, Francesco Emma, Dovilė Ruzgienė, Anna Wasilewska, Irena Bałasz–Chmielewska, Monika Miklaszewska, Małgorzata Stańczyk, Przemysław Sikora, Mieczysław Litwin, Aurelia Morawiec‐Knysak, Ana Teixeira, Gordana Miloševski‐Lomić, Larisa Prikhodina, Rina Rus, Houweyda Jilani, Engin Melek, Ali Düzova, Alper Soylu, Cengiz Candan, Lale Sever, Alev Yılmaz, Neslihan Çiçek, Nurver Akıncı, Sevgı Mır, İsmail Dursun, Yılmaz Tabel, Hülya Nalçacıoğlu, ESCAPE Study Group, GPN Study Group
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.