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

• Verfasst von: Burgmaier, Kathrin [VerfasserIn]
• Verfasst von: Wühl, Elke [VerfasserIn]
• Verfasst von: Schaefer, Franz [VerfasserIn]
• Verfasst von: Doyon, Anke [VerfasserIn]
• Titel: Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)
• Verf.angabe: Kathrin Burgmaier, Gema Ariceta, Martin Bald, Anja Katrin Buescher, Mathias Burgmaier, Florian Erger, Michaela Gessner, Ibrahim Gokce, Jens König, Claudia Kowalewska, Laura Massella, Antonio Mastrangelo, Djalila Mekahli, Lars Pape, Ludwig Patzer, Alexandra Potemkina, Gesa Schalk, Raphael Schild, Rukshana Shroff, Maria Szczepanska, Katarzyna Taranta-Janusz, Marcin Tkaczyk, Lutz Thorsten Weber, Elke Wühl, Donald Wurm, Simone Wygoda, Ilona Zagozdzon, Jörg Dötsch, Jun Oh, Franz Schaefer, Max Christoph Liebau, Anke Doyon & the ARegpKD consortium
• E-Jahr: 2020
• Jahr: 29 September 2020
• Umfang: 12 S.
• Fussnoten: Gesehen am 17.11.2020 ; The ARegpKD consortium: Loai Akram Eid, Klaus Arbeiter, Nadejda Ranguelov, Laure Collard, Aurélie De Mul, Markus Feldkoetter, Tomas Seeman, Julia Thumfart, Franziska Grundmann, Matthias Galiano, Björn Buchholz, Rainer Buescher, Karsten Häffner, Oliver Gross, Wanja Bernhardt, Anke Doyon, Michael Henn, Jan Halbritter, Ute Derichs, Günter Klaus, Bärbel Lange‑Sperandio, Barbara Uetz, Marcus Benz, Andrea Titieni, Hagen Staude, Heinz E. Leichter, Neveen A. Soliman, Luis enrique Lara, Francisco de la Cerda Ojeda, Jerome Harambat, Bruno Ranchin, Marc Fila, Claire Dossier, Olivia Boyer, Matko Marlais, Stella Stabouli, Nakysa Hooman, Francesca Mencarelli, William Morello, Germana Longo, Francesco Emma, Dovile Ruzgiene, Anna Wasilewska, Irena Balasz‑Chmielewska, Monika Miklaszewska, Malgorzata Stanczyk, Przemyslaw Sikora, Mieczyslaw Litwin, Aurelia Morawiec‑Knysak, Ana Teixeira, Gordana Milosevski‑Lomic, Larisa Prikhodina, Rina Rus, Houweyda Jilani, Engin Melek, Ali Duzova, Alper Soylu, Cengiz Candan, Lale Sever, Alev Yilmaz, Neslihan Cicek, Nurver Akinci, Sevgi Mir, Ismail Dursun, Yilmaz Tabel, Hulya Nalcacioglu
• Titel Quelle: Enthalten in: Scientific reports
• Ort Quelle: [London] : Macmillan Publishers Limited, part of Springer Nature, 2011
• Jahr Quelle: 2020
• Band/Heft Quelle: 10(2020) Artikel-Nummer 16025, 12 Seiten
• ISSN Quelle: 2045-2322
• DOI: doi:10.1038/s41598-020-71956-1
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1738886093

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.