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

The ARegPKD consortium, 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 ShroffMaria 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

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Article number16025
JournalScientific Reports
Volume10
Issue number1
DOIs
Publication statusPublished - Dec 1 2020

ASJC Scopus subject areas

  • General

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