RT Journal Article
T1 Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD).
A1 Burgmaier, Kathrin
A1 Ariceta, Gema
A1 Bald, Martin
A1 Buescher, Anja Katrin
A1 Burgmaier, Mathias
A1 Erger, Florian
A1 Gessner, Michaela
A1 Gokce, Ibrahim
A1 König, Jens
A1 Kowalewska, Claudia
A1 Massella, Laura
A1 Mastrangelo, Antonio
A1 Mekahli, Djalila
A1 Pape, Lars
A1 Patzer, Ludwig
A1 Potemkina, Alexandra
A1 Schalk, Gesa
A1 Schild, Raphael
A1 Shroff, Rukshana
A1 Szczepanska, Maria
A1 Taranta-Janusz, Katarzyna
A1 Tkaczyk, Marcin
A1 Weber, Lutz Thorsten
A1 Wühl, Elke
A1 Wurm, Donald
A1 Wygoda, Simone
A1 Zagozdzon, Ilona
A1 Dötsch, Jörg
A1 Oh, Jun
A1 Schaefer, Franz
A1 Liebau, Max Christoph
A1 ARegPKD consortium, 
AB 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.
YR 2020
FD 2020-09-29
LK https://hdl.handle.net/10668/27270
UL https://hdl.handle.net/10668/27270
LA en
DS RISalud
RD Apr 8, 2025