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

TY - JOUR

T1 - Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)

AU - Burgmaier, Kathrin

AU - Ariceta, Gema

AU - Bald, Martin

AU - Buescher, Anja Katrin

AU - Burgmaier, Mathias

AU - Erger, Florian

AU - Gessner, Michaela

AU - Gokce, Ibrahim

AU - König, Jens

AU - Kowalewska, Claudia

AU - Massella, Laura

AU - Mastrangelo, Antonio

AU - Mekahli, Djalila

AU - Pape, Lars

AU - Patzer, Ludwig

AU - Potemkina, Alexandra

AU - Schalk, Gesa

AU - Schild, Raphael

AU - Shroff, Rukshana

AU - Szczepanska, Maria

AU - Taranta-Janusz, Katarzyna

AU - Tkaczyk, Marcin

AU - Weber, Lutz Thorsten

AU - Wühl, Elke

AU - Wurm, Donald

AU - Wygoda, Simone

AU - Zagozdzon, Ilona

AU - Dötsch, Jörg

AU - Oh, Jun

AU - Schaefer, Franz

AU - Liebau, Max Christoph

AU - ARegPKD consortium

PY - 2020/9/29

Y1 - 2020/9/29

N2 - 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.

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.

U2 - 10.1038/s41598-020-71956-1

DO - 10.1038/s41598-020-71956-1

M3 - SCORING: Journal article

C2 - 32994492

VL - 10

SP - 16025

JO - SCI REP-UK

JF - SCI REP-UK

SN - 2045-2322

IS - 1

ER -