Burgmaier, Kathrin, Ariceta, Gema, Bald, Martin, Buescher, Anja Katrin, Burgmaier, Mathias, Erger, Florian, Gessner, Michaela, Gokce, Ibrahim, Koenig, Jens, Kowalewska, Claudia, Massella, Laura, Mastrangelo, Antonio, Mekahli, Djalila, Pape, Lars, Patzer, Ludwig, Potemkina, Alexandra, Schalk, Gesa, Schild, Raphael, Shroff, Rukshana, Szczepanska, Maria, Taranta-Janusz, Katarzyna ORCID: 0000-0002-8762-8866, Tkaczyk, Marcin ORCID: 0000-0003-1753-7560, Weber, Lutz Thorsten, Wuehl, Elke, Wurm, Donald, Wygoda, Simone, Zagozdzon, Ilona, Doetsch, Joerg, Oh, Jun, Schaefer, Franz and Liebau, Max Christoph ORCID: 0000-0003-0494-9080 (2020). Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD). Sci Rep, 10 (1). BERLIN: NATURE RESEARCH. ISSN 2045-2322

Full text not available from this repository.

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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Burgmaier, KathrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ariceta, GemaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bald, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Buescher, Anja KatrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Burgmaier, MathiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Erger, FlorianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gessner, MichaelaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gokce, IbrahimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koenig, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kowalewska, ClaudiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Massella, LauraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mastrangelo, AntonioUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mekahli, DjalilaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pape, LarsUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Patzer, LudwigUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Potemkina, AlexandraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schalk, GesaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schild, RaphaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shroff, RukshanaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Szczepanska, MariaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Taranta-Janusz, KatarzynaUNSPECIFIEDorcid.org/0000-0002-8762-8866UNSPECIFIED
Tkaczyk, MarcinUNSPECIFIEDorcid.org/0000-0003-1753-7560UNSPECIFIED
Weber, Lutz ThorstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wuehl, ElkeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wurm, DonaldUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wygoda, SimoneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zagozdzon, IlonaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doetsch, JoergUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Oh, JunUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schaefer, FranzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Liebau, Max ChristophUNSPECIFIEDorcid.org/0000-0003-0494-9080UNSPECIFIED
URN: urn:nbn:de:hbz:38-318015
DOI: 10.1038/s41598-020-71956-1
Journal or Publication Title: Sci Rep
Volume: 10
Number: 1
Date: 2020
Publisher: NATURE RESEARCH
Place of Publication: BERLIN
ISSN: 2045-2322
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
PERITONEAL-DIALYSIS; UNILATERAL NEPHRECTOMY; 1ST YEAR; INFANTS; ENCODES; RECOMMENDATIONS; HYPOTENSION; MATURATION; MANAGEMENT; NEUROPATHYMultiple languages
Multidisciplinary SciencesMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/31801

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item