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

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dc.authorscopusid57196234704
dc.authorscopusid6602702810
dc.authorscopusid56339294200
dc.authorscopusid35340836700
dc.authorscopusid8655740200
dc.authorscopusid57193547651
dc.authorscopusid55812699300
dc.contributor.authorBurgmaier K.
dc.contributor.authorAriceta G.
dc.contributor.authorBald M.
dc.contributor.authorBuescher A.K.
dc.contributor.authorBurgmaier M.
dc.contributor.authorErger F.
dc.contributor.authorGessner M.
dc.date.accessioned2024-08-04T20:03:30Z
dc.date.available2024-08-04T20:03:30Z
dc.date.issued2020
dc.departmentİnönü Üniversitesien_US
dc.description.abstractTo 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. © 2020, The Author(s).en_US
dc.description.sponsorshipPKD Foundation, PKDF; European Paediatric Neurology Society, EPNS; Bundesministerium für Bildung und Forschung, BMBF: 01GM1515, 01GM1903; Bundesministerium für Bildung und Forschung, BMBF; Universität zu Köln, UoC; Marga und Walter Boll-Stiftung; Bundesministerium für Bildung, Wissenschaft und Forschung, BMBWFen_US
dc.description.sponsorshipWe thank the German Society for Pediatric Nephrology (GPN) and the ESCAPE Network for their support. ML was supported by grants of the GPN, the European Society for Paediatric Nephrology (ESPN), the German PKD foundation, the Koeln Fortune program, the GEROK program of the Medical Faculty of University of Cologne, and the Marga and Walter Boll-Foundation. FS and ML are supported by the the German Federal Ministry of Research and Education (BMBF grant 01GM1515 and 01GM1903). KB was supported by the Koeln Fortune program of the Medical Faculty of University of Cologne and the Marga and Walter Boll-Foundation. This work was generated within the European Reference Network for Rare Kidney Disorders (ERKNet). Open Access funding provided by Projekt DEAL.en_US
dc.identifier.doi10.1038/s41598-020-71956-1
dc.identifier.issn2045-2322
dc.identifier.issue1en_US
dc.identifier.pmid32994492en_US
dc.identifier.scopus2-s2.0-85091924160en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.urihttps://doi.org/10.1038/s41598-020-71956-1
dc.identifier.urihttps://hdl.handle.net/11616/91871
dc.identifier.volume10en_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherNature Researchen_US
dc.relation.ispartofScientific Reportsen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectadverse eventen_US
dc.subjectcohort analysisen_US
dc.subjectdisease exacerbationen_US
dc.subjectfemaleen_US
dc.subjecthemodialysisen_US
dc.subjecthumanen_US
dc.subjectinfanten_US
dc.subjectkidney polycystic diseaseen_US
dc.subjectmaleen_US
dc.subjectnephrectomyen_US
dc.subjectneurologic diseaseen_US
dc.subjectnewbornen_US
dc.subjectpostoperative complicationen_US
dc.subjectrisk factoren_US
dc.subjectCohort Studiesen_US
dc.subjectDisease Progressionen_US
dc.subjectFemaleen_US
dc.subjectHumansen_US
dc.subjectInfanten_US
dc.subjectInfant, Newbornen_US
dc.subjectMaleen_US
dc.subjectNephrectomyen_US
dc.subjectNervous System Diseasesen_US
dc.subjectPolycystic Kidney, Autosomal Recessiveen_US
dc.subjectPostoperative Complicationsen_US
dc.subjectRenal Dialysisen_US
dc.subjectRisk Factorsen_US
dc.titleSevere neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)en_US
dc.typeArticleen_US

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