en
Scientific article
Open access
English

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

CollaboratorsDe Mul, Aurélie
Published inScientific Reports, vol. 10, no. 1, 16025
Publication date2020
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.

Keywords
  • Cohort Studies
  • Disease Progression
  • Female
  • Humans
  • Infant
  • Infant
  • Newborn
  • Male
  • Nephrectomy/adverse effects
  • Nervous System Diseases/epidemiology/etiology
  • Polycystic Kidney
  • Autosomal Recessive/surgery
  • Postoperative Complications/epidemiology/etiology
  • Renal Dialysis/statistics & numerical data
  • Risk Factors
Citation (ISO format)
BURGMAIER, Kathrin et al. Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD). In: Scientific Reports, 2020, vol. 10, n° 1, p. 16025. doi: 10.1038/s41598-020-71956-1
Main files (1)
Article (Published version)
Identifiers
ISSN of the journal2045-2322
136views
29downloads

Technical informations

Creation08/05/2021 10:10:00 AM
First validation08/05/2021 10:10:00 AM
Update time03/16/2023 1:54:52 AM
Status update03/16/2023 1:54:49 AM
Last indexation02/12/2024 12:15:46 PM
All rights reserved by Archive ouverte UNIGE and the University of GenevaunigeBlack