Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)
Open Access
- 29 September 2020
- journal article
- research article
- Published by Springer Science and Business Media LLC in Scientific Reports
- Vol. 10 (1), 1-12
- https://doi.org/10.1038/s41598-020-71956-1
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.Funding Information
- Universität zu Köln
- Marga und Walter Boll-Stiftung
- Bundesministerium für Bildung, Wissenschaft und Forschung (01GM1515, 01GM1515)
- Bundesministerium für Bildung und Forschung (01GM1903)
- European Society for Paediatric Nephrology
- Projekt DEAL
This publication has 34 references indexed in Scilit:
- A systematic review and meta-analysis to revise the Fenton growth chart for preterm infantsBMC Pediatrics, 2013
- Clinical practice recommendations for the care of infants with stage 5 chronic kidney disease (CKD5)Pediatric Nephrology, 2012
- Refractory hypotension after bilateral nephrectomies in a Denys–Drash patient with phenylketonuriaPediatric Nephrology, 2012
- Maturation of Heart Rate and Blood Pressure Variability during Sleep in Term-Born InfantsSleep, 2012
- Maturation-related changes in the pattern of renal sympathetic nerve activity from fetal life to adulthoodExperimental Physiology, 2010
- Postnatal development of baroreflex sensitivity in infancyJournal Of Physiology-London, 2010
- Aggressive respiratory support and unilateral nephrectomy for infants with severe perinatal autosomal recessive polycystic kidney diseaseThe Journal of Pediatrics, 1995
- Age-Related Changes in the Renin-Aldosterone SystemDrugs & Aging, 1993
- Polycystic kidney disease in the first year of lifeThe Journal of Pediatrics, 1987
- Potassium-renin-aldosterone relationships during the first year of lifeThe Journal of Pediatrics, 1977