Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD).
Journal
Scientific reports
ISSN: 2045-2322
Titre abrégé: Sci Rep
Pays: England
ID NLM: 101563288
Informations de publication
Date de publication:
29 09 2020
29 09 2020
Historique:
received:
11
04
2020
accepted:
24
08
2020
entrez:
30
9
2020
pubmed:
1
10
2020
medline:
14
1
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 32994492
doi: 10.1038/s41598-020-71956-1
pii: 10.1038/s41598-020-71956-1
pmc: PMC7525474
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
16025Investigateurs
Loai Akram Eid
(LA)
Klaus Arbeiter
(K)
Nadejda Ranguelov
(N)
Laure Collard
(L)
Aurélie De Mul
(A)
Markus Feldkoetter
(M)
Tomas Seeman
(T)
Julia Thumfart
(J)
Franziska Grundmann
(F)
Matthias Galiano
(M)
Björn Buchholz
(B)
Rainer Buescher
(R)
Karsten Häffner
(K)
Oliver Gross
(O)
Wanja Bernhardt
(W)
Anke Doyon
(A)
Michael Henn
(M)
Jan Halbritter
(J)
Ute Derichs
(U)
Günter Klaus
(G)
Bärbel Lange-Sperandio
(B)
Barbara Uetz
(B)
Marcus Benz
(M)
Andrea Titieni
(A)
Hagen Staude
(H)
Heinz E Leichter
(HE)
Neveen A Soliman
(NA)
Luis Enrique Lara
(LE)
Francisco de la Cerda Ojeda
(F)
Jerome Harambat
(J)
Bruno Ranchin
(B)
Marc Fila
(M)
Claire Dossier
(C)
Olivia Boyer
(O)
Matko Marlais
(M)
Stella Stabouli
(S)
Nakysa Hooman
(N)
Francesca Mencarelli
(F)
William Morello
(W)
Germana Longo
(G)
Francesco Emma
(F)
Dovile Ruzgiene
(D)
Anna Wasilewska
(A)
Irena Balasz-Chmielewska
(I)
Monika Miklaszewska
(M)
Malgorzata Stanczyk
(M)
Przemyslaw Sikora
(P)
Mieczyslaw Litwin
(M)
Aurelia Morawiec-Knysak
(A)
Ana Teixeira
(A)
Gordana Milosevski-Lomic
(G)
Larisa Prikhodina
(L)
Rina Rus
(R)
Houweyda Jilani
(H)
Engin Melek
(E)
Ali Duzova
(A)
Alper Soylu
(A)
Cengiz Candan
(C)
Lale Sever
(L)
Alev Yilmaz
(A)
Neslihan Cicek
(N)
Nurver Akinci
(N)
Sevgi Mir
(S)
Ismail Dursun
(I)
Yilmaz Tabel
(Y)
Hulya Nalcacioglu
(H)
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