Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)
Küçük Resim Yok
Tarih
2020
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Nature Research
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
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. © 2020, The Author(s).
Açıklama
Anahtar Kelimeler
adverse event, cohort analysis, disease exacerbation, female, hemodialysis, human, infant, kidney polycystic disease, male, nephrectomy, neurologic disease, newborn, postoperative complication, risk factor, Cohort Studies, Disease Progression, Female, Humans, Infant, Infant, Newborn, Male, Nephrectomy, Nervous System Diseases, Polycystic Kidney, Autosomal Recessive, Postoperative Complications, Renal Dialysis, Risk Factors
Kaynak
Scientific Reports
WoS Q Değeri
Scopus Q Değeri
Q1
Cilt
10
Sayı
1
