Maintenance Peritoneal Dialysis in Children With Autosomal Recessive Polycystic Kidney Disease: A Comparative Cohort Study of the International Pediatric Peritoneal Dialysis Network Registry

Akarkach, Abdelaziz; Burgmaier, Kathrin; Sander, Anja; Hooman, Nakysa; Sever, Lale; Cano, Francisco; Zambrano, Pedro; Bilge, İlmay; Flynn, Joseph T.; Yavaşcan, Önder; Vallés, Patricia G.; Munárriz, Reyner Loza; Patel, Hiren P.; Serdaroğlu, Erkin; Koch, Vera H.; Suarez, Ángela; Galanti, Monica; Celedon, Claudia Gonzalez; Rébori, Anabella; Kari, Jameela A.; Wong, Cynthia; Elenberg, Ewa; Rojas, Luisa Fernanda Quiroga; Warady, Bradley A.; Liebau, Max C.; Schaefer, Franz

doi:10.1053/j.ajkd.2019.10.009

Cited by 9 publications

(2 citation statements)

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“…Despite the challenges of fluid management in anephric infants, PD remains the preferred modality for ARPKD infants 11 . A recent analysis showed that technique survival in ARPKD is comparable to that observed in other early-onset kidney diseases, although PD in ARPKD may require smaller fill volumes and/or more cycles 27 . Our analysis emphasizes in particular the association of documented pronounced hypotensive episodes with severe neurological complications in ARPKD patients receiving early bilateral nephrectomies and underlines the recommendation of very tight blood pressure control for all anephric children on PD.…”

Section: Discussionmentioning

confidence: 92%

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

Burgmaier

Ariceta

Bald

et al. 2020

Sci Rep

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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.

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Section: Discussionmentioning

confidence: 92%

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

Burgmaier

Ariceta

Bald

et al. 2020

Sci Rep

Self Cite

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“…Concerns exist about the feasibility of peritoneal dialysis in ARPKD with enlarged kidneys. A recent study from the International Pediatric Peritoneal Dialysis Network (IPPN) registry therefore systematically compared peritoneal dialysis in children with ARPKD ( n = 79) with control groups suffering from congenital nephrotic syndrome ( n = 79) and CAKUT phenotypes ( n = 158) [ 52 ]. Patients were matched for age and time on dialysis.…”

Section: Perinatal and Early Aspects Of Clinical Presentationmentioning

confidence: 99%

Early clinical management of autosomal recessive polycystic kidney disease

Liebau

2021

Pediatr Nephrol

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Autosomal recessive polycystic kidney disease (ARPKD) is a rare but highly relevant disorder in pediatric nephrology. This genetic disease is mainly caused by variants in the PKHD1 gene and is characterized by fibrocystic hepatorenal phenotypes with major clinical variability. ARPKD frequently presents perinatally, and the management of perinatal and early disease symptoms may be challenging. This review discusses aspects of early manifestations in ARPKD and its clincial management with a special focus on kidney disease.

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Autosomal Recessive Polycystic Kidney Disease

Liebau

Guay‐Woodford²

2022

Pediatric Nephrology

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Maintenance Peritoneal Dialysis in Children With Autosomal Recessive Polycystic Kidney Disease: A Comparative Cohort Study of the International Pediatric Peritoneal Dialysis Network Registry

Cited by 9 publications

References 10 publications

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

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

Early clinical management of autosomal recessive polycystic kidney disease

Autosomal Recessive Polycystic Kidney Disease

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