Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

ESCAPE Study Group, GPN Study Group, ARegPKD consortium

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Objective: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. Study design: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. Results: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. Conclusions: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.

Original languageEnglish
Pages (from-to)22-28.e6
JournalJournal of Pediatrics
Volume199
DOIs
Publication statusPublished - 2018

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Autosomal Recessive Polycystic Kidney
Dialysis
Oligohydramnios
Apgar Score
Kidney
Counseling
Respiration
Prenatal Diagnosis
Registries
Dependency (Psychology)
Cysts
Regression Analysis

Keywords

  • ciliopathy
  • oligohydramnios
  • PKHD1
  • renal replacement therapy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease. / ESCAPE Study Group; GPN Study Group; ARegPKD consortium.

In: Journal of Pediatrics, Vol. 199, 2018, p. 22-28.e6.

Research output: Contribution to journalArticle

ESCAPE Study Group ; GPN Study Group ; ARegPKD consortium. / Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease. In: Journal of Pediatrics. 2018 ; Vol. 199. pp. 22-28.e6.
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abstract = "Objective: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. Study design: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. Results: Thirty-six out of 385 children (9.4{\%}) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5{\%} (95{\%} CI, 0.5{\%}-4.1{\%}) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3{\%} (95{\%} CI, 22.2{\%}-44.5{\%}) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. Conclusions: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.",
keywords = "ciliopathy, oligohydramnios, PKHD1, renal replacement therapy",
author = "{ESCAPE Study Group} and {GPN Study Group} and {ARegPKD consortium} and Kathrin Burgmaier and Kevin Kunzmann and Gema Ariceta and Carsten Bergmann and Buescher, {Anja Katrin} and Mathias Burgmaier and Ismail Dursun and Ali Duzova and Loai Eid and Florian Erger and Markus Feldkoetter and Matthias Galiano and Michaela Ge{\ss}ner and Heike Goebel and Ibrahim Gokce and Dieter Haffner and Nakysa Hooman and Bernd Hoppe and Augustina Jankauskiene and Guenter Klaus and Jens K{\"o}nig and Mieczyslaw Litwin and Laura Massella and Djalila Mekahli and Engin Melek and Sevgi Mir and Lars Pape and Larisa Prikhodina and Bruno Ranchin and Raphael Schild and Tomas Seeman and Lale Sever and Rukshana Shroff and Soliman, {Neveen A.} and Stella Stabouli and Malgorzata Stanczyk and Yilmaz Tabel and Katarzyna Taranta-Janusz and Sara Testa and Julia Thumfart and Rezan Topaloglu and Weber, {Lutz Thorsten} and Dorota Wicher and Elke W{\"u}hl and Simone Wygoda and Alev Yilmaz and Katarzyna Zachwieja and Ilona Zagozdzon and Klaus Zerres and Francesco Emma",
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language = "English",
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T1 - Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease

AU - ESCAPE Study Group

AU - GPN Study Group

AU - ARegPKD consortium

AU - Burgmaier, Kathrin

AU - Kunzmann, Kevin

AU - Ariceta, Gema

AU - Bergmann, Carsten

AU - Buescher, Anja Katrin

AU - Burgmaier, Mathias

AU - Dursun, Ismail

AU - Duzova, Ali

AU - Eid, Loai

AU - Erger, Florian

AU - Feldkoetter, Markus

AU - Galiano, Matthias

AU - Geßner, Michaela

AU - Goebel, Heike

AU - Gokce, Ibrahim

AU - Haffner, Dieter

AU - Hooman, Nakysa

AU - Hoppe, Bernd

AU - Jankauskiene, Augustina

AU - Klaus, Guenter

AU - König, Jens

AU - Litwin, Mieczyslaw

AU - Massella, Laura

AU - Mekahli, Djalila

AU - Melek, Engin

AU - Mir, Sevgi

AU - Pape, Lars

AU - Prikhodina, Larisa

AU - Ranchin, Bruno

AU - Schild, Raphael

AU - Seeman, Tomas

AU - Sever, Lale

AU - Shroff, Rukshana

AU - Soliman, Neveen A.

AU - Stabouli, Stella

AU - Stanczyk, Malgorzata

AU - Tabel, Yilmaz

AU - Taranta-Janusz, Katarzyna

AU - Testa, Sara

AU - Thumfart, Julia

AU - Topaloglu, Rezan

AU - Weber, Lutz Thorsten

AU - Wicher, Dorota

AU - Wühl, Elke

AU - Wygoda, Simone

AU - Yilmaz, Alev

AU - Zachwieja, Katarzyna

AU - Zagozdzon, Ilona

AU - Zerres, Klaus

AU - Emma, Francesco

PY - 2018

Y1 - 2018

N2 - Objective: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. Study design: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. Results: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. Conclusions: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.

AB - Objective: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis. Study design: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life. Results: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys. Conclusions: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.

KW - ciliopathy

KW - oligohydramnios

KW - PKHD1

KW - renal replacement therapy

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