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 language | English |
|---|---|
| Pages (from-to) | 22-28.e6 |
| Journal | Journal of Pediatrics |
| Volume | 199 |
| DOIs | |
| Publication status | Published - 2018 |
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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 journal › Article
}
TY - JOUR
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
UR - http://www.scopus.com/inward/record.url?scp=85046668768&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85046668768&partnerID=8YFLogxK
U2 - 10.1016/j.jpeds.2018.03.052
DO - 10.1016/j.jpeds.2018.03.052
M3 - Article
AN - SCOPUS:85046668768
VL - 199
SP - 22-28.e6
JO - Journal of Pediatrics
JF - Journal of Pediatrics
SN - 0022-3476
ER -