Burgmaier, Kathrin, Kunzmann, Kevin, Ariceta, Gema, Bergmann, Carsten, Buescher, Anja Katrin, Burgmaier, Mathias, Dursun, Ismail, Duzova, Ali, Eid, Loai, Erger, Florian ORCID: 0000-0002-2768-1702, Feldkoetter, Markus, Galiano, Matthias, Gessner, Michaela, Goebel, Heike, Gokce, Ibrahim ORCID: 0000-0002-6896-5162, Haffner, Dieter, Hooman, Nakysa, Hoppe, Bernd, Jankauskiene, Augustina, Klaus, Guenter, Koenig, Jens, Litwin, Mieczyslaw ORCID: 0000-0002-5241-2483, Massella, Laura, Mekahli, Djalila ORCID: 0000-0003-0954-6088, Melek, Engin, Mir, Sevgi, Pape, Lars, Prikhodina, Larisa ORCID: 0000-0002-0702-4932, Ranchin, Bruno, Schild, Raphael, Seeman, Tomas, Sever, Late, Shroff, Rukshana, Soliman, Neveen A., Stabouli, Stella, Stanczyk, Malgorzata ORCID: 0000-0001-6613-1642, Tabel, Yilmaz, Taranta-Janusz, Katarzyna ORCID: 0000-0002-8762-8866, Testa, Sara, Thumfart, Julia ORCID: 0000-0003-1162-5295, Topaloglu, Rezan, Weber, Lutz Thorsten, Wicher, Dorota ORCID: 0000-0002-8360-0006, Wuehl, Elke, Wygoda, Simone, Yilmaz, Alev, Zachwieja, Katarzyna, Zagozdzon, Ilona, Zerres, Klaus, Doetsch, Joerg, Schaefer, Franz and Liebau, Max Christoph ORCID: 0000-0003-0494-9080 (2018). Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease. J. Pediatr., 199. S. 22 - 35. NEW YORK: MOSBY-ELSEVIER. ISSN 1097-6833

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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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Burgmaier, KathrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kunzmann, KevinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Ariceta, GemaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bergmann, CarstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Buescher, Anja KatrinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Burgmaier, MathiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dursun, IsmailUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Duzova, AliUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Eid, LoaiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Erger, FlorianUNSPECIFIEDorcid.org/0000-0002-2768-1702UNSPECIFIED
Feldkoetter, MarkusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Galiano, MatthiasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gessner, MichaelaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Goebel, HeikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gokce, IbrahimUNSPECIFIEDorcid.org/0000-0002-6896-5162UNSPECIFIED
Haffner, DieterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hooman, NakysaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hoppe, BerndUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jankauskiene, AugustinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klaus, GuenterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koenig, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Litwin, MieczyslawUNSPECIFIEDorcid.org/0000-0002-5241-2483UNSPECIFIED
Massella, LauraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mekahli, DjalilaUNSPECIFIEDorcid.org/0000-0003-0954-6088UNSPECIFIED
Melek, EnginUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mir, SevgiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pape, LarsUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Prikhodina, LarisaUNSPECIFIEDorcid.org/0000-0002-0702-4932UNSPECIFIED
Ranchin, BrunoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schild, RaphaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Seeman, TomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sever, LateUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shroff, RukshanaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Soliman, Neveen A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stabouli, StellaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stanczyk, MalgorzataUNSPECIFIEDorcid.org/0000-0001-6613-1642UNSPECIFIED
Tabel, YilmazUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Taranta-Janusz, KatarzynaUNSPECIFIEDorcid.org/0000-0002-8762-8866UNSPECIFIED
Testa, SaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thumfart, JuliaUNSPECIFIEDorcid.org/0000-0003-1162-5295UNSPECIFIED
Topaloglu, RezanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Weber, Lutz ThorstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wicher, DorotaUNSPECIFIEDorcid.org/0000-0002-8360-0006UNSPECIFIED
Wuehl, ElkeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wygoda, SimoneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Yilmaz, AlevUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zachwieja, KatarzynaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zagozdzon, IlonaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zerres, KlausUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doetsch, JoergUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schaefer, FranzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Liebau, Max ChristophUNSPECIFIEDorcid.org/0000-0003-0494-9080UNSPECIFIED
URN: urn:nbn:de:hbz:38-177293
DOI: 10.1016/j.jpeds.2018.03.052
Journal or Publication Title: J. Pediatr.
Volume: 199
Page Range: S. 22 - 35
Date: 2018
Publisher: MOSBY-ELSEVIER
Place of Publication: NEW YORK
ISSN: 1097-6833
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
GENOTYPE-PHENOTYPE CORRELATIONS; PKHD1 MUTATIONS; CLINICAL-EXPERIENCE; ARPKD; PROTEIN; ENCODES; GENETICSMultiple languages
PediatricsMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/17729

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