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

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dc.authoridErger, Florian/0000-0002-2768-1702
dc.authoridLiebau, Max Christoph/0000-0003-0494-9080
dc.authoridSikora, Przemyslaw/0000-0002-5698-6863
dc.authoridJankauskiene, Augustina/0000-0001-7767-2102
dc.authoridPape, Lars/0000-0002-3635-6418
dc.authoridWicher, Dorota/0000-0002-8360-0006
dc.authoridShroff, Rukshana/0000-0001-8501-1072
dc.authorwosidThumfart, Julia/T-7828-2017
dc.authorwosidErger, Florian/I-4298-2019
dc.authorwosidLiebau, Max Christoph/JCP-4310-2023
dc.authorwosidBAYAZIT, AYSUN K/E-8696-2018
dc.authorwosidSikora, Przemyslaw/ABE-1814-2021
dc.authorwosidDursun, Ismail/AAW-7097-2020
dc.authorwosidJankauskiene, Augustina/AFQ-3737-2022
dc.contributor.authorBurgmaier, Kathrin
dc.contributor.authorKunzmann, Kevin
dc.contributor.authorAriceta, Gema
dc.contributor.authorBergmann, Carsten
dc.contributor.authorBuescher, Anja Katrin
dc.contributor.authorBurgmaier, Mathias
dc.contributor.authorDursun, Ismail
dc.date.accessioned2024-08-04T20:44:28Z
dc.date.available2024-08-04T20:44:28Z
dc.date.issued2018
dc.departmentİnönü Üniversitesien_US
dc.description.abstractObjective 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.en_US
dc.description.sponsorshipGerman Society for Pediatric Nephrology (GPN); ESCAPE Network; GPN; European Society for Paediatric Nephrology (ESPN); German PKD foundation; Koeln Fortune program; GEROK program of the Medical Faculty of University of Cologne; Marga and Walter Boll-Foundation; German Federal Ministry of Research and Education (BMBF) [01GM1515, 01KN1106]; Koeln Fortune program of the Medical Faculty of University of Cologne; Deutsche Forschungsgemeinschaft (DFG) Collaborative Research Centre (SFB) [KIDGEM 1140]; Egyptian Group for Orphan Renal Diseases (EGORD)en_US
dc.description.sponsorshipSupported by the German Society for Pediatric Nephrology (GPN) and the ESCAPE Network. M.C.L. received grant support from GPN, the European Society for Paediatric Nephrology (ESPN), the German PKD foundation, the Koeln Fortune program, the GEROK program of the Medical Faculty of University of Cologne, and the Marga and Walter Boll-Foundation. C.B., D.H., J.K., F.S., and M.C.L. are supported by the the German Federal Ministry of Research and Education (BMBF grant 01GM1515). The Pediatric Study Center Cologne was supported by the German Federal Ministry of Research and Education (BMBF grant 01KN1106). K.B. received support from the Koeln Fortune program of the Medical Faculty of University of Cologne. C.B. receives support from the Deutsche Forschungsgemeinschaft (DFG) Collaborative Research Centre (SFB) KIDGEM 1140. N.S. receives support from the Egyptian Group for Orphan Renal Diseases (EGORD). The other authors declare no conflicts of interest.en_US
dc.identifier.doi10.1016/j.jpeds.2018.03.052
dc.identifier.endpage+en_US
dc.identifier.issn0022-3476
dc.identifier.issn1097-6833
dc.identifier.pmid29753540en_US
dc.identifier.scopus2-s2.0-85046668768en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage22en_US
dc.identifier.urihttps://doi.org/10.1016/j.jpeds.2018.03.052
dc.identifier.urihttps://hdl.handle.net/11616/98275
dc.identifier.volume199en_US
dc.identifier.wosWOS:000439478200007en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherMosby-Elsevieren_US
dc.relation.ispartofJournal of Pediatricsen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectGenotype-Phenotype Correlationsen_US
dc.subjectPkhd1 Mutationsen_US
dc.subjectClinical-Experienceen_US
dc.subjectArpkden_US
dc.subjectProteinen_US
dc.subjectEncodesen_US
dc.subjectGeneticsen_US
dc.titleRisk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Diseaseen_US
dc.typeArticleen_US

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