Demographic and clinical characteristics of children with autosomal dominant polycystic kidney disease: a single center experience

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dc.authoridBerdeli, afig/0000-0002-4791-8367
dc.authoridAlparslan, Caner/0000-0002-7046-8907
dc.authoridYavascan, Onder/0000-0002-3582-5075
dc.authoridArslansoyu Camlar, Secil/0000-0002-2402-0722
dc.authorscopusid7801347643
dc.authorscopusid8294173700
dc.authorscopusid57194828196
dc.authorscopusid47461055000
dc.authorscopusid57223274691
dc.authorscopusid35114625600
dc.authorscopusid26428015700
dc.authorwosidsoyaltın, eren/GSD-7757-2022
dc.authorwosidAlparslan, Caner/E-6832-2015
dc.contributor.authorDemir, Belde Kasap
dc.contributor.authorMutlubas, Fatma
dc.contributor.authorSoyaltin, Eren
dc.contributor.authorAlparslan, Caner
dc.contributor.authorArya, Merve
dc.contributor.authorAlaygut, Demet
dc.contributor.authorCamlar, Secil Arslansoyu
dc.date.accessioned2023-01-12T20:16:07Z
dc.date.available2023-01-12T20:16:07Z
dc.date.issued2021
dc.departmentN/A/Departmenten_US
dc.description.abstractBackground/aim: In children with autosomal dominant polycystic kidney disease (ADPKD), clinical manifestations range from severe neonatal presentation to renal cysts found by chance. We aimed to evaluate demographic, clinical, laboratory findings, and genetic analysis of children with ADPKD. Materials and methods: We evaluated children diagnosed with ADPKD between January 2006 and January 2019. The diagnosis was established by family history, ultrasound findings, and/or genetic analysis. The demographic, clinical, and laboratory findings were evaluated retrospectively. Patients < 10 years and >= 10 years at the time of diagnosis were divided into 2 groups and parameters were compared between the groups. Results: There were 41 children (M/F: 18/23) diagnosed with ADPKD. The mean age at diagnosis was 7.2 +/- 5.1 (0.6-16.9) years and the follow-up duration was 59.34 perpendicular to 40.56 (8-198) months. Five patients (12%) were diagnosed as very early onset ADPKD. All patients had a positive family history. Genetic analysis was performed in 29 patients (PKD1 mutations in 21, PKD2 mutations in 1, no mutation in 3). Cysts were bilateral in 35 (85%) of the patients. Only one patient had hepatic cysts. No valvular defect was defined in 12 patients detected. Only 1 patient had hypertension. None of them had chronic kidney disease. No difference could be demonstrated in sex, laterality of the cysts, maximum cyst diameter, cyst or kidney enlargement, follow-up duration, or GFR at last visit between Groups 1 and 2. Conclusion: The majority of children with ADPKD had preserved renal functions and slight cyst enlargement during their follow-up. However, they may have different renal problems deserving closed follow-up.en_US
dc.identifier.doi10.3906/sag-2009-79
dc.identifier.endpage777en_US
dc.identifier.issn1300-0144
dc.identifier.issn1303-6165
dc.identifier.issue2en_US
dc.identifier.pmid33315352en_US
dc.identifier.scopus2-s2.0-85105491384en_US
dc.identifier.scopusqualityQ3en_US
dc.identifier.startpage772en_US
dc.identifier.trdizinid484695en_US
dc.identifier.urihttps://doi.org/10.3906/sag-2009-79
dc.identifier.urihttps://search.trdizin.gov.tr/tr/yayin/detay/484695
dc.identifier.urihttps://hdl.handle.net/11454/78651
dc.identifier.volume51en_US
dc.identifier.wosWOS:000646281600050en_US
dc.identifier.wosqualityQ3en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakTR-Dizinen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherTubitak Scientific & Technical Research Council Turkeyen_US
dc.relation.ispartofTurkish Journal of Medical Sciencesen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectAutosomal dominant polycystic kidney diseaseen_US
dc.subjectchildrenen_US
dc.subjectGlomerular Hyperfiltrationen_US
dc.subjectTolvaptanen_US
dc.subjectVolumeen_US
dc.subjectRisken_US
dc.titleDemographic and clinical characteristics of children with autosomal dominant polycystic kidney disease: a single center experienceen_US
dc.typeArticleen_US

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