The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome

Basit Öğe Kaydı
dc.authoridDuzova, Ali/0000-0002-4365-2995
dc.authoridYUKSEL, SELCUK/0000-0001-9415-1640
dc.authoridAğbaş, Ayşe/0000-0002-3658-8622
dc.authoridÖzçakar, Zeynep Birsin/0000-0002-6376-9189
dc.authoridSaygili, Seha Kamil/0000-0002-2424-6959
dc.authoridOZDEMIR, EMINE GULSAH/0000-0002-0015-5665
dc.authorwosidDuzova, Ali/AAB-7692-2021
dc.authorwosidYUKSEL, SELCUK/C-5473-2015
dc.authorwosidDursun, Ismail/AAW-7097-2020
dc.authorwosidAğbaş, Ayşe/AAH-3650-2019
dc.authorwosidÖzçakar, Zeynep Birsin/AAG-1760-2020
dc.authorwosidBAYAZIT, AYSUN K/E-8696-2018
dc.authorwosidSaygili, Seha Kamil/GLU-6742-2022
dc.contributor.authorOzdemir, Gulsah
dc.contributor.authorGulhan, Bora
dc.contributor.authorSukur, Eda Didem Kurt
dc.contributor.authorAtayar, Emine
dc.contributor.authorAtan, Raziye
dc.contributor.authorDursun, Ismail
dc.contributor.authorOzcakar, Zeynep Birsin
dc.date.accessioned2024-08-04T20:10:21Z
dc.date.available2024-08-04T20:10:21Z
dc.date.issued2023
dc.departmentİnönü Üniversitesien_US
dc.description53rd Annual Meeting of European-Society-for-Pediatric-Nephrology (ESPN) -- SEP 16-19, 2021 -- Amsterdam, NETHERLANDSen_US
dc.description.abstractBackground. Alport syndrome (AS) is characterized by progressive kidney disease. There is increasing evidence that renin-angiotensin-aldosterone system (RAAS) inhibition delays chronic kidney disease (CKD) while the effectiveness of immunosuppressive (IS) therapy in AS is still uncertain. In this study, we aimed to analyze the outcomes of pediatric patients with X-linked AS (XLAS) who received RAAS inhibitors and IS therapy.Methods. Seventy-four children with XLAS were included in this multicenter study. Demographic features, clinical and laboratory data, treatments, histopathological examinations, and genetic analyses were analyzed retrospectively.Results. Among 74 children, 52 (70.2%) received RAAS inhibitors, 11 (14.9%) received RAAS inhibitors and IS, and 11 (14.9%) were followed up without treatment. During follow-up, glomerular filtration rate (GFR) decreased <60 ml/min/1.73 m2 in 7 (9.5%) of 74 patients (M/F=6/1). In male patients with XLAS, kidney survival was not different between RAAS and RAAS+IS groups (p=0.42). The rate of progression to CKD was significantly higher in patients with nephrotic range proteinuria and nephrotic syndrome (NS), respectively (p=0.006, p=0.05). The median age at the onset of RAAS inhibitors was significantly higher in male patients who progressed to CKD (13.9 vs 8.1 years,Conclusions. RAAS inhibitors have beneficial effects on proteinuria and early initiation of therapy may delay the progression to CKD in children with XLAS. There was no significant difference between the RAAS and RAAS+IS groups in kidney survival. AS patients presenting with NS or nephrotic range proteinuria should be followed upen_US
dc.description.sponsorshipEuropean Soc Pediat Nephrolen_US
dc.identifier.doi10.24953/turkjped.2022.735
dc.identifier.endpage468en_US
dc.identifier.issn0041-4301
dc.identifier.issue3en_US
dc.identifier.pmid37395965en_US
dc.identifier.scopus2-s2.0-85164209626en_US
dc.identifier.scopusqualityQ3en_US
dc.identifier.startpage456en_US
dc.identifier.trdizinid1186276en_US
dc.identifier.urihttps://doi.org/10.24953/turkjped.2022.735
dc.identifier.urihttps://search.trdizin.gov.tr/yayin/detay/1186276
dc.identifier.urihttps://hdl.handle.net/11616/92727
dc.identifier.volume65en_US
dc.identifier.wosWOS:001025408400011en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakTR-Dizinen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherTurkish J Pediatricsen_US
dc.relation.ispartofTurkish Journal of Pediatricsen_US
dc.relation.publicationcategoryKonferans Öğesi - Uluslararası - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectAlport syndromeen_US
dc.subjectcyclosporin Aen_US
dc.subjectimmunosuppressive therapyen_US
dc.subjectnephrotic syndromeen_US
dc.subjectRAAS inhibitorsen_US
dc.titleThe outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndromeen_US
dc.typeConference Objecten_US

Dosyalar

Koleksiyon

WoS İndeksli Yayınlar Koleksiyonu
PubMed İndeksli Yayın Koleksiyonu
Scopus İndeksli Yayınlar Koleksiyonu
TR-Dizin İndeksli Yayınlar Koleksiyonu