Please use this identifier to cite or link to this item: https://hdl.handle.net/11499/52098
Title: The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome
Authors: Özdemir, Emine Gülşah
Gülhan, Bora
Kurt Şükür, Eda Dideme
Atayar, Emine
Atan, Raziye
Dursun, İsmail
Özçakar, Zeynep Birsin
Saygılı, Seha Kamil
Soylu, Alper
Söylemezoğlu, Oğuz
Yılmaz, Alev
Karabay, Bayazıt
Kara Eroğlu, Fehime
Demir, Belde
Yüksel, Selçuk
Yılmaz, Tabel
Ağbaş, Ayşe
Düzova, Ali
Hayran, Mutlu
Özaltın, Fatih
Topaloğlu, Rezan
Keywords: Alport syndrome
cyclosporin A
immunosuppressive therapy
nephrotic syndrome
RAAS inhibitors
angiotensin receptor antagonist
creatinine
dipeptidyl carboxypeptidase inhibitor
drug
mycophenolate mofetil
RAAS inhibitor
serum albumin
steroid
tacrolimus
unclassified drug
dipeptidyl carboxypeptidase inhibitor
Alport syndrome
Article
child
disease exacerbation
edema
electron microscopy
estimated glomerular filtration rate
female
focal glomerulosclerosis
follow up
genetic analysis
genetic variation
hearing impairment
hematuria
histopathology
human
hyperlipidemia
hypoalbuminemia
immunosuppressive treatment
kidney biopsy
kidney failure
major clinical study
male
missense mutation
monotherapy
nephrotic syndrome
outcome assessment
protein creatinine ratio
proteinuria
questionnaire
renin angiotensin aldosterone system
survival
survival analysis
survival rate
chronic kidney failure
clinical trial
genetics
immunosuppressive treatment
multicenter study
nephritis
physiology
proteinuria
renin angiotensin aldosterone system
retrospective study
Angiotensin-Converting Enzyme Inhibitors
Child
Humans
Immunosuppression Therapy
Male
Nephritis, Hereditary
Proteinuria
Renal Insufficiency, Chronic
Renin-Angiotensin System
Retrospective Studies
Publisher: Turkish National Pediatric Society
Abstract: Background. 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, p=0.003). 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 up more carefully considering the risk of early progression to CKD. © 2023, Turkish National Pediatric Society. All rights reserved.
URI: https://hdl.handle.net/11499/52098
https://doi.org/10.24953/turkjped.2022.735
https://search.trdizin.gov.tr/yayin/detay/1186276
ISSN: 0041-4301
Appears in Collections:PubMed İndeksli Yayınlar Koleksiyonu / PubMed Indexed Publications Collection
Scopus İndeksli Yayınlar Koleksiyonu / Scopus Indexed Publications Collection
Tıp Fakültesi Koleksiyonu
TR Dizin İndeksli Yayınlar Koleksiyonu / TR Dizin Indexed Publications Collection
WoS İndeksli Yayınlar Koleksiyonu / WoS Indexed Publications Collection

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