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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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