TY  - JOUR
TI  - The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome
AB  - 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.
AU  - Dursun, Ismail
AU  - Gülhan, Bora
AU  - Kurt Şükür, Eda Didem
AU  - ATAN, RAZİYE
AU  - Özçakar, Z.Birsin
AU  - Soylu, Alper
AU  - Karabay Bayazit, Aysun
AU  - Eroglu, Fehime K.
AU  - YUKSEL, SELCUK
AU  - Tabel, Yılmaz
AU  - Ağbaş, Ayşe
AU  - DÜZOVA, ALI
AU  - Hayran, Mutlu
AU  - Topaloglu, Rezan
AU  - Ozaltin, Fatih
AU  - Saygili, Seha Kamil
AU  - Kasap-Demir, Belde
AU  - OZDEMIR, EMINE GULSAH
AU  - Atayar, Emine
AU  - Soylemezoglu, Oguz
AU  - Yilmaz, Alev
DO  - 10.24953/turkjped.2022.735
PY  - 2023
JO  - Turkish Journal of Pediatrics
VL  - 65
IS  - 3
SN  - 0041-4301
SP  - 456
EP  - 468
DB  - TRDizin
UR  - http://search/yayin/detay/1186276
ER  -