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

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.

Anahtar Kelime: Alport syndrome cyclosporin A immunosuppressive therapy nephrotic syndrome RAAS inhibitors

Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık

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APA	OZDEMIR E, Gülhan B, Kurt Şükür E, Atayar E, ATAN R, Dursun I, Özçakar Z, Saygili S, Soylu A, Soylemezoglu O, Yilmaz A, Karabay Bayazit A, Eroglu F, Kasap-Demir B, YUKSEL S, Tabel Y, Ağbaş A, DÜZOVA A, Hayran M, Ozaltin F, Topaloglu R (2023). The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. , 456 - 468. 10.24953/turkjped.2022.735
Chicago	OZDEMIR EMINE GULSAH,Gülhan Bora,Kurt Şükür Eda Didem,Atayar Emine,ATAN RAZİYE,Dursun Ismail,Özçakar Z.Birsin,Saygili Seha Kamil,Soylu Alper,Soylemezoglu Oguz,Yilmaz Alev,Karabay Bayazit Aysun,Eroglu Fehime K.,Kasap-Demir Belde,YUKSEL SELCUK,Tabel Yılmaz,Ağbaş Ayşe,DÜZOVA ALI,Hayran Mutlu,Ozaltin Fatih,Topaloglu Rezan The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. (2023): 456 - 468. 10.24953/turkjped.2022.735
MLA	OZDEMIR EMINE GULSAH,Gülhan Bora,Kurt Şükür Eda Didem,Atayar Emine,ATAN RAZİYE,Dursun Ismail,Özçakar Z.Birsin,Saygili Seha Kamil,Soylu Alper,Soylemezoglu Oguz,Yilmaz Alev,Karabay Bayazit Aysun,Eroglu Fehime K.,Kasap-Demir Belde,YUKSEL SELCUK,Tabel Yılmaz,Ağbaş Ayşe,DÜZOVA ALI,Hayran Mutlu,Ozaltin Fatih,Topaloglu Rezan The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. , 2023, ss.456 - 468. 10.24953/turkjped.2022.735
AMA	OZDEMIR E,Gülhan B,Kurt Şükür E,Atayar E,ATAN R,Dursun I,Özçakar Z,Saygili S,Soylu A,Soylemezoglu O,Yilmaz A,Karabay Bayazit A,Eroglu F,Kasap-Demir B,YUKSEL S,Tabel Y,Ağbaş A,DÜZOVA A,Hayran M,Ozaltin F,Topaloglu R The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. . 2023; 456 - 468. 10.24953/turkjped.2022.735
Vancouver	OZDEMIR E,Gülhan B,Kurt Şükür E,Atayar E,ATAN R,Dursun I,Özçakar Z,Saygili S,Soylu A,Soylemezoglu O,Yilmaz A,Karabay Bayazit A,Eroglu F,Kasap-Demir B,YUKSEL S,Tabel Y,Ağbaş A,DÜZOVA A,Hayran M,Ozaltin F,Topaloglu R The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. . 2023; 456 - 468. 10.24953/turkjped.2022.735
IEEE	OZDEMIR E,Gülhan B,Kurt Şükür E,Atayar E,ATAN R,Dursun I,Özçakar Z,Saygili S,Soylu A,Soylemezoglu O,Yilmaz A,Karabay Bayazit A,Eroglu F,Kasap-Demir B,YUKSEL S,Tabel Y,Ağbaş A,DÜZOVA A,Hayran M,Ozaltin F,Topaloglu R "The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome." , ss.456 - 468, 2023. 10.24953/turkjped.2022.735
ISNAD	OZDEMIR, EMINE GULSAH vd. "The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome". (2023), 456-468. https://doi.org/10.24953/turkjped.2022.735

APA	OZDEMIR E, Gülhan B, Kurt Şükür E, Atayar E, ATAN R, Dursun I, Özçakar Z, Saygili S, Soylu A, Soylemezoglu O, Yilmaz A, Karabay Bayazit A, Eroglu F, Kasap-Demir B, YUKSEL S, Tabel Y, Ağbaş A, DÜZOVA A, Hayran M, Ozaltin F, Topaloglu R (2023). The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. Turkish Journal of Pediatrics, 65(3), 456 - 468. 10.24953/turkjped.2022.735
Chicago	OZDEMIR EMINE GULSAH,Gülhan Bora,Kurt Şükür Eda Didem,Atayar Emine,ATAN RAZİYE,Dursun Ismail,Özçakar Z.Birsin,Saygili Seha Kamil,Soylu Alper,Soylemezoglu Oguz,Yilmaz Alev,Karabay Bayazit Aysun,Eroglu Fehime K.,Kasap-Demir Belde,YUKSEL SELCUK,Tabel Yılmaz,Ağbaş Ayşe,DÜZOVA ALI,Hayran Mutlu,Ozaltin Fatih,Topaloglu Rezan The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. Turkish Journal of Pediatrics 65, no.3 (2023): 456 - 468. 10.24953/turkjped.2022.735
MLA	OZDEMIR EMINE GULSAH,Gülhan Bora,Kurt Şükür Eda Didem,Atayar Emine,ATAN RAZİYE,Dursun Ismail,Özçakar Z.Birsin,Saygili Seha Kamil,Soylu Alper,Soylemezoglu Oguz,Yilmaz Alev,Karabay Bayazit Aysun,Eroglu Fehime K.,Kasap-Demir Belde,YUKSEL SELCUK,Tabel Yılmaz,Ağbaş Ayşe,DÜZOVA ALI,Hayran Mutlu,Ozaltin Fatih,Topaloglu Rezan The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. Turkish Journal of Pediatrics, vol.65, no.3, 2023, ss.456 - 468. 10.24953/turkjped.2022.735
AMA	OZDEMIR E,Gülhan B,Kurt Şükür E,Atayar E,ATAN R,Dursun I,Özçakar Z,Saygili S,Soylu A,Soylemezoglu O,Yilmaz A,Karabay Bayazit A,Eroglu F,Kasap-Demir B,YUKSEL S,Tabel Y,Ağbaş A,DÜZOVA A,Hayran M,Ozaltin F,Topaloglu R The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. Turkish Journal of Pediatrics. 2023; 65(3): 456 - 468. 10.24953/turkjped.2022.735
Vancouver	OZDEMIR E,Gülhan B,Kurt Şükür E,Atayar E,ATAN R,Dursun I,Özçakar Z,Saygili S,Soylu A,Soylemezoglu O,Yilmaz A,Karabay Bayazit A,Eroglu F,Kasap-Demir B,YUKSEL S,Tabel Y,Ağbaş A,DÜZOVA A,Hayran M,Ozaltin F,Topaloglu R The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome. Turkish Journal of Pediatrics. 2023; 65(3): 456 - 468. 10.24953/turkjped.2022.735
IEEE	OZDEMIR E,Gülhan B,Kurt Şükür E,Atayar E,ATAN R,Dursun I,Özçakar Z,Saygili S,Soylu A,Soylemezoglu O,Yilmaz A,Karabay Bayazit A,Eroglu F,Kasap-Demir B,YUKSEL S,Tabel Y,Ağbaş A,DÜZOVA A,Hayran M,Ozaltin F,Topaloglu R "The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome." Turkish Journal of Pediatrics, 65, ss.456 - 468, 2023. 10.24953/turkjped.2022.735
ISNAD	OZDEMIR, EMINE GULSAH vd. "The outcomes of renin-angiotensin-aldosterone system inhibition and immunosuppressive therapy in children with X-linked Alport syndrome". Turkish Journal of Pediatrics 65/3 (2023), 456-468. https://doi.org/10.24953/turkjped.2022.735