İdiyopatik nefrotik sendromlu çocuklarda uzun dönem sonuçlar: tek merkez deneyimi

Year 2023, Volume: 48 Issue: 2, 697 - 705, 02.07.2023

Gönül Parmaksız

https://doi.org/10.17826/cumj.1243700

Abstract

Amaç: İdiyopatik nefrotik sendrom (İNS) çocuklarda yaygın bir glomeruler hastalıktır. Steroid tedavisinin yanıtına göre steroide hassas nefrotik sendrom (SHNS) veya steroid dirençli nefrotik sendrom (SDNS) olarak sınıflandırılır. SHNS hastaları steroid tedavisinde normal böbrek fonksiyonları ile tam remisyon elde ederken, SDNS hastaları bunun aksine olumlu yanıt vermez ve son dönem böbrek hastalığı gelişme riski yüksektir. Bu çalışmanın amacı İNS’li çocukların uzun dönem sonuçlarını değerlendirmektir.
Gereç ve Yöntem: Merkezimizde İNS tanısı alan çocukların tıbbi kayıtları geriye dönük olarak değerlendirildi. Demografik bilgileri, laboratuvar bulguları, tedavi yanıtları ve klinik seyri analiz edildi.
Bulgular: Çalışmaya ortalama yaşı 11.1±4.1 (3.5-18) olan 91 çocuk (%64, erkek) dahil edildi. Ortalama tanı yaşı 5.2±3.8 yıl (1-16.2) ve ortalama izlem süreleri 5.7±2.8 yıl (2-12) idi. Hastaların 68 (%75)’i steroide hassas, 23 (%25)’ü steroide dirençli idi. Steroide hassas olanların 18 (%31)’i steroide bağımlı, 12 (%20)’si sık tekrarlayan, 29 (%49)’u seyrek tekrarlayan idi. Böbrek biyopsi 29 (%32) hastaya yapıldı, %59’unun histopatolojik bulgusu fokal segmental glomeruloskleroz idi. Tüm hastaların %94’ünde tam remisyon sağlandı, SHNS hastalarının tamamı, SDNS hastaların %74’ü tam remisyonda idi. SDNS hastalarının %9’unda son dönem böbrek yetmezliği gelişti.
Sonuç: Steroid cevabı İNS’da son dönem böbrek yetmezliğine ilerlemeyi azalttığından iyi prognoz göstergesidir.

Keywords

Nefrotik sendrom, steroid yanıtı, çocuklar

Project Number

KA22/520

Long-term outcomes in children with idiopathic nephrotic syndrome: a single center experience

Abstract

Purpose: Idiopathic nephrotic syndrome (INS) is a common glomerular disease observed in children. Depending on their response to steroids, patients can be classified either as having steroid-sensitive nephrotic syndrome (SSNS) or steroid-resistant nephrotic syndrome (SRNS). Whereas SSNS patients respond favorably to steroid treatment and achieve a complete remission with normal renal functions, SRNS patients do not, and are at high risk of developing end-stage renal diseases (ESRD). The aim of this study was to analyze the long-term outcomes of patients with INS.
Materials and Methods: In our medical center, records of children diagnosed with INS were evaluated. Demographic information, laboratory results, response to treatment, and clinical progression were analyzed.
Result: Ninety-one children (64% male) with a mean age of 11.1±4.1 years (3.5-18) were included in the patient cohort, with a mean age of diagnosis of 5.2±3.8 years (1-16.2) and a mean follow-up period of 5.7±2.8 years (2-12). Sixty-eight (75%) patients had SSNS, and 23 (25%) patients had SRNS. Among the SSNS patients, 18 (31%) were steroid-dependent, 12 (20%) were frequently relapsing, and 29 (49%) were infrequently relapsing. Renal biopsy was performed on 29 (32%) patients, 59% had focal segmental glomerulosclerosis. The complete remission rate was 94% for all patients, with 100% for SSNS and 74% for SRNS. ESRD was developed for 9% of patients with SRNS.
Conclusion: The response to steroid treatment serves as a valuable prognostic indicator for INS as it plays a pivotal role in mitigating the risk of progression toward end-stage renal failure.

Keywords

Nephrotic syndrome, steroid response, children

Supporting Institution

Baskent University Research Fund

Project Number

KA22/520

Thanks

I would like to thank Professor Dr. Zekiye Aytül Noyan at the Pediatric Nephrology Department for Dr. Turgut Noyan Research and Training Center of Başkent University.

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