Deniz KARAKAYA, Fatma YAZILITAŞ, Evrim KARGIN ÇAKICI, Tülin GÜNGÖR, Evra ÇELİKKAYA, Mehmet BÜLBÜL

Çocukluk Çağı Fokal Segmental Glomerülosklerozis: Tek Merkez Deneyimi

Amaç: Fokal segmental glomerüloskleroz (FSGS), çocuklarda nefrotik sendromun (NS) yaygın nedenlerinden biridir. Bu çalışma, primer FSGS'li çocukların demografik verilerini, klinik seyrini, tedavisini ve böbrek sonuçlarını belirlemeyi ve tek merkez deneyimini raporlamayı amaçlamaktadır. Gereç ve Yöntemler: Üçüncü basamak bir pediatric bakım hastanesinde Temmuz 2005 ile Temmuz 2019 arasında primer FSGS tanısı alan 38 hastanın uzun vadeli sonuçlarına ilişkin retrospektif bir çalışmadır. Bulgular: Fokal segmental glomerüloskleroz tanısı olan 38 çocuk (23 kız ve 15 erkek hasta) dahil edildi ve ortalama tanı yaşı 8.5 ± 4.2 yıldı. Ortalama takip süresi 4.8 ± 4.1 (1-14.6) yıldı. On yedi (%44.7) hasta steroide dirençli NS ve 21 (%55.3) hasta steroide duyarlı NS [12 (%31.6) steroid bağımlı NS ve 9 (%23.7) hasta sık tekrarlayan NS] idi. Başvuru anında bu gruplar arasında yaş, cinsiyet, hematüri, serum albumin ve idrar protein düzeyi açısından anlamlı fark yoktu (p > 0.05). Uzun süreli takipte SRNS’li hastaların %47'sinin tam remisyon, %23.5'inin kısmi remisyon ve %29.4 'ünün de tüm tedavilere dirençli olduğu görüldü. Hastaların %15.8'sinde SDBH gelişmişti. Kötü prognoz için risk faktörleri, başvuruda hipertansiyon (HT) varlığı, kadın cinsiyet ve başlangıç tedavisine yanıtsızlık olarak belirlendi. Sonuç: Çocukluk çağında FSGS, tedaviye yanıt ve prognozda değişkenlik göstermektedir. Bu çalışmada prognozu etkileyen risk faktörleri ile ilgili verilerimizi sunduk.

Anahtar Kelimeler:

pediatri, nefrotik sendrom, fokal segmental glomerulosklerozis

Pediatric Focal Segmental Glomerulosclerosis: A Single-Center Experience

Aim: Focal segmental glomerulosclerosis (FSGS) is one of the common causes of nephrotic syndrome (NS) in children. This study aims to determine the demographical data, clinical course, treatment and renal outcome of children with primary FSGS and report the experience of a single center. Material and Methods: A retrospective study of the long-term outcome of 38 patients with diagnosis of primary FSGS at a tertiary pediatric care hospital from the period July 2005 to July 2019 was conducted. Results: The study included 38 patients (23 female and 15 male) with FSGS, and the mean age at diagnosis was 8.5 ± 4.2 years. The mean follow-up duration was 4.8± 4.1 (1-14.6) years. Seventeen (44.7%) patients were steroid-resistant NS (SRNS) and 21 (55.3%) patients were steroid-sensitive NS (SSNS) [12 (31.6%) steroid-dependent NS (SDNS) and 9 (23.7%) frequently relapsing NS (FRNS)]. There was no significant difference between these groups in age, gender, hematuria, serum albumin and urine protein level at presentation (p > 0.05). Long-term follow-up showed that 47% of SRNS patients achieved complete remission, 23.5% partial remission and 29.4% resistant to all therapies. ESRD was developed 15.8% of the FSGS patients. Risk factors for poor prognosis were the presence of hypertension (HT) at admission, female gender, and unresponsiveness to initial treatment. Conclusion: Focal segmental glomerulosclerosis in childhood, shows changes in response to treatment and prognosis. In this study, we presented our data on risk factors affecting prognosis.

Keywords:

pediatric, nephrotic syndrome, focal segmental glomerulosclerosis,

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