TY - JOUR T1 - Pediatric Focal Segmental Glomerulosclerosis: A Single-Center Experience TT - Çocukluk Çağı Fokal Segmental Glomerülosklerozis: Tek Merkez Deneyimi AU - Karakaya, Deniz AU - Yazılıtaş, Fatma AU - Kargın Çakıcı, Evrim AU - Güngör, Tülin AU - Çelikkaya, Evra AU - Bülbül, Mehmet PY - 2022 DA - June DO - 10.18663/tjcl.1077175 JF - Turkish Journal of Clinics and Laboratory JO - TJCL PB - DNT Ortadoğu Yayıncılık A.Ş. WT - DergiPark SN - 2149-8296 SP - 280 EP - 284 VL - 13 IS - 2 LA - en AB - Aim: Focal segmental glomerulosclerosis (FSGS) is one of the common causes of nephrotic syndrome (NS) in children. Thisstudy aims to determine the demographical data, clinical course, treatment and renal outcome of children with primaryFSGS 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 ata 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 patientsachieved complete remission, 23.5% partial remission and 29.4% resistant to all therapies. ESRD was developed 15.8% ofthe 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. Inthis study, we presented our data on risk factors affecting prognosis. KW - pediatric KW - nephrotic syndrome KW - focal segmental glomerulosclerosis N2 - Amaç: Fokal segmental glomerüloskleroz (FSGS), çocuklarda nefrotik sendromun (NS) yaygın nedenlerinden biridir. Buçalışma, primer FSGS'li çocukların demografik verilerini, klinik seyrini, tedavisini ve böbrek sonuçlarını belirlemeyi ve tekmerkez deneyimini raporlamayı amaçlamaktadır.Gereç ve Yöntemler: Üçüncü basamak bir pediatric bakım hastanesinde Temmuz 2005 ile Temmuz 2019 arasında primerFSGS 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 yaşı8.5 ± 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) hastasteroide duyarlı NS [12 (%31.6) steroid bağımlı NS ve 9 (%23.7) hasta sık tekrarlayan NS] idi. Başvuru anında bu gruplararasında yaş, cinsiyet, hematüri, serum albumin ve idrar protein düzeyi açısından anlamlı fark yoktu (p > 0.05). Uzun sürelitakipte SRNS’li hastaların %47'sinin tam remisyon, %23.5'inin kısmi remisyon ve %29.4 'ünün de tüm tedavilere dirençliolduğu 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 etkileyenrisk faktörleri ile ilgili verilerimizi sunduk. CR - 1. Marcelo M. Abrantes, Luis Sergio B. Cardoso, et al. Clinical course of 110 children and adolescents with primary focal segmental glomerulosclerosis. Pediatr Nephrology. 2006; 21: 482–9 CR - 2. Beşbaş N, Ozaltin F, Emre S, et al. Clinical course of primary focal segmental glomerulosclerosis (FSGS) in Turkish children: a report from the Turkish Pediatric Nephrology FSGS Study Group. Turk J Pediatr. 2010; 52: 255-61 CR - 3. Shakeel S, Mubarak M, Kazi JI. 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Am J Kidney Dis. 1998; 32: 72–9 CR - 20. Ponticelli C, Edefonti A, Ghio L, et al. Cyclosporin versus cyclophosphamide for patients with steroid-dependent and frequently relapsing idiopathic nephrotic syndrome: a multicentre randomized controlled trial. Nephrol Dial Transplant. 1993; 8: 1326–32 UR - https://doi.org/10.18663/tjcl.1077175 L1 - https://dergipark.org.tr/tr/download/article-file/2267774 ER -