Pediatric Focal Segmental Glomerulosclerosis: A Single-Center Experience

Öz

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.

Anahtar Kelimeler

• pediatric
• nephrotic syndrome
• focal segmental glomerulosclerosis

Çocukluk Çağı Fokal Segmental Glomerülosklerozis: Tek Merkez Deneyimi

Öz

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

Kaynakça

1. 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
2. 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
3. 3. Shakeel S, Mubarak M, Kazi JI. Frequency and clinicopathological correlations of histopathological variants of idiopathic focal segmental glomerulosclerosis in nephrotic adolescents. J Pak MedAssoc. 2014; 64: 322-6
4. 4. Bulut IK, Taner S, Keskinoglu A, et al. Long-Term Follow-up Results of Renal Transplantation in Pediatric Patients With Focal Segmental Glomerulosclerosis: A Single-Center Experience. Transplant Proc. 2019; 51: 1064-9
5. 5. Ahmed M. El-Refaey, Ashraf Bakr, Ayman Hammad, et al. Primary focal segmental glomerulosclerosis in Egyptian children: a 10- year single-centre experience Pediatr Nephrol. 2010; 25: 1369–73
6. 6. J Rivera Roja 1, M Pérez, A Hurtado, et al. Factors predicting for renal survival in primary focal segmental glomerulosclerosis. Nefrologia. 2008; 28: 439-46
7. 7. Sozeri B, Mir S, Mutlubas F, et al.Term results of pediatric patients with primary focal and segmental glomerulosclerosis. Saudi J Kidney Dis Transpl. 2010; 21: 87-92
8. 8. Manel Jellouli, Kamel Abidi, Mouna Askri, et al. Focal segmental glomerulosclerosis in children. Tunis Med. 2016; 94: 356-9

9. 9. Arbus GS, Poucell S, Bacheyie GS, et al. Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: three types of clinical response. J Pediatr. 1982; 101: 40–5
10. 10. Yoshikawa N, Ito H, Akamatsu R, et al. Focal segmental glomerulosclerosis with and without nephrotic syndrome in children. J Pediatr 1986; 109: 65–70
11. 11. Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003; 362: 629–39
12. 12. Burgess E. Management of focal segmental glomerulosclerosis: evidence-based recommendations. Kidney Int Suppl. 1999; 55: 26–32
13. 13. Asiri S. Abeyagunawardena, Neil J. Sebire, R. Anthony Risdon, et al. Predictors of long-term outcome of children with idiopathic focal segmental glomerulosclerosis. Pediatr Nephrol. 2007; 22: 215-21
14. 14. Habashy D, Hodson EM, Craig JC. Interventions for steroidresistant nephrotic syndrome: a systematic review. Pediatr Nephrol 2003; 18: 906–12
15. 15. K V Lieberman, A Tejani. A randomized double-blind placebocontrolled trial of cyclosporine in steroid-resistant idiopathic focal segmental glomerulosclerosis in children. J Am Soc Nephrol. 1996; 7: 56-63
16. 16. Debbie S. Gipson, Hyunsook Chin, Trevor P. Presler , et al. Differential risk of remission and ESRD in childhood FSGS. Pediatr Nephrol. 2006; 21: 344–9
17. 17. Sorof JM, Hawkins EP, Brewer ED, et al. Age and ethnicity affect the risk and outcome of focal segmental glomerulosclerosis. Pediatr Nephrol. 1998; 12: 764-8.
18. 18. Nehus EJ, Goebel JW, Succop PS, et al. Focal segmental glomerulosclerosis in children: multivariate analysis indicates that donor type does not alter recurrence risk. Transplantation. 2013; 96: 550
19. 19. Cattran DC, Rao P. Long-term outcome in children and adults with classic focal segmental glomerulosclerosis. Am J Kidney Dis. 1998; 32: 72–9
20. 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