İdiyopatik nefrotik sendromlu çocuklarda uzun dönem sonuçlar: tek merkez deneyimi

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.

Anahtar Kelimeler:

Nefrotik sendrom, steroid yanıtı, çocuklar

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

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,

PDF

___

Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003;362:629-39.
Clark AG, Barratt TM. Steroid responsive nephrotic syndrome. Pediatric Nephrology, 4 th edition (Eds TM Barratt, ED Avner, WE Harmon): 731-45. Baltimore, Lippincott Williams& Wilkins, 1999.
Noone DG, Iijima K, Parekh R. Idiopathic nephrotic syndrome in children Lancet. 2018;392:61-74.
Hjorten R, Anwar Z, Reidy KJ. Long-term Outcomes of childhood onset nephrotic syndrome. Front Pediatr. 2016;4:53.
Trautmann A, Schnaidt S, Lipska-Zietkiewicz BS, Bodria M, Ozaltın F, Emma F et al. Long-term outcome of steroid-resistant nephrotic syndrome in children. J Am Soc Nephrol. 2017;28:3055-65.
Inaba A, Hamasaki Y, Ishikura K, Hamada R, Sakai T, Hataya H et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children. Pediatr Nephrol. 2016;31:425-34.
International Study of Kidney Disease in Children. Primary nephrotic syndrome in children: clinical significance of histopathologic variants of minimal change and of diffuse mesangial hypercellularity. A Report of the International Study of Kidney Disease in Children. Kidney Int. 1981;20:765-71.
Hacıhamdioğlu DÖ, Kalman S, Gök F. Long-term results of children diagnosed with idiopathic nephrotic syndrome, single center experience. Türk Ped Arş. 2015;50:37-44.
Mishra OP, Abhinay A, Mishra RN, Prasad R, Pohl M. Can we predict relapses in children with idiopathic steroid-sensitive nephrotic syndrome? J Trop Pediatr. 2013;59:343-9.
Özlü SG, Demircin G, Tökmeci N, Yılmaz AÇ, Aydoğ Ö, Bülbül M et al. Long-term prognosis of idiopathic nephrotic syndrome in children. Ren Fail. 2015;37:672-7.
Dossier C, Delbent JD, Boyer O, Daoud P, Mesples B, Pellegrino B et al. Five-year outcome of children with idiopathic nephrotic syndrome: The NEPHROVIR population-based cohort study. Pediatr Nephrol. 2019;34:671-8.
Fakhouri F, Bocquet N, Taupin P, Presne C, Gagnadoux MF, Landais P et al. Steroid-sensitive nephrotic syndrome: from childhood to adulthood. Am J Kidney Dis. 2003;41:550-7.
Skrzypczyk P, Panczyk-Tomaszewska M, Roszkowska-Blaim M, Wawer Z, Bienias B, Zajaczkowska M et al. Long-term outcomes in idiopathic nephrotic syndrome: from childhood to adulthood. Clin Nephrol. 2014;81:166-73.
Vats AN. Genetics of idiopathic nephrotic syndrome. Indian J Pediatr. 2005;72:777-83.
Gbadegesin RA, Adeyemo A, Webb NJA, Greenbaum LA, Abeyagunawardena A, Thalgahagoda S et al. HLA-DQA1 and PLCG2 are candidate risk loci for childhood-onset steroid-sensitive nephrotic syndrome. J Am Soc Nephrol. 2015;26:1701-10.
Caridi G, Trivelli A, Sanna-Cherchi S, Perfumo F, Ghiggeri GM. Familial forms of nephrotic syndrome. Pediatr Nephrol. 2010;25:241-52.
Beata S, Lipska BS, Iatropoulos P, Maranta R, Caridi G, Ozaltın F, the PodoNet Consortium. Genetic screening in adolescents with steroid resistant nephrotic syndrome. Kidney Int. 2013;84:206-13.
Santin S, Bullich G, Tazon-Vega B, Garcia-Maset R, Gimenez I, Silva I et al. clinical utility of genetic testing in children and adults with steroid-resistant nephrotic syndrome. Clin J Am Soc Nephrol. 2011;6:1139-48.
Hodson EM, Alexander SI, Graf N. Steroid sensitive nephrotic syndrome. Pediatric Kidney Disease, 2nd edition (Eds DF Geary, F Schaefer): 419-53. Berlin Heidelberg, Springer-Verlag, 2016.
Shatat IF, Becton LJ, Woroniecki RP. Hypertension in childhood nephrotic syndrome. Front Pediatr. 2019;7:287.
Banaszak B, Banaszak P. The increasing incidence of initial steroid resistance in childhood nephrotic syndrome. Pediatr Nephrol. 2012;27:927-32.
Gulati S, Sengupta D, Sharma RK, Sharma A, Gupta RK, Singh U et al. Steroid resistant nephrotic syndrome: Role of histopathology. Indian Pediatr. 2006;43:55-60.
International Study of Kidney Disease in Children. Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney Int. 1978;13:159-65.
Moustafa BH, Tolba OA. Immunosuppressive therapy in children with steroid-resistant, frequently-relapsing, and steroid-dependent idiopathic nephrotic syndrome: a single center experience. Electron Physician. 2016;8:2039-47.
Nammalwar BR, Vijayakumar M, Prahlad N. Experience of renal biopsy in children with nephrotic syndrome. Pediatr Nephrol. 2006;21:286-8.
Larkins N, Kim S, Craig J, Hodson E. Steroid-sensitive nephrotic syndrome: an evidence-based update of immunosuppressive treatment in children. Arch Dis Child. 2016;101:404-8.
Carter SA, Mistry S, Fitzpatrick J, Banh T, Hebert D, Langlois V et al. Prediction of short- and long-term outcomes in childhood nephrotic syndrome. Kidney Int Rep. 2019;5:426-34.
Sureshkumar P, Hodson EM, Willis NS, Barzi F, Craig JC. Predictors of remission and relapse in idiopathic nephrotic syndrome: a prospective cohort study. Pediatr Nephrol. 2014;29:1039-46.
Taktak A, Çiçek N. Could steroid dependency be predicted at the first attack in idiopathic nephrotic syndrome? J Child. 2020;20:96-9.
Benz MR, Toenshoff B, Weber LT. Treatment of children with frequently relapsing steroid-sensitive nephrotic syndrome: recent trial results. Clin. Invest. 2014;4:1043-54.
Lombel RM, Gipson DS, Hodson EM. Kidney Disease: Improving Global Outcomes. Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr. Nephrol. 2013;28:415-26.
Tullus K, webb H, Bagga A. Management of steroid-resistant nephrotic syndrome in children and adolescents. Lancet Child Adolesc Health. 2018;2:880-90.
Abeyagunawardena AS, Sebire NJ, Risdon RA, Dillon MJ, Rees L, Van’t Hoff W et al. Predictors of long-term outcome of children with idiopathic focal segmental glomerulosclerosis. Pediatr Nephrol. 2007;22:215-21.
Mekahli D, Liutkus A, Ranchin B, Yu A, Bessenay L, Girardin E et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study. Pediatr Nephrol. 2009;24:1525-32.
Pokrajac D, Kamber AH, Karasalihovic Z. Children with steroid-resistant nephrotic syndrome: a single-center experience. Mater Sociomed. 2018;30:84-8.