İdiyopatik Nefrotik Sendromda İlk Atakta Steroid Bağımlılığı Öngörülebilir mi?

Amaç: İdiyopatik nefrotik sendrom (İNS) çocuklarda en sık görülen glomerüler hastalık olup, %80’i steroide duyarlıdır. Steroide duyarlı nefrotik sendrom (SDNS) hastalarının yaklaşık yarısı steroid bağımlı nefrotik sendromudur (SBNS). Bu çalışmanın amacı ilk atağında başvuran SDNS hastalarında, steroid bağımlılığını tedavi öncesi öngörebilecek risk faktörlerini belirlemektir. Gereç ve Yöntem: Çocuk Nefroloji Kliniğimize Eylül 2016-Eylül 2018 tarihleri arasında ilk atak İNS ile başvuran ve steroid tedavisine yanıtlı hastalar geriye dönük olarak değerlendirildi. Hastaların demografik özellikleri, poliklinik kan basıncı ölçümü, kan albümin ve kreatinin değeri, beyaz küre sayısı, lenfosit sayısı, lenfosit/beyaz küre oranı, spot idrar protein/kreatinin oranı, steroid tedavisi başlanmasını takiben remisyona girme süresi ve atak başlatıcısı kaydedildi. Bulgular: Hastaların 23’ü SDNS, 16’sı SBNS tanısı aldı. Başvuru yaşı ortalaması SDNS grubunda 77,34±32 ay, SBNS grubunda 73,62±27,32 ay idi (p=0,690). İki grup arasında kan albümin, kreatinin, lenfosit sayısı ve lenfosit/ beyaz küre oranı açısından anlamlı fark saptanmadı. Atak başlatıcısı SBNS grubunda daha fazla görüldü (p=0,001) ve en sık tespit edilen atak başlatıcısı üst solunum yolu enfeksiyonu idi. Spot idrar protein/kreatinin oranı, kan beyaz küre seviyesi SBNS grubunda anlamlı yüksek saptandı (p=0,014, p=0,004) ve remisyona girme süresi SBNS grubunda daha uzundu (17,5 güne 9 gün) (p=0,000). Sonuç: İdiyopatik NS’de, üst solunum yolu enfeksiyonuyla ilk atağın tetiklenmesi, başvuru anında ağır proteinüri saptanması ve geç remisyona girme süresi SBNS gelişmesi açısından risk faktörleri olabilir. Bu hastalarda steroid ayırıcı tedavilerin daha erken gündeme alınması, steroid tedavisinin istenmeyen uzun dönem yan etkilerinden koruyacaktır.

Anahtar Kelimeler:

Nefrotik Sendrom, Steroid Tedavisi, Steroid Bağımlı Nefrotik Sendrom, Çocuk

Could Steroid Dependency Be Predicted at the First Attack in Idiopathic Nephrotic Syndrome?

Objective: Idiopathic nephrotic syndrome (INS) is the most common glomerular disease in children. Approximately 80% of INS is steroid sensitive (SSNS), and half of SSNS patients are steroid dependent (SDNS). This study determines the risk factors that may predict steroid dependence in patients with SSNS. Material and Method: Patients with SSNS who followed in our Pediatric Nephrology clinic between September 2016 and September 2018 were retrospectively evaluated. The demographic characteristics, albumin, creatinine values, blood pressure, white blood cell, lymphocyte count, lymphocyte/white blood cell ratio, urine protein–creatinine ratio, time to remission, and triggers of the attack were recorded. Results: Twenty-three patients were diagnosed as SSNS and 16 as SDNS. The mean age at presentation was 77.34±32 months in the SSNS group and 73.62±27.32 months in the SDNS group (p=0.690). No significant difference was observed between the two groups in terms of blood albumin, creatinine, lymphocyte count, and lymphocyte/white blood cell ratio. Trigger initiating the attack was more common in the SDNS group (p=0.001). The most common trigger was upper respiratory tract infection. Urine protein/creatinine ratio and white blood cell levels were significantly higher in the SDNS group (p=0.014, p=0.004, respectively), and the time to remission was also longer in the SDNS group (p=0.001). Conclusion: Upper respiratory tract infection may be a trigger, and severe proteinuria and late remission time may be risk factors for the development of SDNS in INS. Early use of steroid-sparing agents in these patients can prevent long-term adverse effects of steroid therapy.

Keywords:

Nephrotic syndrome, steroid treatment, steroid depended nephrotic syndrome, child,

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1. Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet 2003 Aug 23;362(9384):629-39.
2. Niaudet P, Boyer O. Idiopathic nephrotic syndrome in children: Clinical aspects in a textbook of pediatric nephrology. In: Avner D et al. (eds). 7th ed; 2016. pp. 839-82.
3. Kang HG, Cheong HI. Nephrotic syndrome: what’s new, what’s hot? Korean J Pediatr 2015 Aug;58(8):275-82.
4. Gipson DS, Massengill SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD, et al. Management of childhood onset nephrotic syndrome. Pediatrics 2009 Aug;124(2):747-57.
5. Park SJ, Shin JI. Complications of nephrotic syndrome. Korean J Pediatr 2011 Aug;54(8):322-8.
6. Sinha A, Hari P, Sharma PK, Gulati A, Kalaivani M, Mantan M, et al. Disease course in steroid sensitive nephrotic syndrome. Indian Pediatr 2012 Nov;49(11):881-7.
7. Dawman L, Mehta A, Sharawat IK, Yadav R. Risk factors for steroid dependency in children with idiopathic nephrotic syndrome in India. Indian J Pediatr 2016 Mar;83(3):261.
8. Andersen RF, Thrane N, Noergaard K, Rytter L, Jespersen B, Rittig S. Early age at debut is a predictor of steroid-dependant and frequently relapsing nephrotic syndrome. Pediatr Nephrol 2010 Jul;25(7):1299-304.
9. Kabuki N, Okugawa T, Hayakawa H, Tomizawa S, Kasahara T, Uchiyama M. Influence of age at onset on the outcome of steroid-sensitive nephrotic syndrome. Pediatr Nephrol 1998 Aug;12(6):467-70.
10. Abdel-Hafez MA, Abou-El-Hana NM, Erfan AA, El-Gamasy M, Abdel- Nabi H. Predictive risk factors of steroid-dependent nephrotic syndrome in children. J Nephropathol 2017Jul;6(3):180-6.
11. Dakshayani B, Lakshmanna M, Premalatha R. Predictors of frequent relapsing and steroid-dependent nephrotic syndrome in children. Turk Pediatri Ars 2018 Mar 1;53(1):24-30.
12. Rai VK, Awasthi S, Venkatesh V. Prediction of early response to steroids in nephrotic syndrome patients aged between 2 and 10 years. Clin Epidem and Glob Health 2017 Dec;5(4):203-8.
13. MacDonald NE, Wolfish N, McLaine P, Phipps P, Rossier E. Role of respiratory viruses in exacerbations of primary nephrotic syndrome. J Pediatr 1986 Mar;108(3):378-82.
14. Takahashi S, Wada N, Murakami H, Funaki S, Inagaki T, Harada K, et al. Triggers of relapse in steroid-dependent and frequently relapsing nephrotic syndrome. Pediatr Nephrol 2007 Feb;22(2):232-6.
15. Letavernier B, Letavernier E, Leroy S, Baudet-Bonneville V, Bensman A, Ulinski T. Prediction of high-degree steroid dependency in pediatric idiopathic nephrotic syndrome. Pediatr Nephrol 2008 Dec;23(12):2221-6.
16. Niaudet P. Long-Term outcome of children with steroid-sensitive idiopathic nephrotic syndrome. Clin J Am Soc Nephrol 2009 Oct;4(10):1547-8.
17. 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 Mar;41(3):550-7.