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ÇOCUKLUK ÇAĞI İDİOPATİK NEFROTİK SENDROMLARINDA RELAPSLARA ETKİ EDEN FAKTÖRLER VE PROGNOZ ÜZERİNE ETKİSİ

THE FACTORS THAT AFFECT THE RELAPSES AND THEIR PROGNOSTIC SIGNIFICANCE IN CHILDHOOD IDIOPATHIC NEPHROTIC SYNDROME

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 1995

Key Words:

Keywords (Original Language):

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Abstract (2. Language): 
Retrospective analysis of 113 children with idio-pathic nephrotic syndrome (INS) was performed bet¬ween January 1990 and January 1995. The mean age of the patients was 3.9 years and male/female ratio was 1.3. All the patients had edema at first admission while 60 had infection. Steroid therapy was started on 109 patients. One hundred and two patients had re¬mission while 7 (6.4 %) patients did not respond. At least one relaps developed in 46.9 % of patients and most of the first relapses occured within the first year. Occurence of relapses was found to be more frequent in the patients who received albumin infusion. Num¬ber of relapses in the patients whose follow up period is more than two years were higher in male patients and whose initiation of the disease is after 3 years of age. Thirty four percentage of the relapses were associated with infection. Two patients died because ofin-fection (1.8 %). Fifteen patients had spontaneous re¬mission in different stages of the disease and 8 pati¬ents (7.1 %) developed frequent relapses. Fifteen pati¬ents with frequent relapses or steroid dependent and steroid resistant nephrotic syndrome were given cyclophosphamide therapy and 3 nonresponders were administered chlorambucil with a good result. In this study we showed once more that INS is frequent in childhood, infection is an important factor that affect the mortality and morbidity of the disease, relapses are more frequent in males and in patients whose di¬sease onset is after 3 years of age and with appropria¬te therapy its mortality is low with a good prognosis
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Abstract (Original Language): 
Ocak 1990 ile Ocak 1995 tarihleri arasında idio-patik nefrotik sendrom (İNS) tanısı alan 113 çocuk retrospektif olarak incelendi. Vakalarının yaş ortala¬ması 3.9, erkek/kız oranı ise 1.3 idi. Hastaların hep¬sinde ilk başvuruda ödem mevcut olup 60 (% 53.1) vakada enfeksiyon tesbit edildi. Steroid tedavisi başlanan 109 hastanın 102'si remisyona girdi, 7 hasta (% 6.4) ise tedaviye cevap vermedi. Hastaların % 46.9'unda en az bir kere relaps gözlendi ve ilk relaps-lar en sık ilk bir yıl içinde gelişti. Albumin infüzyonu yapılan hastalarda relaps oranı daha yüksek bulunur¬ken, izlem süreleri iki yılın üzerinde olan 64 hastada relapsların erkeklerde ve üç yaş üzerinde hastalık başlayanlarda daha sık olduğu gözlendi. Relapsların % 34.4'ü enfeksiyon ile ilişkili bulundu. İki hasta en¬feksiyon nedeniyle kaybedildi (% 1.8). On beş hasta değişik dönemlerde spontan remisyona girdi. Sekiz hastada (% 7.1) ise sık relaps gözlendi. Steroide ba¬ğımlı, sterolde dirençli ve sık relaps gösteren toplam 15 İNS'lu vakaya oral siklofosfamid tedavisi uygulan¬dı. Siklofosfamide cevap vermeyen üç hastada ise klo-rambusil tedavisi ile remisyon sağlandı. Sonuç olarak bu çalışma ile İNS'un çocukluk yaş grubunda sık gö¬rüldüğü, enfeksiyonların hastalığın mortalite ve mor-bitesini etkileyen önemli bir faktör olduğu, erkeklerde ve üç yaş üzerindeki hastalık başlayanlarda relaps riskinin daha fazla olduğu, uygun tedavi ile mortalite oranının düşük, prognozun iyi olduğu gösterildi.
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  • Turkish

REFERENCES

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