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SON DÖNEM BÖBREK YETMEZLİĞİ NEDENİ GLOMERÜLOKİSTİK BÖBREK HASTALIĞI (OLGU SUNUMU)

GLOMERULOCYSTIC KIDNEY DISEASE AS A CAUSE OF END-STAGE RENAL FAILURE (CASE REPORT)

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Abstract (2. Language): 
Glomerulocysüc kidney disease (GCKD) is an unusual form of cystic renal diseases in childhood characterized pathologically by cystic dilatations in Bowman's space. GCKD may be relatively underreported in children with end-stage renal disease (ESRD) because definitive diagnostic testing with a renal biopsy is not routinely performed in patiens with ESRD. We present a case of GCKD in a 5-year-old girl who presented with ESRD and underwent chronic peritoneal dialysis. Additional findings in this patient were cerebral atrophy, mental retardation and hypermetropia that has previosly been unreported in patients with this disease. The clinical features of this rare condition and the diagnostic approach are reviewed. In conclusion, although rare, GCKD should be considered in the differential diagnosis of ESRD in childhood.
Abstract (Original Language): 
Glomerülokistik böbrek hastalığı (GKBH) patolojik olarak Bowman boşluğunda- kistik dilatasyonlarla seyreden renal kistik hastalıkların nadir bir tipidir. Her yaşta görülebilmekle birlikte özellikle çocukluk yaş grubunun bir hastalığı olarak kabul edilmektedir. Son dönem böbrek yetmezliği (SDBY) tablosunda başvuran ve renal replasman tedavisine alınan hastalarda böbrek biyopsisi genellikle yapılmadığından, kronik böbrek yetmezliği nedenleri arasında GKBH insidansı relatif olarak çok düşük görünmektedir. Bu yazıda, SDBY tablosunda başvuru ile kronik periton diyalizi programına alınan ve tanısı böbrek biyopsisi ile konan GKBHlı 5 yaşında bir kız olgu sunulmuştur. Ayrıca literatürde bildirilen GKBH'ya eşlik eden ekstrarenal anomaliler dışında olgumuzda kortikal atrofı, mental retardasyon ve hipermetropi saptanmıştır. Nadir görülen bu hastalıkta klinik bulgular ve tanış al yaklaşım gözden geçirilmiştir. Sonuç olarak çocukluk yaş grubunda SDBY ayırıcı tanısında nadir bir durum olan GKBH da akla getirilmelidir.
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REFERENCES

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