Abstract
Primer hiperokzaluri (PH), okzalatin asiri sistemik uretimine yol acan, nadir gorulen gliokzalat metabolizma bozuklugudur. Okzalatin asiri uretimi ve idrarla atiliminin artmasi, tekrarlayan uriner sistem taslari ve nefrokalsinozis ile sonuclanir. Vucutta okzalat birikimi sistemik okzalozise neden olur ve glomeruler filtrasyon hizi ilerleyici bobrek tutulumuna bagli olarak azalir. Infantil donemde PH tanisi alan ve bobrek yetmezligi gelisen iki vaka sunulmustur.
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