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ÇOCUKLARDA PRİMER HİPEROKZALÜRİYE BAĞLI KRONİK BÖBREK YETMEZLİĞİ

CHRONIC RENAL FAILURE DUE TO HYPER OXALURIA IN PEDIATRIC PATIENTS

Journal Name:

  • Türk Nefroloji, Diyaliz ve Transplantasyon Dergisi

Publication Year:

  • 2003

Key Words:

Keywords (Original Language):

Author Name
Abstract (2. Language): 
Primary hyperoxaluria is a rarely encountered disease characterised by recurrent urolithiasis, nephrocalcinosis, and oxalate deposition in almost all tissues, generally inherited in autosomal recessive fashion and the important complications of which can be prevented by early liver transplantation. Here we presented 3 boys with primary hyperoxaluria and early onset end stage renalfailure aged 9, 10 and 14 years , in order to impress on the importance of early treatment. All the patients, treated for urolithiasis since their early childhood, had disturbed renal functions and anemia. Their renal ultrasonography revealed hyperechogenic kidneys and multiple stones. The urinary oxalate excretion of the three cases were all detected high (828 mmol/day, 500 mmol/day, 1390 mmol/day) and urinary citrate excretion low (52 mg/day, 50 mg/day and 124 mg/day). Together with the supportive treatment Pyridoxine and Potasium citrate were prescribed to the patients. The pateints receiving continuous renal replacement theapy have been followed up for 1-4 years.
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Abstract (Original Language): 
Primer hiperokzalüri tekrarlayan ürolitiazis, nefrokahinozis ve tüm vücutta okzalat birikimi ile karakterize, genellikle otozomal resessif kalıtım ile geçen, nadir görülen bir hastalık olup erken dönemde karaciğer transplantasyonu ile önemli komplikasy onlar önlenebilmektedir. Burada, erken dönemde son dönem böbrek yetmezliğine giren 9, 10 ve 14 yaşlarında primer hiperokzalürili üç erkek hasta erken tanı ve tedavinin önemini vurgulamak üzere takdim edildi. Küçük yaşlardan itibaren ürolitiazis nedeniyle tedavi gören hastaların ilk başvuruda böbrek fonksiyonları bozuk, anemileri mevcuttu. Renal ultrasonografılerinde böbrekler hiperekojen ve multipl taşlar içermekteydi. Üç vakanın da idrarda okzalat atılımı yüksek (828 mmol/gün, 500 mmoUgün, 1390 mmoU gün), sitrik asit atılımı düşük (52 mg/gün, 50 mg/gün ve 124 mg/gün) bulundu. Vakalara destekleyici tedavinin yanısıra pridoksin ve K sürat başlandı. Sürekli renal replasman tedavisine alınan hastalar 1-4 yıldır takip edilmektedir.
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  • 2
104-108
  • Turkish

REFERENCES

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