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A TİPİK HEMOLİTİK ÜREMİK SENDROM: BİR OLGU SUNUMU

ATYPICAL HEMOLYTIC UREMIC SYNDROME: REPORT OF A PEDIATRIC CASE

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Abstract (2. Language): 
Atypical hemolytic uremic syndrome (HUS) is a rare disease in childhood. The morbidity and mortality of this disease is found high. In this report, a 13 years old boy who had five times of HUS attacks was presented. The family history revealed that his uncle was on hemodialysis for chronic renal failure. The patient had acute renal failure, hemolytic anemia, thrombocytopenia, and dark urine when he was first hospitalized. HUS was diagnosed based upon his clinical and laboratory findings. During the follow-up, he developed three more attacks when he was five, six, nine and ten years old which all required admission to the hospital. The last attack occurred when he was ten years old and plasmapheresis required for five times. He had a quick response to it. We suggest that plasmapheresis is an effective treatment approach in cases with atypical HUS.
Abstract (Original Language): 
Atipik hemolitik Uremik sendrom (HÜS) çocukluk çağında nadir görülen morbiditesi ve mortalitesi yüksek bir klinik tablodur. Bu çalışmada 13 yaşında beş kez HÜS atakları ile izlediğimiz ve amcasına kronik böbrek yetmezliği nedeni ile hemodiyaliz uygulanan bir erkek olgu sunuldu. İlk defa iki yaşında akut böbrek yetmezliği, hemolitik anemi, trombositopeni, idrar renginde koyulaşma yakınmaları ile başvurdu. Bu bulgularla HÜS olarak değerlendirildi. HÜS atakları beş, altı, dokuz ve on yaşlarında yineledi. İzlemdeki olgu son atağında on yaşında olup, aynı yakınmalar ile başvurdu. Hipertansiyon, azotemi, trombositopeni, anemi ve oligüri saptanan olguya beş kez plazmaferez uygulandı. Hastada plazmaferez tedavisi ile daha kısa sürede klinik yanıt alındı. Atipik HÜS tedavisinde plazmaferezin etkin bir tedavi modeli olabileceğini düşünmekteyiz.
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REFERENCES

References: 

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