Atipik Hemolitik Üremik Sendrom

Yıl 2014, , 108 - 115, 01.07.2014

Zeynep Nagehan Yürük Yıldırım Alev Yılmaz

https://doi.org/10.5222/j.child.2014.108

Öz

Hemolitik üremik sendrom HÜS mikroanjiopatik hemolitik anemi, trombositopeni ve akut böbrek hasarının bir arada görüldüğü bir hastalıktır. Hastaların %90’ında klinik tablodan diyareye yol açan Shiga toksin üreten Eschericha Coli STEC sorumludur. Geleneksel olarak “tipik HÜS” olarak adlandırı- lan bu hastalarda iyi bir destek tedavi sağlandığında prognoz yüz güldürücüdür. Hastaların geri kalan %10’u diyare ilişkili olmayan “atipik HÜS” aHÜS olarak adlandırılır. Günümüzde aHÜS’ün patogenezinde kompleman sisteminin regülasyon bozuklukları ve kobalamin metabolizma bozuklukları gibi bir- çok nedenin yer aldığı gösterilmiştir. Bu yeni bilgiler ışığında aHÜS tedavisinde de yeni yaklaşımlar geliştirilmiştir. Bu makalede hastalığın tanı ve tedavisinde güncel bilgiler değer- lendirilmiştir

Anahtar Kelimeler

HÜS, aHÜS, hemolitik üremik sendrom, diyare, eculizumab

Atypical Hemolytic Uremic Syndrome

Öz

Hemolytic uremic syndrome HUS is characterized by conco- mitancy of hemolysis, thrombocytopenia, and acute kidney injury. The majority of the cases 90% are due to diarrhea D+HUS caused by Shiga toxin-producing Escherichia coli STEC . The prognosis is generally well in HUS patients when appropriate supportive treatment is provided The remaining 10% of the patients have not usually diarrhea and this entity is called atypical HUS aHUS . Nowadays many causes in the etiology of aHUS are discovered, the most important of these being dysregulation of the complement system, and disorders of cobalamine metabolism. In the light of these information novel approaches in the treatment of aHUS have been develo- ped. In this report, current information related to the diagnosis and treatment of HUS have been evaluated

Anahtar Kelimeler

HUS, aHUS, hemolytic uremic syndrome, diarrhea, eculizumab

Kaynakça

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