Ayşe Seda PINARBAŞI, Sibel YEL, Neslihan GÜNAY, Hakan POYRAZOĞLU, Aynur GENCER BALABAN, İsmail DURSUN, Zübeyde GÜNDÜZ, Ruhan DÜŞÜNSEL

Hemolitik Üremik Sendrom; 10 Yıllık Tek Merkez Deneyimi

Amaç: Hemolitik üremik sendrom (HÜS) mikroanjiopatik hemolitik anemi, trombositopeni ve akut böbrek hasarı ile karakterize bir tablodur. Şiga toksini üreten Escherchia coli ilişkili HÜS (STEC-HÜS) çocuklarda en sık nedendir. Farklı dönemlerde salgınlar şeklinde karşımıza çıkan HÜS vakalarında son yıllarda merkezimizde de artış görülmüştür ve bu yazımızda on yıllık HÜS ile takipli hastaların klinik özellikleri değerlendirilmiştir. Araçlar ve Yöntem: 2008-2018 yılları arasında HÜS tanısı alan hastalar çalışmaya alındı. Hastaların demografik özellikleri, başvuru klinik bulguları, başvuru anında ve hastalığın akut fazında hematolojik ve biyokimyasal bulguları, kompleman değerleri, HÜS’e bağlı organ tutulumları, uygulanan tedaviler ve böbrek yerine koyma tedavileri değerlendirildi. Bulgular: Kırk üç hasta çalışmaya alındı. Hastaların %86’sında ishal öyküsü vardı ve bu hastaların yarısında ishal kanlı idi. Nörolojik tutulum hastaların %30’unda vardı. On sekiz hastaya ekulizumab tedavisi verildi. Ekulizumab tedavisi alan hastaların proteinüri, hipertansiyon gibi böbrek hastalığının progresyonunu gösteren parametreleri daha belirgin idi. İki hastada takipte son dönem böbrek yetmezliği gelişti. Sonuç: Akut böbrek hasarının önemli bir nedeni olan HÜS çocuk hastalarda en sık ishal ilişkili olarak karşımıza çıkmaktadır. Nörolojik tutulum en sık böbrek dışı tutulum şeklidir. Hastaların çoğunda (%70) böbrek yerine koyma tedavisine gereksinim duyulmakta ve ekulizumab tedavisini progresyonu kötü olan olgularda daha sık kullanılmaktadır.

Anahtar Kelimeler:

Hemolitik üremik sendrom, Ekulizumab, Pediatri

Hemolytic Uremic Syndrome; 10 Year Single Center Experience

Purpose: Hemolytic uremic syndrome (HUS) is a clinical syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Shiga toxin producing Escherchia coli-associated HUS (STEC-HUS) is the most common cause in children. In recent years, an increase has been observed in our center in the cases of HUS, which appeared as outbreaks in different periods, and in this study, the clinical characteristics of patients with HUS were evaluated.Materials and Methods: Patients diagnosed with HUS between 2008-2018 were included in the study. The demographic characteristics of the patients, clinical findings at admission, hematological and biochemical findings at the time of admission and in the most active period of the disease, complement values, organ involvement due to HUS, applied treatments and kidney replacement treatments were evaluated.Results: Forty-three patients were included in the study. 86% of the patients had a history of diarrhea and half of these patients had diarrhea with blood. Neurological involvement was present in 30% of the patients. Eighteen patients were given eculizumab treatment. Parameters showing the progression of kidney disease such as proteinuria and hypertension were more prominent in patients who received eculizumab treatment. End-stage kidney disease developed in two patients during follow-up.Conclusion: HUS, which is an important cause of acute kidney damage, is most frequently associated with diarrhea in pediatric patients. Neurological involvement is the most common extra-renal manifestation. Kidney replacement therapy is required in most patients (70%) and eculizumab therapy is used more frequently in severe cases.

Keywords:

Hemolytic uremic syndrome, Eculizumab, Pediatrics,

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