göknar, nilüfer bezmialem vakıf üniversitesi, Turkey , göknar, nilüfer bağcılar eğitim ve araştırma hastanesi, Turkey , öktem, faruk bezmialem vakıf üniversitesi, Turkey , demir, ayşegül doğan bezmialem vakıf üniversitesi, Turkey , vehapoğlu, aysel bezmialem vakıf üniversitesi, Turkey , küçükkoç, mehmet bezmialem vakıf üniversitesi, Turkey

Eculizumab Experience of a Single Center in Treatment of Atypical Hemolytic Uremic Syndrome

27380

Objectives: Hemolytic uremic syndrome (HUS) is defined by the presence of hemolytic anemia, thrombocytopenia, and acute renal failure. Atypical HUS (aHUS) is characterized by a relapsing course, high risk of permanent renal damage, and dysregulation in the complement pathway. Plasma exchange/ infusions may temporarily correct hematologic variables, but as the underlying complement dysregulation persists, end stage renal disease or death occurs in 33-40% of patients. In recent years, eculizumab has been used in the treatment of aHUS with high success rates. In this study we report seven pediatric cases with aHUS successfully treated with eculizumab. Methods: From January 2012 to December 2015, we investigated seven pediatric patients who had been diagnosed as aHUS and treated with eculizumab. Age, gender, presenting symptoms, presence of diarrhea, laboratory data on admission and follow-up, need for dialysis, response to plasma exchange, response to eculizumab, and outcome were recorded. Results: Diarrhea was present in four cases but the investigation about Enterohemorrhagic Echerichia coli (EHEC) was negative in stool specimens. Oliguria/anuria was present in six cases. Four of the seven children received plasma exchange. Five children needed dialysis. Despite vaccination, one patient had severe sepsis and meningitis as a complication of eculizumab. All had full recovery of hematologic parameters. Except one children none had erythrocyte transfusion after treatment. On the last follow up, none of the patients were on dialysis. Conclusion: Our results showed that eculizumab is a life-saving and successful treatment for use in cases of aHUS. However severe complications such as meningitis should be kept in mind before treatment.

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Hemolytic Uremic Sydrome , Thrombocytopenia , Eculizumab , Dialysis

Dicle Medical Journal

507