Abstract:

Aim: Hemolytic Uremic Syndrome (HUS) is characterized as microangiopathic hemolytic anemia, trombocytopenia and acute kidney injury. This study aimed to evaluate etiological factors, follow-up and treatment results of the patients with HUS who were followed-up at Pediatric Nephrology Department of our center. Method: Nineteen patients who were admitted to Department of Pediatric Emergency and Pediatric Nephrology Units between December 2010 and December 2020 with HUS diagnosis were included in the study. Demographic and clinical features, treatments and extra-renal involvement of the patients were collected retrospectively from the records. Results: Mean age of the patients was 28.8±33.6 months. Atypic and STEC-HUS was diagnosed in 10 (52.6%) and nine (47.4%) of the patients, respectively. Hypertension developed in 16 (84.2%) of the patients. Fresh frozen plasma infusions were given to 18 (94.7%) patients. Eculizumab were given to three patients with atypic HUS (8/10, 80%). Plasma exchange were applied to three patients (3/19, 15.8%). Renal replecament therapy was applied to three of the patients with STEC-HUS (3/9, 33.3%) and to all of the patients with atypic HUS. Extra-renal involvement was observed in one patient with STEC-HUS (1/19, 11.1%) and in all patients with atypic HUS. Two patients with atypic HUS died due to gastrointestinal bleeding and heart failure, respectively. Chronic kidney disease was developed in three patients with atypic HUS. Conclusion: Hemolytic uremic syndrome is an important etiology for acute kidney injury in children. Hypertension is commonly accompanied with HUS. The rate of extra-renal involvement, requirement of renal replacement therapy and mortality were high in patients with atypic HUS.

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