1096A: USE OF ECULIZUMAB FOR CARDIAC DYSFUNCTION IN TYPICAL HEMOLYTIC UREMIC SYNDROME: A CASE REPORT

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INTRODUCTION: Hemolytic uremic syndrome (HUS) is a microangiopathic hemolytic anemia with thrombocytopenia and acute kidney injury. Children who develop extra-renal disease are at increased risk of death from neurologic or cardiac complications with the incidence of myocarditis between 1 to 5%. The activation of the alternative complement cascade during Shiga toxin-producing E. coli (STEC) HUS is thought to contribute to the extra-renal manifestations of HUS but to what extent remains unclear. Eculizumab, a monoclonal IgG2/4 antibody, prevents the formation of the terminal complement pathway. It is routinely used in the treatment of atypical HUS. Although its use on typical HUS has not demonstrated benefit, there are reported cases of improvement in cases with neurologic involvement. Despite these promising results in limited patients, Eculizumab has rarely been described for the use of cardiac manifestations resulting from STEC HUS. DESCRIPTION: We describe a 22 month old male with typical STEC positive HUS complicated by acute kidney injury requiring continuous renal replacement and myocarditis. Laboratory workup was remarkable for creatinine of 3.29mg/dl, BUN of 73 mg/dl, WBC 34.3K, Hgb 10.1 g/dl, platelets of 104K. CRP was 47.86mg/L, procalcitonin 29.5ng/ml, hypocomplementemia and transaminitis. On day 2 of admission patient acutely decompensated requiring intubation and inotropic support. Echocardiography revealed moderate to severely decreased left ventricular function with ejection fraction of 32% with associated troponinemia and elevated brain natriuretic peptide. Due to failure of improvement with standard therapy, decision was made to trial Eculizumab 600 mg induction dose with maintenance dose of 300 mg as is protocol for atypical HUS. 24 hours after first dose, repeated echo showed improvement in cardiac function to low-normal with ejection fraction of 55% and decreased inotropic support. Patient was weaned off inotropic support and successfully extubated. He continued renal replacement therapy until improvement of renal function and was discharged home without significant complications. DISCUSSION: Eculizumab could be a useful therapy for extrarenal manifestations of STEC HUS, particularly in severe cases with continued decompensation and lack of improvement with standard therapy.

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