Case report: Hemolytic uremic syndrome with multiple organ damage

Abstract: Typical HUS is one of the most common causes of the community acquired acute kidney failure in young children. It is characterized by the microangiopathic hemolytic anemia, thrombocytopenia and renal insufficiency. The most common etiological factor is STEC infection, which primary manifests with diarrhea. Life-threatening multiple organ damage (MOD) with central nervous systems (CNS) involvement develops in some cases, and then the treatment of monoclonal antibody might be considered. A previously healthy 2 years old girl was hospitalized in Children’s Clinical University Hospital with febrile temperature for 4 days, bloody diarrhea, abdominal pain and decreased diuresis followed by anuria. Laboratory data revealed leukocytosis, anemia, thrombocytopenia, serum creatinine – 365.46 mcmol/L, GFR-11.9 ml/min/1.73 m2 by Schwartz Equation. EHEC serotype O26 was found in the feces. The diagnosis of the tHUS was established. The disease progressed. Patient had arterial hypertension, prolonged episodes of nausea, vomiting, elevated glucose level. Neurological impairment involved behavioral disturbances and left side hemiparesis. Ischemic lesions in the basal ganglia and brainstem were demonstrated by magnetic resonance imaging. According to the development of MOD with CNS involvement, the therapy of Eculizumab was started. After that patients neurological state and renal function slightly improved, however complete recovery was not made. This case shows that monoclonal antibody can be used when the tHUS presents with CNS involvement. However, in this case therapy with Eculizumab was started after 8 weeks from the onset of the disease, and that may explain why patients complete recovery was not made.

Authors:
Ieva Karklina-Kravale, Edite Jeruma, Sandra Derkevica

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