A devastating case of diarrhea-associated hemolytic uremic syndrome associated with extensive cerebral infarction; why we need to do better

Abstract

A 4-year-old girl with diarrhea-associated hemolytic uremic syndrome (D+HUS) was transferred to the PICU of our center due to deteriorating renal function and neurological involvement. On admission, a comatous child was seen with hypoventilation and she was placed on mechanical ventilation. Hemodialysis was commenced but plasma exchange was discontinued due to repeated hypersensitivity reactions. A trial of eculizumab was given in light of the worsening of her neurologic condition with development of a pyramidal syndrome and deepening of the coma. Hematological and renal improvement were noted but severe neurologic involvement persisted. MRI revealed extensive bilateral zones of corticocerebral infarction and neurological damage proved to be irreversible. Diarrhea-associated hemolytic uremic syndrome is a common cause of Acute Kidney Injury associated with severe short- and long-term complications. Neurologic involvement is frequent but often reversible. Currently, no effective treatment strategies are available and a paucity of data exists concerning the efficacy of potential treatment options such as early plasma exchange, eculizumab, and high dose corticosteroids. A concerted effort is needed to early identify patients at risk for poor outcome with trials aimed at evaluating the efficacy of potential treatment options for this subgroup.

Keywords:

• Acute kidney injury
• diarrhea-associated hemolytic uremic syndrome
• neurological dysfunction
• cerebral infarct