Benjamin Flam1, Peter Sackey2,3, Andreas Berge4, Anne C Zachau5, Bo Brink6, Sigrid Lundberg6,7. 1. Department of Anesthesiology, Surgical Services and Intensive Care Medicine, Karolinska University Hospital, Solna, Stockholm, Sweden. benjamin.flam@karolinska.se. 2. Department of Anesthesiology, Surgical Services and Intensive Care Medicine, Karolinska University Hospital, Solna, Stockholm, Sweden. 3. Section of Anesthesiology and Intensive Care, Department of Physiology and Pharmacology, Karolinska Institutet, Stockholm, Sweden. 4. Department of Infectious Disease, Karolinska University Hospital, Solna, Stockholm, Sweden. 5. Department of Neurology, Karolinska University Hospital, Stockholm, Sweden. 6. Department of Nephrology, Karolinska University Hospital, Stockholm, Sweden. 7. Division of Renal Medicine, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden.
Abstract
BACKGROUND: Diarrhea-associated hemolytic uremic syndrome (HUS) is characterized by acute kidney injury with microangiopathic hemolytic anemia and thrombocytopenia with a diarrhea prodrome, typically caused by Shiga-like toxin-producing Escherichia coli. Supportive management is generally recommended. CASE REPORT: A 58-year-old female with diarrhea-associated HUS developed delayed-onset severe neurological manifestations including coma, status epilepticus, and subcortical magnetic resonance imaging signal alterations. Rescue treatment with immunoglobulin (Ig)G depletion through immunoadsorption was followed by significant improvement in neurological and renal function. The patient recovered with only minimal sequelae. CONCLUSION: Delayed-onset neurological abnormalities may occur in diarrhea-associated HUS. Novel specific treatment options include IgG depletion through immunoadsorption. Severe clinical and imaging findings do not preclude a good outcome.
BACKGROUND:Diarrhea-associated hemolytic uremic syndrome (HUS) is characterized by acute kidney injury with microangiopathic hemolytic anemia and thrombocytopenia with a diarrhea prodrome, typically caused by Shiga-like toxin-producing Escherichia coli. Supportive management is generally recommended. CASE REPORT: A 58-year-old female with diarrhea-associated HUS developed delayed-onset severe neurological manifestations including coma, status epilepticus, and subcortical magnetic resonance imaging signal alterations. Rescue treatment with immunoglobulin (Ig)G depletion through immunoadsorption was followed by significant improvement in neurological and renal function. The patient recovered with only minimal sequelae. CONCLUSION: Delayed-onset neurological abnormalities may occur in diarrhea-associated HUS. Novel specific treatment options include IgG depletion through immunoadsorption. Severe clinical and imaging findings do not preclude a good outcome.
Authors: Andreas Greinacher; Sigrun Friesecke; Peter Abel; Alexander Dressel; Sylvia Stracke; Michael Fiene; Friedlinde Ernst; Kathleen Selleng; Karin Weissenborn; Bernhard M W Schmidt; Mario Schiffer; Stephan B Felix; Markus M Lerch; Jan T Kielstein; Julia Mayerle Journal: Lancet Date: 2011-09-02 Impact factor: 79.321