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      Cryptic activity of atypical hemolytic uremic syndrome and eculizumab treatment.

      Pediatrics

      Antibodies, Monoclonal, Humanized, adverse effects, therapeutic use, Biological Markers, blood, Child, Child, Preschool, Combined Modality Therapy, Complement Activation, drug effects, Complement Factor H, genetics, immunology, DNA Mutational Analysis, Diagnosis, Differential, Haptoglobins, metabolism, Hemolytic-Uremic Syndrome, diagnosis, drug therapy, Humans, Infant, Kidney Function Tests, L-Lactate Dehydrogenase, Longitudinal Studies, Male, Plasma, Plasmapheresis, Platelet Count, Proteinuria, Purpura, Thrombotic Thrombocytopenic, Secondary Prevention

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          Abstract

          Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening disease often related to uncontrolled complement activation. The use of eculizumab has changed the management and the outcome of aHUS, becoming the frontline treatment of the acute disease and for the prevention of relapses. We report the case of a male patient with aHUS due to complement factor H gene mutation who was shifted from plasmatherapy to eculizumab for preventing disease relapses. The shift to eculizumab was associated with a significant decrease in proteinuria, revealing disease activity otherwise unsuspected, being the classic criteria of disease activity (platelet, haptoglobin, LDH, schistocytes), all in the normal range.The condition of proteinuria as the only sign of thrombotic microangiopathy activity is here designated as "cryptic activity of aHUS." Copyright © 2014 by the American Academy of Pediatrics.

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          Journal
          24843058
          10.1542/peds.2013-2921

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