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Plasmaferese prolongada num caso de síndrome hemolítico urémico atípico

Dr Bernardo O. Faria, Dr Jesús Garrido, Dr Tânia Sousa, Dr Carlos Soares, Dr Sérgio Lemos, Dr Délia A. Osuna, Dr Edgar L. Gomes

Hospital de São Teotónio, E.P.E., Viseu

Abstract

The hemolytic-uremic syndrome can present itself in the classical form associated to diarrhea or in the atypical form, not associated to diarrhea. It´s pathogenesis is well defined in the first, most cases following enteric infection with Shiga toxin - producing bacteria, but remains unclear in the latter. Urinary tract infection is described as a possible cause of hemolytic-uremic syndrome. Here we describe a case of a 50 year-old woman, admitted to our hospital with fever and the diagnosis of acute obstructive renal failure. She was submitted to mechanical desobstruction and started antibiotherapy for suspected urosepsis. Laboratory tests showed renal failure, hemolytic anemia, thrombocytopenia, LDH elevation, and schistocytes in blood smear, which suggested the diagnosis of hemolytic uremic syndrome. She started prompt treatment with daily plasmapheresis and hemodialysis. She recovered from renal failure after 20 days of treatment, although with persistence of thrombocytopenia and elevated LDH. As a result corticosteroid therapy was associated. She had slow analytical recovery, needing 35 plasmapheresis sessions during 2 months of hospital stay. After 1 year of follow-up, there was no disease recurrence despite several Escherichia coli urinary tract infections, and there was confirmation of full recovery of renal failure. Atypical hemolytic-uremic syndrome can be a complex disease with difficult treatment. These cases need to be carefully examined for foci other than the gastrointestinal tract, namely the urinary tract. Despite associated risks, prolonged plasmapheresis showed in this case to be effective, given the resolution of the acute clinical manifestations and the complete recovery of the renal failure.

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