title: Atypical hemolytic-uremic syndrome: a case report and literature review.
abstract: BACKGROUND
  Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by hemolysis, thrombocytopenia, and renal dysfunction. It is a disease related to genetic mutations in the alternative complement pathway and has a distinct pathophysiology but is difficult to differentiate from other thrombotic microangiopathies.
  
  
  METHODS
  We present a case of a 59-year-old female patient who presented with accelerated hypertension, acute renal failure, hemolysis, and encephalopathy. She was managed with antihypertensive medication, but her encephalopathy did not improve. Evaluation resulted in our impression of the disease being atypical hemolytic-uremic syndrome. The patient continued to be managed with good blood pressure control and later was started on eculizumab, but evaluation of response to therapy was hindered by the patient's non-compliance with therapy and follow-up appointments.
  
  
  CONCLUSIONS
  We have a very limited understanding of the genetics and epidemiology of atypical HUS, and the overlapping clinical features sometimes delay diagnosis and initiation of appropriate treatment of this rare disease.
authors.affiliation: Department of Internal Medicine, Bronx Lebanon Hospital Center, Bronx, NY, USA.
authors.forename: Arsalan
authors.initials: A
authors.lastname: Rafiq
authors.affiliation: Department of Internal Medicine, Bronx Lebanon Hospital Center, Bronx, NY, USA.
authors.forename: Hassan
authors.initials: H
authors.lastname: Tariq
authors.affiliation: Department of Internal Medicine, Bronx Lebanon Hospital Center, Bronx, NY, USA.
authors.forename: Naeem
authors.initials: N
authors.lastname: Abbas
authors.affiliation: Department of Internal Medicine, Bronx Lebanon Hospital Center, Bronx, NY, USA.
authors.forename: Roopalekha
authors.initials: R
authors.lastname: Shenoy
mesh_terms: D000465:Algorithms; D065766:Atypical Hemolytic Uremic Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008875:Middle Aged; D011379:Prognosis