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Atypical Hemolytic Uremic Syndrome (aHUS)

aHUS is a complement mediated renal and rare disease, characterized by haemolytic microangiopathic anemia, thrombocytopenia, and renal impairment. Mutations in complement regulatory proteins (loss of function or even gain-of-function) seem to have a major role in this disease, that can only become evident after an additional immune insult / trigger.

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Test your knowledge

  1.  A 52 year old man was transferred to the renal unit with a four day history of fatigue, shortness of breath, headache and malaise.
    On examination, he was pale with multiple petechiae on his arms and legs. Blood pressure was 175/90.
    Investigations:
    Haemoglobin 84g/L (130-180)
    Platelet count 3x109/L (150-400)
    Prothrombin time 13s (11.5-15.5)
    International normalised ratio 1.1 (<1.4)
    Serum creatinine 1100 µmol/L (60-110)
    Serum lactate dehydrogenase 891U/L (10-250)
    Blood film showed schistocytes
    Which is the next most appropriate test to guide management?
    To answer to these question click here.
  2.  A 37 year old man with end stage renal failure, secondary to atypical haemolytic uraemic syndrome (aHUS), is being worked up to receive a live donor kidney from his wife. He has two children and asks about the likelihood of them inheriting his condition.
    A mutation in the gene encoding which of the following proteins most commonly predisposes to aHUS?
    To answer to these question click here.
  3.  A 41 year old woman with end stage renal failure, secondary to atypical haemolytic uraemic syndrome (aHUS), is being worked up to receive a live donor kidney from her sister. She asks about the likelihood of recurrence of aHUS in her transplanted kidney.
    A genetic mutation in which protein is least associated with recurrence of aHUS in the transplanted kidney?
    To answer to these question click here.

 


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