These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis.
    Author: Laurence J, Haller H, Mannucci PM, Nangaku M, Praga M, Rodriguez de Cordoba S.
    Journal: Clin Adv Hematol Oncol; 2016 Nov; 14 Suppl 11(11):2-15. PubMed ID: 27930620.
    Abstract:
    Atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA), is a rare, life-threatening, systemic disease. When unrecognized or inappropriately treated, aHUS has a high degree of morbidity and mortality. aHUS results from chronic, uncontrolled activity of the alternative complement pathway, which activates platelets and damages the endothelium. Two-thirds of aHUS cases are associated with an identifiable complement-activating condition. aHUS is clinically very similar to the other major TMAs: Shiga toxin-producing Escherichia coli (STEC)-HUS, thrombotic thrombocytopenic purpura (TTP), and disseminated intravascular coagulation (DIC). The signs and symptoms of all the TMAs overlap, complicating the differential diagnosis. Clinical identification of a TMA requires documentation of microangiopathic hemolysis accompanied by thrombocytopenia. DIC must be recognized and treated before it is possible to discriminate among the other 3 major TMAs. STEC-HUS can be excluded through testing for Shiga toxin-producing E. coli. aHUS can be distinguished from TTP on the basis of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity, with a severe decrease characteristic of TTP. This test, as both an activity assay and an inhibitor assay, should be ordered before the initiation of plasma therapy in any patient presenting with a TMA. Finally, it is important to recognize that aHUS remains a clinical diagnosis, but in complex scenarios, tissue biopsy may be a useful adjunct in diagnosis.
    [Abstract] [Full Text] [Related] [New Search]