+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis

, : Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis. Clinical Advances in Hematology & Oncology 14 Suppl 11(11): 2-15

NlmCategory="UNASSIGNED">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.


PMID: 27930620

Other references

González-García, M.; Cuacos, Mía.; González-Sánchez, Mónica.; Puertas, Mía.J.; Vega, J.M., 2011: Painting the rye genome with genome-specific sequences. We used rye-specific repetitive DNA sequences in fluorescence in situ hybridization (FISH) to paint the rye genome and to identify rye DNA in a wheat background. A 592 bp fragment from the rye-specific dispersed repetitive family R173 (named UCM60...

Bielecki, C.; Bocklitz, T.W.; Schmitt, M.; Krafft, C.; Marquardt, C.; Gharbi, A.; Knösel, T.; Stallmach, A.; Popp, J., 2013: Classification of inflammatory bowel diseases by means of Raman spectroscopic imaging of epithelium cells. We report on a Raman microspectroscopic characterization of the inflammatory bowel diseases (IBD) Crohn's disease (CD) and ulcerative colitis (UC). Therefore, Raman maps of human colon tissue sections were analyzed by utilizing innovative che...

Hall, J.P.; Jones, L.L., 1973: Costs of solid waste management in rural communities in Texas. A 1969 amendment to the Texas Solid Waste Disposal Act established minimum legal standards for disposal operations in rural Texas communities. The findings are presented from a survey used to obtain information on cost and quality characteristics...

Constable, P.D.;, G.; Hull, B.L.; Rings, D.M.; Morin, D.E.; Nelson, D.R., 1997: Intussusception in cattle: 336 cases (1964-1993). Medical records of cattle admitted to 17 veterinary medical teaching hospitals in North America were reviewed. 336 cattle with intussusception were identified; 281 had small intestinal, 7 had ileocolic, 12 had caecocolic, and 36 had colocolic intu...

Fabian, E.; Havránek, F.; Stork, A.; Fabianová, J., 1971: Lipoprotein lipase and postheparin esterase activity and postheparin increase in free fatty acids after the short-term administration of a sequential contraceptive. American Journal of Obstetrics and Gynecology 109(8): 1212-1213

Munro, Cindy L., 1998: The rat model of endocarditis. The rat model of endocarditis is a well established experimental protocol which closely approximates human native valve endocarditis. The rat model of endocarditis has been used to examine the role of particular streptococcal virulence factors, to...

Kuznetsov, V.V.; Andreeva, E.N.; Prostakov, N.S., 1995: Synthesis and pesticide activity of 1,2,3,4-tetrahydroquinoline. Some new 4-methyl- containing propyl, phenyl and pyridyl radicals at C-2 position were synthesized from readily available N-aryl-N-alkenylamines. The tatters and tetrahydroquinolines obtained were tested for bactericida...

Stulz, P.; Zimmerli, W.; Mihatsch, J.; Grädel, E., 1989: Recurrent infective endocarditis in idiopathic hypertrophic subaortic stenosis. A patient with idiopathic hypertrophic subaortic stenosis suffered from five episodes of infective endocarditis of the mitral valve. At least two of these episodes were caused by Lactobacillus species, a microorganism known to be difficult to erad...

Todisco, T.; Eslami, A.; Baglioni, S.; Dottorini, M.; Sposini, T.; Wunderlich, E.; Knoch, M., 1997: Characterization and biological activity of new aerosolized drugs in pneumology. European Respiratory Journal Supplement 10(25): 235S, Sept

Papademetriou, Mk, 1976: Percentage fruit set in avocados (Persea americana Mill.). Yearbook of the California Avocado Society for the year: 9 135-142