U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Shiga toxin-associated hemolytic uremic syndrome

MedGen UID:
383843
•Concept ID:
C1856143
•
Disease or Syndrome
Synonym: Typical hemolytic-uremic syndrome
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019536
OMIM®: 235400
Orphanet: ORPHA90038

Definition

A rare thrombotic microangiopathy characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction that is usually associated with prodromal enteritis caused by <i>Shigella dysentriae</i> type 1 or <i>E. Coli</i>. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVShiga toxin-associated hemolytic uremic syndrome

Professional guidelines

PubMed

Shiga Toxin-Associated Hemolytic Uremic Syndrome: Specificities of Adult Patients and Implications for Critical Care Management.
Travert B, Rafat C, Mariani P, Cointe A, Dossier A, Coppo P, Joseph A
Toxins (Basel) 2021 Apr 26;13(5) doi: 10.3390/toxins13050306. PMID: 33925836Free PMC Article
The efficacy of recombinant human soluble thrombomodulin for the treatment of shiga toxin-associated hemolytic uremic syndrome model mice.
Suyama K, Kawasaki Y, Miyazaki K, Kanno S, Ono A, Ohara S, Sato M, Hosoya M
Nephrol Dial Transplant 2015 Jun;30(6):969-77. Epub 2015 Feb 17 doi: 10.1093/ndt/gfv004. PMID: 25694534
Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS).
Scheiring J, Andreoli SP, Zimmerhackl LB
Pediatr Nephrol 2008 Oct;23(10):1749-60. Epub 2008 Aug 13 doi: 10.1007/s00467-008-0935-6. PMID: 18704506Free PMC Article

Recent clinical studies

Etiology

Shiga Toxin-Associated Hemolytic Uremic Syndrome in Adults, France, 2009-2017.
Travert B, Dossier A, Jamme M, Cointe A, Delmas Y, Malot S, Wynckel A, Seguin A, Presne C, Hie M, Benhamou Y, Ribes D, Choukroun G, Grangé S, Hertig A, Le Gall EC, Galicier L, Daugas E, Bouadma L, Weill FX, Azoulay E, Fakhouri F, Veyradier A, Bonacorsi S, Hogan J, Frémeaux-Bacchi V, Rondeau E, Mariani-Kurkdjian P, Coppo P; Centre de Référence des Microangiopathies Thrombotiques2
Emerg Infect Dis 2021;27(7):1876-1885. doi: 10.3201/eid2707.204638. PMID: 34152955Free PMC Article
Severely ill pediatric patients with Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS) who suffered from multiple organ involvement in the early stage.
Luna M, Kamariski M, Principi I, Bocanegra V, Vallés PG
Pediatr Nephrol 2021 Jun;36(6):1499-1509. Epub 2020 Nov 17 doi: 10.1007/s00467-020-04829-4. PMID: 33205220
Is Plasma Exchange Efficacious in Shiga Toxin-Associated Hemolytic Uremic Syndrome? A Narrative Review of Current Evidence.
Keenswijk W, Raes A, De Clerck M, Vande Walle J
Ther Apher Dial 2019 Apr;23(2):118-125. Epub 2018 Nov 19 doi: 10.1111/1744-9987.12768. PMID: 30324646
Early Differentiation of Shiga Toxin-Associated Hemolytic Uremic Syndrome in Critically Ill Adults With Thrombotic Microangiopathy Syndromes.
Joseph A, Rafat C, Zafrani L, Mariani-Kurkdjian P, Veyradier A, Hertig A, Rondeau E, Mariotte E, Azoulay E
Crit Care Med 2018 Sep;46(9):e904-e911. doi: 10.1097/CCM.0000000000003292. PMID: 29979220
Shiga toxin-associated hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: distinct mechanisms of pathogenesis.
Tarr PI
Kidney Int Suppl 2009 Feb;(112):S29-32. doi: 10.1038/ki.2008.615. PMID: 19180128

Diagnosis

Heparin-induced thrombocytopenia following Shiga-toxin-associated hemolytic uremic syndrome: a case report.
Rigamonti E, Bonora T, Ventresca M, Cippà P
J Med Case Rep 2022 Oct 23;16(1):384. doi: 10.1186/s13256-022-03627-w. PMID: 36273193Free PMC Article
Shiga Toxin-Associated Hemolytic Uremic Syndrome: Specificities of Adult Patients and Implications for Critical Care Management.
Travert B, Rafat C, Mariani P, Cointe A, Dossier A, Coppo P, Joseph A
Toxins (Basel) 2021 Apr 26;13(5) doi: 10.3390/toxins13050306. PMID: 33925836Free PMC Article
Shiga Toxin-Associated Hemolytic Uremic Syndrome: A Narrative Review.
Joseph A, Cointe A, Mariani Kurkdjian P, Rafat C, Hertig A
Toxins (Basel) 2020 Jan 21;12(2) doi: 10.3390/toxins12020067. PMID: 31973203Free PMC Article
Anti-factor H autoantibodies assay.
Dragon-Durey MA, Blanc C, Roumenina LT, Poulain N, Ngo S, Bordereau P, Frémeaux-Bacchi V
Methods Mol Biol 2014;1100:249-56. doi: 10.1007/978-1-62703-724-2_20. PMID: 24218265
Shiga toxin-associated hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: distinct mechanisms of pathogenesis.
Tarr PI
Kidney Int Suppl 2009 Feb;(112):S29-32. doi: 10.1038/ki.2008.615. PMID: 19180128

Therapy

Heparin-induced thrombocytopenia following Shiga-toxin-associated hemolytic uremic syndrome: a case report.
Rigamonti E, Bonora T, Ventresca M, Cippà P
J Med Case Rep 2022 Oct 23;16(1):384. doi: 10.1186/s13256-022-03627-w. PMID: 36273193Free PMC Article
Is Plasma Exchange Efficacious in Shiga Toxin-Associated Hemolytic Uremic Syndrome? A Narrative Review of Current Evidence.
Keenswijk W, Raes A, De Clerck M, Vande Walle J
Ther Apher Dial 2019 Apr;23(2):118-125. Epub 2018 Nov 19 doi: 10.1111/1744-9987.12768. PMID: 30324646
Shiga toxin associated hemolytic uremic syndrome.
Keir LS
Hematol Oncol Clin North Am 2015 Jun;29(3):525-39. doi: 10.1016/j.hoc.2015.01.007. PMID: 26043390
Anti-factor H autoantibody-associated hemolytic uremic syndrome: review of literature of the autoimmune form of HUS.
Dragon-Durey MA, Blanc C, Garnier A, Hofer J, Sethi SK, Zimmerhackl LB
Semin Thromb Hemost 2010 Sep;36(6):633-40. Epub 2010 Sep 23 doi: 10.1055/s-0030-1262885. PMID: 20865640
Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS).
Scheiring J, Andreoli SP, Zimmerhackl LB
Pediatr Nephrol 2008 Oct;23(10):1749-60. Epub 2008 Aug 13 doi: 10.1007/s00467-008-0935-6. PMID: 18704506Free PMC Article

Prognosis

Shiga Toxin-Associated Hemolytic Uremic Syndrome: Specificities of Adult Patients and Implications for Critical Care Management.
Travert B, Rafat C, Mariani P, Cointe A, Dossier A, Coppo P, Joseph A
Toxins (Basel) 2021 Apr 26;13(5) doi: 10.3390/toxins13050306. PMID: 33925836Free PMC Article
Is Plasma Exchange Efficacious in Shiga Toxin-Associated Hemolytic Uremic Syndrome? A Narrative Review of Current Evidence.
Keenswijk W, Raes A, De Clerck M, Vande Walle J
Ther Apher Dial 2019 Apr;23(2):118-125. Epub 2018 Nov 19 doi: 10.1111/1744-9987.12768. PMID: 30324646
Shiga toxin-associated hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: distinct mechanisms of pathogenesis.
Tarr PI
Kidney Int Suppl 2009 Feb;(112):S29-32. doi: 10.1038/ki.2008.615. PMID: 19180128
Treatment and outcome of Shiga-toxin-associated hemolytic uremic syndrome (HUS).
Scheiring J, Andreoli SP, Zimmerhackl LB
Pediatr Nephrol 2008 Oct;23(10):1749-60. Epub 2008 Aug 13 doi: 10.1007/s00467-008-0935-6. PMID: 18704506Free PMC Article
Shiga toxin-associated hemolytic uremic syndrome: absence of recurrence after renal transplantation.
Ferraris JR, Ramirez JA, Ruiz S, Caletti MG, Vallejo G, Piantanida JJ, Araujo JL, Sojo ET
Pediatr Nephrol 2002 Oct;17(10):809-14. Epub 2002 Aug 10 doi: 10.1007/s00467-002-0936-9. PMID: 12376808

Clinical prediction guides

Shiga Toxin-Associated Hemolytic Uremic Syndrome in Adults, France, 2009-2017.
Travert B, Dossier A, Jamme M, Cointe A, Delmas Y, Malot S, Wynckel A, Seguin A, Presne C, Hie M, Benhamou Y, Ribes D, Choukroun G, Grangé S, Hertig A, Le Gall EC, Galicier L, Daugas E, Bouadma L, Weill FX, Azoulay E, Fakhouri F, Veyradier A, Bonacorsi S, Hogan J, Frémeaux-Bacchi V, Rondeau E, Mariani-Kurkdjian P, Coppo P; Centre de Référence des Microangiopathies Thrombotiques2
Emerg Infect Dis 2021;27(7):1876-1885. doi: 10.3201/eid2707.204638. PMID: 34152955Free PMC Article
Is Plasma Exchange Efficacious in Shiga Toxin-Associated Hemolytic Uremic Syndrome? A Narrative Review of Current Evidence.
Keenswijk W, Raes A, De Clerck M, Vande Walle J
Ther Apher Dial 2019 Apr;23(2):118-125. Epub 2018 Nov 19 doi: 10.1111/1744-9987.12768. PMID: 30324646
The efficacy of recombinant human soluble thrombomodulin for the treatment of shiga toxin-associated hemolytic uremic syndrome model mice.
Suyama K, Kawasaki Y, Miyazaki K, Kanno S, Ono A, Ohara S, Sato M, Hosoya M
Nephrol Dial Transplant 2015 Jun;30(6):969-77. Epub 2015 Feb 17 doi: 10.1093/ndt/gfv004. PMID: 25694534
Outcome of renal transplantation in patients with non-Shiga toxin-associated hemolytic uremic syndrome: prognostic significance of genetic background.
Bresin E, Daina E, Noris M, Castelletti F, Stefanov R, Hill P, Goodship TH, Remuzzi G; International Registry of Recurrent and Familial HUS/TTP
Clin J Am Soc Nephrol 2006 Jan;1(1):88-99. Epub 2005 Nov 2 doi: 10.2215/CJN.00050505. PMID: 17699195
Shiga toxin-associated hemolytic uremic syndrome: absence of recurrence after renal transplantation.
Ferraris JR, Ramirez JA, Ruiz S, Caletti MG, Vallejo G, Piantanida JJ, Araujo JL, Sojo ET
Pediatr Nephrol 2002 Oct;17(10):809-14. Epub 2002 Aug 10 doi: 10.1007/s00467-002-0936-9. PMID: 12376808

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...