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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.
 
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

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
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
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

Nester CM, Feldman DL, Burwick R, Cataland S, Chaturvedi S, Cook HT, Cuker A, Dixon BP, Fakhouri F, Hingorani SR, Java A, van de Kar NCAJ, Kavanagh D, Leung N, Licht C, Noris M, O'Shaughnessy MM, Parikh SV, Peyandi F, Remuzzi G, Smith RJH, Sperati CJ, Waldman M, Walker P, Vivarelli M
Kidney Int 2024 Sep;106(3):326-336. doi: 10.1016/j.kint.2024.05.021. PMID: 39174192
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
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
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
Tarr PI
Kidney Int Suppl 2009 Feb;(112):S29-32. doi: 10.1038/ki.2008.615. PMID: 19180128

Diagnosis

Nester CM, Feldman DL, Burwick R, Cataland S, Chaturvedi S, Cook HT, Cuker A, Dixon BP, Fakhouri F, Hingorani SR, Java A, van de Kar NCAJ, Kavanagh D, Leung N, Licht C, Noris M, O'Shaughnessy MM, Parikh SV, Peyandi F, Remuzzi G, Smith RJH, Sperati CJ, Waldman M, Walker P, Vivarelli M
Kidney Int 2024 Sep;106(3):326-336. doi: 10.1016/j.kint.2024.05.021. PMID: 39174192
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
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
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
Tarr PI
Kidney Int Suppl 2009 Feb;(112):S29-32. doi: 10.1038/ki.2008.615. PMID: 19180128

Therapy

Hambrick HR, Short K, Askenazi D, Krallman K, Pino C, Yessayan L, Westover A, Humes HD, Goldstein SL
Blood Purif 2023;52(9-10):812-820. Epub 2023 Aug 22 doi: 10.1159/000531963. PMID: 37607519
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
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
Keir LS
Hematol Oncol Clin North Am 2015 Jun;29(3):525-39. doi: 10.1016/j.hoc.2015.01.007. PMID: 26043390
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

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
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
Ko H, Maymani H, Rojas-Hernandez C
J Med Case Rep 2016 Jun 15;10:175. doi: 10.1186/s13256-016-0970-z. PMID: 27301547Free PMC Article
Tarr PI
Kidney Int Suppl 2009 Feb;(112):S29-32. doi: 10.1038/ki.2008.615. PMID: 19180128
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

Clinical prediction guides

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
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
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
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
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

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