Infection-related hemolytic uremic syndrome

MedGen UID:: 1826072
•Concept ID:: C5680165
•: Disease or Syndrome

Synonyms:	infection-related hemolytic uremic syndrome; Infection-related HUS; Infection-related HUS (hemolytic uremic syndrome)
SNOMED CT:	Infection-related HUS (hemolytic uremic syndrome) (1269270002); Infection-related hemolytic uremic syndrome (1269270002)
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:0034103
Orphanet:	ORPHA544482

Definition

A rare type of hemolytic uremic syndrome (HUS) characterized by the triad of hemolytic anemia due to generalized thrombotic microangiopathy, thrombocytopenia and acute kidney injury. The disease most commonly occurs after acute gastroenteritis due to Shiga toxin-producing enterohemorrhagic Escherichia coli or Shigella dysenteriae. Other infectious causes of HUS include Streptococcus pneumoniae, HIV, Mycoplasma pneumoniae, Histoplasmosis and Coxsackie virus. [from SNOMEDCT_US]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVInfection-related hemolytic uremic syndrome

Congenital Systemic Disorder
- Abnormality of the genitourinary system
  - Abnormality of the urinary system
    - Abnormality of the urinary system physiology
      - Abnormal renal physiology
        Hemolytic-uremic syndrome
        Infection-related hemolytic uremic syndrome
        Shiga toxin-associated hemolytic uremic syndrome
        Streptococcus pneumoniae-associated hemolytic uremic syndrome

Recent clinical studies

Etiology

Complement-driven hemolytic uremic syndrome.

Leon J, LeStang MB, Sberro-Soussan R, Servais A, Anglicheau D, Frémeaux-Bacchi V, Zuber J
Am J Hematol 2023 May;98 Suppl 4:S44-S56. doi: 10.1002/ajh.26854. PMID: 36683290

A community outbreak of haemolytic-uraemic syndrome in children occurring in a large area of northern Italy over a period of several months.

Tozzi AE, Niccolini A, Caprioli A, Luzzi I, Montini G, Zacchello G, Gianviti A, Principato F, Rizzoni G
Epidemiol Infect 1994 Oct;113(2):209-19. doi: 10.1017/s0950268800051645. PMID: 7925660 Free PMC Article

See all (2)

Diagnosis

Treatment of hemolytic uremic syndrome related to Bordetella pertussis infection -is plasma exchange or eculizumab use necessary?

Saida K, Ogura M, Kano Y, Ishimori S, Yoshikawa T, Nagata H, Sato M, Kamei K, Ishikura K
BMC Nephrol 2018 Dec 17;19(1):365. doi: 10.1186/s12882-018-1168-y. PMID: 30558570 Free PMC Article

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Therapy

Real-world safety profile of eculizumab in patients with paroxysmal nocturnal hemoglobinuria, atypical hemolytic uremic syndrome, or generalized myasthenia gravis: an integrated analysis of post-marketing surveillance in Japan.

Nishimura JI, Kawaguchi T, Ito S, Murai H, Shimono A, Matsuda T, Fukamizu Y, Akiyama H, Hayashi H, Nakano T, Maruyama S
Int J Hematol 2023 Oct;118(4):419-431. Epub 2023 Jul 29 doi: 10.1007/s12185-023-03630-x. PMID: 37515657

Treatment of hemolytic uremic syndrome related to Bordetella pertussis infection -is plasma exchange or eculizumab use necessary?

Saida K, Ogura M, Kano Y, Ishimori S, Yoshikawa T, Nagata H, Sato M, Kamei K, Ishikura K
BMC Nephrol 2018 Dec 17;19(1):365. doi: 10.1186/s12882-018-1168-y. PMID: 30558570 Free PMC Article

Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome.

Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, Bingham C, Cohen DJ, Delmas Y, Douglas K, Eitner F, Feldkamp T, Fouque D, Furman RR, Gaber O, Herthelius M, Hourmant M, Karpman D, Lebranchu Y, Mariat C, Menne J, Moulin B, Nürnberger J, Ogawa M, Remuzzi G, Richard T, Sberro-Soussan R, Severino B, Sheerin NS, Trivelli A, Zimmerhackl LB, Goodship T, Loirat C
N Engl J Med 2013 Jun 6;368(23):2169-81. doi: 10.1056/NEJMoa1208981. PMID: 23738544

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Prognosis

Complement-driven hemolytic uremic syndrome.

Leon J, LeStang MB, Sberro-Soussan R, Servais A, Anglicheau D, Frémeaux-Bacchi V, Zuber J
Am J Hematol 2023 May;98 Suppl 4:S44-S56. doi: 10.1002/ajh.26854. PMID: 36683290

Dominant C3 glomerulopathy: new roles for an old actor in renal pathology.

Pirozzi N, Stoppacciaro A, Menè P
J Nephrol 2018 Aug;31(4):503-510. Epub 2017 Nov 18 doi: 10.1007/s40620-017-0458-y. PMID: 29151252

A community outbreak of haemolytic-uraemic syndrome in children occurring in a large area of northern Italy over a period of several months.

See all (3)