raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:544482

Infection-related hemolytic uremic syndrome

Synonyms: Infection-related HUS

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
All ages
Status
active

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, and most commonly occurring after acute gastroenteritis due to Shiga toxin-producing enterohemorrhagic <i>Escherichia coli</i> or <i>Shigella dysenteriae</i>. Other infectious causes of HUS include <i>Streptococcus pneumoniae</i>, HIV, <i>Mycoplasma pneumoniae</i>, Histoplasmosis, and Coxsackie virus.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enInfection-related hemolytic uremic syndromeInfection-related HUS
frSyndrome hémolytique et urémique dû à une infectionSHU dû à une infection
deInfektions-assoziiertes hämolytisch-urämisches SyndromInfektion-bedingtes HUS
esSíndrome hemolítico urémico asociado a infecciónSHU asociado a infección
itSindrome emolitico-uremica correlata ad infezioneSEU correlata alle infezioni
nlInfectie-gerelateerd hemolytisch-uremisch syndroomInfectie-gerelateerd HUS
ptSíndrome hemolítico-urémico infecção-relacionadoSHU infecção-relacionado
plZespół hemolityczno-mocznicowy związany z infekcją= en
csHemolyticko-uremický syndrom související s infekcíHUS související s infekcí
ukІнфекційний гемолітико-уремічний синдром, повязаний з інфекцієюHUS, повязаний з інфекцією
zh感染性溶血性尿毒症综合征= en
trEnfeksiyon-ilişkili hemolitik üremik sendromEnfeksiyon-ilişkili HUS

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D59.3NTBTattributedvalidated
ICD-113A21.YNTBTindexvalidated
MONDO0034103Evalidated
UMLSC5680165Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

Children (2):

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyValidatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

52 annotations · validated 2021-06-09
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0001919Acute kidney injury
HP:0012378Fatigue
HP:0001878Hemolytic anemia
HP:0000980Pallor
HP:0032169Severe infection
HP:0001873Thrombocytopenia
Frequent (79-30%)
HP:0032155Abdominal cramps
HP:0002027Abdominal pain
HP:0011115Abnormality of chemokine secretion
HP:0011116Abnormality of interferon secretion
HP:0000707Abnormality of the nervous system
HP:0100282Acute colitis
HP:0011037Decreased urine output
HP:0002014Diarrhea
HP:0001945Fever
HP:0030783Increased circulating interleukin 6 concentration
HP:0001974Increased total leukocyte count
HP:0011947Respiratory tract infection
Occasional (29-5%)
HP:0100519Anuria
HP:0031864Bacteremia
HP:0025085Bloody diarrhea
HP:0002094Dyspnea
HP:0000969Edema
HP:0002153Hyperkalemia
HP:0000822Hypertension
HP:0100735Hypertensive crisis
HP:0002901Hypocalcemia
HP:0002902Hyponatremia
HP:0001287Meningitis
HP:0012819Myocarditis
HP:0002018Nausea
HP:0012593Nephrotic range proteinuria
HP:0100520Oliguria
HP:0001733Pancreatitis
HP:0002102Pleuritis
HP:0002090Pneumonia
HP:0004372Reduced consciousness
HP:0005208Secretory diarrhea
HP:0031691Severe viral infection
HP:0002013Vomiting
Very rare (<4-1%)
HP:0030049Brain abscess
HP:0100704Cerebral visual impairment
HP:0001259Coma
HP:0000819Diabetes mellitus
HP:0005244Gastrointestinal infarctions
HP:0007430Generalized edema
HP:0001269Hemiparesis
HP:0031368Intestinal perforation
HP:0002576Intussusception
HP:0011919Pleural empyema
HP:0001250Seizure
HP:0003095Septic arthritis

Run the differential on the 6 obligate / very frequent terms

Annotation source: 25593915[PMID]_27768015[PMID]_28242109[PMID]_31232894[PMID]_28711159[PMID]

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Sources for this card