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Start free with EleplanShiga toxin-associated hemolytic uremic syndrome
ORPHA:90038Clinical subtype
Also called D+ HUS · EHEC-HUS · Hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli · Hemolytic uremic syndrome with diarrhea · STEC-HUS · Shiga-like toxin-associated HUS · Stx-HUS · Typical HUS · Typical hemolytic uremic syndrome
What it is
A rare thrombotic microangiopathy characterized by mechanical hemolytic anemia, thrombocytopenia, and renal dysfunction that is usually associated with prodromal enteritis caused by Shigella dysentriae type 1 or E. Coli.
Key facts
- Prevalence
- 1-9 / 100 000 (annual incidence, United States)
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Clinical subtype
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
10Sometimes5–29%
16- Abnormality of the nervous system
- Acute colitis
- Bloody diarrhea
- Dehydration
- Excessive daytime somnolence
- Hemoglobinuria
- Hypertension
- Hypokalemia
and 8 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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