Haemolytic uraemic syndrome
Description
- Thrombotic microangiopathy triad: microangiopathic haemolytic anaemia + thrombocytopenia + acute kidney injury
- Renal-predominant - the discriminator from TTP, which is neurological-predominant
| STEC-HUS ("typical") | Complement-mediated (atypical) HUS | Secondary TMA | |
|---|---|---|---|
| Share | ~90% of childhood HUS | ~5-10% | Common in adults |
| Trigger | *Shiga toxin - E. coli O157:H7 (and O104:H4), Shigella dysenteriae*** | Complement regulatory defect + a trigger | Drugs, pregnancy, malignancy, transplant, infection, autoimmune |
| Prodrome | Bloody diarrhoea | Often none | Underlying condition |
| Age | Children <5 | Any; ~60% before adulthood | Adults |
| Treatment | Supportive | Complement inhibition | Treat the cause |
| Outcome | Mostly recovers | Relapsing, progressive CKD | Variable |
- *Never a diagnosis of exclusion made slowly* - the immediate task is to separate it from TTP, because TTP needs plasma exchange within hours
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