A 10yo girl presents with pallor and features of renal failure. She has hematuria as well as
proteinuria. The serum urea and creat are elevated. These symptoms started after an episode of
bloody diarrhea 4days ago. What is the most probable dx?
a. TTP
b. HUS
c. ITP
d. HSP
e. ARF
answer: B
Haemolytic–uraemic syndrome
This is the commonest cause of AKI in children in Europe and the USA. It typically has a seasonal variation with peaks in the summer and autumn months. It presents with a triad of:
• microangiopathic haemolytic anaemia;
• thrombocytopenia;
• acute renal failure.
Two forms of HUS are recognized.
• Atypical/sporadic:
• not diarrhoea-associated (D– HUS);
• often familial.
• Epidemic form:
• diarrhoea-associated (D+ HUS);
• commonly associated with verocytotoxic producing E. coli
Clinical features
Severe thrombocytopenia: purpura, epistaxis, hematuria, hemoptysis, GI bleed
Acute renal failure
Gut
• Prodrome of bloody diarrhoea.
• Rectal prolapse.
• Haemorrhagic colitis.
• Bowel wall necrosis and perforation.
Pancreas (occurs in <10%)
• Glucose intolerance/insulin-dependent diabetes mellitus.
• Pancreatitis.
• Liver jaundice.
• Neurological Irritability to frank encephalopathy.
Myocarditis (rare)
Investigations
• FBC + film.
• Blood cultures.
• U&E.
• LFTs.
• E. coli polymerase chain reaction (PCR).
• Stools: microscopy and culture.
Treatment
Early liaison with a paediatric nephrology unit is required, as early dialysis may be needed. Management is mainly supportive and directed at treating the clinical features of HUS. Antibiotics for underlying E. coli infection are not indicated.
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