Haemolytic Uraemic Syndrome (HUS)

Haemolytic uraemic syndrome (HUS) is a combination of three things: destruction of red blood cells, a low platelet count and acute kidney injury. Tiny clots form in the smallest blood vessels, above all in the kidneys.

The common form follows a gut infection with Shiga toxin producing E. coli (STEC) and mainly affects young children. Atypical HUS is a rare disorder of the complement system that can strike at any age and tends to recur. Both are medical emergencies managed in hospital.

Symptome

• Diarrhoea, often bloody, with stomach cramps, beginning 5 to 10 days before the kidney problems
• Pallor, tiredness and irritability
• Passing much less urine, or none
• Puffy face, hands or feet
• Bruising or tiny skin bleeds
• Yellow tinge to the skin
• High blood pressure
• Drowsiness, confusion or seizures in severe cases

Ursachen und Risikofaktoren

STEC infection, most famously E. coli O157, comes from undercooked minced beef, unpasteurised milk or juice, contaminated salad and sprouts, petting farms and person to person spread. About 5 to 15 percent of infected children develop HUS. Antibiotics and anti-diarrhoeal medicines during the diarrhoeal phase may increase that risk. Atypical HUS arises from inherited or acquired faults in complement regulation and is triggered by infection, pregnancy, transplantation or certain medicines. Invasive pneumococcal infection is a further rare cause in children.

Wie es diagnostiziert wird

• Blood count and film: Anaemia with fragmented red cells (schistocytes) and low platelets.
• Haemolysis markers: High LDH, low haptoglobin, raised bilirubin, with a negative direct antiglobulin test.
• Kidney function and urine tests: Raised creatinine, blood and protein in the urine.
• Stool culture and Shiga toxin PCR: Identifies STEC; the sample should be sent early, as the organism clears quickly.
• ADAMTS13 activity: Distinguishes HUS from thrombotic thrombocytopenic purpura, which needs different urgent treatment.
• Complement studies and genetics: Requested when no STEC is found or the illness recurs.

Behandlungsmöglichkeiten

• Supportive hospital care: Careful fluid and salt balance, blood pressure control and nutrition. This is the mainstay for STEC-HUS.
• Dialysis: Needed for a period by around half of children with STEC-HUS. Most come off it as the kidneys recover.
• Red cell transfusion: For significant anaemia. Platelets are avoided unless there is active bleeding or a procedure.
• Complement inhibitors: Eculizumab or ravulizumab for atypical HUS, started promptly. Meningococcal vaccination and antibiotic cover are required.
• Plasma exchange: A bridge while the diagnosis is uncertain or where complement inhibitors are unavailable.
• Long-term follow-up: Blood pressure, urine protein and kidney function checked for years, since late problems can surface after apparent recovery.

Wenn es dringend ist

Bloody diarrhoea in a child, followed by pallor, reduced urine, swelling, unusual sleepiness or a seizure, needs immediate assessment at the nearest hospital with paediatric facilities. This is never a situation for travel.

Reisen nach Türkiye zur Behandlung

Acute HUS is treated in the nearest capable hospital and a patient in that phase must not fly. Travel has a limited later role: complement genetic testing and a specialist opinion after recovery, advice about relapse risk before pregnancy, or transplant planning in atypical HUS, where complement inhibitor cover has to be arranged in advance. Long-term monitoring stays with your local kidney team.

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