Haemolytic uraemic syndrome

Overview 
Theory 
Diagnosis 
Management 
Follow up 
Resources 

Key diagnostic factors

common

diarrhoea, especially bloody diarrhoea

Diarrhoea precedes the diagnosis of Shiga toxin-producing Escherichia coli (STEC) HUS in 91% of cases, and bloody diarrhoea is seen in 57% of cases.[37][38] HUS is diagnosed in approximately 15% of children with STEC infections, approximately 5 to 10 days after the onset of diarrhoea.[8][9][13][14]

childhood, especially age <5 years

The incidence of Shiga toxin-producing Escherichia coli (STEC) HUS is highest in children <5 years of age.[7]

Other diagnostic factors

uncommon

known community outbreak of Shiga toxin-producing E coli

Although large epidemics of E coli O157:H7 have been reported, most cases are sporadic or occur in small clusters.[8][9][10]

history of ingestion of food that may have been contaminated with Shiga toxin-producing E coli

There may be a history of ingestion of undercooked meat, cheese, poultry, vegetables, or water that has been contaminated with Shiga toxin-producing E coli.[20][21]

unusual adverse effect following treatment with ciclosporin, some chemotherapy agents, targeted cancer agents, and quinine

Thrombotic microangiopathy has been associated with ciclosporin and other immunosuppressive drugs (ticlopidine, quinine, mitomycin).[26] It is also infrequently associated with other chemotherapeutic agents and miscellaneous drugs.[19][27]

pregnancy or postpartum status

Most typically seen in primiparas who present at a median of 26 days post delivery, but can be difficult to distinguish from other causes of thrombocytopenia.[28][29]

unusual adverse effect following bone marrow transplant

Thrombotic microangiopathy occurs as an adverse effect in 5% to 15% of patients following allogeneic bone marrow transplant and <1% of patients following autologous bone marrow transplant, generally several months after the procedure.[25]

family history of possible HUS-like syndrome

An estimated 50% of patients with familial and sporadic HUS are found to have defects in one of the components of the alternative complement pathway.[6]

Risk factors

strong

ingestion of contaminated food or water

Although the ingestion of undercooked meat has received the most widespread attention, outbreaks of Shiga toxin-producing Escherichia coli(STEC) HUS have been related to the ingestion of many other contaminated substances, including cheese, poultry, vegetables, or water.[20][21]

known community outbreak of toxicogenic E coli

Although large epidemics of E coli O157:H7 have been reported, most cases are sporadic or occur in small clusters.[8][9][10]

exposure to infected individuals in institutional settings

Person-to-person transmission of Shiga toxin-producing E coli (STEC) has been described in day-care centres, nursing homes, and other institutions.[22][23]

genetic predisposition (atypical HUS)

Seen in familial syndromes and some sporadic cases, this can be autosomally dominant or recessive. Abnormalities have been found in the quantity and function of factor H, factor I, and membrane co-factor protein, all of which are involved in the regulation of complement.[6][24]

weak

bone marrow transplant

Thrombotic microangiopathy occurs in 5% to 15% of patients following allogeneic bone marrow transplant and <1% of patients following autologous bone marrow transplant, generally several months after the procedure.[25]

exposure to ciclosporin, some chemotherapy agents, targeted cancer agents, and quinine

Thrombotic microangiopathy has been associated with ciclosporin and other immunosuppressive drugs (e.g., ticlopidine, quinine, mitomycin).[26] It is also infrequently associated with other chemotherapeutic agents and other miscellaneous drugs.[27] There have been reports of thrombotic microangiopathy associated with the use of targeted cancer agents (e.g., immunotoxins, monoclonal antibodies, and tyrosine kinase inhibitors).[19]

pregnancy- or postpartum-related