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Fitzpatrick (BMJ Vol 318, Page 684) is to be commended for reviewing
some of the important features of Haemolytic uraemic syndrome (HUS) and
its link to E coli O157 infection. From our experience of the 1996 Central
Scotland outbreak we think that some points require clarification.
We feel that this article gives a paediatric perspective on this
disease and fails to recognise the different manifestations of E coli O157
related thrombotic microangiopathy (TMA) in children and adults. Firstly
it is important to recognise that in the context of E coli O157 infection,
Shiga toxin mediated TMA leads to the overlapping syndromes of HUS and
thrombotic thrombocytopaenic purpura (TTP) [1]. We have taken the term HUS
when used in this editorial to refer to the syndrome of HUS/TTP. We would
disagree with the statement that HUS after E coli O157 infection occurs
generally in children, as is well reported that HUS/TTP occurs equally at
both extremes of age. The Central Scotland outbreak demonstrated this
quite clearly. The incidence of HUS/TTP was 33% in children (less than 15
years of age), 34% in the elderly (over 65 years of age) and 13% in cases
between these ages. Fitzpatrick states that in the Scottish outbreak
deaths occurred mainly in the elderly, whereas deaths occurred exclusively
in patients over 69 years of age in whom the mortality rate was 28%. Most
patients who died had HUS/TTP and the mortality rate associated with this
condition was 42%. It is however erroneous to say that deaths were from
renal failure as only three of the 18 deaths were from acute renal
failure. Cardiorespiratory and neurological complications were more common
causes of death. It is also our observation, from the Central Scotland
outbreak, that the syndrome of HUS/TTP seems to follow a different pattern
in adults as opposed to children. Children with HUS/TTP developed acute
renal failure as an early manifestation of this syndrome and we agree that
dialysis is the most appropriate and effective treatment in this age
group. In adults however, renal disease progressed more slowly and adults
were more likely to develop neurological or cardiorespiratory problems
before the onset of acute renal failure requiring dialysis. We believe
therefore that a therapeutic window for the management of non-renal
sequelae of HUS/TTP exists in adult patients. Our experience of the use of
Therapeutic Plasma Exchange [TPE] in adults within this window period
suggests that it may be effective in reducing the progression of HUS/TTP
to acute renal failure and death [In Press]. We would therefore challenge
the statement that no specific intervention for HUS/TTP exists. We concede
that the role of TPE needs to be determined definitively. In summary
therefore we feel that it is important not to extrapolate that the
accepted management of HUS/TTP in children can be extended to adults in
whom the disease has different clinical manifestations and a dramatically
different mortality rate.
Re: Haemolytic uraemic syndrome and E coli O157
Fitzpatrick (BMJ Vol 318, Page 684) is to be commended for reviewing
some of the important features of Haemolytic uraemic syndrome (HUS) and
its link to E coli O157 infection. From our experience of the 1996 Central
Scotland outbreak we think that some points require clarification.
We feel that this article gives a paediatric perspective on this
disease and fails to recognise the different manifestations of E coli O157
related thrombotic microangiopathy (TMA) in children and adults. Firstly
it is important to recognise that in the context of E coli O157 infection,
Shiga toxin mediated TMA leads to the overlapping syndromes of HUS and
thrombotic thrombocytopaenic purpura (TTP) [1]. We have taken the term HUS
when used in this editorial to refer to the syndrome of HUS/TTP. We would
disagree with the statement that HUS after E coli O157 infection occurs
generally in children, as is well reported that HUS/TTP occurs equally at
both extremes of age. The Central Scotland outbreak demonstrated this
quite clearly. The incidence of HUS/TTP was 33% in children (less than 15
years of age), 34% in the elderly (over 65 years of age) and 13% in cases
between these ages. Fitzpatrick states that in the Scottish outbreak
deaths occurred mainly in the elderly, whereas deaths occurred exclusively
in patients over 69 years of age in whom the mortality rate was 28%. Most
patients who died had HUS/TTP and the mortality rate associated with this
condition was 42%. It is however erroneous to say that deaths were from
renal failure as only three of the 18 deaths were from acute renal
failure. Cardiorespiratory and neurological complications were more common
causes of death. It is also our observation, from the Central Scotland
outbreak, that the syndrome of HUS/TTP seems to follow a different pattern
in adults as opposed to children. Children with HUS/TTP developed acute
renal failure as an early manifestation of this syndrome and we agree that
dialysis is the most appropriate and effective treatment in this age
group. In adults however, renal disease progressed more slowly and adults
were more likely to develop neurological or cardiorespiratory problems
before the onset of acute renal failure requiring dialysis. We believe
therefore that a therapeutic window for the management of non-renal
sequelae of HUS/TTP exists in adult patients. Our experience of the use of
Therapeutic Plasma Exchange [TPE] in adults within this window period
suggests that it may be effective in reducing the progression of HUS/TTP
to acute renal failure and death [In Press]. We would therefore challenge
the statement that no specific intervention for HUS/TTP exists. We concede
that the role of TPE needs to be determined definitively. In summary
therefore we feel that it is important not to extrapolate that the
accepted management of HUS/TTP in children can be extended to adults in
whom the disease has different clinical manifestations and a dramatically
different mortality rate.
1.Mead PS, Griffin PM. Escherichia coli O157:H7. Lancet 1998;
352:1207-12.
Competing interests: No competing interests