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Haemolytic uraemic syndrome (HUS) is a rare thromboembolic complication observed in patients with cancer. It is characterised by the clinical triad of acute renal failure, microangiopathic haemolytic anaemia and thrombocytopaenia. It may be associated with a variety of aetiologies, including chemotherapeutic agents such as mitomycin, cisplatin, bleomycin, 5-fluorouracil and, most recently, gemcitabine. We report a 70-year-old patient treated with gemcitabine who developed haemolytic uraemic syndrome.

Diarrhoea-associated haemolytic uraemic syndrome develops in about 5 to 10% of children with haemorrhagic colitis due to Escherichia coli (E. coli) O157:H7 and is a common cause of acute renal failure in childhood. Endothelial cell damage, white blood cell activation and platelet-endothelial cell interactions are important in the pathogenesis. Meticulous supportive care, with attention to nutrition and fluid, and electrolyte balance, is important. Dialysis is necessary in many children. Public health follow-up is important to minimise the spread of E. coli O157:H7, which is transmitted by person-to-person, as well as through contaminated food products. 20-year follow-up studies report that 75% of children recover without any clinically significant long term sequelae. Chronic renal failure is reported in about 5% of children.

Fewer than 10% of children with Escherichia coli O157:H7 enteritis develop hemolytic-uremic syndrome (HUS).To determine whether circulating leukocytes are independent risk markers of developing HUS during E. coli O157:H7 enteritis.We reviewed the charts of all children with culture-proved E. coli O157:H7 infections seen at Sainte-Justine Hospital between 1987 and 1997. Epidemiologic data, laboratory indices and circulating leukocytes counts were noted. HUS diagnosis was validated with independent HUS patient lists from the pediatric nephrology services of tertiary care hospitals in the Montreal metropolitan area. The date of onset of enteritis was determined by two independent observers. Leukocyte counts were compared among the following independent groups: (1) uncomplicated O157:H7 enteritis (Group 1); (2) O157:H7 enteritis with the subsequent development of HUS (Group 2); (3) HUS already present at the time of medical consultation (Group 3).There were 369 children with E. coli O157:H7 infection. A complete blood count was not performed in 114 (31%) patients. Observers disagreed on the date of onset of gastroenteritis in 34 (9%) children only (kappa 0.92). The study population thus included 221 patients: Group 1, n = 161; Group 2, n = 27; and Group 3, n = 33. Patients developing HUS (Group 2) presented greater total leukocyte (P0.008), polymorphonuclear (P0.008) and monocyte (P0.07) counts than those with an uncomplicated course (Group 1). Logistic regression analysis showed that young age [odds ratio (OR), 0.98; 95% confidence interval (CI), 0.96 to 0.99], duration of enteric prodromeor =3 days (OR 4.8, 95% CI 1.13 to 20.7) and initial leukocytosis (OR 1.22, 95% CI, 1.11 to 1.35) were independent predictors of HUS.Based on the variables identified above, further studies are needed to determine whether the inflammatory response of the host represents only a marker of the severity of gastrointestinal infection or whether, alternatively, it is a pathophysiologic factor that leads to HUS.

Complement factor H auto-antibodies (CFH-ab) are a rare cause (6–10%) of atypical haemolytic uraemic syndrome (aHUS). All observations previously described were retrospective and occurred in children or teenagers. We report the first case of aHUS associated with anti-CFH antibodies in an adult patient who was successfully treated by plasma exchange, corticosteroids and rituximab.

Verocytotoxin-producing Escherichia coli (VTEC) O157 can cause haemolytic diarrhea and haemolytic uraemic syndrome, and most outbreaks caused by this strain have been food or water related. Cattle are known to be major reservoirs for VTEC including E. coli O157, and this strain has also been isolated from drinking water, treated water and sewage. E. coli O157 was frequently detected in natural river water by immunological and molecular biological methods, even if it was not detected by traditional culture methods. To clarify the physiological state of E. coli O157, flow cytometry was applied with the DVC or the vital staining method. Physiolocially active E. coli O157 was found to be distributed in natural river water. Bacteriophages carrying the sltII gene were also detected in sewage, and they could transform E. coli O157. These results indicate that monitoring not only bacterial cells but also slt genes is necessary to clarify the dynamics of E. coli O157 in natural environments, and examination of the viable but non-culturable state of E. coli O157 is also essential to prevent disease outbreaks.

Pulmonary haemorrhage is a potentially life-threatening event that may occur in patients with pulmonary-renal syndromes. These syndromes have typically been thought to occur in small-vessel vasculitides, such as ANCA-mediated disease, Goodpasture's disease and other autoimmune conditions including systemic lupus erythematosus or anti-phospholipid antibody syndrome. Here, we present a rare cause for pulmonary haemorrhage with associated renal failure—atypical haemolytic-uraemic syndrome. In this case, renal biopsy was integral to providing a diagnosis and guiding therapy.

Correspondence to: Dr. Toshiyuki Miyata Department of Molecular Pathogenesis National Cerebral and Cardiovascular Center 5-7–1 Fujishiro-dai, Suita, Osaka 565–8565, Japan Tel.: +81 6 6833 5012, Fax: +81 6 6835 1176 E-mail: miyata@ncvc.go.jp Financial support: This work was supported in part by grants-in-aid from the Ministry of Health, Labour and Welfare of Japan, the Japan Society for the Promotion of Science, and the Takeda Science Foundation.

Haemolytic-uraemic syndrome (HUS) is a clinical syndrome characterized by acute haemolytic anaemia with fragmented erythocytes, thrombocytopenia and acute renal failure. It is one of the leading causes of acute renal failure in childhood. HUS in children can be divided into the so-called typical, diarrhoea-associated HUS, and atypical HUS, which is not preceded by acute gastroenteritis. Infection with verocytotoxin-producing Escherichia coli is the main cause of diarrhoea-associated HUS. In this chapter the pathogenesis of diarrhoea-associated HUS and the role of verocytotoxin-producing Escherichia coli in this form of HUS is emphasized.

Haemolytic uraemic syndrome thrombocytopenic thrombotic purpura (HUS/TTP) remains an incompletely understood complex disease process that involves many organs. It was first described, as thrombocytopenic purpura, by Moschcowitz in 1924 (1). Since that time the prognosis of this disease has improved considerably, the short term mortality declining from 90% to 10%. Challenging questions remain, however, about clinical classification, pathophysiology, and therapy of the initial episode and later relapse. The present art and science of classification, pathophysiology and therapy, plus recommendations for future approaches are presented in this paper.

The haemolytic uraemic syndromes (HUS) are a heterogenous group of disorders which occur throughout the world and are the commonest cause of acute renal failure in childhood in North America and Western Europe. They are characterized by a microangiopathic haemolytic anaemia, thrombocytopenia and renal failure? Two broad subgroups are now recognized: the first is common in children and is associated with a diarrhoeal prodrome (D+ HUS), whereas the second is much rarer in childhood and not associated with antecedent diarrhoea (D- HUS). 2 D+ HUS is synonymous with typical, prototypic, epidemic or enteropathic HUS and D- HUS with atypical or sporadic disease.

Background. Renal insufficiency is increasingly common in end-stage liver disease and allocation of livers to this category of patient has escalated. The frequency of combined liver-kidney transplantation (CLKT) has consequently increased. Indications for CLKT in children differ from those for adults and typically include rare congenital conditions; subsequently limited numbers of this procedure have been performed in paediatric patients worldwide. Scant literature exists on the subject. Methods. Subsequent to institutional approval, a retrospective chart analysis of all paediatric CLKTs performed at the Transplant Unit, Wits Donald Gordon Medical Centre, University of the Witwatersrand, Johannesburg, South Africa between January 2005 and July 2013 was conducted. Results. Defining children as younger than 18 years of age, 43 patients had received a liver transplant since 2005, of whom 8 received a CLKT. Indications included autosomal recessive polycystic kidney disease ( n =3), primary hyperoxaluria type 1 ( n =4) and heterozygous factor H deficiency with atypical haemolytic uraemic syndrome ( n =1). Graft combinations included whole liver and one kidney ( n =5), whole liver and two kidneys ( n =1) and left lateral liver segment and one kidney ( n =2), all from deceased donors. Patient age ranged from 4 to 17 years (median 9) and included 4 females and 4 males. Weight ranged from 13 to 42 kg (median 22.5). We describe one in-hospital mortality. The remaining 7 patients were long-term survivors with a survival range from 6 to 65 months. Conclusions. Although rarely indicated in children, CLKT is an effective treatment option, appropriately utilising a scarce resource and significantly improving quality of life in the recipient.

The monoclonal anti-C5 antibody eculizumab has been successfully tested in atypical haemolytic-uraemic syndrome (aHUS), with or without mutations in the regulatory proteins of the alternative pathway of the complement, and less convincingly in enterohaemorrhagic Escherichia coli-associated HUS. Here, we report a patient with mitomycin-C-induced HUS unresponsive to plasma exchanges. Eculizumab infusion was followed by a dramatic improvement of haematological parameters and renal function, suggesting a role of complement blockade in the management of refractory, drug-related HUS.

Denmark faced an outbreak of verocytotoxin-producing E. coli (VTEC) O157:H7 infections in autumn 2012. Thirteen cases were diagnosed of which eight had haemolytic uraemic syndrome (HUS). Epidemiological investigations suggested ground beef as the vehicle of the outbreak. The outbreak strain had a rare toxin gene subtype profile: eae, vtx1a and vtx2a, and a high proportion of HUS (62%) among cases, a finding previously linked with the outbreak subtype profile. Toxin subtyping can be useful to identify high risk VTEC strains.

Background: Atypical haemolytic uraemic syndrome (aHUS) results from uncontrolled complement system activation. Complement factor H gene mutations are common causes of aHUS. Plasmatherapy, including plasma infusions and/or plasma exchanges, has been tried in this Setting with various successes. At present, we lack a specific marker to monitor functional factor H deficiency-related aHUS. Methods: We report the use of factor H functional assay in three patients-with atypical haemolytic uraemic syndrome. This assay is based on the requirement of soluble complement regulators that bind sheep red cells to prevent haemolysis. As factor H is highly abundant in the plasma, its defect results in haemolysis. Factor H activity was also measured among plasma donors. Results: One patient suffered from a plasma-dependent form of atypical haemolytic uraemic,syndrome, Plasma exchanges restored higher factor H activity-and were associated With a 15-months-disease-free period. In the two other patient, one With-a failingrenal graft and the-other-on chronic dialysis,,a bout of thrombotic micro-angiopathy was preceded by a drop of haemolytic activity below normal values. Plasma from healthy donors (N = 65) showed only minimal variations of Factor H activity (mean activity: 98.3%, SD=4.0). Conclusion: These preliminary data suggest that factor H activity could be of interest in both the diagnosis and the treatment by plasmatherapy of factor H-related aHUS.

Verocytotoxigenic Escherichia coli O157:H7 is responsible for outbreaks worldwide, causing haemorrhagic colitis characterised by abdominal pain and bloody diarrhoea, and can lead to haemolytic uraemic syndrome (HUS) which can, in turn, result in kidney failure. Outbreaks are often linked to inadequately cooked or prepared meat and other foodstuffs. E. coli O157:H7 has also been responsible for a number of serious waterborne outbreaks2 including more than 2000 cases (with 7 deaths) in Walkerton, Canada in 2000. Previous work has shown E. coli O157:H7 to survive in potable water for extended periods of time4 and to be incorporated into associated biofilms.

Three cases of haemolytic-uraemic syndrome in the Hunter area were reported in February 1995. An investigation was initiated to identify any verocytotoxic Escherichia coli in clinical samples that could be associated with the development of the disease. Escherichia coli O6:H- and O2:H7 were isolated from Case 1. No organisms were identified for Case 2, and Case 3 samples yielded Campylobacter jejuni. In addition, efforts were made to trace sources of any such pathogens in food samples or in the environment generally. Shiga-like toxins were found in meat products sampled from butchers' shops patronised by the families of the three cases. However, it was not found possible to match stool samples with samples of food from sources used by the families of the children. Environmental factors seemed likely to have played a significant role in the development of haemolytic-uraemic syndrome in Case 3. It is suggested that the incidence of the disease may be reduced by increasing the frequency of testing of meat products for Shiga-like toxins I and II and through educational and research programs.

SUMMARYA 3-year study of Escherichia coli infections in Grampian Region was conducted to ascertain the incidence, document clinical sequelae and identify at-risk groups. Approximately 30000 stools from patients with acute diarrhoea were screened for E. coli O157, and an epidemiological questionnaire filled in for each patient whose stool was positive. Eighty-three patients were studied. The annual incidence was 6 per 100000. Proportionately more infections occurred in people involved in agriculture. Evidence was seen of case-to-case transmission, and contamination of a water supply. Eight cases developed haemolytic uraemic syndrome (HUS). There were 2 deaths due to HUS and 2 due to haemorrhagic colitis (HC). Symptomatic E. coli infection is relatively common in the Grampian Region, more common in the agricultural community, and is the main cause of HUS in this Region.

Antibiotic use in infection with Shiga-toxin-producing strains of Escherichia coli (E coli) is thought to increase the risk of developing haemolytic-uraemic syndrome (HUS). One paediatric study concluded that E coli O157:H7-infected patients who had received antibiotic therapy were 17 times more likely to progress to HUS than those who had not. Quinolones are among those incriminated. In vitro experiments suggest toxin induction of 80-fold with ciprofloxacin and E coli O104:H4. We report here the case of a 44-year-old man returning from Hamburg who presented with a 5 day history of bloody diarrhoea which had worsened after starting ciprofloxacin. A severe illness of overlapping HUS and thrombotic thrombocytopaenic purpura (TTP) ensued, with neurological complications requiring ventilation and intensive care admission. Stool sample eventually confirmed E coli O104:H4. Although the patient made a good recovery following treatment with haemofiltration and plasma exchange with fresh frozen plasma (FFP), ciprofloxacin may have exacerbated his clinical course.

SUMMARYShiga toxin-producingEscherichia coli(STEC) is an important cause of gastroenteritis (GE) and haemolytic uraemic syndrome (HUS). Incidence of STEC illness is largely underestimated in notification data, particularly of serogroups other than O157 (‘non-O157’). Using HUS national notification data (2008–2012, excluding 2011), we modelled true annual incidence of STEC illness in Germany separately for O157 and non-O157 STEC, taking into account the groups’ different probabilities of causing bloody diarrhoea and HUS, and the resulting difference in their under-ascertainment. Uncertainty of input parameters was evaluated by stochastic Monte Carlo simulations. Median annual incidence (per 100 000 population) of STEC-associated HUS and STEC-GE was estimated at 0·11 [95% credible interval (CrI) 0·08-0·20], and 35 (95% CrI 12-145), respectively. German notification data underestimated STEC-associated HUS and STEC-GE incidences by factors of 1·8 and 32·3, respectively. Non-O157 STEC accounted for 81% of all STEC-GE, 51% of all bloody STEC-GE and 32% of all STEC-associated HUS cases. Non-O157 serogroups dominate incidence of STEC-GE and contribute significantly to STEC-associated HUS in Germany. This might apply to many other countries considering European surveillance data on HUS. Non-O157 STEC should be considered in parallel with STEC O157 when searching aetiology in patients with GE or HUS, and accounted for in modern surveillance systems.

Thrombotic microangiopathy: Inhibition of the lectin complement pathway reduces renal injury in mice with STEC–HUS

Cancer-associated microangiopathic haemolytic anaemia (CA-MAHA) is a syndrome characterised by Coombs-negative haemolytic anaemia and thrombocytopenia. It is primarily seen in advanced solid tumours and is distinct from thrombotic thrombocytopenic purpura/haemolytic uraemic syndrome. Diagnosis is often delayed and patients have a high mortality. We present the case of CA-MAHA in a patient with metastatic breast cancer treated successfully with early initiation of chemotherapy. In addition, we report longitudinal laboratory evaluation of circulating tumour cells and microparticles and suggest a hypothesis for the mechanism behind CA-MAHA.

A 62-year-old man presented to our department with gastroenteritis. He was found to have decreased platelets and deranged renal function. A diagnosis of haemolytic uraemic syndrome was eventually made. The disease usually presents nonspecifically with gastroenteric symptoms and systemic upset at the initial stage. Without a high index of suspicion, the diagnosis can easily be missed. (Hong Kong j.emerg.med. 2003; 10:54-56)

SummaryThe Oklahoma Thrombotic Thrombocytopenic Purpura-Haemolytic Uraemic Syndrome (TTPHUS) Registry has a 24 year record of success for collaborative clinical research, education, and patient care. This article tells the story of how the Registry began and it describes the Registry’s structure and function. The Registry provides a model for using a cohort of consecutive patients to investigate a rare disorder. Collaboration between Oklahoma, United States and Bern, Switzerland has been the basis for successful interpretation of Registry data.Registry data have provided new insights into the evaluation and management of TTP. Because recovery from acute episodes of TTP has been assumed to be complete, the increased prevalence of hypertension, diabetes, depression, and death documented by long-term follow-up was unexpected. Registry data have provided opportunities for projects for students and trainees, education of physicians and nurses, and also for patients themselves. During our follow-up, patients have also educated Registry investigators about problems that persist after recovery from an acute episode of TTP. Most important, Registry data have resulted in important improvements for patient care.

Background The shiga toxin-producing E. coli (STEC) O104:H4 caused a major outbreak in Germany in spring 2011. STEC are usually susceptible to common antibiotics. However, antibiotic treatment of STEC-infected patients is not recommended because STEC may enhance production and release of shiga toxins (STX) in response to antibiotics, which eventually enhances the frequency and severity of clinical symptoms, including haemolytic uraemic syndrome (HUS) and fatalities. Results We characterized the response to antibiotics of STEC O104:H4 isolates from two HUS patients during the German STEC outbreak in spring 2011 in comparison to the common STEC O157:H7. Liquid cultures of STEC O157:H7 and O104:H4 were incubated with graded dilutions of the antibiotics ciprofloxacin, meropenem, fosfomycin, gentamicin, rifampicin, and chloramphenicol. At defined times of antibiotic treatment, transcriptional activation of the STX2 gene, contents of STX and STX-activity in the culture supernatants were quantified. Unlike the common serotype O157:H7, STEC O104:H4 does not release STX in response to therapeutic concentrations of ciprofloxacin, meropenem, fosfomycin, and chloramphenicol. Conclusions In future outbreaks, the response of the respective epidemiologic STEC strain to antibiotics should be rapidly characterized in order to identify antibiotics that do not enhance the release of STX. This will eventually allow clinical studies tackling the question whether antibiotic treatment impacts on the eradication of STEC, clinical course of disease, and frequency of carriers.

Shiga toxin-producing Escherichia coli (STEC) strains of O157:H7 serotype are a predominant cause of haemolytic uraemic syndrome (HUS) worldwide, but strains of non-O157 serotypes can also be associated with serious disease. Some of them are associated with outbreaks of HUS, others with sporadic cases of HUS, and some with diarrhoea but not with outbreaks or HUS. A large number of STEC serotypes isolated from ruminants and foods have never been associated with human disease. In this study we characterize a STEC strain belonging to serotype O171:H25 that is responsible for a case of HUS. This strain has a single Shiga toxin gene encoding Stx2 toxin, and hlyA gene, but is eae-negative.

Clostridium septicum infection in humans is rare and this is the second case report of focal C septicum brain abscesses associated with Escherichia coli 0157 haemolytic uraemic syndrome (HUS). The case presented in a child who lived on a rural farm. The abscesses initially progressed despite 7emsp14;months of appropriate antibiotic therapy and repeated image-guided aspiration. After definitive resection and prolonged antibiotic therapy, there was no recurrence. This case reminds us of this rare but important neurological complication of HUS and supports consideration of early definitive neurosurgical intervention in similar unusual cases.

Aims: To estimate disability-adjusted life years (DALY) and cost of illness (COI) associated with the gastrointestinal bacterial pathogens Campylobacter and verocytotoxin-producing Escherichia coli (VTEC) in Sweden and to investigate the impact of variability in health outcomes, data availability, and different assumptions about underreporting on DALY. Methods: Data from the Swedish notification system, public databases, and the literature were used to estimate COI and DALY. DALY was modelled using a deterministic and a stochastic approach, the latter describing variation in health outcomes between individuals. Effects of different assumptions about underreporting of gastroenteritis were evaluated in separate scenarios. Results: COI and DALY were greater for Campylobacter than for VTEC. Years of life lost due to haemolytic uraemic syndrome and years lived with gastroenteritis constituted most of DALY for VTEC and Campylobacter, respectively. Productivity losses due to gastroenteritis constituted the main cost associated with both pathogens. Degree of underreporting had a greater impact on DALY for Campylobacter, due to higher estimated incidence of gastroenteritis associated with campylobacteriosis. Conclusions: Pathogen-specific health outcomes and data quality may influence the preferred modelling approach. There was a fair agreement between modelling approaches, but the stochastic model reflected the contribution of some rare health outcomes not captured in the deterministic model. Health outcomes excluded due to lack of data lead to an underestimation of the total burden associated with the pathogens. Increased knowledge, especially on the degree of underreporting and the contribution of the pathogens to sequelae, is needed to further improve public health burden estimates for these pathogens in Sweden.

From May to July 2011, one of the largest reported outbreaks of haemolytic uraemic syndrome (HUS) and bloody diarrhoea caused by the Shiga toxin-producing Escherichia coli (STEC) O104:H4 occurred in Germany and France. The hypothetical origin of the outbreak strain was a combined enteroaggregative E. coli and an enterohaemorrhagic E. coli with the ability to resist multi-antibiotics and produce Shiga-toxin 2. The combination of aggregative ability, antibiotic resistance and the production of Shiga-toxin 2 significantly affected the severity of the symptoms presented. Since humans may be the primary reservoir, it is likely that contamination could have occurred through contact with infected individuals. Farm food safety management, and hand hygiene training programmes are crucial to primary production to prevent or reduce risks of contamination.

Background: The first cases of the European epidemic of Shiga toxin-producing Escherichia coli O104:H4 (STEC-O104:H4) infection were reported in Germany in April 2011. Objectives: To characterize the 2011 STEC-O104:H4 outbreak and its management. A literature review is made to assess the state of the art in STEC-haemolytic-uraemic syndrome (HUS) epidemiology, pathogenesis, management and prognosis, focusing on critically ill adults. Methods: References were obtained from the European Center for Disease Control and World Health Organization epidemiological updates, in addition to a PubMed search covering the period from 1980 to August 2011, including all published work on STEC-014:H4 and reviews on HUS management and prognosis. Results: The epidemic originated from a bean and seed sprouts farm in Lower Saxony, and was caused by the O104:H4 strain - a highly antibiotic resistant, hybrid enteroaggregative - Shiga toxin producing E. coli strain (STEC). The infection was characterized by increased HUS (25%) and a higher mortality rate. STEC enteritis and HUS are associated with significant mortality and morbidity, especially amongst patients with severe renal and neurological disorders. Management should center on prompt kidney protection by maintaining adequate renal perfusion, in addition to avoiding diuretics and nephrotoxic agents. Conclusions: The published studies regarding antibiotic treatment lack good quality evidence. However, recent data suggest a potential modulating effect that explains the conflicting data but moreover suggests that azithromycin might be of use. Neutralizing monoclonal antibodies are a promising new therapy for STEC-HUS, with currently ongoing studies. Other treatments have not been shown to be superior to supportive therapy alone.

Background Shiga toxin-producing Escherichia coli (STEC) are an important cause of gastroenteritis in Australia and worldwide and can also result in serious sequelae such as haemolytic uraemic syndrome (HUS). In this paper we describe the epidemiology of STEC in Australia using the latest available data. Methods National and state notifications data, as well as data on serotypes, hospitalizations, mortality and outbreaks were examined. Results For the 11 year period 2000 to 2010, the overall annual Australian rate of all notified STEC illness was 0.4 cases per 100,000 per year. In total, there were 822 STEC infections notified in Australia over this period, with a low of 1 notification in the Australian Capital Territory (corresponding to a rate of 0.03 cases per 100,000/year) and a high of 413 notifications in South Australia (corresponding to a rate of 2.4 cases per 100,000/year), the state with the most comprehensive surveillance for STEC infection in the country. Nationally, 71.2% (504/708) of STEC infections underwent serotype testing between 2001 and 2009, and of these, 58.0% (225/388) were found to be O157 strains, with O111 (13.7%) and O26 (11.1%) strains also commonly associated with STEC infections. The notification rate for STEC O157 infections Australia wide between 2001-2009 was 0.12 cases per 100,000 per year. Over the same 9 year period there were 11 outbreaks caused by STEC, with these outbreaks generally being small in size and caused by a variety of serogroups. The overall annual rate of notified HUS in Australia between 2000 and 2010 was 0.07 cases per 100,000 per year. Both STEC infections and HUS cases showed a similar seasonal distribution, with a larger proportion of reported cases occurring in the summer months of December to February. Conclusions STEC infections in Australia have remained fairly steady over the past 11 years. Overall, the incidence and burden of disease due to STEC and HUS in Australia appears comparable or lower than similar developed countries.

Summary In the haemolytic uraemic syndrome (HUS), platelet microthrombi and deposition of fibrin in glomeruli may contribute to the development of renal failure. The balance of procoagulant and fibrinolytic activities in the renal vasculature may therefore have an important role. We measured plasminogen-activator inhibitor (PAI) activity in the plasma of 81 children with diarrhoea-associated (D+) HUS, and found elevated PAI activity in many patients. When we categorized patients by need for dialysis, only the dialysed group had significantly higher levels of activity, compared with a group of normal controls. We also compared PAI activity with patient outcome after one year, and found that those with a poor outcome had significantly higher PAI activity than those with a good outcome. We suggest that plasma PAI activity may be an acute marker for dialysis requirement in HUS, and may have prognostic value for long-term outcome. The possible role of PAI in the pathogenesis of HUS requires further investigation.

Introduction: Haemolytic uremic syndrome (HUS) is a disease characterised by microangiopathic haemolytic anaemia, thrombocytopenia and renal impairment. It can be either typical HUScaused by shiga toxin producing enterohemorrhagic Escherichia coli or atypical HUS- caused by mutation in the gene for complement proteins (1). HUS should be considered in patients presenting with jaundice to the emergency room in the setting of the above mentioned triad. Case Report: This case report is about a 41 year old lady who presented with jaundice, thrombocytopenia, anemia and renal failure. In the course of investigating the cause of her symptoms, a diagnosis of HUS was made and she was commenced on therapeutic plasma exchange and hemodialysis. Conclusion: The main lesson is to raise awareness of HUS as a possible cause of jaundice among physicians, especially those in the emergency care.

International audience; Comment on: Characterisation of the Escherichia coli strain associated with an outbreak of haemolytic uraemic syndrome in Germany, 2011: a microbiological study. [Lancet Infect Dis. 2011]

International audience; Following the outbreak of haemolytic uraemic syndrome (HUS) on June 2011 in south-western France, household transmission due to Escherichia coli O104:H4 was suspected for two cases who developed symptoms 9 and 10 days after onset of symptoms of the index case. The analysis of exposures and of the incubation period is in favour of a secondary transmission within the family. Recommendations should be reinforced to prevent person-to-person transmission within households.

At the beginning of May an outbreak of bloody diarrhoea and haemolytic uraemic syndrome (HUS) began in Germany. During the succeeding months following the initial outbreak in Germany, thousands of infections occurred resulting in 877 cases of haemolytic uraemic syndrome (HUS) with 32 deaths and 3,043 cases of enterohaemorrhagic Escherichia coli (EHEC) with 16 deaths