Cases Of HUS Research Articles

Clinical Outcomes and Virulence Factors of Shiga Toxin-Producing Escherichia coli (STEC) from Southern Alberta, Canada, from 2020 to 2022.

Shiga toxin-producing Escherichia coli (STEC) can cause severe clinical disease in humans, particularly in young children. Recent advances have led to greater availability of sequencing technologies. We sought to use whole genome sequencing data to identify the presence or absence of known virulence factors in all clinical isolates submitted to our laboratory from Southern Alberta dated 2020-2022 and correlate these virulence factors with clinical outcomes obtained through chart review. Overall, the majority of HUS and hospitalizations were seen in patients with O157:H7 serotypes, and HUS cases were primarily in young children. The frequency of virulence factors differed between O157:H7 and non-O157 serotypes. Within the O157:H7 cases, certain virulence factors, including espP, espX1, and katP, were more frequent in HUS cases. The number of samples was too low to determine statistical significance.

Shiga Toxin-Producing Escherichia coli Strains from Romania: A Whole Genome-Based Description.

The zoonotic Shiga toxin-producing Escherichia coli (STEC) group is unanimously regarded as exceptionally hazardous for humans. This study aimed to provide a genomic perspective on the STEC recovered sporadically from humans and have a foundation of internationally comparable data. Fifty clinical STEC isolates, representing the culture-confirmed infections reported by the STEC Reference Laboratory between 2016 and 2023, were subjected to whole-genome sequencing (WGS) analysis and sequences were interpreted using both commercial and public free bioinformatics tools. The WGS analysis revealed a genetically diverse population of STEC dominated by non-O157 serogroups commonly reported in human STEC infections in the European Union. The O26:H11 strains of ST21 lineage played a major role in the clinical disease resulting in hospitalisation and cases of paediatric HUS in Romania surpassing the O157:H7 strains. The latter were all clade 7 and mostly ST1804. Notably, among the Romanian isolates was a stx2a-harbouring cryptic clade I strain associated with a HUS case, stx2f- and stx2e-positive strains, and hybrid strains displaying a mixture of intestinal and extraintestinal virulence genes were found. As a clearer picture emerges of the STEC strains responsible for infections in Romania, further surveillance efforts are needed to uncover their prevalence, sources, and reservoirs.

Work-related Road Traffic Crashes in Malaysia: A Comparison between Fatal and Temporary Injury Cases

Introduction: Work-related road traffic crashes have significant implications for both employers and employees, leading to devastating consequences such as fatalities, injuries, and property damage. Despite the increasing focus on road safety research, little attention has been given to identifying factors associated with work-related road crashes. This paper explores and compares the characteristics of fatal cases (FOT) with the characteristics of temporary injury cases (HUS) in work-related road traffic crashes. Materials and methods: Seven years of work-related road crash claim compensation data between 2014 and 2020 was obtained from the Social Security Organization (SOCSO). A total of 546 cases were involved, including 56 FOT and 490 HUS cases. Categorical data analysis techniques were employed to examine the distinctions between FOT and HUS cases. Results: The results indicate a lower representation of transportation sectors in FOT cases, with the corresponding odds approximately 84% lower. Conversely, small and middle enterprises exhibit an odds ratio of about 5.1 times higher in FOT compared to HUS cases. Conclusion: The findings from this study can serve as proactive measures for employers in addressing work-related road traffic crashes. This includes enhancing road safety awareness programs specifically focusing on groups engaged in nighttime shifts and those involved in sending and pickup duties. Considering the distinct risk profiles of large enterprises versus small and medium enterprises, future studies are necessary to clarify the effectiveness of the road safety initiatives undertaken by both types of enterprises.

АТИПИЧНЫЙ ГЕМОЛИТИКО-УРЕМИЧЕСКИЙ СИНДРОМ У ДЕТЕЙ В РЕСПУБЛИКЕ БЕЛАРУСЬ: ПЕРВЫЙ ОПЫТ КОМПЛЕМЕНТБЛОКИРУЮЩЕЙ ТЕРАПИИ

Atypical hemolytic uremic syndrome (aHUS) is one of the most complex and severe forms of thrombotic microangiopathy with an unfavorable outcome in its natural course. The purpose of this research was to study the epidemiology, demographics, course and outcomes of aHUS in comparison with patients with typical hemolytic uremic syndrome (tHUS) as well as to conduct a personal analysis of all cases of aHUS and evaluate the response to therapy, including comple-ment-blocking with Eculizumab. Materials and methods used: the study was longitudinal, obser-vational and included all 349 cases of HUS in children in Belarus in 2015-2023. A comparative analysis of demographic and clinical data, laboratory results and outcomes was performed in pa-tients with HUS hospitalized at the Center for Pediatric Nephrology and Renal Replacement Therapy of the Republic of Belarus (Minsk, Belarus). Results: of 349 HUS cases, the proportion of aHUS was 3,7% (13). Children with aHUS were older (4,6 (1,6; 6,7) v. 2,7 (1,7; 4,8) y/o with tHUS) due to the portion of patients older than 5 y/o (46%). In aHUS, severe central nervous system disorders were more often observed (62% v. 14%, p=0,002), the development of critical conditions requiring transfer to mechanical ventilation (39% v. 9%, p=0,006), prolonged anuria with the need for dialysis therapy in 100% of cases and severe adverse outcomes (death or end-stage renal disease) during natural course (66,0% v. 2,7%, p<0,001). The most common trigger for aHUS was acute intestinal infections in 54% of cases. A genetic study was performed in 9 (69%) children with aHUS and in 8 (89%) of them the CFHR3/CFHR1 deletions were detected: in 4 cases (3 hetero- and a single homozygous) and heterozygous pathogenic and probably patho-genic gene mutations, CFHR5 in 2 cases, CFH in a single case and combined CD46 and CFI in a single case as well. Antibodies to complement factor H were detected in 5 patients, 3 of which had a CFHR3/CFHR1 deletion. Therapy with a biosimilar of the original Eculizumab showed its effectiveness in aHUS: in 3 (100%) children it was possible to prevent the return of the disease in the kidney graft, in single patient who did not receive prophylaxis it was able to block the recur-rence of aHUS in the transplanted kidney while preserving its function, in 3 (100%) patients with late administration of treatment the urine output was restored after prolonged anuria (24, 33 and 59 days); in a single child with the antibody variant of aHUS, with early administration of the drug, renal function was preserved. No complications of complement blocking therapy were not-ed. Conclusions: the introduction into clinical practice of a biosimilar of the original Eculizumab in children in Belarus has significantly improved the outcomes and prognosis of the disease.

Thrombotic Microangiopathy in Solid Organ Transplantation

Thrombotic Microangiopathy (TMA) is a syndrome characterized by microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. The presence of schistocytes on peripheral smear, a negative Coombs test, elevated lactate dehydrogenase, increased reticulocyte count and low haptoglobin are often the clues for MAHA. The microvascular process often targets vasculature in kidneys, brain, gastrointestinal system, heart, and skin. A timely diagnosis and treatment are often crucial to prevent severe end organ damage and death. TMA is classified into primary and secondary forms. Primary TMA includes TTP and complement mediated or atypical hemolytic uremic syndrome (aHUS), often related to a mutation or deficiency and clinically expressed in the setting of a precipitant condition. Secondary TMA is a manifestation of underlying disorder and can occur in clinical scenarios associated with autoimmune disease, malignancy, infections, SOT (Solid Organ Transplant), pregnancy, HSCT (Hematopoietic Stem Cell Transplantation), medications, or methylmalonic acidemia. Transplant associated TMA (TA-TMA) can be complement mediated or aHUS and could be related to the ischemic reperfusion injury, induction regimen, calcineurin inhibitor (CNI) use, mammalian target of rapamycin (MTOR) inhibitor use, or could be infection related. Cost, access, and turnaround time are often the limitations for certain TTP and complement specific testing. Treatment should not be delayed while waiting for such tests. Treatment must be individualized based on the underlying cause of TMA. Terminal complement blockade utilizing monoclonal antibodies directed against C5 complement is the treatment for complement mediated TMA. C5 inhibitors have also been used successfully in treatment of secondary HUS cases where, unlike aHUS, defects in complement cannot be demonstrated. Such treatment has demonstrated improvement in renal function, MAHA and platelet counts.

Quantitative microbial risk assessment of haemolytic uremic syndrome associated with Argentinean kosher beef consumption in Israel.

The aim of this study was to perform a quantitative microbial risk assessment (QMRA) of Shiga toxin-producing Escherichia coli hemolytic uremic syndrome (STEC-HUS) linked to the consumption of Kosher beef produced in Argentina and consumed in Israel in children under 14 years. A probabilistic risk assessment model was developed to characterize STEC prevalence and contamination levels in the beef supply chain (cattle primary production, cattle transport, processing and storage in the abattoir, for export and at retail, and home preparation and consumption). The model was implemented in Microsoft Excel 2016 with the @Risk add-on package. Results of 302 surveys with data collected in Israel were as follows: 92.3% of people consumed beef, mostly at home, and 98.2% preferred levels of cooking that ensured STEC removal from the surface of beef cuts. The preferred degree of ground beef doneness was "well-done" (48.2%). Cooking preference ranged from red to "medium-well done" (51.8%). Median HUS probability from Argentinean beef cut and ground beef consumption in children under 14 years old was <10-15 and 8.57x10-10, respectively. The expected average annual number of HUS cases and deaths due to beef cut and ground beef consumption was zero. Risk of infection and HUS probability correlated with salting effect on E. coli count, processing raw beef before vegetables, ways of storage and refrigeration temperature at home, joint consumption of salad and beef cuts, degree of beef doneness and cutting board washing with detergent after each use with beef and vegetables. The STEC-HUS risk in Israel from consumption of bovine beef produced in Argentina was negligible. The current QMRA results were similar to those of previous beef cut consumption QMRA in Argentina and lower than any of the QMRA performed worldwide in other STEC-HUS linked to ground beef consumption. This study confirms the importance of QMRA to estimate and manage the risk of STEC-HUS from beef consumption. The impact variables identified in the sensitivity analysis allowed us to optimize resources and time management, to focus on accurate actions and to avoid taking measures that would not have an impact on the risk of STEC-HUS.

A Case of Macrophage Activation Syndrome (MAS) and Atypical HUS in Newely Diagnosed SLE

A Case of Macrophage Activation Syndrome (MAS) and Atypical HUS in Newely Diagnosed SLE

Outbreak of hemolytic uremic syndrome with unusually severe clinical presentation caused by Shiga toxin-producing Escherichia coli O26:H11 in France

BackgroundIn spring 2019, an outbreak of Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (STEC HUS) occurred in France. Epidemiological investigations made by Santé publique France in connection with microbiological investigations at the national reference center for STEC promptly identified a common exposure to consumption of raw cow's milk cheese, and confirmed a cluster affiliation of the E. coli O26:H11 outbreak strain. Here, we report the clinical characteristics of the patients, the treatment used, as well as the outcome at 1 month. MethodPatients with STEC HUS linked to the E. coli O26:H11 outbreak strain were identified from the national surveillance network of pediatric STEC HUS cases coordinated by Santé publique France.Clinical data were analyzed from the patients’ hospital records obtained from the treating physicians. ResultsOverall, 20 pediatric cases of STEC HUS linked to the outbreak strain were identified.Their median age of the patients was 16 months (range: 5–60). Most of them presented with diarrhea but none had received prior antibiotherapy. A total of 13 patients required dialysis; 10 patients and four patients had central nervous system (CNS) and cardiac involvement, respectively. No deaths occurred.At the 1-month follow-up, only two patients had a decreased glomerular filtration rate, below 80 mL /min/1.73m2 and four had hypertension. One patient had neurological sequelae. ConclusionThe E. coli O26:H11 strain identified as the cause of an STEC HUS outbreak in France in spring 2019 is notable for the initial severe clinical presentation of the patients, with a particularly high frequency of CNS and cardiac involvement similar to the German E. coli O104:H4 outbreak described in 2011. However, despite the initial severity, the 1-month outcome was favorable in most cases. The patients’ young age in this outbreak highlights the need to improve information and caregiver awareness regarding consumption of at-risk foods by young children as key preventive measures against STEC infections.

Successful Use of Belatacept in Three Lung Transplant Recipients for Unique Indications

<h3>Introduction</h3> Belatacept is a selective costimulatory blocker approved for immunosuppression after kidney transplantation. Data for its use in other solid organ transplants shows mixed results. We present 3 cases of its efficacy in lung transplant as an alternative agent for maintenance therapy in unique contexts. <h3>Case Report</h3> Patient #1 is a 40 year old male who received bilateral lung transplant for ILD from scleroderma complicated by AKI requiring hemodialysis with partial renal recovery at discharge. Over the next 7 months, his renal function declined and with a creatinine of 3.9 mg/dL, belatacept was initiated in combination with a lowered tacrolimus goal of 5 - 6 ng/mL to minimize further nephrotoxicity. 2 months into this course, his creatinine improved to 3.2 mg/dL without untoward effect on the allograft. Patient #2 is a 47 year old female who underwent a third retransplant for CLAD. Prior to the third transplant, her immunosuppression consisted of sirolimus, tacrolimus, and prednisone. Mycophenolate mofetil was avoided due to prior skin cancer. Given her history of rejection and high PRA, she was started on belatacept to supplement tacrolimus and prednisone post-op. 22 months post-transplant she has low level DSA and normal cfDNA levels with a normally functioning graft. Patient #3 is a 66 year old male with a right lung transplant for IPF. His course was complicated by TTP and HUS secondary to calcineurin inhibitors, which reoccurred on rechallenge with mTOR inhibitors. He was subsequently started on belatacept along with mycophenolate mofetil and prednisone. He has been on this regimen for 2 years without recurrent side effects, cytopenia, or renal dysfunction. <h3>Summary</h3> Belatacept is renal sparing, which is beneficial as standard immunosuppression regimens include calcineurin inhibitors that are nephrotoxic. Its utility to prevent rejection was shown when evaluated by DSA and cfDNA levels. In the rare case of TTP and HUS, these phenomena did not reoccur. It is imperative to recognize the risks with this agent. PTLD has occurred in EBV-seronegative recipients. There has been an association of viral infections, including CMV. ACR has been documented in heart and kidney recipients. In conclusion, belatacept has a role as an alternative immunosuppressive component in the appropriate clinical context.

Task force for the diagnosis of thrombotic microangiopathy / Força-tarefa para o diagnóstico de microangiopatia trombótica

Background: Thrombotic microangiopathy triggers microangiopathic hemolytic anemia, thrombocytopenia, and organic injuries. It is a rare and serious condition that demands specific approaches and strategies to recognize it promptly, especially in critically ill patients. The task force could be an effective model. Methods: An evaluative study of the impact of a task force in establishing a routine for the diagnosis of TMA in ICUs. A standard flowchart was used for 90 consecutive days and a low platelet count was the driver for the collection of epidemiological and laboratory data. Suspicion of typical HUS was proven after analyzing haptoglobin, LDH, creatinine, ADAMTS-13 enzyme, and Shiga toxin levels. Meetings were also held with ICU personnel and the self-explanatory flowchart was fine tuned. Results: There were 490 patients (55.9% male and 44.1% female) aged 59.5 ± 21.13 years. Forty-two percent of patients in the sample had thrombocytopenia. Out of the 42 patients, five (1.02%) had TTP, three (0.6%) were presumed to have atypical HUS, and none had typical HUS. The task force remained in touch with researchers to assess diagnoses and three other suspected cases of atypical HUS underwent specific treatment.Discussion: Only 1.62% of the sampled patients had TMA. Despite its complexity, the task force model is useful in situations in which higher technology is the norm. Conclusion: The use of a task force has proven to be effective in establishing a diagnostic practice for a health condition deemed rare or exceedingly rare.

Peripheral nervous system manifestations of Shiga toxin-producing E. coli-induced haemolytic uremic syndrome in children

BackgroundThe Neurological involvement is the most common extra-renal complication of Shiga toxin-producing E. coli-hemolytic uremic syndrome (HUS) or typical HUS. On brain magnetic resonance examination, main neurological signs encompass acute lesions of the basal ganglia and the white matter, which could usually regress after Eculizumab infusion. In contrast, peripheral nervous system (PNS) manifestations in typical HUS are very rare and, when occurring, they require a careful management of neurological sequelae and an intensive multidisciplinary neuro-rehabilitation program.Case presentationHere, we present two pediatric cases of severe and complicated typical HUS with PNS manifestations who required therapeutic treatment and an intensive multidisciplinary neuro-rehabilitation program.In both cases, PNS manifestations were followed by the recovery from typical HUS-related severe central neurological damage and manifested mainly with marked bilateral motor deficit and hyporeflexia/areflexia in the lower limbs. The peripheral polyneuropathy was treated with immunosuppressive therapy (methylprednisolone boluses, i.v. immunoglobulins, plasma exchange), followed by a prolonged intensive neuro-rehabilitation program. After 8 months of rehabilitation, both patients gained complete functional recovery.ConclusionsPNS manifestations during typical HUS are a rare event and potentially leading to severe disability. A timely clinical assessment is mandatory to set up a prompt therapeutic and rehabilitation program and to obtain a complete clinical and functional recovery.

118 A Case of Concurrent HELLP and Pregnancy Associated Atypical HUS (aHUS) During Postpartum Period

118 A Case of Concurrent HELLP and Pregnancy Associated Atypical HUS (aHUS) During Postpartum Period

Complement Genetic Variants and FH Desialylation in S. pneumoniae-Haemolytic Uraemic Syndrome.

Haemolytic Uraemic Syndrome associated with Streptococcus pneumoniae infections (SP-HUS) is a clinically well-known entity that generally affects infants, and could have a worse prognosis than HUS associated to E. coli infections. It has been assumed that complement genetic variants associated with primary atypical HUS cases (aHUS) do not contribute to SP-HUS, which is solely attributed to the action of the pneumococcal neuraminidase on the host cellular surfaces. We previously identified complement pathogenic variants and risk polymorphisms in a few Hungarian SP-HUS patients, and have now extended these studies to a cohort of 13 Spanish SP-HUS patients. Five patients presented rare complement variants of unknown significance, but the frequency of the risk haplotypes in the CFH-CFHR3-CFHR1 region was similar to the observed in aHUS. Moreover, we observed desialylation of Factor H (FH) and the FH-Related proteins in plasma samples from 2 Spanish and 4 Hungarian SP-HUS patients. To analyze the functional relevance of this finding, we compared the ability of native and “in vitro” desialylated FH in: (a) binding to C3b-coated microtiter plates; (b) proteolysis of fluid-phase and surface-bound C3b by Factor I; (c) dissociation of surface bound-C3bBb convertase; (d) haemolytic assays on sheep erythrocytes. We found that desialylated FH had reduced capacity to control complement activation on sheep erythrocytes, suggesting a role for FH sialic acids on binding to cellular surfaces. We conclude that aHUS-risk variants in the CFH-CFHR3-CFHR1 region could also contribute to disease-predisposition to SP-HUS, and that transient desialylation of complement FH by the pneumococcal neuraminidase may have a role in disease pathogenesis.

Core and Accessory Genome Comparison of Australian and International Strains of O157 Shiga Toxin-Producing Escherichia coli.

Shiga toxin-producing Escherichia coli (STEC) is a foodborne pathogen, and serotype O157:H7 is typically associated with severe disease. Australian STEC epidemiology differs from many other countries, as severe outbreaks and HUS cases appear to be more often associated with non-O157 serogroups. It is not known why Australian strains of O157 STEC might differ in virulence to international strains. Here we investigate the reduced virulence of Australian strains. Multiple genetic analyses were performed, including SNP-typing, to compare the core genomes of the Australian to the international isolates, and accessory genome analysis to determine any significant differences in gene presence/absence that could be associated with their phenotypic differences in virulence. The most distinct difference between the isolates was the absence of the stx2a gene in all Australian isolates, with few other notable differences observed in the core and accessory genomes of the O157 STEC isolates analyzed in this study. The presence of stx1a in most Australian isolates was another notable observation. Acquisition of stx2a seems to coincide with the emergence of highly pathogenic STEC. Due to the lack of other notable genotypic differences observed between Australian and international isolates characterized as highly pathogenic, this may be further evidence that the absence of stx2a in Australian O157 STEC could be a significant characteristic defining its mild virulence. Further work investigating the driving force(s) behind Stx prophage loss and acquisition is needed to determine if this potential exists in Australian O157 isolates.

The prevalence and genomic context of Shiga toxin 2a genes in E. coli found in cattle.

Shiga toxin-producing Escherichia coli (STEC) that cause severe disease predominantly carry the toxin gene variant stx2a. However, the role of Shiga toxin in the ruminant reservoirs of this zoonotic pathogen is poorly understood and strains that cause severe disease in humans (HUSEC) likely constitute a small and atypical subset of the overall STEC flora. The aim of this study was to investigate the presence of stx2a in samples from cattle and to isolate and characterize stx2a-positive E. coli. In nationwide surveys in Sweden and Norway samples were collected from individual cattle or from cattle herds, respectively. Samples were tested for Shiga toxin genes by real-time PCR and amplicon sequencing and stx2a-positive isolates were whole genome sequenced. Among faecal samples from Sweden, stx1 was detected in 37%, stx2 in 53% and stx2a in 5% and in skin (ear) samples in 64%, 79% and 2% respectively. In Norway, 79% of the herds were positive for stx1, 93% for stx2 and 17% for stx2a. Based on amplicon sequencing the most common stx2 types in samples from Swedish cattle were stx2a and stx2d. Multilocus sequence typing (MLST) of 39 stx2a-positive isolates collected from both countries revealed substantial diversity with 19 different sequence types. Only a few classical LEE-positive strains similar to HUSEC were found among the stx2a-positive isolates, notably a single O121:H19 and an O26:H11. Lineages known to include LEE-negative HUSEC were also recovered including, such as O113:H21 (sequence type ST-223), O130:H11 (ST-297), and O101:H33 (ST-330). We conclude that E. coli encoding stx2a in cattle are ranging from strains similar to HUSEC to unknown STEC variants. Comparison of isolates from human HUS cases to related STEC from the ruminant reservoirs can help identify combinations of virulence attributes necessary to cause HUS, as well as provide a better understanding of the routes of infection for rare and emerging pathogenic STEC.

P0391STUDY OF ANTI COMPLEMENT FACTOR H MEDIATED DISEASE AT A TERTIARY CARE CENTRE

Abstract Background and Aims To study the clinical profile and outcome of patients with anti complement factor H mediated disease at our centre. Method We conducted a prospective study over 24 months ( August 2016 to July 2018) to assess the clinical profile and outcome of patients with anti complement factor H mediated disease at our tertiary care centre. We had a total of 18 cases during the study period. All patients were followed up to assess their response to therapy. Results A total of 28 cases of atypical HUS were seen during the study period of which anti factor H antibody was elevated in 18 (64.2%). All patients presented with hypertension with active urinary sediments and rapidly progressive renal failure. Mean serum creatinine at presentation was 6.8 +/- 1.2 mg/dl and all patients were oliguric at presentation and required haemodialysis. Anti complement factor H antibody was elevated in all patients with a mean of 549 +/- 90 AU/ml. (normal - 0 to 100 AU/ml).Renal biopsy showed thrombotic microangiopathy in 12 patients (66.6%) while features were suggestive of C3 glomerulopathy in 6 patients (33.3%). All patients received induction with plasmapheresis and pulse steroids followed by oral steroids at 1mg / kg with IV cyclophosphamide 500mg/ m 2 monthly for 6 months . This was followed by maintenance immunosuppression with azathioprine +/- oral steroids. 10 patients (55.5%) attained complete remission. Patients with anti complement factor H mediated disease presenting with TMA had better prognosis compared to patients presenting with C3 glomerulopathy. Also patients in the paediatric age group showed good response to treatment Conclusion Our study shows that anti complement factor H mediated disease is more common in the paediatric age group with good response to plasmapheresis and immunosuppression in the form of oral steroids and cyclophosphamide. Patients presenting with TMA have better prognosis compared to C3 glomerulopathy

Pediatric Pneumococcal Hemolytic Uremic Syndrome Treated with Sequence Tandem Therapeutic Plasma Exchange and Continuous Venovenous Hemodiafiltration: A Case Report

AbstractHemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Approximately 5% of HUS cases are associated with Streptococcus pneumoniae infections (pHUS). Treatment includes supportive care with appropriate antimicrobial therapy, fluid and blood product resuscitation, and renal replacement therapy. We presented a case of a 22-month-old previously healthy girl, who was hospitalized at University Hospital of Split. Left-sided pneumonia and sepsis caused by S. pneumoniae were confirmed. The course of illness was complicated with development of pHUS. Since the pathogenesis of pHUS is only partially understood, the treatment remains controversial. Our patient was successfully treated with daily sequence tandem continuous venovenous hemodiafiltration and therapeutic plasma exchange with albumins, along with other supportive measures. Therefore, in our opinion, plasmapheresis should be considered as a part of standard treatment of children with pHUS. Additionally, the incidence of pHUS appears to be increasing. S. pneumoniae is a particularly important among pediatric pathogens and it can cause wide spectrum of illnesses. Therefore, due to the significant burden of invasive pneumococcal disease, pneumococcal vaccination should be encouraged.

Sociodemographic and clinical risk factors for paediatric typical haemolytic uraemic syndrome: retrospective cohort study

ObjectivesHaemolytic uraemic syndrome (HUS) following Shiga toxin-producing Escherichia coli (STEC) infection is the the most common cause of acute renal failure among children in the UK. This study explored differential...

Shiga toxin-producing Escherichia coli in household members of children with hemolytic uremic syndrome.

The objective is to establish the frequency of STEC infections in household contacts of HUS patients. We studied 292 household contacts of 82 HUS patients attended from 2010 to 2018. In HUS cases, diagnostic criteria were (1) isolation and characterization of STEC strains, (2) detection of free fecal Shiga toxin (FFStx), and (3) detection of anti-O serogroup-specific antibodies. Contacts were studied by screening of stx genes by polymerase chain reaction and/or STEC isolation from stool samples. Clonal relation of STEC strains was established by pulsed-field gel electrophoresis (PFGE). Frequencies of HUS patients without STEC isolation with STEC-positive contacts were determined. Serotypes and stx-genotypes in patients and contacts were analyzed. Thirty (36.6%) HUS patients had 36 STEC-positive contacts. Fourteen (38.8%) were children, 20 adults, and 2 dogs. One sibling developed HUS, 6 contacts had gastrointestinal symptoms, and the rest were asymptomatic. In 5 of 30 HUS patients, STEC infection could not be confirmed, and 2 cases were diagnosed only by FFStx detection. Of the remaining 23 HUS patients, 16 had E. coli O157 and 7 E. coli O145 infection. Serotype and/or stx-genotype concordance was established in 19 (83%) of 23 HUS patients and their contacts. Five HUS cases and their contacts studied by PFGE showed macrorestriction patterns with more than 90% similarity. Nearly one third of HUS patients had STEC-positive family contacts, and one third of them were children. Early identification is important to prevent ongoing contamination among family and institutional contacts and to facilitate prompt detection of HUS in STEC-positive contacts.

Haemolytic uraemic syndrome associated with non shiga toxin-producing Escherichia coli bacteraemia: a case report

BackgroundHaemolytic uraemic syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by predominant renal involvement. Several types of HUS can be distinguished: the most frequent « typical » HUS, due to shiga toxin producing Escherichia coli (STEC), “atypical” HUS due to complement alternative pathway dysregulation and “secondary” HUS associated with various diseases/conditions, the classification of which is still subject to debate.Case presentationWe report a case of HUS following E.coli prostatitis and bacteraemia in an adult male. He presented with severe renal and neurological involvement. Initially considered as a “typical” HUS, the condition was treated by antibiotics. No other specific treatment for HUS was administered. The outcome was favorable. We eventually identified a non shiga toxin producing E.coli. Genetic testing of the complement alternative pathway revealed a rare – potentially pathogenic – variant of factor H. This constitutes a possible factor of susceptibility for atypical HUS, suggesting that E.coli infection may be the trigger.ConclusionThis case raises the question of complement exploration for HUS associated with infections, in order to classify such cases of HUS in accordance with their underlying pathophysiological mechanisms.