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Shiga toxin–associated hemolytic uremic syndrome (HUS) is responsible for approximately 90% of cases of HUS in children, and supportive care remains the backbone of therapy.
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With early fluid resuscitation in the enteritis phase, outcomes for Shiga toxin–associated HUS have improved; however, up to 25% of patients develop chronic kidney disease, hypertension, or proteinuria.
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Streptococcus pneumoniae is likely an underrepresented cause of HUS, responsible for about 5% of cases in children, most
Hemolytic Uremic Syndrome
Section snippets
Key points
Epidemiology and Pathomechanism
HUS associated with STEC (STEC-HUS) is classically a disease of children, with a peak incidence from 3 to 5 years of age. In the United States and Europe, the predominant pathogen is E coli O157:H7, whereas Shigella dysenteriae type 1 remains a predominant cause of disease in other countries.5 The overall incidence of HUS is 1 to 2 cases per 100,000 per year, with most cases attributed to STEC-HUS.3 In children presenting with E coli enterocolitis, approximately 15% will progress to the
Epidemiology and Pathomechanism
Estimated to account for approximately 5% of all cases of HUS in children, and 38% to 43% of HUS not caused by Shiga toxin,36 pneumococcal HUS or S pneumoniae-associated HUS (SP-HUS) has an annual incidence of approximately 0.06 per 100,000 children less than 18 years of age.3 The incidence of HUS following invasive pneumococcal infections is estimated to be about 0.4% to 0.6%, most commonly occurring after pneumonia, particularly complicated by empyema, or meningitis.37 With the introduction
Epidemiology and Pathomechanism
aHUS, also termed complement-mediated TMA,4 is responsible for approximately 5% to 10% of HUS seen in children. Overall incidence is estimated at 2 per 1,000,000 individuals in the United States, with 70% of children having their first episode before 2 years of age, and with equal frequency in boys and girls when onset is in childhood.2, 43 The disease is frequently sporadic despite heterozygous pathogenic variants in complement genes often identified in the patient and one of their healthy
Epidemiology and Pathomechanism
Cobalamin C hemolytic uremic syndrome is a form of HUS that is a rare autosomal recessive disorder of cobalamin (vitamin B12) metabolism that causes TMA and multiorgan dysfunction in infants, although there is 1 reported case with presentation in adulthood.2, 4 Its incidence is estimated at 1 in 100,000 live births66 with more than 300 cases described.67 The mutations are on the MMACHC gene encoding the methylmalonic aciduria and homocystinuria type C protein, leading to hyperhomocysteinemia,
Clinical features
The clinical presentations of cobalamin C deficiency vary considerably and are generally divided into early-onset and late-onset disease, with early-onset disease having a more severe phenotype.66 Early-onset disease usually manifests within the first year of life and has multisystem involvement, presenting with feeding difficulties, failure to thrive, somnolence/lethargy, and hypotonia.66 Patients with late-onset disease are rare and can present any time from childhood to adulthood, generally
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Hemolytic uremic syndrome with central nervous system manifestations, a case report and literature review
2023, Radiology Case ReportsThrombotic microangiopathies in critically ill children: The MATUCIP registry in Spain
2023, Anales de PediatriaCluster analysis and geospatial mapping of antibiotic resistant Escherichia coli O157 in southwest Nigerian communities
2022, One HealthCitation Excerpt :Community spread and poor detection of Escherichia coli O157-producing shiga toxin (stx), characterized with antibiotic resistance intensify the disease burden associated with hemorrhagic colitis and threatening systemic infections [1]. Production of shiga toxin and other virulent factors enhances severe clinical manifestations involving vascular endothelial cells, resulting in vasculitis, hemolytic uremic syndrome, abdominal pain, and rarely, thrombotic thrombocytopenic purpura [2,3]. Shiga toxin producing Escherichia coli O157 mostly initiates an inflammatory cascade leading to leukocyte aggregation, apoptosis, platelet aggregation, microthrombic formation, hemolysis, renal dysfunction and diffuse vasculitic injury resulting to multiple organ failures [4].
Uncommon Causes of Acute Kidney Injury
2022, Critical Care ClinicsCitation Excerpt :It can be caused by infection with either STX producing Escherichia coli (STEC) serotype 0157:H7 or Shigella dysenteriae type 1.77–79 Other causes include infection with Streptococcus pneumoniae (SpHUS),80–83 influenza,84 H1N1,85,86 HIV,87 and inborn errors of metabolism including cobalamin C deficiency88,89 and diacylglycerol kinase epsilon (DGKE) deficiency.90,91 Secondary forms of HUS also occur in various disease states and conditions including bone marrow or solid organ transplant, malignancy, autoimmune disorders, malignant hypertension, and drug-associated HUS.92–96
Eculizumab in Shiga toxin-producing Escherichia coli hemolytic uremic syndrome: a systematic review
2024, Pediatric NephrologyAutonomic activity and cardiovascular system risk assessment in pediatric patients with hemolytic uremic syndrome
2024, European Journal of Pediatrics
Disclosure Statement: The authors have nothing to disclose.