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Haemolytic Uraemic Syndrome (hus) In Children

19 Questions
Haemolytic Uraemic Syndrome (hus) In Children

This quiz is based on a comprehensive overview of the existing literature on the epidemiology, etiology, pathogenesis, clinical presentation, diagnosis and management of HUS. It highlights the ongoing controversy regarding the role of antibiotics during the acute enteric phase of the classical HUS and the challenges involved in the management of the atypical diseases.

Questions and Answers
  • 1. 
    Which of the following identifiable risk factors do not suggest vulnerability for developing HUS?
    • A. 

      Age under 4 years

    • B. 

      Fever

    • C. 

      Leukocytosis

    • D. 

      Elevated C-Reactive protein

    • E. 

      Deranged clotting

  • 2. 
    What proportion of children infected with enterohemorrhagic Escherichia coli (STEC) O157:H7 develops HUS?
    • A. 

      5%-15%

    • B. 

      20-40%

    • C. 

      50-70%

    • D. 

      2 - 7%

    • E. 

      None

  • 3. 
    What proportion of children with HUS requires renal replacement therapy (dialysis) in the acute phase?
    • A. 

      50-80%

    • B. 

      20-40%

    • C. 

      40-50%

    • D. 

      5%-15%

    • E. 

      None

  • 4. 
    • A. 

      Potentially harmful

    • B. 

      May increase the release of Shiga-like toxins during the diarrheal illness

    • C. 

      May decrease the duration of E. coli O157:H7 infection

    • D. 

      Associated with high mortality and/or longer duration of diarrhoea

    • E. 

      There are some antibiotics, especially fosfomycin, which may reduce the risk of developing HUS

  • 5. 
    • A. 

      Avoid use of antibiotics unless HUS is associated with an entero-invasive species of Shigella

    • B. 

      Anti-hypertensive therapy may be necessary

    • C. 

      Blood transfusion to raise haemoglobin up to 70 g/L (not to normal)

    • D. 

      Avoid anti-motility agents, narcotics and non-steroidal anti-inflammatory drugs during the acute phase

    • E. 

      Dialysis may be required if anuria persist longer than 24-48 hrs

  • 6. 
    Non-O157 E. coli may account for up to 20%-50% of all STEC infections?
    • A. 

      Yes

    • B. 

      No

  • 7. 
    HUS can also be caused by Shigellosis, Streptococcus pneumoniae, Clostridium difficile, Salmonella, Yersinia, Campylobacter species, varicella, echovirus, coxsackie A and B and human immunodeficiency virus (HIV)?
    • A. 

      YES

    • B. 

      NO

  • 8. 
    Approximately 5%-10% of all HUS cases have an "atypical" or recurrent course?
    • A. 

      YES

    • B. 

      NO

  • 9. 
    Causes for aHUS include complement disorders, disorders interfering with the degradation of von Willebrand factor (VWF), the cobalamin metabolism, pregnancy – (HELLP syndrome); drugs such as many chemotherapeutic agents, autoimmune and other disorders?
    • A. 

      YES

    • B. 

      NO

  • 10. 
    A small group of aHUS in post bone marrow transplantation period may be caused by immunosuppressive drugs including cyclosporine, or following exposure to herbicides or pesticides and severe scorpion envenomation?
    • A. 

      YES

    • B. 

      NO

  • 11. 
    Factors associated with good outcome of renal transplant in patients with non-Shiga toxin-associated HUS (non-Stx-HUS) include:
    • A. 

      Presence of a factor H (CFH) mutation

    • B. 

      Presence of factor I (IF) mutation

    • C. 

      Proteinuria and hypertension

    • D. 

      Chronic renal failure

    • E. 

      Presence of membrane co-factor protein (MCP) mutation

  • 12. 
    • A. 

      They require dialysis therapy more often

    • B. 

      More likely to require prolonged hospitalisation or have persistent thrombocytopenia

    • C. 

      Less likely to require platelet transfusions and need less packed red blood cell transfusions

    • D. 

      Infection caused by S pneumoniae accounts for nearly 40% of cases of non-enteropathic HUS

    • E. 

      The long-term prognosis for recovery of renal function appears to be good

  • 13. 
    How can you describe the most characteristic pathologic processes in HUS?
    • A. 

      Shiga-like toxins (Stx-1 and Stx-2) produced by STEC are responsible for the systemic complications of HUS

    • B. 

      Shiga toxins bind to host cells which express the neutral glycolipid receptors bearing a terminal globotriaosylceramide (Gb3) moiety

    • C. 

      The basis for the multi-organ system disease in HUS is endothelial cells damage via the inhibition of protein synthesis, activation to produce inflammatory mediators, and amplification of the pro-thrombogenic state

    • D. 

      Young children express low levels of Stx receptors in their renal glomeruli

    • E. 

      Stx receptors may become down-regulated by the effects of LPS and cytokines in adults and older children

  • 14. 
    What is the case fatality rate of HUS?        
    • A. 

      Between 50% and 80%

    • B. 

      Between 30% and 50%

    • C. 

      Between 2% and 7%

    • D. 

      Between 12% and 27%

    • E. 

      Not fatal

  • 15. 
    Which of the following statements are true regarding Complications of hus?
    • A. 

      Complications in the acute phase of HUS can include intussusception, renal failure, hypertension, encephalopathy, pancreatitis and seizures

    • B. 

      Insulin-dependent diabetes mellitus may present as a long term complication

    • C. 

      2.5% to 10% will either die during the acute illness or have permanent renal failure

    • D. 

      Patients with atypical HUS are more likely to develop complications with recurrent disease episodes, severe hypertension, pancreatitis, hepatitis, cardiac failure, chronic and end-stage renal failure

    • E. 

      Patients with genetic HUS have an alarmingly high risk of graft loss from disease recurrence or thrombosis

  • 16. 
    • A. 

      Moderate leucocytosis and haemolytic anemia with elevated reticulocyte count

    • B. 

      Presence of schistocytes (fragmented, deformed, irregular, or helmet-shaped red cells) in the peripheral blood smear

    • C. 

      Abnormal prothrombin time (PT), activated partial thromboplastin time (aPTT), and fibrinogen levels

    • D. 

      Markedly elevated blood urea nitrogen (BUN) and creatinine

    • E. 

      Sterile Blood cultures

  • 17. 
    • A. 

      Appropriate personal and public hygiene procedures

    • B. 

      Prompt isolation of index cases of ETEC-associated diarrhea is recommended

    • C. 

      Immunization of cattle to reduce the prevalence of STEC colonization

    • D. 

      Development of human monoclonal antibodies against Stx and plant-based oral vaccines based on genetically modified Stx (toxoid)

    • E. 

      Early antibiotic treatment for patients with STEC infection

  • 18. 
    Characteristics of atypical HUS include:
    • A. 

      Atypical- or non-Shiga toxin-associated HUS can be either sporadic or familial

    • B. 

      Up to 50% of cases of aHUS involve abnormalities of the complement regulatory genes including factor H (CFH), membrane cofactor protein (MCP; CD46), and factor I (IF)

    • C. 

      Other cases are caused by Von Willebrand factor-cleaving protease (ADAMTS 13) mutations, as well as intracellular defect in vitamin B12 metabolism

    • D. 

      Factor B mutants leading to formation of hyper-functional C3-convertase and mutations of serum thrombomodulin occurs in about 5% of patients with atypical HUS

    • E. 

      Patients with the CFH mutation have the most severe prognosis, with 60% of them developing end-stage renal disease (ESRD) or dying within one year

  • 19. 
    Describe some specific clinical prognostic factors for patients with HUS.
    • A. 

      The functional state of PMN

    • B. 

      Duration of anuria or oliguria

    • C. 

      Renal function assessment for a minimum of one year after HUS should be routinely conducted on all patients

    • D. 

      Long-term follow-up is required for children with proteinuria, hypertension, abnormal ultrasound and/or impaired GFR at 1 year

    • E. 

      The presence of factor H (CFH) and factor I (IF) mutations have been associated with a high incidence of graft failure while membrane cofactor protein (MCP) is associated wit a more favourable outcome

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