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Hemolytic uremic syndrome in children (HUS)

Hemolytic uremic syndrome in children (HUS)

 

Hemolytic uremic syndrome in children (HUS)

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HUS is currently recognised by the characteristic triad of:

1)    Thrombocytopenia,

2)    Acute kidney injury (AKI).

3)    Microangiopathic Hemolytic anemia (MAHA),

Although all paediatric HUS exhibit the characteristic triad of findings describing HUS, there’re an underlying several aetiologies of HUS that reflected on variable presentations, managements, & survival. A classifying system is applied to describe the various aetiologies of HUS and is classified into primary & secondary:

·         Primary: include disorders that result in dysregulated complement (C)& rarely mutated proteins in the coagulation pathway & cobalamin metabolism.

·         Secondary: include infectious disorders (e.g., Shiga toxin-producing Escherichia coli [STEC] & Pneumococcus). Other rare causes include AB formation due to C proteins, drug intoxication in paediatric SOT recipients & children with malignancy; SE of quinine, & oral contraceptive pills; & complications in SLE ptns & APA syndrome, or pregnancy.  

 

STEC HUS resembling > 90 % of paediatric HUS. It is usually preceded by a prodromal illness with abd. pain, vomiting, & diarrhoea. Pneumococcal-related HUS is the 2nd  most common infectious disorder > HUS. It is mainly in young children & infants who usually present with pneumonia. Most ptns undergo DX therapy, & extra-renal sequalae are common. Almost 10 % MR & another 10 % of ptns with pneumococcal-related HUS progressing to ESRD.  

Complement-mediated HUS accounts for 5-10 % of HUS paediatric ptns and mainly related to genetic mutations for C proteins C3, CD46 (known before as membrane cofactor protein [MCP]), and C factors H, I, & B. Acquired C dysregulation due to AB to C proteins has also been greatly implicated in the evolution of C -mediated HUS.  

 

Dgx:of HUS is clinically relied on the presence of the classical triad of MAHA, thrombocytopenia, & AKI. Confirmatory laboratory testing includes CBC (e.g., HB/Hct & platelet count) and peripheral blood smear, Kidney function testing, & urinalysis.  

DD: for HUS includes disorders that present with concomitant findings of anemia, thrombocytopenia, & AKI. These include DIC, TTP, & inborn error of vit. B12. Further evaluation is required to recognise the underlying cause & DD HUS from others.  

1.    Historical clue is useful in Dgx STEC (PH of STEC exposure or episodic HUS in family member), C-mediated HUS (non-current FH of HUS or past history (PH) of HUS), or a secondary cause (e.g., PH of predisposing medical disorder or drug).

2.    In addition, lab testing includes coagulation profile, survey for STEC, blood culture of blood and other pertinent bodily fluids, C studies, & cobalamin profile.

The initial management of HUS is mainly supportive, as follows:

1)    RBCs transfusion for anemia if the HB level reaches 6-7 g/dL or Hct <18 %.

2)    Platelet transfusion for ptns having significant or life-threatening bleeding or if an invasive intervention is planned.

3)    Proper fluid & electrolyte correction to keep adequate intravascular optimization & avoiding electrolyte alterations.

4)    Holding nephrotoxic agents or implicating in the development of HUS.

5)    Commencing DX in ptns with symptomatic uraemia, azotaemia (BUN >80 mg/dL [29 mmol/L]), fluid overload, or electrolyte abnormalities resistant to medical therapy.

6)    Commencing proper nutrition.

 

Use of other maneuverers is related on the underlying cause of HUS.

  • STEC HUS.  
  • C-mediated HUS.  
  • Pneumococcal-related HUS: initial empiric AB therapy in ptns with pneumococcal-related HUS. Due to the increased prevalence of AB-resistant strains of pneumococcus, covering should include both vancomycin + broad-spectrum cephalosporin (e.g., cefotaxime or ceftriaxone) while awaiting C&S results.

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