This document provides an overview of hemolytic uremic syndrome (HUS). It begins with background information, noting that HUS is characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. It is most common in children under 5. HUS is then classified as typical (diarrhea-associated) or atypical. The etiopathogenesis of typical HUS involves shiga toxin-producing bacteria like E. coli damaging endothelial cells. Clinically, HUS presents with anemia, oliguria, hematuria, and hypertension. Investigations show anemia, thrombocytopenia, and schistocytes on blood smear. Management involves supportive care, antibiotics, and plasma therapy. The

1. HEMOLYTIC UREMIC SYNDROME
• Presenter- Hassani Bakari
• Facilitator- Dr. Joshua( Pediatrician)

2. OUTLINE OF THE PRESENTATION
• Background
• Classification of the hemolytic uremic syndrome
• Etiopathogenesis
• Clinical features
• Investigation
• Management

3. • BACKGROUND
• It is a clinical syndrome characterized by
 macroangiopathic hemolytic anemia.
Thrombocytopenia
Acute kidney failure
• it is the most common cause of AKI in children.
• Predominantly affect children( under 5)

4. TYPES OF HUS
• Into two broad categories
Typical HUS- diarrhea associated (commonest one
Atypical HUS- non diarrhea associated( infrequent and most
severe)

5. infection associated Systemic disease
associated
Drug induced Genetic
• Verotoxin/shiga
like toxin
producing E.coli(0
157:H7), Shiga
toxin producing
shigella(STPSD
1)
• Systemic Lupus
Erythematosus
• Quinine(most
common)
• Cytotoxic drugs
such as Cisplatin,
mitomycin C
• Von Willebrand
Factor-cleaving
protease
deficiency(ADAM
TS 13)
• Neuraminidase
producing
S.pneumonia
• Anti phospholipid
syndrome
• Cyclosporine • Complement H
and I deficiency
• HIV
CLASSIFICATION OF HUS BASED ON ETIOLOGY

6. E.coli

7. SOURCES OF INFECTION
• Milk and animal products( poorly cooked beef ,pork,
lamb)
• Human feco-oral transmission
• Vegetables ,salads drinking water contaminated with the
bacteria

8. ETIOPATHOGENESIS –typical HUS
• Typical / Diarrhea associated
• Commonly due to shiga toxin producing shigella dysenteriae type 1(developing nations)
• And Escherichia coli serotype 0157:H7 ( commonly ,In Developed nations)
• Shiga toxin or shiga like toxin cause endothelia damage
• Leading to platelets adhesion, aggregation, and activation
• Leading to narrowing of small vessels in glomerular due to formed thrombi
• This cause damage to passing RBC leading to hemolytic anemia

9. ETIOPATHOGENESIS –typical HUS………….
• Formed thrombi reduce GFR leading to AKI
• Consumptive thrombocytopenia as platelets adhere to damaged endothelial

10. Pathogenesis of typical HUS

11. CLINICAL FEATURES
• HUS develops 5-10 days after onset of diarrhea, abdominal pain
and fever
• Then acute anemia(pallor)
• Oliguria
• Hematuria and Hypertension

12. CLINICAL FEATURES……..
• Complications of fluid overload( Pulmonary edema,
Hypertensive encephalopathy)
• Neurological manifestation such as
 irritability
Seizures
Encephalopathy
• Bleeding manifestation ( possible but rare)

13. INVESTIGATIONS
• CBC-anemia, thrombocytopenia and reticulocytosis
• Peripheral blood smear (schistocytes)
• LDH
• Bilirubin level-unconjugated
• RFT-Cr and BUN
• Urine analysis –hematuria, proteinuria, hemoglobinuria

14. INVESTIGATION…..(schistocytes)

15. INVESTIGATION…….
• To check for EHEC or Shigella from stool culture
• From urine culture(Rarely HUS can arise from UTI due to
E.Coli

16. MANAGEMENT OF HUS
• Supportive
• Antibiotics
• Plasma therapy

17. a) SUPPORTIVE
• It is cornerstone of primary approach
• If there is fluid deficit-fluid supplementation is of paramount
importance
• If the is fluid overload –fluid restriction
• Correct electrolyte imbalance-hyponatremia, hyperkalemia

18. a) supportive care…….
• Early initiation of dialysis if –(a ,e ,i, o ,u )
• If significant anemia is present –give PRBCs very carefully
• Platelet transfusion- not generally required( to be considered
only in uncontrolled severe bleeding)

19. (b) Use of antibiotics
• Use of antimicrobial agent{contradicting)- no evidence of
benefit
• [c] Plasma infusion in severe cases
• Complement replacements

20. PROGNOSIS OF HUS
• Immediate outcome of classical HUS is relative better than
aHUS
• It has less than 5% mortality with good supportive care
• 10-30% develop CKD
• 5-10% develop ESRD in the next 10yrs

21. •THANK YOU

22. REFERENCES
• Journal of nephrology and therapeutic-review of articles on HUS by
Jagabandhu et al
• Current diagnosis and treatment book 22 edition
• Medscape