HUS and SLE - Pediatrics Flashcards
definition of Hemolytic Uremic syndrome
HUS is a potentially life-threatening disease. It comprises:
* Acute renal failure
* Microangiopathic haemolytic anaemia (red cell fragments and schistocytes on blood film)
* Thrombocytopenia
Causes of HUS
- Diarrhoea-positive HUS
* Verotoxin producing Escherichia coli 0157 (10% of this organism result in HUS)
* Other diarrhoea infection, e.g. shigella (Shiga toxin), salmonella, campylobacter - Diarrhoea-negative HUS
* Familial disease: children with presenting age < 1 have a poor prognosis.
* Complement: factor H deficiency
* Drug related: cyclosporin.
Clinical features of diarrhoea-positive disease
- Usually, < 5 years old
- Bloody diarrhoea; may resolve, then 5–10 days later oliguria, pallor, lethargy and petechiae
- Hypertension and hyperkalaemia are major causes of morbidity.
- Other organs may be damaged (leading to fits or coma, or pancreatitis)
Investigations of HUS
- Blood: CBC and film (microangiopathic haemolytic anaemia,thrombocytopaenia), Coagulation screen (normal), Serum creatinine, calcium, phosphate
- Urine analysis: mild haematuria and proteinuria
- Stool analysis: with culture and sensitivity
- C3 level
- Factor H level
- Genetic study
Management of HUS
- Supportive: fluid status assessment ± diuretics
- Hyperkalaemia: treatment as necessary.
- Dialysis: if needed
- Blood transfusion: rarely platelets are used.
- Plasmapheresis and immunosuppressive in resistant cases
- Recently monoclonal antibodies against complement C5
NB: Long term follow-up is essential to look for hypertension and CRF.
Risk factors of SLE
o Genetic predisposition.
o Hormonal role may exist.
o Environmental: exposure to UVR of the sun.
o Females > males, usually > 8 years.
Clinical picture of SLE (Diagnostic criteria)
- Malar rash; fixed erythema over malar eminence.
- Photosensitivity; unusual rash on exposure to sun light.
- Discoid rash.
- Painless oral ulcers
- Arthritis.
- Serositis: Pleurisy or pericarditis with or without effusion.
- Renal: - proteinuria > 3+ or cellular casts e.g. RBCs casts.
- Neurologic: - seizures or psychosis.
- Hematologic: Hemolytic anemia or Leukopenia or Thrombocytopenia.
- Immunologic disorders: Anti- DNA or Anti-Smith or Anti-phospholipid antibodies.
- Anti-nuclear antibody (ANA).
Investigations in SLE + ttt
-CBC, KFT, LFT, reticulocytic count and coombs test for haemolytic anemia
-Urine analysis, protein creatinine ratio
-C3 and C4 usually consumed in activity
-ESR may be very high over 100
-Immunological work up ANA, anti DNA positive
-Renal biopsy in case of lupus nephritis
Treatment: Steroid therapy and Immunosuppressive drugs
