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Nephrotic syn with thromboembolism

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  • 1. DEPARTMENT OF PEDIATRIC NEPHROLOGY DEPARTMENT OF PEDIATRIC NEUROLOGY MEHTA’S CHILDREN HOSPITAL
  • 2.
    • Complications of NS are
    • Infection
    • Acute renal failure
    • Dyselectrolytemia
    • Fluid overload disorders
    • Bone mineral loss
    • Stunted growth
    • Hypertension
    • Thromboembolism
  • 3.
    • 3⅟₂ years old, Male
    • Diagnosed as NS in April 2008
    • Remission after 12 weeks of oral prednisolone therapy
    • 1 st relapse in March 2009 responded to daily prednisolone therapy
  • 4.
    • Two weeks later when in remission on alternate day prednisolone therapy came with
    • Headache
    • Poor oral intake
    • Progressively increasing lethargy
  • 5.
    • Mild dehydration
    • HR: 98/min, volume good
    • BP: 100/70
    • Fundus normal
    • Systemic examination: essentially normal
  • 6.
    • Urine albumin: Neg
    • Serum albumin: 3.7g/dl
    • Creatinine: 0.5mg/dl
    • Sodium: 137meq/l
    • Calcium: 9.4mg/dl
    • WBC count: 11,500 cmm
    • Polymorphs 60%, Lymphocytes 37%
    • Hb 14, PCV 40%
    • Platelet count: 5.04lacs/cmm
  • 7.  
  • 8.
    • In absence of
    • Dyselectrolytemia
    • Hypertension
    • Hypovolemia
    • CT Brain was done
  • 9.
    • Thrombosis of superior sagital sinus with possible extension to Right transverse sinus
  • 10.  
  • 11.
    • Protein C: 204 (67-195%)
    • Protein S: 101 (77-143%)
    • Anti Thrombin III: 158 (70-122%)
  • 12.
    • Low molecular weight Heparin
    • IV Diuretics for raised ICP
    • Oral anticonvulsants
  • 13.
    • After 1 week of treatment he did not improve symptomatically
    • MRI Brain with MR Venogram was done
  • 14.
    • Thrombosis of superior sagital sinus, torcula, Right transverse sinus and Right sigmoid sinus
  • 15.
    • Oral anticoagulants with dose adjustment was done to maintain INR between 2-3 and was discharged
  • 16.
    • COMPLETE RECOVERY OF RIGHT LATERAL RECTUS PALSY
  • 17.
    • Incidence of Thromboembolism in children with NS is 1.8-5%
    • Bryce A, et al. Epidemiology and risk factors for thromboembolic complications of childhood nephrotic syndrome: A midwest pediatric nephrology consortium study. J Pediatr 2009;155:105-10
    • Mahmoodi BK, ten Kate MK, Waanders F, Veeger NJ, Brouwer JL, Vogt L. High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: results from a large retrospective cohort study.  Circulation . Jan 15 2008;117(2):224-30. 
  • 18.
    • Platelet hyper-aggregability
    • Hypercoagulation
    • Decreased endogenous anti-coagulants
    • Decreased activity of fibrinolytic system
    • Endothelial cell injury
  • 19.
    • Hyperviscosity
    • Hyperlipidemia
    • RBC hyperaggregation
  • 20.
    • Renal vein thrombosis (30-40%)
    • Deep vein thrombosis (15-20%)
    • Pulmonary embolism
    • Mesenteric vein thrombosis
    • Cerebral venous thrombosis (1-2%)
    • Arterial thrombosis
  • 21.
    • Children >12 years of age
    • Congenital NS
    • Severe proteinuria
    • Previous episodes of thrombosis/DVT
    • Central line access
    • SLE
  • 22.
    • Hypertension
    • Hypovolemia
    • Dyselectrolytemia
    • Acute renal failure
    • CNS infection
    • Thromboembolism
  • 23.