nephrotic syn with thromboembolism

DEPARTMENT OF PEDIATRIC NEPHROLOGYDEPARTMENT OF PEDIATRIC NEUROLOGYMEHTA’S CHILDREN HOSPITAL

Complications of NS are1.Infection2.Acute renal failure3.Dyselectrolytemia4.Fluid overload disorders5.Bone mineral loss6.Stunted growth 7.Hypertension8.Thromboembolism

3⅟₂ years old, MaleDiagnosed as NS in April 2008Remission after 12 weeks of oral

prednisolone therapy1st relapse in March 2009 responded

to daily prednisolone therapy

Two weeks later when in remission on alternate day prednisolone therapy came with

Headache Poor oral intakeProgressively increasing lethargy

Mild dehydrationHR: 98/min, volume goodBP: 100/70Fundus normalSystemic examination: essentially

normal

Urine albumin: NegSerum albumin: 3.7g/dlCreatinine: 0.5mg/dlSodium: 137meq/lCalcium: 9.4mg/dlWBC count: 11,500 cmmPolymorphs 60%, Lymphocytes 37%Hb 14, PCV 40%Platelet count: 5.04lacs/cmm

In absence of DyselectrolytemiaHypertension Hypovolemia CT Brain was done

Thrombosis of superior sagital sinus with possible extension to Right transverse sinus

Protein C: 204 (67-195%)Protein S: 101 (77-143%)Anti Thrombin III: 158 (70-122%)

Low molecular weight Heparin IV Diuretics for raised ICPOral anticonvulsants

After 1 week of treatment he did not improve symptomatically

MRI Brain with MR Venogram was done

Thrombosis of superior sagital sinus, torcula, Right transverse sinus and Right sigmoid sinus

Oral anticoagulants with dose adjustment was done to maintain INR between 2-3 and was discharged

COMPLETE RECOVERY OF RIGHT LATERAL RECTUS PALSY

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. 

Platelet hyper-aggregabilityHypercoagulation Decreased endogenous anti-

coagulantsDecreased activity of fibrinolytic

systemEndothelial cell injury

Hyperviscosity HyperlipidemiaRBC hyperaggregation

Renal vein thrombosis (30-40%)Deep vein thrombosis (15-20%)Pulmonary embolismMesenteric vein thrombosisCerebral venous thrombosis (1-2%)Arterial thrombosis

Children >12 years of ageCongenital NSSevere proteinuriaPrevious episodes of thrombosis/DVTCentral line accessSLE

HypertensionHypovolemiaDyselectrolytemiaAcute renal failureCNS infectionThromboembolism