Nephrotic syndrome

History:-
             Edema- around the orbits first then around the legs, ascites, and on the dependent parts of the body, hydrocele and hydrothorax.
        Decreased urine output.
        Infections
        Hypertension occurs if there is  significant lesions in glomerulus.
          
Signs-
      Massive proteinuria- 3 + TO 4+ ( 1 gm/m*2 per day)
      Hypoabuminemia ( less than 2.5 g/ dl)
      Hyperlipidemia
      Hematuria
      Hypertension.

Etiology:

    Idiopathic -90 percent
  Other causes are psgn, amiloidosis, lupus etc..

Types
   1) (Low molecular weight nephrotic syndrome)- (highly selective proteinuria) albumin
   2) (High molecular wt nephrotic syndrome)- globulin

1)Minimal change disease

2)Nephrotic syndrome with significant lesion.

    A) Steroid sensitive nephrotic syndrome.

   B) Steroid resistant nephrotic syndrome.

Pathology
      Obscure
Theories-  IgM mediated glomerular damage.
     Petrubance of cell mediated immunity causing damage to glomerular epithelium causing leakage.
    
Investigation-
       Proteinuria- 3+ TO 4+
       Hematuria - gross - significant lesions.
      Low serum albumin<1g/ dl
      White plasma due to hyperlipidemia ( due to increased production of beta lipoproteins by liver)
     IgG  reduced IgM elevated.

Tests
      Urine analysis
      Complete blood count
     Tuberculin test
      Anti streptolysin O
     Blood biochemistry- urea createnin, albumin, globulin, lipids.
     Anti nuclear antibodies- lupus
     C3.

Treatment-
       High protein diet
       Low sodium intake
       Decreased lipids intake.

    In case of significant edema
       Diuretics- furasemide (1-4 mg/ kg / day)
       Spirinolactone(2-3 mg/kg/day)
Steroid therapy- prednisone
    Prednisolone.
( 2 mg/kg for 6 weeks)
(1.5 mg/kg for next 6 w in alternate days as morning dose)

Parents- explain about the disease to parents and regular check for protein levels should be checked by them.

Relapse-
      Significant relapse 3- 4+ proteinuria
  2 mg/kg/ day till no protein can be found in urine for 3 consequtive days.
Then 1.5 mg/ kg for 5- 6 wks.

Steroid dependent-
      Low doses of steroids.
      Steroid sparring agents-
         Levamisol- 2.5 mg/ kg every alternative days, Till Steroid level is reduced to .3 - .5 mg/ kg. Continue for 6 mts then stop steroids.
  Side eff- leukopenia

Cyclophospomide- 12 wk course, side eff- leukopenia, vomiting.

Cyclosporin.

Steroid resistant-
   Prednisone + Cyclosporin alt days.

ACE inhibitors- enlapril -0.3 - 0.6 mg/ kg.

Complications of nephrotic syndrome-

Edema, steroid toxicity, thrombotic complications, infections, hypovolumia, acute renal failure.