NS is a glomerular disorder characterised by

a tetrad of:Proteinuria more than1g/m2/24hr

Hypoprotinuria ( albumin less than2.5gm/dl)

Hypercholestrolemiamore than220mg/dl

Edema

CLASSIFICATIONo Idiopathic nephrotic syndrome (90% of cases)

Minimal change nephrotic syndrome Nephrotic syndrome with mesangial proliferation Nephrotic syndrome with focal sclerosis

o Nephrotic syndrome secondary to glomerulonephritis (10% of cases) Membranous glomerulopathy MPGN OTHRS as SLE and HSP

o Congenital nephrotic syndrome AR presenting at birth or during the 1st 6 months

• The nephritic syndrome is a clinical condition characterized by:

Heavy proteinuria: Hypoalbuminemia : Oedema: Hypercholestrolemia ABSENCE OF nephritic manifestations as hematuria, h

ypertension, renal failure and hypocomplementemia . Good response to steroids.

• Commenest type of nephrotic syndrome

• Age : peak incidence 2-6 years ( can occur in 1st year or in adults).

• Sex: more in boys( male to female ratio is 2: 1)

Etiology and Pathogensis

GFR

Glomerular basement membrane (GBM)

Pathogenesis of nephrotic diseases

• LIGHT MICROSCOPY : glomeruli appear normal.

• IMMUNOFLURESCENCENT MICROSCOPY : negative n( no deposition of immune complexes.

• ELECTRON MICROSCOPY : retraction of fppt processes of podocytes

The initial attack and subsequent relapses may follow a viral upper respiratory infection.

Edema.

Weight gain.

Diminshed urinary output.

Respiratory difficulty.

Diarrhea.

Normal blood pressure.

Manifestations of complications.

Oedema• Around the eyes in the morning, and around the

ankles in the evening

• There is permanent swelling of ankles and face

• Severe: With increasing edema, ascites and genital edema may appear, followed by pleural effusions

• The edema remains soft and pit:

• Increase suscebtability to infection.

• Hypercoagulation and thrombosis arterial and venous thrombosis )

• Why are nephrotic patients more susceptible to infection?

Decrease immunity Edema fluid is a good culture medium. Immunosupressive therapy.

• Most common organism: Strept. Pneumoniae. Gram negative organisms.

• Most common sites of infection Peritonitis (commonest ) Sepsis, pneumonia, cellulitis, urinary tract infection.

• Manifestations of infection during steroid therapy: minimal signs of infection ( mild fever may be the only sign )

Ascites+fever =suspect peritonitis & do culture of ascitic fluid

• Due to increase prothrompotic factors and decrease fibinolytic factors, most common site is renal vein thrombosis

Lab findings

• Urine analysis

• Serum and blood

• Renal functions

Protinuria Exceeds 40mg/m2/hr

1gm/m2/24hrs3or4+ by dip stick

Spot urine protein to creatinine exceeds 2 or 3

Other causes of edema and hypoprotinaemia

Kwashiorkor and marasmic kwash.

Acute nephritic syndrome may present with marked edema and proteinuria

• The two principle lines of treatment are

Effort to reduce edema.Specific therapy with prednisone

• Hospitalisation, investigations and exclusion of contraindications to steroids

• Physical activity• Diet and fluids.• Diuretics are used cautiously

(over dose of duritics hypovolemia Hypotension Iatrogenic shock)

• Prednisone : 60mg/m2/day given in 3 divided daily doses, for 4 weeks then start alternate day therapy.

• Alternate day therapy : prednisone 40mg/m2/day taken as single morning dose with break fast . The alternate day therapy is then tappered slowly and discontiuned over the next 3 months.

• Relapse :• Proteinuria more than 3+ and edema

• Daily steroids is given until proteinuria is negative or only trace by dip stick for 3 consecutive days ,then the patient is shifted to alternate day therapy and treatment is tapered over 2 months

Classification according to response to steroids

• Steroid responsive :Children who respond to treatment within 8

weeks of treatment.

Children with no relapses Children with relapses

Infrequent relapserFrequent relapserSteroid dependent

Infrequent relapser : relapse less than 4 times in a 12 months period.

Frequent relapser: relapse more than 4 times within 12 months period

Steroid dependent: relapse while on alternate day therapy or eithin 14-28

days of stopping treatment

Classification according to response to steroids

• Steroid resistant:

Patients who fail to respond to treatment within 8 weeks of treatment.

Cytotoxic drugs( as cyclophosphamide)

• Indications :

• Corticosteroid toxicity in frequent relapsers and steroid dependent,

• Steroid resisrent nephrotic syndrome (after renal biopsy )

Vaccines

• Pneumococcal and varicilla vaccines may be given once the child is in remission.