nephrotic syndrome n.
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Nephrotic Syndrome. Etiology Idiopathic nephrotic syndrome (90%) Minimal change disease (85%) Focal segmental glomerulosclerosis (10%) Mesangial proliferation (5%) Glomerulonephritis; Membranous nephropathy; and membranoproliferative glomerulonephritis (10%).

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nephrotic syndrome
Nephrotic Syndrome
  • Etiology
  • Idiopathic nephrotic syndrome (90%)
    • Minimal change disease (85%)
    • Focal segmental glomerulosclerosis (10%)
    • Mesangial proliferation (5%)
  • Glomerulonephritis; Membranous nephropathy; and membranoproliferative glomerulonephritis (10%)
idiopathic nephrotic syndrome
Idiopathic Nephrotic Syndrome
  • Approximately 90% of children with nephrotic syndrome have idiopathic nephroticsyndrome
  • Male:female ratio (2:1)
  • Peak incidence: 2 – 6 yr
  • The initial episode and subsequent relapses may follow minor infections and, occasionally, reactions to insect bites, bee stings, or poison ivy.
characteristics
Characteristics
  • Proteinuria
    • >3.5 g/24 hr in adults
    • >40 mg/m2/hr in children
    • Spot urine protein to creatinine ratio >2.0
  • Hypoalbuminemia
    • <2.5 g/dl
  • Edema
  • Hyperlipidemia
proteinuria
Proteinuria
  • Results from increased permeability of glomerular basement membrane (GBM) to plasma protein
slide9
Degrees of proteinuria

Types of proteinuria

Selective proteinuria: where proteins of low molecular weight .such as albumin, are excreted more readily than protein of HMW

Non selective :

LMW+HMW are lost in urine

  • Mildless than 0.5g/m2/day
  • Moderate 0.5 – 2g/m2/day
  • Severemore than 2g/m2/day
hypoalbuminemia
Hypoalbuminemia
  • Due to hyperproteinuria
    • Mainly albumin
edema
Edema
  • plasma oncotic pressure  transudation of fluid from the intravascular compartment to the interstitial space
  • intravascular volume renal perfusion (activates RAAS) tubular reabsorption of sodium water retention
hyperlipidemia
Hyperlipidemia
  • Hypoalbuminemia stimulates generalized hepatic protein synthesis
  • Diminished catabolism of lipids
complications
Complications
  • Infection (major)
    • Bacterial peritonitis – most frequent
    • Sepsis
    • Pneumonia
    • Cellulitis
    • UTI
  • Commonly caused by S. pneumoniae and E.coli
complications1
Complications
  • All children with nephrotic syndrome should receive polyvalent pneumococcal vaccine (if not previously immunized), ideally administered when the child is in remission and off of daily prednisone therapy.
  • Nonimmunenephrotic children in relapse exposed to varicella should receive varicella zoster immune globulin (VZIG) within 72 hr of exposure.
complications2
Complications
  • Thromboembolic events
    • increased prothrombotic factors (fibrinogen, thrombocytosis, hemoconcentration, relative immobilization)
    • decreased fibrinolytic factors (urinary losses of antithrombin ill, proteins C and S)
  • Prophylaxis is not indicated
prognosis
Prognosis
  • Steroid-responsive nephroticsyndrome
    • Repeated relapses
    • Decrease in frequency as the child grows older
prognosis1
Prognosis
  • Children who respond to steroids rapidly and those who have no relapses during the first 6 mo after diagnosis tend to follow an infrequently relapsing course
prognosis2
Prognosis
  • Steroid-resistant nephroticsyndrome
    • most often caused by focal segmental glomerulosclerosis
    • Generally have a much poorer prognosis
prognosis3
Prognosis
  • Recurrent nephrotic syndrome develops in 30-50% of transplant recipients with focal segmental glomerulosclerosis.
prevention
Prevention
  • Cannot be totally prevented
    • Usually follows minor infections, reactions to insect bites, bee stings, or poison ivy
biopsychosocial
BioPsychoSocial
  • It is important to indicate to the family that:
    • The child with steroid-responsive nephrotic syndrome will not develop chronic renal failure
    • The disease is generally not hereditary, and
    • The child (in the absence of prolonged cyclophosphamide therapy) will remain fertile.
biopsychosocial1
BioPsychoSocial
  • To minimize the psychological effects of the condition, the physician should emphasize that the child should be considered normal when in remission and may have unrestricted diet and activity, without the need for urine testing for protein.
biopsychosocial2
BioPsychoSocial
  • Affected children may attend school and participate in physical activities as tolerated.