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Nephrotic Syndrome (Nephrosis) - PowerPoint PPT Presentation


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Nephrotic Syndrome (Nephrosis). Characteristics : Proteinuria ( urine protein loss > 2 gm/day ) Hypo-proteinemia ( serum albumin < 2.5 gm/dL ) Edema Hyperlipidemia Etiology : Idiopathic nephrotic syndrome (90 %) Minimal-change disease (85 %)

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nephrotic syndrome nephrosis
Nephrotic Syndrome (Nephrosis)
  • Characteristics:

Proteinuria ( urine protein loss > 2 gm/day )

Hypo-proteinemia ( serum albumin < 2.5 gm/dL )

Edema

Hyperlipidemia

  • Etiology :
  • Idiopathic nephrotic syndrome (90 %)

Minimal-change disease (85 %)

Focal sclerosis (10 %)  End-stage renal failure

Mesangial proliferation ( 5 %)

  • Glomerulonephphritis; Membranous nephropathy &

Membranoproliferative glomerulonephritis (10 %)

nephrotic syndrome nephrosis2
Nephrotic Syndrome (Nephrosis)
  • Pathology :
  • Idiopathic nephrotic syndrome (90 %)

Minimal-change disease (85 %)

miminal increase in Mesangial cells & matrix

95 % : respond to corticosteroid

Focal sclerosis (progressive) (10 %)  End-stage renal failure

normal or mesangial proliferation

juxta-medullary segmental scarring

20 % : respond to corticosteroid

Mesangial proliferation ( 5 %)

diffuse increase in Mesangial cells & matrix

mesangial IgM & C3 deposits

50~60 % : respond to corticosteroid

nephrotic syndrome nephrosis3
Nephrotic Syndrome (Nephrosis)
  • Clinical manifestations :
  • Idiopathic nephrotic syndrome (90 %)

Boys : girls = 2 : 1

peak age = 2 ~ 6 yr

initial episode & subsequent relapses :

follow an apparent Viral URI

Edema : eyes, lower extremities “ pitting ”

Weight gain

Ascites or Pleural effusions (dependent sites)

Declining urine output

Anorexia

Abdominal pain

Diarrhea

nephrotic syndrome nephrosis4
Nephrotic Syndrome (Nephrosis)
  • Diagnosis :

Urinalysis = 3+ ~ 4+ proteinuria

Microscopic hematuria

Gross hematuria (rare)

low Ccr

Urine Protein loss > 2 gm/day

Serum albumin < 2.5 gm/dL

decreased Total serum Ca

Normal C3

*

nephrotic syndrome nephrosis5
Nephrotic Syndrome (Nephrosis)
  • Complications :

Infections

Spontaneous peritonitis (Streptococcus ; GNB )

* Polyvalent pneumococcal vaccine

Sepsis

Pneumonia

Cellulitis

Urinary tract infections

Arterial & Venous thrombosis

* inhibitors of frinolysis

* decreased plasma Anti-thrombin III

* increased PLT aggregation

* deficiency of coagulation factors IX, XI & XII

nephrotic syndrome nephrosis6
Nephrotic Syndrome (Nephrosis)
  • Treatments :

Divided-dose therapy

time needed for response to prednisolone : 2 wk

* response: urine becames free of protein

* relapse :

recurrence of edema, not simply of proteinuria

Intermittent proteinuria that resolves spontaneously

Alternate-day therapy

5 days after the urine becomes free of protein

continued 3~6 mo

nephrotic syndrome nephrosis7
Nephrotic Syndrome (Nephrosis)
  • Treatments :

* Steroid-resistant :

Proteinuria > 2+ , after 1 mo of continuous daily

divided-dose prednisolone

* Steroid-dependent :

respond to daily divided-dose therapy, but

having relapses shortly after switching to or

after terminating alternate-day therapy

nephrotic syndrome nephrosis8
Nephrotic Syndrome (Nephrosis)
  • Treatments :

Corticosteroid toxicity :

Cushingoid appearance

Hypertension

Growth failure

Immunosuppressive drugs : ( WBC > 5000/mm3 )

Prolong the duration of remission

prevent relapses in children with frequency

relapsing nephrotic syndrome

* Steroid-resistent group:

Pulse methylprednisolone

Immunosuppressive drugs

nephrotic syndrome nephrosis9
Nephrotic Syndrome (Nephrosis)
  • Treatments :

Most children with steroid-responsive nephrosis

have repeated relapses till the disease resolves

spontaneously toward

the end of the 2nd decade of life

*