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 (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 (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 (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 (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 (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 (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 (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 (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