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