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Nephrotic Syndrome in Children : Causes, Symptoms, Diagnosis, & Treatment

Nephrotic Syndrome in Children : Definition, Etiology, Pathophysiology, Signs, Symptoms, Investigations, & Management

Definition

  • Nephrotic Syndrome is a kidney disorder that presents with collection of characteristic symptoms.

Components

Five components

1. Massive proteinuria

Urine protein excretion > 40 mg/m2/hour

OR

Spot urine Protein/Creatinine > 2:1

OR

Urine dipstick : 3+ or 4+ urinary protein

2. Hypoalbuminemia

Serum albumin < 2.5 g/dl

3. Edema

  • Periorbital puffiness more in the morning.

4. Hyperlipidemia

Serum cholesterol > 250 mg/dl

5. Hypercoagulability of blood

Types

Features Primary (Idiopathic) Secondary
Category Minimal change disease Nephrotic syndrome with significant glomerular lesions ⇓ FSGS > MPGN
Occurrence 80% cases 20% cases
Age group 2-6 year <1 year, >10 year
Symptoms Hematuria, Hypertension ⇓ Rare to absent Hematuria, Hypertension ⇓ Common
Response Excellent response to steroids Variable response to steroids

Pathophysiology

1. Massive proteinuria is because of damage to podocyte-slit membrane complex.

  • Minimal change disease

T-cell activation

Reversible effacement of foot process of podocytes

  • Other lesion

Inflammatory damage

Podocytes/slit membrane/GBM/ or all

  • Congenital nephrotic syndrome

Absence of proteins forming slit membrane

2. Edema

  • Major cause: Overfill hypothesis ⇒ Primary Na+/H2O retention (RSA axis activation)
  • Minor/contributory/initiating cause: Underfill hypothesis ⇒ Decrease in oncotic pressure

Congenital nephrotic syndrome

  • Onset < 3 months
  • High mortality

Types

  • Finnish type: NPHS-1 gene on chromosome 29 ⇒ Nephrin
  • Classical SRNS: NPHS-2 gene on chromosome 1 ⇒ Podocin

Treatment

  • Supportive

Complications

1. Infections

  • Most common : Spontaneous bacterial peritonitis (SBP)

Most common cause in children: Pneumococcus

  • Pneumonia
  • Cellulitis

2. Thrombosis

Most common site

  • Children: Cortical dural veins
  • Adults: Renal vein

3. Growth retardation

4. Anemia

5. Rickets

6. Iatrogenic cushing syndrome

Relevant Terminologies

  • Remission: When urine protein becomes nil or 1+ for 3 consecutive days.
  • Relapse: Patient goes into remission initially but again develops urine protein excretion 3+ or 4+ for 3  consecutive days.
  • Frequently relapsing nephrotic syndrome (FRNS): When more than 2 relapses in 6 months OR more than 3 relapses in one year.
  • Steroid dependent nephrotic syndrome (SDNS): Patient stays in remission on daily full dose steroids but relapses as soon as steroids started on alternate days or within 14 days of stopping steroids.
  • Steroid resistant nephrotic syndrome (SRNS): When failure to attain remission despite 4 weeks of daily full dose of steroids.

Treatment

A. First episode (6+6)

Oral steroid

2 mg/kg daily × 6 week

1.5 mg/kg alternate days × 6 week

Stop

B. Relapse (2+6)

Oral steroid

2 mg/kg daily × 2 week

1.5 mg/kg alternate days × 4 week

Stop

C. FRNS / SDNS / Any relapse with steroid toxicity

Daily oral steroid

+

Steroid sparing agent

D. SRNS

Drug of choice: Calcineurin inhibitors

↓If fails

Monoclonal antibody

Vaccination

Two vaccines are mandatory in childhood nephrotic syndrome

Pneumococcal vaccine

Varicella vaccine

  • Dosing time: 1st dose 4 week after last dose of steroids

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