Nephrotic Syndrome in Children

Definitions :

Response: U.Alb trace/ neg for 3 days.

Relapse: U.Alb > 3+ for 3 days

FRNS: >2 relapses in 6 months or > 4 in 1 year

SDNS: Two consecutive relapses while tapering steroids or within 14 days of stopping steroids.

SRNS: U.Prot >3+ after 4 weeks of steroid therapy

Classification :

Primary NS:

Secondary NS: SLE, HSP, Lymphoma

Congenital NS: TORCH infection

Infantile NS: Finnish (DMS)

Nephrotic Syndrome in Children :

Incidence: 90-100 per million population

Age: 2- 6years

More common in males

95% children primary glomerular disease- MCNS

Clinical Manifestations :

Periorbital Oedema

Oedema feet

Distension of abdomen

Genital Oedema

Check Blood pressure

Presence of Infection:Redness, Tenderness , Skin Rash, Joints & Systemic involvement

Side Effects of Steroids :

Steroid Facies

Hypertension

Gastritis

Cataracts

Hyperglycemia

Proximal Myopathy

Hyperglycemia

Osteoporosis

Educate the Parents :

Check Urine protein at home

Bring child early to avoid complications

Maintain a diary

Avoid Extra Salt in diet

When child has proteinuria, avoid extra protein

Explain that disease might have relapses in future

Avoid vaccines when child is on steroids

 

 

Treatment of Edema :

Salt restriction

Daily weight chart & I/O Chart must

Diuretics: Frusemide, Spironolactone, Metolazone

IV Albumin

Investigations :

Urine Routine CBC, ESR S.Protein, S.Albumin S.Creatinine, S.Cholesterol

X Ray Chest MT

No need of 24 hrs Urine protein Urine protein/ Creatinine spot ratio

USG not necessary in every child

Nephrotic Syndrome is characterized by :

Nephrotic Range proteinuria

Hypoproteinemia

Oedema

Hypercholesterolemia

Higher Incidence of Infections, Thrombosis & Side effects of Therapy

Indications for kidney Biopsy :

Age at presentation <1yr or > 10 yrs

Atypical features like high BP, Persistently elevated S.Creat or gross hematuria

Low C3

Steroid resistant NS

Cyclosporine Treatment

Treatment of First episode :

Treat the infection if present

If no infection then consider steroids 2 mg/kg/day for 6 weeks followed by 1.5 mg/kg every alternate day for 6 weeks

Give Calcium supplements & antacids

SRNS :

Could be due to Genetic disorder

Early or late SRNS

Drugs useful are Cyclosporine, pulse steroids, ACEI or receptor blockers

Treatment of edema

Statins for hypercholestoloremia