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Management of Nephrotic Syndrome

Paediatrics CME
by

Guru Bhoojhawon

on 6 May 2011

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Transcript of Management of Nephrotic Syndrome

Management Of Nephrotic Syndrome Guru V Bhoojhawon
Pre Registration House Officer
Department of Pediatrics
Dr A. G. Jeetoo Hospital A child with Nephrotic Syndrome Periorbital Edema Ascites Hydrocele Hydrothorax Oligouria
Blood Pressure
Infection
Hypercoagulable states Investigations Heavy Proteinuria
+++ to ++++ Urine Examination Hyaline Casts Granular Casts Serum Albumin levels
Serum Cholesterol levels
Blood Urea & Creatinine Milky Appearance of Plasma Ig G
Ig M
C3
Renal Biopsy Recommended in children with atypical features:
Age < 1yr
Age > 10yrs
Persistent Hematuria
Low C3
Hypertension
Impaired Renal Function
Proteinuria despite appropriate steroid therapy Treatment Admit
High Protein Diet
Free fluid Intake
Diet with no added salt
Intravenous Albumin in case of depleted vascular space
Penicillin prophylaxis
Low Dose Aspirin
Nephrotic Charting
Corticosteroids for initial episode
Relapses Anuria,
Hypotension,
Poor skin perfusion with skin mottling,
Poor capillary return,
Gross genital edema

Use of Frusemide Dry Fish Eggs Pulses Milk Oral penicillin 12.5 mg/kg/dose bd (prophylaxis) while oedematous.

If the child is profoundly ill or appears to have sepsis use cefotaxime 50 mg/kg/dose 6-hourly to a maximum of 2 g/dose (to cover Strep pneumoniae, H influenzae and E coli).
Weight
Abdominal circumference
Blood Pressure
Urine Protein Corticosteroids Prednisolone
60 mg/m2 per day as a single dose up to (max 80 mg/day) for 4 weeks. Then:
40 mg/m2 per alternate day for 4 weeks.
20 mg/m2 per alternate day for 4 weeks.
15 mg/m2 per alternate day for 4 weeks.
10 mg/m2 per alternate day for 4 weeks.
5 mg/ m2 per alternate day for 4 weeks.

OR

Prednisolone 2mg/kg/day for 6weeks, 1.5mg/kg/day on alternate days for 6 weeks Investigations to monitor treatment Complete Blood Count - Hematocrit levels
Serum electrolytes
Serum Calcium
BUN Relapses Over 75% of patients will experience at least one relapse,
usually in the setting of an intercurrent illness.

A relapse is defined as proteinuria ++++ or +++ for 4 days.
Lower levels of transient proteinuria with fever do not require re-treatment.

Infrequent Relapses
Frequent Relapses

Parent Education
Prednisolone 60 mg/m2 per day till proteinuria dip test result in 0, trace or +.
Then:
40 mg/m2 alternate day for 2 weeks.
20 mg/m2 alternate day for 2 weeks.
15 mg/m2 alternate day for 2 weeks.
10 mg/m2 alternate day for 2 weeks.
5 mg/m2 alternate day for 2 weeks.

If oedema recurs also restart penicillin and aspirin. Steroid Dependent
Steroid Resistant Options for frequent relapses or steroid dependence Alternate day prednisolone 0.3-0.7 mg/kg; duration: 9-12 months*

Levamisole 2-2.5 mg/kg and prednisolone 1.5 mg/kg on alternate days; duration 1-2 years*; prednisolone dose tapered and discontinued

Cyclophosphamide 2mg/kg daily and prednisolone 1.5mg/kg alternate day; duration: 12 weeks

Cyclosporin 5mg/kg daily and prednisolone 1-1.5 mg/kg alternate days; duration: 1-3years*; prednisolone dose tapered

*duration of treatment may be prolonged ...........04...........08...........12...........16...........20...........24......... 60 40 20 15 10 5 Prednisolone in mg/m2 Number of Weeks of treatment ...........trace...........02...........04...........06...........08...........10.......... 0 + 60 40 20 15 10 5 Prednisolone in mg/m2 Number of Weeks Complications in Nephrotic Syndrome Infections Thrombotic Complications Acute Renal Failure Steroid Toxicity Due to the nephrotic state & corticosteroid therapy

Streptococcus pneumoniae
Gram negative organism
Varicella

Children may present with:
Peritonitis
Cellulitis
Pneumonia
Meningitis

Immunization with pneumococcal and varicella vaccine Hypercoagulable state

Thrombosis of renal, pulmonary, and cerebral veins are uncommon but well recognized complications.

Femoral vein thrombosis may follow venepuncture.

Only superficial veins should be used for blood sampling.
Rapid diuresis or AGE may aggravate hypovolemia and precipitate renal failure.

Appropriate preventive measures and judicious fluid replacement is necessary. Repeated and prolonged doses often result in significant steroid toxicity.
Cushingoid features
Short Stature
Hypertension
Osteoporosis
Subcapsular cataract

Timely use of steroid sparing medications is essential to prevent such effects.
Differential Diagnosis Primary (or Idiopathic) Nephrotic Syndrome
Amyloidosis
Vasculitis
Systemic Lupus Erythematosus
Post Infectious Glomerulonephritis
Hepatitis B Nephropathy Minimal Change Nephrotic Syndrome Nephrotic Syndrome with Significant lesions Mesangial Proliferative GN
Focal Segmental glomerulosclerosis
Membranoproliferative GN Thank you...
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