A short presentation about Nephrotic syndrome on the pediatrics population.

1. College of Health Sciences & Medicine,
School of Health Sciences, Department of
Pediatrics & Child Health Nursing
Nephrotic Syndromein Children
Aklilu Endalamaw (By Aklilu Endalamaw (Assistance professor of
Pediatrics and Child Health Nursing)
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2. Nephrotic syndrome
• It caused by renal diseases that increase the permeability
of theglomerular filtration barrier.
• Characterized by four of clinicalfeatures
a. Nephrotic range proteinuria – Urinary protein
excretion > 50 mg/kg/day.
b. Hypoalbuminemia – Serum albumin concentration < 3g/dl
(30g/l).
c. Hyperlipidemia
d. Edema
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3. Distribution
• Recorded at all ages but most common among children
1.5-6 years.
• Common on males (Male > females, 2:1 ratio).
• Higher in developing countries
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4. Types with etiology
1. Primary NS: No recognized systemic disease. Theseare patients
with Idiopathic NS (No glomerular inflammation on renal biopsy)
and Primary glomerulonephritis (Active sediment and
glomerular inflammationon biopsy).
2. Idiopathic NS: Minimal Change Disease, Focal Segmental
Glomeruloscrelosis, Membranoproliferative
glomerulonephritis, Mesangial proliferation. Membranous
nephropathy
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5. Cont…
3. Secondary NS: Infections [ Viral (HBV, HCV & HIV), Bacterial
(Streptococcal) and parasites (Malaria), syphilis and toxoplasmosis]
and
• Systemic conditions [Autoimmune, vasculitis), Metabolic
(Diabetes & amyloidosis), Malignancies (Leukemia & lymphomas),
Hematological (SCA), Obesity.]
• Allergy – bee sting, cow’s milk, pollen, house dust.
• Drugs – NSAIDs, Ampicillin, penicillamine, gold, captopril and
lithium.
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6. Cont…
4. Hereditary NS: Below one year
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7. Mechanism
Different Etiologies
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Increase
permeability
of glomerulus
a. Increase protein & lipoprotien
in the
liver…..>
b. Decrease oncotic pressure
Decrease oncotic pressure leads to fluid shift from
intravascular compartment to extracellular
compartment…>
• A decreased intravascular volume leads to
a. increased ADH…..> increase water reabsorption in
collecting ducts..>
b. Decrease renal perfusion pressure…> Activate
renin angiotensin aldosterone system…> increase
tubular reabsorption of Na+ & H2O
c. …>

8. Clinical manifestations
• Periorbital puffiness: more marked inthe morning
and later generalized
• Scrotal edema
• Pleural effusion and Ascites are latefeatures
• Decreased urine output
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9. Laboratory result
• Laboratory analysis of Urine
I. Proteinuria +3 or +4
II. Urinary protein excretion (>40mg/m2/hour)
III. 24 hours urinary protein : creatinine >3
IV. Microscopic hematuria in 10%
V. Pus cells in underlying UTI
VI. Cellular casts except Minimal Change Disease type
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10. Cont…
• Blood analysis
1. Serum: Albumin <3g/dl and Cholesterol > 250mg/dl
2. CBC usually normal, raised ESR
Other
• Chest X-ray to rule out pleural effusion
• Renal biopsy: Frequent relapses, Steroid toxicity, Secondary NS,
Gross hematuria, Hypertension, renal insufficiency
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11. Management
• Diet: Adequate in proteins andcalories, Fluid and salt restriction in
edema
• Monitoring BP in hypovolemia
• Diuretics: massive ascites, pleural effusion andsevere genital edema
• Ambulation &Anti coagulants for thromboembolism
• ACEI if hypertension
• Calcium carbonate and vitamin D if Osteoporosis
• Emphasis to prevent complications: Infections, pneumonia, UTI,
sepsis, cellulitis, arterial & venous thrombosis, steroids adverse
effects
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12. Drug therapy
• Steroids: 2mg/kg (max 60) daily for 6 weeks or
1.5mg/kg (max 40) alternating days for 6 weeks (If
attained remission)
• If NS relapsed with persistent +3-4 needs steroids:
2mg/kg (max 60) daily until remission, then 1.5 mg/kg on
A/D for 4 weeks.
• For steroid dependent and frequent relapses: Long term
steroid therapy (0.3-0.7 mg/kg on A/D for 9-18 months).
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13. Keynotes
• Nephrotic syndrome is an indicators of the presence
of underlying disease s though there is sometimes
idiopathic.
• Most of children withidiopathic NS are steroid sensitive.
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Thank you for wathing!