About Disease
Introduction
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Nephrotic syndrome is the clinical manifestation of glomerular disease associated with heavy (nephrotic-range) proteinuria.
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Nephrotic range proteinuria is defined as proteinuria >3.5 gm/24 hr or a urine protein:creatinine ratio >2.
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It is characterized by hypoalbuminemia ( <2.5 gm/dL), oedema and hyperlipidemia (200mg/dL). ​
Cause/Risk:
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Primary/Idiopathic Nephrotic Syndrome.
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Infections: Hepatitis B and C, Malaria, HIV.
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Drugs: NSAID'S, Antibiotics.
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Malignant Disease: Lymphoma, Wilms tumor, Leukemia.
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Immunologic Disorders: SLE.
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Signs & Symptoms:
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Bloating
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Puffy Eyes
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Generalized body swelling (oedema pitting type) around the eyes and ankles.
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Weight gain
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Fatigue
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Loss of Appetite
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Foamy urine
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Pleural Effusion
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Complications:
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Infections: Sepsis, Pneumonia, Peritonitis and UTI.
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Thromboembolism
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Cardiovascular complications: Hyperlipidemia, Hypercholesteremia, Hypoalbuminemia, Hypertension, Hypothyroidism.
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Anemia: Microcytic, Hypochromic, Iron-deficiency.
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Acute Renal Failure.
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Oedema.
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How Old Are The Children When The Symptoms Begin:
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Congenital Nephrotic Syndrome: From birth to 3 months.
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Infantile Nephrotic Syndrome: From 3 to 12 months.
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Childhood Nephrotic Syndrome: From 12 months or older.
Types:
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Primary Nephrotic Syndrome: this is caused by three different types of kidney disease namely; minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy.
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Secondary Nephrotic Syndrome: this is caused due to namely; systemic disease (IgA vasculitis and lupus), infections (hepatitis B, hepatitis C, HIV and malaria), blood disease (leukemia, lymphoma, sickle cell disease) and NSAID's.
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Congenital Nephrotic Syndrome: this is caused due to genetic variants, infection present on or before birth (syphilis, toxoplasmosis).
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Classification:
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Steroid Sensitive Nephrotic Syndrome: patient's who enter remission in response to corticosteroid treatment alone.
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Steroid Resistant Nephrotic Syndrome: patient's who fail to enter remission after 8 weeks of corticosteroid treatment.
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Steroid Dependent Nephrotic Syndrome: patient's who enter complete remission but develop relapse either while still receiving steroids or discontinuation of treatment.
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Frequently Relapsing Nephrotic Syndrome: patient's who enter complete remission in response to steroids and remain in remission after discontinuation of treatment but develop frequent relapses (4 times in 12 months).
Diagnosis:
Tests and procedures used to diagnose Pediatric Nephrotic Syndrome include
ROUTINE INVESTIGATIONS
HAEMATOLOGY TESTS
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Hb
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Total WBC count
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Differential leucocyte count
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URINE ANALYSIS
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Protein Dipstick
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Sugar
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Microscopy
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Culture sensitivity
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BIOCHEMISTRY
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​Blood urea
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Serum cholesterol
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Serum electrolytes
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Serum creatinine
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SPECIFIC INVESTIGATIONS
URINE
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24 hour Urine Protein: To confirm Nephrotic range proteinuria (>40mg/sqm/24hr, urine protein:creatinine ratio >2).
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HbsaAg
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MANTOUX TEST
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Evaluation of epidemiological status before treatment with corticosteroids.
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CHEST X-RAY
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To check for pleural effusion.
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RENAL BIOPSY
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To check the prognosis of the patient.
Clinical Manifestations For Nephrotic Syndrome In Paediatrics
ABOUT DRUGS
MEDICAL ATTENTION
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Admit to hospital on first presentation.
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If the child is profoundly ill or appears to have sepsis treat accordingly.
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Managing oedema.
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Steroid therapy (Prednisolone).
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Immunosuppressant (Cyclosporine, Cyclophosphamide).
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Prophylaxis against complications (Infection & Gastritis).