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NEPHROTIC SYNDROME
INTRODUCTION
• Common cause for hospitalization among
children.
• It’s a syndrome caused by renal disease that
increases the permeability across the
glomerular filtration barrier.
DEFINITION
A group of clinical findings characterized by-
• Proteinuria
• Hypoalbuminemia
• Hyperlipidemia
• Edema
Sometimes – hematuria, hypertension &
reduced glomerular filtration rate.
INCIDENCE
• 2-7 cases per 100,000 children per year
• Higher in underdeveloped countries (South
east Asia)
• Occurs at all ages but prevalent in children
between the ages 1.5-6 years.
• It affects more boys than girls, 2:1 ratio.
CLASSIFICATION
1. Idiopathic Nephrotic Syndrome-
• Majority cases in childhood.
• Autoimmune phenomenon
• Two types-
a. Minimal change NS
b. Significant change NS-
i. Focal segmental glomerulosclerosis
ii. Membranous glomerulonephritis
iii. Membranoproliferative glomerulonephritis
iv. Rapidly progressive glomerulonephritis
2. Secondary Nephrotic Syndrome-
• About 10% in children of all cases.
• Secondary to disease condition like
glomerulonephritis, DM , SLE, malaria,
hepatitis B, HIV, etc.
3. Congenital Nephrotic Syndrome-
• Rare but serious condition.
• Associated with congenital anomalies of
kidney.
• Autosomal recessive disease
• Severe renal insufficiency and urinary
infections.
4. Infantile Nephrotic Syndrome-
• Major causes-
• NPHS2
• Diffuse mesengial sclerosis
ETIOLOGY & RISK FACTORS
Primary Renal cause-
• Minimal change nephropathy
• Glomerulosclerosis
• Acute post streptococcal glomerulonephritis
• Immune complex glomerulonephritis.
Systemic cause-
• Infections
• Toxins- Hg, Bi, Au
• Allergic- pollen, food allergy
• CVS- SCD,CHF, renal vein thrombosis
• Malignancies- Leukemia
• Others- systemic lupus erythematosus
PATHOPHYSIOLOGY
Alteration in glomerular basement membrane
Altered glomerular protein permeability
Massive proteinuria
Decreased oncotic pressure
Decreased vascular volume
Decreased renal blood flow
Activation of renin -angiotensin –aldosterone system & release of ADH
Tubular Na and water reabsorption
EDEMA
CLINICAL MANIFESTATION
• Periorbital puffiness
• Pitting edema over legs
• Generalised edema
• Ascites
• Hypertension
• Loss of appetite
• Weight gain
• Pleural effusion
• hydrothorax
• Hydrocele
• Hematuria
• Weakness
• Fever
• Joint pain
DIAGNOSIS
• Palpation
• Urine analysis-Dipstick test for proteinuria
(>3.5g/lt/day)
• Blood test-
Blood total serum albumin- reduced
serum albumin-reduced (2.5 g/dl)
Serum globulin-normal or increased
Cholesterol-increased
Serum creatinine-normal or elevated
Serum complement level-normal or low
• Needle biopsy of kidney
• Renal ultrasound
• ECG
• KUB-X ray
• Renal scan
• Intravenous urogram
MEDICAL MANAGEMENT
• Steroid therapy-
Prednisolone(drug of choice)
Daily dose 2mg/kg/day in 2-3 divided doses for
atleast 4-6 weeks
• Antibiotic therapy-
Penicillin antibiotics
• Diuretics-
Furosemide (1-44 mg/kg/day in 2 divided doses)
• Albumin infusion-
1 g/kg/day
• Immunosuppressive drugs-
Cyclophosphamide(2mg/kg daily for 12 weeks)
SURGICAL MANAGEMENT
Renal transplantation
• Dietary management-
High protein
High carbohydrate
Sodium restricted
Water restricted occasionally.
COMPLICATION
• Acute renal failure
• Renal vein thrombosis
• Atherosclerosis
• Chronic kidney disease
• Fluid overload
• Congestive heart failure
• Pulmonary edema
• infections
NURSING MANAGEMENT
• Excess fluid volume related to decreased kidney
function as evidenced by pitting edema or
periorbital puffiness.
• Imbalanced nutrition less than body requirement
related to damaged metabolism as evidenced by
anorexia,loss of protein,rejection of low salt diet.
• Fatigue related to discomfort as evidenced by
lethargy, extreme edema.
• Risk for infection related to immunosuppressive
drugs
PROGNOSIS
• Early diagnosis and appropriate treatment
80% children recover
• 10-15% becomes complicated with chronic
renal failure
• 2-4% cases may have fatal outcome
THANK YOU

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Nephrotic Syndrome

  • 2. INTRODUCTION • Common cause for hospitalization among children. • It’s a syndrome caused by renal disease that increases the permeability across the glomerular filtration barrier.
  • 3. DEFINITION A group of clinical findings characterized by- • Proteinuria • Hypoalbuminemia • Hyperlipidemia • Edema Sometimes – hematuria, hypertension & reduced glomerular filtration rate.
  • 4. INCIDENCE • 2-7 cases per 100,000 children per year • Higher in underdeveloped countries (South east Asia) • Occurs at all ages but prevalent in children between the ages 1.5-6 years. • It affects more boys than girls, 2:1 ratio.
  • 5. CLASSIFICATION 1. Idiopathic Nephrotic Syndrome- • Majority cases in childhood. • Autoimmune phenomenon • Two types- a. Minimal change NS b. Significant change NS- i. Focal segmental glomerulosclerosis ii. Membranous glomerulonephritis iii. Membranoproliferative glomerulonephritis iv. Rapidly progressive glomerulonephritis
  • 6. 2. Secondary Nephrotic Syndrome- • About 10% in children of all cases. • Secondary to disease condition like glomerulonephritis, DM , SLE, malaria, hepatitis B, HIV, etc.
  • 7. 3. Congenital Nephrotic Syndrome- • Rare but serious condition. • Associated with congenital anomalies of kidney. • Autosomal recessive disease • Severe renal insufficiency and urinary infections.
  • 8. 4. Infantile Nephrotic Syndrome- • Major causes- • NPHS2 • Diffuse mesengial sclerosis
  • 9. ETIOLOGY & RISK FACTORS Primary Renal cause- • Minimal change nephropathy • Glomerulosclerosis • Acute post streptococcal glomerulonephritis • Immune complex glomerulonephritis.
  • 10. Systemic cause- • Infections • Toxins- Hg, Bi, Au • Allergic- pollen, food allergy • CVS- SCD,CHF, renal vein thrombosis • Malignancies- Leukemia • Others- systemic lupus erythematosus
  • 11. PATHOPHYSIOLOGY Alteration in glomerular basement membrane Altered glomerular protein permeability Massive proteinuria
  • 12. Decreased oncotic pressure Decreased vascular volume Decreased renal blood flow Activation of renin -angiotensin –aldosterone system & release of ADH Tubular Na and water reabsorption EDEMA
  • 13. CLINICAL MANIFESTATION • Periorbital puffiness • Pitting edema over legs • Generalised edema • Ascites • Hypertension • Loss of appetite • Weight gain
  • 14. • Pleural effusion • hydrothorax • Hydrocele • Hematuria • Weakness • Fever • Joint pain
  • 15. DIAGNOSIS • Palpation • Urine analysis-Dipstick test for proteinuria (>3.5g/lt/day) • Blood test- Blood total serum albumin- reduced serum albumin-reduced (2.5 g/dl) Serum globulin-normal or increased Cholesterol-increased Serum creatinine-normal or elevated Serum complement level-normal or low • Needle biopsy of kidney
  • 16. • Renal ultrasound • ECG • KUB-X ray • Renal scan • Intravenous urogram
  • 17. MEDICAL MANAGEMENT • Steroid therapy- Prednisolone(drug of choice) Daily dose 2mg/kg/day in 2-3 divided doses for atleast 4-6 weeks • Antibiotic therapy- Penicillin antibiotics • Diuretics- Furosemide (1-44 mg/kg/day in 2 divided doses)
  • 18. • Albumin infusion- 1 g/kg/day • Immunosuppressive drugs- Cyclophosphamide(2mg/kg daily for 12 weeks) SURGICAL MANAGEMENT Renal transplantation
  • 19. • Dietary management- High protein High carbohydrate Sodium restricted Water restricted occasionally.
  • 20. COMPLICATION • Acute renal failure • Renal vein thrombosis • Atherosclerosis • Chronic kidney disease • Fluid overload • Congestive heart failure • Pulmonary edema • infections
  • 21. NURSING MANAGEMENT • Excess fluid volume related to decreased kidney function as evidenced by pitting edema or periorbital puffiness. • Imbalanced nutrition less than body requirement related to damaged metabolism as evidenced by anorexia,loss of protein,rejection of low salt diet. • Fatigue related to discomfort as evidenced by lethargy, extreme edema. • Risk for infection related to immunosuppressive drugs
  • 22. PROGNOSIS • Early diagnosis and appropriate treatment 80% children recover • 10-15% becomes complicated with chronic renal failure • 2-4% cases may have fatal outcome