Download - Nephrotic syndrome
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NS is a glomerular disorder characterised by
a tetrad of:Proteinuria more than1g/m2/24hr
Hypoprotinuria ( albumin less than2.5gm/dl)
Hypercholestrolemiamore than220mg/dl
Edema
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CLASSIFICATIONo Idiopathic nephrotic syndrome (90% of cases)
Minimal change nephrotic syndrome Nephrotic syndrome with mesangial proliferation Nephrotic syndrome with focal sclerosis
o Nephrotic syndrome secondary to glomerulonephritis (10% of cases) Membranous glomerulopathy MPGN OTHRS as SLE and HSP
o Congenital nephrotic syndrome AR presenting at birth or during the 1st 6 months
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• The nephritic syndrome is a clinical condition characterized by:
Heavy proteinuria: Hypoalbuminemia : Oedema: Hypercholestrolemia ABSENCE OF nephritic manifestations as hematuria, h
ypertension, renal failure and hypocomplementemia . Good response to steroids.
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• Commenest type of nephrotic syndrome
• Age : peak incidence 2-6 years ( can occur in 1st year or in adults).
• Sex: more in boys( male to female ratio is 2: 1)
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Etiology and Pathogensis
GFR
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Glomerular basement membrane (GBM)
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Pathogenesis of nephrotic diseases
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• LIGHT MICROSCOPY : glomeruli appear normal.
• IMMUNOFLURESCENCENT MICROSCOPY : negative n( no deposition of immune complexes.
• ELECTRON MICROSCOPY : retraction of fppt processes of podocytes
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The initial attack and subsequent relapses may follow a viral upper respiratory infection.
Edema.
Weight gain.
Diminshed urinary output.
Respiratory difficulty.
Diarrhea.
Normal blood pressure.
Manifestations of complications.
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Oedema• Around the eyes in the morning, and around the
ankles in the evening
• There is permanent swelling of ankles and face
• Severe: With increasing edema, ascites and genital edema may appear, followed by pleural effusions
• The edema remains soft and pit:
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• Increase suscebtability to infection.
• Hypercoagulation and thrombosis arterial and venous thrombosis )
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• Why are nephrotic patients more susceptible to infection?
Decrease immunity Edema fluid is a good culture medium. Immunosupressive therapy.
• Most common organism: Strept. Pneumoniae. Gram negative organisms.
• Most common sites of infection Peritonitis (commonest ) Sepsis, pneumonia, cellulitis, urinary tract infection.
• Manifestations of infection during steroid therapy: minimal signs of infection ( mild fever may be the only sign )
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Ascites+fever =suspect peritonitis & do culture of ascitic fluid
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• Due to increase prothrompotic factors and decrease fibinolytic factors, most common site is renal vein thrombosis
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Lab findings
• Urine analysis
• Serum and blood
• Renal functions
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Protinuria Exceeds 40mg/m2/hr
1gm/m2/24hrs3or4+ by dip stick
Spot urine protein to creatinine exceeds 2 or 3
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Other causes of edema and hypoprotinaemia
Kwashiorkor and marasmic kwash.
Acute nephritic syndrome may present with marked edema and proteinuria
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• The two principle lines of treatment are
Effort to reduce edema.Specific therapy with prednisone
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• Hospitalisation, investigations and exclusion of contraindications to steroids
• Physical activity• Diet and fluids.• Diuretics are used cautiously
(over dose of duritics hypovolemia Hypotension Iatrogenic shock)
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• Prednisone : 60mg/m2/day given in 3 divided daily doses, for 4 weeks then start alternate day therapy.
• Alternate day therapy : prednisone 40mg/m2/day taken as single morning dose with break fast . The alternate day therapy is then tappered slowly and discontiuned over the next 3 months.
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• Relapse :• Proteinuria more than 3+ and edema
• Daily steroids is given until proteinuria is negative or only trace by dip stick for 3 consecutive days ,then the patient is shifted to alternate day therapy and treatment is tapered over 2 months
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Classification according to response to steroids
• Steroid responsive :Children who respond to treatment within 8
weeks of treatment.
Children with no relapses Children with relapses
Infrequent relapserFrequent relapserSteroid dependent
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Infrequent relapser : relapse less than 4 times in a 12 months period.
Frequent relapser: relapse more than 4 times within 12 months period
Steroid dependent: relapse while on alternate day therapy or eithin 14-28
days of stopping treatment
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Classification according to response to steroids
• Steroid resistant:
Patients who fail to respond to treatment within 8 weeks of treatment.
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Cytotoxic drugs( as cyclophosphamide)
• Indications :
• Corticosteroid toxicity in frequent relapsers and steroid dependent,
• Steroid resisrent nephrotic syndrome (after renal biopsy )
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Vaccines
• Pneumococcal and varicilla vaccines may be given once the child is in remission.
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