minimal change disease
DESCRIPTION
minimal change disease presentation, diagnosis and update management.TRANSCRIPT
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Minimal change GN
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Nothing in light microscopy.
Diffuse effacement of the epithelia cell foot processes
No immune deposition
2 – 8 ys & 10% in adults
NSAID & malignancies
Presentation :NS.
Renal biopsy for diagnosis
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MCD1. 1- 2 cells per capillary tuft
2. Capillary lumen is open
3. Normal thickness of capillary wall
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MCD
Silver stain
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Electron microscopy
Ultrastructurally, the only glomerular abnormality apparent is effacement of the podocyte foot processes
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SSNS
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Relapsing SSNS
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SRNS
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MCD relapses after Cyclosporine cessation
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Treatment Characterized by remission & relapse
Good response to steroids
Spontaneous remission in 5%
Respond to treatment:Primary Responder non RelapserPR infrequent RLPR frequent RLSecondary non RespondersP non-responders late RespondersNon –respondersSteroids dependent
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Steroids
Corner stone in treatment
50% of children respond within the first 2/52
Almost all within 8/52
Adults 10 – 25 % & renal biopsy is mandatory for diagnosis
25% of adults need 12 – 16 weeks to completely remit.
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Objectives
Speed induction of remission.
Avoid serious complications.
Prevent or minimize relapses
Avoid or minimize side effects of drugs
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Symptomatic treatment
Oedema : Salt & water retention . Loop diuretics Salt free Alb
Thrombosis : Mobilization, aspirin & dipyridamole
Infections
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If the proteinuria persisted beyond the first month the steroid may be boosted or the patient given a daily inj of methylpredisolone for three days.
30 % are cured by this treatment.
Infrequent & frequent relapses describe 10 – 20 % & 40 – 50 % respectively.
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Cyclophosphamide :
started after steroid induced remission in a dose of 2 mg/kg/day for a total duration of 12 weeks
Longer remission if used for 12 instead of 8/52
The response has -ve correlation with HLA-DR7.
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Blood count ( 3000 mm3)
The total dose is away below the gonadotoxic level of 300 mg/kg.
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Chlorambucil : 0.2 mg/kg/day for a period of 2/12.
Levamisol :
Immune modulator that gives a longer remission period.
Longer use more than 6/12 is assosiated with GI manifestations, leuocopenia , psoriasis- like cutaneous lesion & leukaemia.
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cyclosporine
Can induce & maintain remission but many patients relapse upon cessation of the drug.
Relapsers respond poorly to another course of the drug
Toxicity follow up & renal biopsy 18/12 from drug initiation is a better guide.
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Steroid resistant cases
Due to genetic factors ( q1) or due to down regulation of glucocorticiods receptors.
Cyclosporine + prednisolone if normal GFR
Cyclophosphamide or chlorambucil in those with low GFR or non responders to the first regimen.