Minimal Change Disease

Minimal change GN

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

MCD1. 1- 2 cells per capillary tuft

2. Capillary lumen is open

3. Normal thickness of capillary wall

MCD

Silver stain

Electron microscopy

Ultrastructurally, the only glomerular abnormality apparent is effacement of the podocyte foot processes 

SSNS

Relapsing SSNS

SRNS

MCD relapses after Cyclosporine cessation

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

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.

Objectives

Speed induction of remission.

Avoid serious complications.

Prevent or minimize relapses

Avoid or minimize side effects of drugs

Symptomatic treatment

Oedema : Salt & water retention . Loop diuretics Salt free Alb

Thrombosis : Mobilization, aspirin & dipyridamole

Infections

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.

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.

Blood count ( 3000 mm3)

The total dose is away below the gonadotoxic level of 300 mg/kg.

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.

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.

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.