Minimal Change Disease

Hoo Jun TingХоо Джун ТингGroup 88

Also Known As• Nil Lesions• Nil Disease• Lipoid nephrosis

Introduction• the most common cause of nephrotic syndrome in

children 4 to 8 yr • comprises 80 to 90% of childhood nephrotic syndrome• renal function is typically normal• prognosis is excellent

Nephrotic Syndrome vs Nephritic Syndrome

Nephrotic Syndrome Nephritic SyndromeHypoalbuminemia Hematuria

Edema Edema

Hyperlipidemia Secondary Hypertension (very often)

Proteinuria ( > 3.5g/day) Proteinuria (<3.5g/day)

Normal diuresis Oligouria

Waxy and fatty casts Conglomerate and protein casts

Etiology• Unknown• In rare cases may occur secondary to

1. drug use (especially NSAIDs)2. hematologic cancers (especially Hodgkin lymphoma)

Symptoms1. Edema• In leg, abdomen but can occur anywhere• Complaint of shoes and clothes can no longer fit

2. Proteinuria• Complaint that urine becomes more frothy or foamy

What to do when you have children with

edema ?

Children with edema

Check urinary protein

No severe proteinuria

No hematuria

CHFKwashiokor

Liver cirrhosisProtein Losing

Enteropathy

With Hematuria

Acute GN

Proteinuria >3.5 g/day

Check serum creatinine, albumin and lipids

Normal albumin

Normal lipdsIncrease

creatinine

Acute GNRPGN

Hypoalbuminemia

Hyperlipidemia

Normal or slightly increase

creatinineNephrotic

syndrome

Several disorders with NS1. Minimal change disease

2. Membranous glomerulonephritis• SLE• Cancer (of lung, rectum, intestine)• Intoxication by inorganic salts ( for eg. Gold salt)• HBV infection• Syphilis• Drug induced – captopril• Metabolic disease – DM, thyroiditis

3. Focal Segmental Glomerulosclerosis• Idiopathic• SLE

Renal Biopsy• To confirm diagnosis• In children – only in atypical cases (when the patient

fails to improve after a trial of corticosteroids treatment) • In adult - In contrast, a renal biopsy is performed in all

adult patients with nephrotic syndrome, before the initiation of treatment for MCD.

Light Microscopy Electron Microscopy

Treatment• Corticosteroids• Initial dose for induction of remission :15-35 mg once daily for

one month• Maintainance dose : 30 mg once on alternate day for one

month then tapering for 1 – 2 years until discontinue

• If not responsive to corticosteroid do renal biopsy

If really is Minimal Change Disease Use alternative treatments

Indication for Alternative Treatments• Use only in corticosteroid nonresponders• In patients with frequent relapse (corticosteroid

dependent) • Patients may never get out of vicious cycle without

alternative treatment

Alternative Treaments1. Cyclophosphamide (Procytox)• 2 to 3 mg/kg once/day for 12 wk

2. Chlorambucil (Leukeran)• 0.15 mg/kg once/day for 8 wk

3. Cyclosporine (Neoral, Gengraf, SandIMMUNE)• 3 mg/kg po bid

Key Points• MCD accounts for most cases of nephrotic syndrome in children

and is usually idiopathic.

• Suspect MCD in children who have sudden onset of nephrotic range proteinuria with normal renal function and a non-nephritic urine sediment.

• Confirm the diagnosis by renal biopsy only in adults and atypical childhood cases.

• Treatment with corticosteroids is usually sufficient.

Thank you