Minimal Change Disease

Minimal Change Disease

• 1.
  Minimal Change Disease HooJun Ting Хоо Джун Тинг Group 88
• 2.
  Also Known As •Nil Lesions • Nil Disease • Lipoid nephrosis
• 3.
  Introduction • the mostcommon 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
• 4.
  Nephrotic Syndrome vsNephritic Syndrome Nephrotic Syndrome Nephritic Syndrome Hypoalbuminemia 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
• 5.
  Etiology • Unknown • Inrare cases may occur secondary to 1. drug use (especially NSAIDs) 2. hematologic cancers (especially Hodgkin lymphoma)
• 6.
  Symptoms 1. Edema • Inleg, abdomen but can occur anywhere • Complaint of shoes and clothes can no longer fit 2. Proteinuria • Complaint that urine becomes more frothy or foamy
• 7.
  What to dowhen you have children with edema ?
• 8.
  Children with edema Check urinary protein Nosevere proteinuria No hematuria CHF Kwashiokor Liver cirrhosis Protein Losing Enteropathy With Hematuria Acute GN Proteinuria >3.5 g/day Check serum creatinine, albumin and lipids Normal albumin Normal lipds Increase creatinine Acute GN RPGN Hypoalbuminemia Hyperlipidemia Normal or slightly increase creatinine Nephrotic syndrome
• 9.
  Several disorders withNS 1. 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
• 10.
  Renal Biopsy • Toconfirm 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.
• 11.
  Light Microscopy ElectronMicroscopy
• 13.
  Treatment • Corticosteroids • Initialdose 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
• 14.
  Indication for AlternativeTreatments • Use only in corticosteroid nonresponders • In patients with frequent relapse (corticosteroid dependent) • Patients may never get out of vicious cycle without alternative treatment
• 15.
  Alternative Treaments 1. 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
• 16.
  Key Points • MCDaccounts 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.
• 17.
  Thank you