MINIMAL CHANGE DISEASE

MINIMAL CHANGE DISEASE
  • Synonym – Lipoid nephrosis, Foot process disease, Nil deposit disease.
  • It is a benign disorder characterised by diffuse effacement of foot processes of visceral epithelial cells (podocytes) detectable only by EM in the glomeruli that appear virtually normal by light microscope
  • Most frequent cause of nephrotic syndrome in children but less common in adults.
  • Age peak incidence is between 2 & 6 years
  • Immune deposits are absent in glomeruli but immunological basis for disease is suggested due to
    • Clinical association with respiratory infections and prophylactic immunizations
    • Response to corticosteroids and other immunosuppressive therapy
    • Association with other atopic disorders (eczema or rhinitis)
    • Increased incidence in patients with hodgkins lymphoma in whom there is the defect in T-cell mediated immunity)
Pathogenesis
  • Involves immune dysfunction that results in elaboration of factors that damage visceral epithelial cells and cause proteinuria
  • Exact nature of the injury is probably mutation of a renal protein called Nephrin
  • Nephrin resembles an immunoglobulin like cell adhesion receptors that participate in cell-cell and cell matrix interaction. Thus this probably causes adhesion defect to visceral epithelial cells of glomerular basement membrane. This leads to detachment of epithelial cells causing proteinuria
  • Defects in charge barrier may cause proteinuria (not proved)

MORPHOLOGY
  • Light microscopy – Normal glomeruli
  • Electron microscopy –
    • GBM appears normal and no electron dense material is deposited
    • Principal lesion-visceral epithelial cells show uniform diffuse effacement of foot processes and cytoplasm with loss of recognisable intervening slit diaphragms
    • Such change can be seen in the diseases also like membranous glomerulopathy or DM
    • Such change is associated with normal glomeruli on LM than the diagnosis of minimal change diseases is made

Reference : unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/membranous-nephropathy/

  • Cells of proximal tubules are laden with lipids and protein reflecting tubular reabsorption of lipoproteins passing through diseased glomeruli-thus the historical name is lipoid nephrosis
  • Immunofluorescence studies –No immunoglobulin or complement deposits
  • Disease is completely reversible after administration of corticosteroids
Clinical features
    • Massive proteinuria but renal function remaining normal
    • NO HTN or hematuria
    • Proteinuria is highly selective most of the protein being albumin
    • Characteristic feature complete remission by corticosteroids and prognosis is excellent.
References
  • Vinay kumar, Abul K.Abbas, Nelson Fausto, Jon C. Aster. Robbins and Cotran Pathologic basis of disease. 8th edition.
  • Harsh mohan. Text book of Pathology.8th edition.2019
  • A.K.Mandal, Dr. Sharmana Choudhary. Textbook of Pathology for MBBS. Vol II. Second edition 2017.
  • unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/membranous-nephropathy/