tatsnotes.: mindmap - nephrotic Syndrome

mindmap of nephrotic syndrome differentials (Glomerulonephritis)

general characteristics
- clinical complex that includes...
  - massive proteinuria >3.5g /day in adults
    hypoalbuminemia with plasma albumin <3gm/dL
    generalized edema (anasarca)
    hyperlipidaemia and lipiduria
    at early stages, LESS of...
    - Hypertension
    - hematuria
    - azotemia
- - causes
  - children
    - primary disease (95%)
      - Minimmal-change disease (65%)
        IgA nephropathy and others (10%)
        Membranoproliferative GN (10%)
        Focal segmental Glomerurosclerosis (10%)
        membranous GN (5%)
    - systemic (5%)
  - adults
    - primary disease (60%)
      - Focal segmental glomerulosclerosis 35%
        membranous GN 30%
        IgA nephropathy and others 15%
        minimal change d 10%
        membranoproliferative 10%
      systemic (40%)
      - DM
      - Amyloidosis
      - SLE
      - ingestion of drugs
        gold
        
        penicillamine
        
        heroin
      - infections
        malaria
        
        syphilis
        
        hep B
        
        HIV
      - malignancy
- pathogenesis
  - derangement in capillary walls of glomeruli
  - permeability to plasma proteins
  - protein allowed to escape from plasma into glomerular filtrate
  - serum albumin decreased, resulting in hypoalbuminemia
    - increasesed synthesis of lipoproteins in liver
  - resultant edema due to lack of plasma colloid osmotic pressure
  - concomitant drop in plasma volume
    - reduced GFR
  - compensatory secretion of aldesterone
    - along with reduced GFR
      reduction of secretion of NUP
  - promoted retention of salt and water by kidney
    - aggrevate edema
minimal change D
- relatively benign
- most frequent cause of nephrotic $ in children
- morphology
  - as name suggests, minimal change
  - usually no change observed under microscope
  - effacement of podocyte foot process in electron microscope
- pathogenesis
  - not really sure
  - possibly due to T-cell derived factor
- clinical course
  - insiduous onsed of nephrotic $
    no hypertension
    renal function preserved in most cases
    prognosis in children good
    90% respond to corticosteroid therapy
    proteinuria recurs in more then 2/3 of initial respponders
    less than 5% develop CRF after 25 years
    replapses more common in adults,
    responce to steroid slower too
membranous GN
- epidemiology
  - most common in 30-50
- pathogenesis
  - antibodies reacting in situ to endogenous / planted antigens
    direct action of C5b-C9, membrane attack complex of complement
    activation of glomerular mesengial cells and podocytes
    - proteases
    - oxidants
    - damage capillary
      walls
- causes
  - idiopathic (85%)
  - secondary
    - infections
      - chronic hep B
      - Syphilis
      - Schistosomiasis
      - malaria
    - malignant tumours
      - lung cancer
      - colon
      - melanoma
    - SLE / other autoimmune conditions
    - Exposure to inorganic salts
      - Gold
      - mercury
    - drugs
      - penicillamine
      - captopril
      - NSAIDs
- morphology
  - diffuse thickening in the GBM
  - subepithelial deposits
    - spike and dome pattern
  - effacement of foot processes
  - granular deposits of immunoglobulins
- clinical course
  - insidious development of nephrotic syndrome
  - lesser degree of proteinuria
  - proteinuria nonselective
  - does not usually respond to steroids
  - prognosis
    - proteinuria persisit in over 60%
      only about 40% suffer progressive disesase with renal failure after 2-20years
      additional 10-30 more benign course with partial / complete remissoin
membranoproliferative GN
- general characteristics
  - 5-10% of cases of idiopathic nephrotic $ in children & adults.
  - some present only with hematuria / proteinuria in non-nephrotic stage
  - others have combined nephrotic/nephritic picture.
  - 2 major types - MPGN1 and MPGN2.
- pathogenesis
  - MPGN1
    - circulating immune complexes akin to chronic serum sickness
    - inciting antigen not known
    - occurs in association with...
      - hepatitis B
        hepetitis C
        SLE
        infected AV shunts
        extrarenal infections with
        persistant / episodic antigenemia
  - MPGN2
    - also known as dense-deposit disaese
    - less clear
    - appears to be due to excess complement activation
- morphology
  - lobular appearance
  - proliferation of mesangial and endothelial cell
  - basement membrane thickening
  - tram track appearance of glomerullar capillary
- clinical course
  - principal mode of presentation (-50%) is nephrotic $
    may begin as acute nephritis / mild proteinuria
    prognosis poor
    - 40% progress to ESRD
      30% variable degrees
      of renal insufficiency
      30% persistant nephrotic $
      without renal failure
    may occur in association
    with other systemic diseases
    - SLE
    - hep B
    - hepC
    - chronic liver disease
    - chronic bacterial infections
    - cryoglobunemia
focal and segmental
- morphology
  - sclerosis affecting some but not all glomeruli
  - only involving only segments of each affected glomerulus
  - this histological picture can occur associated with...
    - association to other known conditions
      - HIV infection
      - heroin abuse
      secondary event in other forms of GN
      - igA nephropathy
      maladaptation after nephron loss
      in inherited / congenital forms
      resulting fromm mutations
      affecting cytoskeletal / related
      protins expressed in podocytes
      - nephrin
      primary disease
- clinical course
  - unlike MCD, higher incidence of hematuria and hypertension
  - proteinusria nonselective
  - corticosteroid thrapy responce poor
  - 50% develop End-stage renal failure within 10years of diagnosis
  - adults prognosis worse
- pathogenesis
  - unknown

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Sunday 22 May 2011

mindmap - nephrotic Syndrome

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