L58 + 59 Flashcards
If you saw a hypercellular glomerulus, what would be happening in what you see?
Prolif of intracap glomerular cells
Crescents = prolif of extracap glomerular cells
What happens to get sclerosis in the kidney?
GBM collapse + mesangial expansion
Damage to segmental structure
Get scar due to collagen deposition
What happens to get hyalinosis in the kidney?
Accum protein in sclerotic areas in BV walls
What disease process thickens the GBM itself?
Diabetes (vs immune complex deposition thickening)
What is global vs segmental glomerulus damage?
Global - entire thing involved
Segmental - part of tuft involved w/ other part patent
Use the name: what is focal segmental glomerulo-sclerosis?
Focal - dotted throughout kidney
Segmental - only part glomerulus affected
Which cells are mediators of injury to the glomerulus?
Impt ones: neutrophils + mesangial cells
What are the 3 soluble mediators of injury at the glomerulus?
Complement
Fibrinogen
TGF beta
Clinical presentation nephritic syndromes
Hematuria - RBC casts Mild proteinuria Periorbital edema Azotemia Oliguria HTN + edema
Pathogenesis of post-infectious glomerular nephritis?
Strep pyogenes infection: impetigo > pharyngitis
Immune complex pathogenesis
Post-infectious GN LM, IF, EM
LM: hypercellular (nephrotic)
GN: granular
EM: sub-epi humps of Ig
What other labs will indicate post-strep GN
+ ASO
↓C3
What is rapidly progressive GN? What are the categories of disease?
Crescent Renal failure progressing in wks/mos Diseases may RESULT in RPGN 1. Immune complex mediated = granular IF 2. Anti-GBM = linear IF 3. Pauci-immune = nothing on IF
Treat post-strep GN; what is the complication if not treated?
Will resolve on own if treat 1ary infection in kids
Can progress to RPGN
What disease has anti-GBM antibodies?
Goodpature’s: T2 HST rxn
Linear IF
What diseases have granular IF and can progress to RPGN?
Immune complex disorders
- Post-strep GN
- SLE (IgG)
What disease are RPGN pauci-immune?
- Wegener’s: c-ANCA
- Microscopic polyarteritis (small vessel vasculitis) p-ANCA
- Polyarteritis nodosa
- Churg Strauss
Which diseases that can result in RPGN are reno-pulmonary diseases?
Goodpastures
Wegener’s
Microscopic polyangitis
What is ANCA?
Anti-neurtophilic cytoplasmic autoantibodies
c-ANCA = cytoplasmic staining
p-ANCA = perinuclear staining
What is the ANCA in Goodpastures?
Confounder - doesn’t matter!
Treat Goodpasture’s
Plasmapheresis + steroids + cytotox drug
Treat RPGN
Steroids + cytotoxic drugs
What are the characteristics of nephrotic syndrome?
Proteinuria > 3.5 g/d
Hypoalbumin
Hyperlipidemia
Edema
What is the most common cause of nephrotic syndrome in kids?
1ary
Minimal change disease
What is membranous glomerulopathy?
1ary nephropathy- long onset
Ig @ sub-epi
Diffuse thickening of glomerular capillary walls
Idiopathic
Membranous glomerulopathy LM, EM, IF
LM: diffuse thickening
EM: sub-epi spike and dome
What is minimal change disease? LM, IF, EM
Kids: response to steroids
Lose negative change in GBM
ONLY see on EM: effacement of podocytes
Presentation of minimal change disease
Selective proteinuria: lose albumin but not globulins
What might minimal change disease progress to?
Focal seg glomerulo-sclerosis
Clinico-pathological definition of focal-seg GS
Proteinuria (nephrotic) non-selective
FSGS
- Focal makes biopies hard
Effacement of foot processes
Usually FSGS is primary and idiopathic. What are 3 secondary causes?
Focal scarring from another GN Reflux nephropathy Disproportion body:renal mass HIV Drug induced: heroin
Treat FSGS
Not gonna - poor steroid response
Likely progress renal failure
High recurrence in transplant pts
What is IgA nephropathy?
Nephrotic - recurrent hematuria
IgA deposits in mesangium -> alternative complement
What is a systemic IgA disease that can cause IgA nephropathy, vasculitis, and skin purpura?
Henoch-Schoenlein purpura
Presentation Alport’s
Males
Nephritis - hematuria w/ RBC casts
Deafness
Pathogenesis Alport’s
T4 collagen mutation - don’t see any of this collagen on skin biopsy
X GBM synthesis
What is thin membrane disease?
Asymptom hematuria
EM: diffuse thinning of GBM
Is membrano-proliferative glomerulo-nephritis nephrotic or nephritic? Types
Mixed - kids/young adults
- Immune complex mediated - Hep C/B, SLE
- Complement - mutations or Abs vs complement regulating proteins
- W/ thrombotic microangiopathy
Does SLE cause nephritis or nephrotic?
Nephritis
IF: look for all Ig, complement components
