I decided to save some time by learning only 2 of the main ones - post-infective, IgA nephropathy.
Nephritic Syndrome
- IgA nephropathy (berger Disease)
- general features
- affects young adults and children most commonlyone of most common causes of recurrent microscopic / gross hematuriamost common glomerular disease revealed by renal biopsies worldwidesome consider it to be a localized
variant of Henoch-Schonlein purpura- skin - purpuric rash
- GI tract - abd. pain
- Joints - Arthritis
- kidneys
- pathogenesis
- IgA production and clearance may be the culprit
- IgA level increased in 50% of patients with IgA nephropathy
- IgA synthesis in responce to an external stimulus
- lead to depots in mesangium
- actiavation of alternative complement pathway
- initiate glomerular injury
- morphology
- diffuse mesangial proliferation
- mesangial deposits of IgA
- clinical course
- episode of gross hematuria
- last several days
- subside, only to recur every few months
- often assoc. with loin pain
- 30-40% have only microscopic hematurea
- 5-10% develop a typical acute nephritic $.
- within 1-2 days of a nonspecific URTI
- slow progress to CRF in 25-50% of cases in 20years time.
- episode of gross hematuria
- general features
- Hereditary nephritis
- Alport syndrome
- associated with
- deafness
- various eye disorders
- lens dislocation
- posterior cataracts
- corneal dystrophy
- associated with
- Alport syndrome
- TTP-HUS
- membranoproliferative
- Henoch-Schonlein purpura
- Poststreptococcal (Acute postinfectious) GN
- general characteristics
- typically caused by glomerular depots of immune complexesresulting in diffuse proliferation and swelling of resident glomerular cellsfrequent infiltration of leukocytes - esp. neutrophilsinciting antigen
- exogenous
- poststreptococcal GN
- pneumococcal
- staphylococcal
- mumps
- measles
- VZV
- Hep B & C
- endogenous
- SLE
- exogenous
- pathogenesis
- typical features of
immune complex depot- hypocomplementemia
- granular depots of IgG and complent on GBM
- morphology
- uniformly increased cellularity of glomerular tuftsaffects nearly all glomerulisubepithelial humpscapillary lumen filled with leukocytecytoplasmic granulesgranular depots of IgG and complementthis depots are cleared by 2 months approx
- clinical course
- classic case
- 1-4 weeks after group A strep infection
- pharynx
- skin
- onset of kidney disease abrupt with...
- malaise
- slight fever
- nausea
- nephritic $
- mild to moderate
- hypertension
- azotemia
- gross hematuria
- smoky brown - rather than bright red
- some proteinurea
- serum complement low
- anti-streptolysin O antibody elevated
- prognosis
- most children recover uneventfullysome develop rapidly progressive GN
due to severe injury with crescents or
chronic renal disaease due to secondary scarringin adults- 15-50% develop ESRD over
ensuing few years / 1-2 decades.
- 1-4 weeks after group A strep infection
- classic case
- general characteristics
- general properties
- clinical complex
- acute onsethematuria
- dysmorphic RBC
- RBC casts in urine
oliguriaazotemiahypertensionprotinurea and edema
not as severe as nephrotic $
common morphology
/ pathophysiology- proliferation of cells within glomeruli
- leukocytic infiltrate
- injury of capillary walls
- escape of RBCs
- reduced GFR
- ischemic kidney releasing rennin
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