Term
| Components of Glomerular BM |
|
Definition
Collagen type IV (all layers)
Laminin (most in laminin rarae)
polyanioc proteglycans (in laminin rarae)
Entactin
Fibronectin (laminin rarae)
other glycoproteins |
|
|
Term
|
Definition
acute hematuria
mild-moderate proteinuria
hypertension
|
|
|
Term
|
Definition
heavy proteinuria (>3.5gm/day)
hypoalbuminuria
severe edema
hyperlipidemia
lipiduria
|
|
|
Term
|
Definition
Oliguria or anuria
azotemia (increase BUN and creatininine)
|
|
|
Term
|
Definition
|
|
Term
| Symptoms of renal tubular defects |
|
Definition
polyuria
nocturia
often caused by metabolic disorders |
|
|
Term
|
Definition
1) Diminished Renal Reserve (asympomatic, GFR = 50% of NL)
2) Renal Insufficiency (GF=20-50% of NL, azotemia, anemia, HTN, polyuria, nocturia)
3) Renal Failure (GFR<20% of NL, edema, metabolic acidosis, hypocalcemia)
4) ESRD (GFR <5% of NL)
|
|
|
Term
|
Definition
1. Fluids
-dehydration
-edema
-hyperkalemia
-acidosis
2. Calcium
-inc. phosphate (due to dec. GFR)
-dec. calcium (due to inc. phosphate and dec. 1/25-Vit D)
-inc. PTH (due to dec. Ca++)
-osteodystrophy (like osteomalacia or osteiitis fibrosa cystica)
3. Hematologic
-anemia (dec. EPO and BM depression)
-bleeding diathesis (dec. plt aggregation and ec. plt factor 3 release)
4. CV
-CHF (salt retention)
-HTN (salt retention and renin release)
-pulmonary edema (CHF)
-pericarditis (or pleuritis)
-uremic pneumonitis
5. GI
-N/V
-bleeding
-inflammation
6. Neuromuscular
-myopathy
-peripheral neruopathy
-encephalopathy (sz, stupor, coma)
8. Derm
-sallow color
-pruritis
-dermatitis
|
|
|
Term
| Congential Renal disorders |
|
Definition
most are developmental not genetic
20% of CRF in kids
|
|
|
Term
|
Definition
|
|
Term
|
Definition
usually unilateral
reduced number of renal lobes and pyramids
no scars
|
|
|
Term
|
Definition
usually lower than NL kidney
|
|
|
Term
|
Definition
usually lower poles
anterior to great vessels
1:1000
|
|
|
Term
| Cystic Renal dysplasia (associations) |
|
Definition
sporadic developmental
abnl metanephric differentiation
ureteral atresia
uretal obstruction may cause
|
|
|
Term
|
Definition
GROSS
enlarger irregular mulcticystic kidney
MICRO
-cysts lined w/ flattened epith
-islenads of mesnchyme
-FOCI of CARTILAGE
-immature CTs
-some NL nephrons
|
|
|
Term
| Adult Polycystic Kindey Dz (Associations) |
|
Definition
AD on chrom 16
10% of chronic dialysis
always bilateral
1. 40% have polycystic liver
2. spleen
3. pancreas
4. lung
5. berry aneurysms
6. mitral valve prolapse
7. BM defect
|
|
|
Term
| Adult Polycystic Kindey Dz |
|
Definition
GROSS
-enlarged
-surface = 3-4cm cysts
-fluid -serous or blood
MICRO
-cysts lined with variety of epithelia
-NL parenchyma in between
|
|
|
Term
| Childhood Polycystic Kidney Dz (Associations) |
|
Definition
AR
m.c. perinatal or neonatal -> rapid renal failure
1. liver cysts
2. bile duct proliferation
3. congenital hepatic fibrosis (in juvenile and infantile forms)
|
|
|
Term
| Childhood Polycystic Kidney Dz (morphology) |
|
Definition
GROSS
-enlarged
-smooth surface
-numerous cysts in cortex & medulla (sponge-like)
MICRO
-cysts lined w/ cuboidal epithelia
-dilateion of collecting tubule origin
|
|
|
Term
| Medullary Sponge Kidney (Associations) |
|
Definition
-usually benign
1. infection
2. stones
3. calcification
4. hematuria
|
|
|
Term
| Medullary Sponge Kidney (morphology) |
|
Definition
GROSS
-dilated papillary ducts
MICRO
-cuboidal or transitional epithelia
|
|
|
Term
| Nephronopthisis-uremic medullay cystic dz (UMCD) - (sub-types) |
|
Definition
1. sporadic 20%
2. Familiarl juvenile nephronopthisis - 50% AR
3. Renal-retinal dysplasia 15% AR
4. adult onset meduallary cystic dz 15% AD |
|
|
Term
| Nephronopthisis-uremic medullay cystic dz (UMCD) - (morphology) |
|
Definition
GROSS
-small kidneys
-contracted granular surface
-medullary cysts
-small cortical cysts
MICRO
-flattened to cuboidal epithelia
-cysts surrounded by inflammation or fibrosis
-cortical tubular atrophy
-thickened TBMs
-interstitial fibrosis
-preserved glomerulus
20% of chilhood CRF
|
|
|
Term
| Familiarl Juvenile Nephronophthisis (associations) |
|
Definition
1. salt wasting
2. polyuria
3. growth retardation
4. anemia
|
|
|
Term
| adult-onset medullary cystic dz (associations) |
|
Definition
1. polyuria
2. salt wasting
|
|
|
Term
| Dialysis Associated cystic dz (associations) |
|
Definition
-renal parenchymal tumors
-hematuria
-possibly obstruction
|
|
|
Term
| Dialysis Associated cystic dz (morphology) |
|
Definition
GROSS
-numerous corticl & medullary cysts (.5-2 cm)
- clear fluid
MICRO
-hyperplastic or flattened tubular epithelia
-calcium oxalate crystals
|
|
|
Term
| Simple Cysts (associations) |
|
Definition
-often asymptomatic
-hematuria
-pain due to hemorrhage
-calcification
|
|
|
Term
| Simple Cysts (morphology) |
|
Definition
GROSS
-1-5cm translucent cyst
-glitening surface
-clear fluid
-avascular
-clear fluid
MICRO
-cuboidal to flattened epithelia
|
|
|
Term
|
Definition
chronic glomerulonephritis (causes)
|
|
|
Term
| Glomerular reactions to injury |
|
Definition
1. hyperplasia
2. leukocyte infiltrate
3. GBM thickening
4. hyalinization & sclerosis
|
|
|
Term
|
Definition
PAS
-GMB
-TBM
-mesangial matrix
Silver
-GBM
-TBM |
|
|
Term
| Pathogenesis of Glomerular Injury |
|
Definition
1. in situ IC deposition
2. circulating ICs
3. cell mediated immunity
|
|
|
Term
| Post-Infectious Glomerulonephritis (associations) |
|
Definition
1. 1-2 weeks after strep
2. 6-10 year olds
3. acute nephritis
4. rbc casts in urine
5. better prognosis in kids
6. Group A B-hemolytic strep types 12, 4, 1
7. ASO elevated
8. IC mediated
|
|
|
Term
| Post-Infectious Glomerulonephritis (morphology) |
|
Definition
LM
-diffuse, global, proliferative GN
-exudation
-fibrin w/i capillaries and mesangium
IF
-granular IgG and C3
EM
*Subepithelial dome-shaped humps which do not indent GBM
-subendothelial & mesangial deposits
|
|
|
Term
| Minimal Change Disease (associations) |
|
Definition
AKA lipoid nephrosis
M.C. cause of nephrotic syndrome in children
1. 2-6 yr olds
2. post URI or vaccination
3. -atopy
4. HLA-B8 and DR-7
5. Hodgkin's Dz
6. Treatable w/ steroids
|
|
|
Term
| Minimal Change Disease (morphology) |
|
Definition
LM
-NL or inc. mesangium
IF
-negative or non-specific
EM
- effacement of foot processes
|
|
|
Term
| Focal segmental glomerulosclerosis (associations |
|
Definition
1. nephrotic syndrome
2. Idiopathic
3. Secondary ( AIDS, heroin, obesity)
4. <35 yrs old
5. poor prognosis (even w/ transplant)
|
|
|
Term
| Focal segmental glomerulosclerosis (morphology) |
|
Definition
LM
-focal segmental sclerosis
-enlarged glomeruli
-mesagnial proliferiation & hyalinosis
-initially in juxtamedullary glomeruli
IF
-IgM & C3 in sclerotic portions
EM
-NO dense deposits
-effacement of foot processes
-focal denudation of epithelial cells
-amorphous material and lipid granules in sclerotic areas (hyalinosis)
|
|
|
Term
| Membranous Glomerulonephritis (associations) |
|
Definition
M.C. cause of nephrotic syndrome in adults
2. avg age = 38
3. idiopathic
4. secondary
- cancer (lung, colon, melanoma)
-SLE
-gold or mercury toxicity
-Drugs (captopril, penicillamine)
-Chronic HepB
-syphilis
-malaria
-schistosomiasis
-Diabetes
-thyroiditis
-HLA-DR3 and DR2
5. Ic mediated
6. remitting and relapsing
7. worse in males
8. treat w/ steroids
|
|
|
Term
| Membranous Glomerulonephritis (associations) |
|
Definition
LM
-capillary wall thickening (uniform, diffuse)
-NL cellularity
-silver stain spikes
-trichrome beaded appearance
IF
-diffuse uniform granular capillary wall deposits w/ C3 and IgG
EM
-subepithelial dense deposits
-Staging
I - scattered deposits
II - difuse subetpithelial deposits
III - deposits engulfed by GBM
IV - resolution
|
|
|
Term
| Membranoproliferative GN (Associations) |
|
Definition
1. avg age = 13
2. nephrotic syndrome
3. transplant non-curative
4. 50% progress to CRF in 10 yrs
5. idiopathic
6. Secondary
-SLE
-HepB
-shistosomiasis
-AAT deficiency
-liver dz
-Cancer
|
|
|
Term
|
Definition
IC mediated, more common than Type II
LM
-large, hypercellular glomeruli
-thickened capillary walls (DOUBLE-CONTOUR, TRAM-TRACK)
-mesangial proliferation
-WBC infiltrate
IF
-Granular C3 and C1q in peripheral capillaries and mesangium
EM
-circumferential mesangial interposition
-MASSIVE subendothelial elctron-dense deposits
|
|
|
Term
|
Definition
Alternate C pathway, C3 nephritic factor
LM
-large, hypercellular glomeruli
-thickened capillary walls (DOUBLE-CONTOUR, TRAM-TRACK)
-mesangial proliferation
-WBC infiltrate
IF
-capillary wall, mesangial, and TBM irregular granular C3, no IgG
EM
-slug-shaped dense material in GBM lamina densa
-+/-mesangial deposits
-deposits are not ICs |
|
|
Term
| IgA Nephropathy (Associations) |
|
Definition
(Berger's Dz)
1. recurrent hematuria
2. mild proteinuria
3. young males
4. URTI symptoms
5. HLA-B35 &D4
6. May recur after transplant
7. renal manifestation of HSP
|
|
|
Term
| IgA nephropathy (morphology) |
|
Definition
LM
-variable
-most likely focal segmental proliferative GN
-may be mesangial proliferative or focal segmental glomerulosclerosis
IF
-mesangial IgA
EM
-mesangial & paramesangial dense deposits
-subendothelial deposits (poorer prognosis)
|
|
|
Term
| Rapidly progressive GN (associations) |
|
Definition
(Necrotizing crescentic GN)
1. post-infectious
2. systemic illness
3. idiopathic
4. pulmonary dz
leukocyoclastic angiitis, PAN, Wegener's, Churg-Srauss, Goodpastures, HSP, SLE, cryoglobulinemia
|
|
|
Term
|
Definition
LM
-proliferating parietal epithelial cells displace glomeruli
-foci of necrosis
EM
-variable
IF
see notes
|
|
|
Term
| Focal Proliferative GN (Associations) |
|
Definition
1. SLE
2. PAN
3. HSP
4. Goodpasture's
5. SBE
6. Wegener's
7. IgA |
|
|
Term
|
Definition
GROSS
-contracted granular kidney
-thinned cortices
-increased peripelvic fat
LM
-hyaline obliteration of glomeuli
-acellular eosinophilic masses
-tubular atrophy
-interstitialfibrosis
-arteriolar sclerosis
|
|
|
Term
|
Definition
1. females
2. blacks
3. HLA-DR2 & DR3
4. LE bodies
5. ANA
6. Anti-dsDNA
7. anti-SM
|
|
|
Term
|
Definition
|
|
Term
|
Definition
mesangial lupus GN
1. mildest
2. increased mesangial matrix
3. fullhouse of antibodies
4. small mesangial & paramesangial deposits on EM
|
|
|
Term
|
Definition
Focal proliferative Lupus GN
1. 1-2 tufts of proliferativing cells in glomeruli
2. fibrinoid necrosis
3. thrombi
4. fullhouse antibodies on IF
5. mesangial & subepithelial deposits on EM
6. RETICULOTUBULE INCLUSIONS on EM
|
|
|
Term
|
Definition
Diffuse Proliferative Lupus GN
1. most serious
2. 50% of lupus patients
3. Endo, epithelia, and mesangium all proliferate
4. fullhouse antibodies
5. diffuse mesangial, subendulthial deposits on EM
6. reticulotubule inclusions
7. thickened GBM
|
|
|
Term
|
Definition
Membranous Lupus GN
1. nephrotic syndrome
2. thickened capillary walls on LM
3. fullhouse antibodies
4. EM same as type IV
5. wire-loop lesions
|
|
|
Term
|
Definition
1. skin purpura on extremities
2. abdominal pain
3. vomiting
4. GI bleeding
5. arthralgia
6. hematuria
7. proteinuria, nephrotic syndrome
8. 3-8 years old
9. atopy
10. no EM deposits
|
|
|
Term
|
Definition
1. chronic BE
2. IC deposit
3. proteinuria
4. hematuria
5. nephrotic syndrome
6. variable morphology
|
|
|
Term
|
Definition
| membranous proliferative GN |
|
|
Term
|
Definition
| membranous proliferative GN |
|
|
Term
|
Definition
| Dialysis-Associated Kidney |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Reticulutubule Inclusions |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Uremic Medullary Cystic Dz |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Focal segmental glomerulosclerosis |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
1. nephrotic syndrome or non-nephrotic proteinuria or CRF
2. pyelonephritis
3. papillary necrosis
4. arteriolosclerosis
5. inc. collegan IV
6. inc. fibronectin
7. dec. heparin sulfate
8. DM type I more severe
|
|
|
Term
|
Definition
1. inc. capillary walls
2. diffuse glomerulosclerosis
3. nodular glomerulosclerosis(see below)
-a.k.a. intercapillary GS,
-a.k.a. Kimmelstiel-Wilson Disease
-hyaline masses in periphery of glomeruli
|
|
|
Term
|
Definition
1. Fibrin caps (hyaline inviscreral epithelium)
2. capsular drop (hyaline in parietal epithelium)
3. plasma protein exudate in arterioles
4. efferent arteriolosclerosis (only in DM)
5. tubuluar atrophy
6. interstitial fibrosis
7. Armanni-Epstein change: clear cytoplams in renal tubular cells
8. pyelonephritis
|
|
|
Term
|
Definition
LM
-increase mesangium
-congo red
IF
-thioflavin T positive
EM
-7-10nm fibrillar material(thicker fibers = "non-amyloid fibrillary GN")
-replaces GBM and mesangium
|
|
|
Term
| HIV-Associated Nephropathy |
|
Definition
LM
-collapsing glomerulopathy
-epithelial hyperplasia
-dilated tuules w/ intraluminal hyaline
-tubublointerstitial lymphoplasmocytic infiltrate
EM
-reticulotubule inclusions
-granular change in tubular epithelial nuclei
|
|
|
Term
|
Definition
1. hereditary nephritis
2. nerve deafness
3. eye disorders
4. males
5. hematuria
6. proteinuria
7. onset at 5-20 years
8. CRF at 20-50 yrs
|
|
|
Term
| Alport's Syndrome (morphology) |
|
Definition
LM
-glomerular segmental proliferation
-inc. mesangium
-epithelial foam cells
IF
-C3
EM
-attenuation and thickening of GBM
-SPLITTING of GBM lamina densa
-also of TBM
|
|
|
Term
| Benign Recurrent Hematuria (Associations) |
|
Definition
1. thing BM dz
2. no ear or eye stuff
3. Males
4. <40 yrs
5. avg. age = 10
6. AD
7. previous URTI
|
|
|
Term
| Benign Recurrent Hematuria (morphology) |
|
Definition
LM
-NL or slight inc. mesangium
IF
-no IgA, may have C3, IgG, or IgM
EM
-focal widening/splitting of GBM
-Multifocal THINNING of GBM
|
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Diabetic Arteriolosclerosis |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Non-amyloid Fibrillary GN |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
Fibromuscular Dysplasia
-elastic stain
-no choclesterol clefts, no necrotic debris
-concentric |
|
|
Term
|
Definition
| Thrombotic Microangiopathy |
|
|
Term
|
Definition
| Diffuse Cortical Necrosis |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
| Renal Cell Carcinoma (Clear Cell) |
|
|
Term
|
Definition
| Renal Cell Carcinoma (Papillary) |
|
|
Term
|
Definition
| Renal Cell Carcinoma (Chromophobe) |
|
|
Term
|
Definition
| Renal Cell Carcinoma (sarcomatoid) |
|
|
Term
|
Definition
| Transitional Cell Carcinoma |
|
|
Term
|
Definition
|
|
Term
|
Definition
| Transitional Cell Carcinoma |
|
|
Term
|
Definition
| Transitional Cell Carcinoma |
|
|
Term
|
Definition
| Transitional Cell Carcinom |
|
|
Term
|
Definition
| Transitional Cell Carcinoma |
|
|
Term
|
Definition
| Transitional Cell Carcinoma in Situ |
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| Renal Disorders that recur after transplant (7) |
|
Definition
1) FSGS
2) MPGN
3) IgA nephropathy
4) anti-GBM dz
5) SLE
6) DM
7) HUS
|
|
|
