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158 Cards in this Set

  • Front
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what do you see in Type 3 hypersensitivity?
it is deposition of a soluble antigen-antibody complex in glomeruli

Also known as circulating immune complex nephritis

IF:- granular depostion (C3, C5)
What do you see in a Type 2 hypersensitivy?
Anti- GBM antibody, i.e. an antibody is formed against and antigen in the glomerulus.
IF:- smooth, linear deposit
What do you see in a Type 4 hypersensitivity?
Lymphocte cell mediated immune nephritis
Examples of Type 3 hypersensitivity?
SLE, streptococci, HBV Treponema pallidum, Malaria
Examples of Type 2 hypersensitivity?
Good Pasture's syndrome
hematuria and jaundice
Hemolytic anemia
its actually myoglobinurea not hematurea
hematurea and edema in adults
acute non post strep GN
Hematurea and colicky pain (loin to gorin)
renal stones
hematurea and adults, past h/x of lung and GIT infection
Ig A nephropathy
Hematurea and edema in children
Post strep GN
Hematurea, painless and adult
Bladder tumor:
-TCC
-SCC
Nephrotic Syndrome
- more chronic
- increase in permeability
- increased loss of protein= proteinurea (+4)
- results in hypoalbonumemia- anascra +edema
- hypoalbonumenemia= increase in cholesterol= fat oval bodies and lipidurea
Acute Nephritic Syndrome
very similar to ARF
- Hematurea
- HTN +Azotemia
- Anuria/ Oligurea
- Onset- weeks/months
Pathogenesis of Nephrotic Syndrome
- increase in capillary permeability
- increase in protein loss= proteinurea (+4)
- results in hypoalbonumenimia:
a.) lipidurea - oval fat bodies + increase in cholesterol
b.) edema, anascara= pitting edema- transudate (interstitial fluid)
Complications of Nephrotic Syndrome
- increased chances of infections
- increase in cholesterol= ATH- MI or Angina
-Blood clotting: increased viscosity of blood leads to venous thrombosis
Recurrent Hematuria
- family H/x of CRF and hematurea
- "on and off" blood in the urine
- fresh/ smoky urine
- fresh blood on the microscope RED CASTS
Rapidly Progressive GN
it has the same symptoms as ANS only a much quicker onset. i.e a couple of days
Minimal Change Disease is mostly seen in what age group and is assoc with what
Children (2-6 years)

Nephrotic Syndrome
What is another name of Minimal Change Disease
lipoid nephrosis
What is the pathogenesis of minimal change disease
Loss of normal charge barrier in GBM
Lymphocyte production by T cells (type 4 hypersensitivity)
What is unique about minimal change disease
there is +4 proteinurea ( ONLY ALBUMIN)
What is the morphology of Minimal Change Disease?
LM: no change
IF: no change
EM: effacement of podocytes (foot processes), appearance of fusion of epithelial cell.
What is the urine analysis of Minimal Change Disease?
Lipoid Nephrosis (increase tubular reabsorption of lipoproteins)
- yellow appearance, slightly cloudy
4+proteinurea, oval fat body
What are the lab findings in Minimal Change Disease?
(N) complements
increase in cholestrol
What is the treatment for minimal change disease?
Steroids
what is the definition of diffuse in terms of GN
all the glomeruli are involved
What is the definition of focal in relation to GN
It is a some of the GN
What is the definition of segmental in relation to GN
it is a part of the GN
What is the definition of proliferative GN
it is when there is an increase in no. of cells greater than 20, and the cells are PMN's- there is loss of Bowman's space and decrease in GFR- ARF
What is the definition of membraneous GN
increased thickness of capillary walls e.g. diabetes. Due to Type 3 hypersensitivity causing it to get thicker- deposition of complements
When do you see Membraneous GN and what are the main associations
Most Common Cause of Nephrotic Syndrome in adults
What are the etiologies of Membraneous Glomerulonephritis?
-idiopathic or genetic
-Drug induced -Penicillamine
- Renal transplantation, Heymann Nephritis
- SLE, DM
- Adenocarcinoma
What is the antigen in adenocarcinoma that causes MGN?
Mucin
What is the age group you are most likely to associate with Membraneous Glomerulonephritis?
Adult
If a patient has Wilson's disease, What drug is given to them, and what the ADR's of this drug
Penicillamine
-Membraneous Glomerulonephritis
What is the morphology of Membraneous Glomerulonephritis?
-LM: diffuse thickening of the capillary walls, silver stain spikes
- IF: granular deposits of IgG and C3
-EM: Subepithelial deposits along GBM
What are the lab findings of Membraneous Glomerulonephritis?
- Proteinurea (+4) WBC 2/hpf
- Low complement values
- may loose some RBC casts
What are the clinical ft s of Membraneous Glomerulonephritis?
-Some develop hematuria, and HTN
- 40% progress to renal failure or ESRD after 2-20 years
-10-30% with partial or complete remission or proteinurea
Can you treat Membranous Glomerulonephritis with drugs?
No- has to have a transplant
What are the unique fts of Membraneous that make it very different
It's an e.g. of both nephrotic and acute nephritic syndrome, as it has +4 proteinurea as well as HTN and hematurea and uremia
What is another name for acute post-strep GN?
Proliferative GN, Post infection GN
What is the etiology for acute post strep GN
Beta hemolytic group A streptoccocal infection of throat and skin
Child btw 2-4y/o suffering of throat and skin infection. Develops hematurea. What is the kidney disease they may be suffering of?
Acute Post strep GN
What is the pathogenesis behind Acute post strep GN?
complement-mediated tissue damage with help of PMN
What type of hypersenstivity is involved in acute post strep GN?
Type 3
What are the clinical ft.s of acute post strep GN?
Acute nephritic syndrome
- proliferative (greater than 100 neutrophils)
- decrease in GFR due to decrease in BS's= ARF (oligurea, azotemia, HTN, hematurea)W
What is the diagnostic test one can do to determine if it is a strep infection?
ASO test
What is the morphology of acute post strep GN?
LM: hypercellular, proliferative(many neutrophils), RBC cast in tubules
IF: Granular deposit of Ig G and Ig M or C3 in ALL GLOMERULI!
EM: subepithelial humps
What are the lab findings of acute post strep GN?
Urine: smoky (acid hematin) and dysmorphic RBC
- decrease in serum complement
What are the unique ft s of urine in acute post strep GN?
Dysmorphic RBC- the RBC's will squeeze through the walls
Pt. presents with past hx of sore throat or impetigo(skin infection) followed by hematurea, and they have fever and nausea that occurs suddenly
Acute post Strep GN
If an adult suffers of acute post strep GN what can it progress to?
crescentic GN or chronic GN
What is the etiology of non-strep acute GN?
- HBC,HCV
- pneumococcal pneumona
- SLE, PAN
- Malaria
How do you differentiate between acute post strep and non-post acute strep GN?
ASO titer
What is unique about Crescentic GN?
Rapidly progressing GN- acute nephritic syndrome - develops ARF within days
What are the cells that are more prominent in crescentic GN?
Proliferation:
- epithelial cells
- monocytes
- fibrin
- visceral cells
What are the c/f of crescentic GN
ANS/RPGN to ARF
prognosis depends on no. of crescent of kidney= greater than 50% crescents but bad prognosis
What is the stain used to see crescentic GN
PAS +ve
What were the ft s of Type 1 Crescentic GN
- presence of anti-GBM antibody in serum- reacts with alveolar capillary also
- pulmonary alveolar hemorrhage= hemoptysis
-hematurea
what are the IF findings of type 1 Crescentic GN?
Smooth, linear deposit of Ig G or C3 on GBM (type 2 hypersensitivity)
What is another name of type 1 GN?
Good Pasture syndrome
What are the causes of type 2 GN?
SLE (arthritis, anemia, butterfly rash, photosensitivity)
What are the IF findings in type 2 GN?
granular deposition of Ig G and C3 in sub epithelial spaces (type 3 hypersensitivity)
What are the serum findings of Type 2 GN?
ANA(+), antismith antibody, dsDNA
What is another name for type 3 Crescentic GN?
Pauci-immune
What are the diseases' associated with type 3 GN?
Wegner's granulomatosis (c-ANCA) and Polyarteritis Nodosa
What kind of necrosis is seen in Type 3 GN associated with PAN
patchy fibrinoid necrosis
What are the serum findings of type 3 GN?
(N) complements- absence of immune reaction
What are the LM findings of type 3 GN?
glomerular crescent + vasculitis= hematurea, hemoptysis, ARF
What is the etiology of Ig A nephropathy?
Hx of RTI or diarrhea- release of Ig A
-children or young adult
What syndrome is associated with Ig A nephropathy?
Recurrent hematurea
Pt presents with upper respiratory tract infection, rash on body and hematurea, what is ur diagnosis
Ig A nephropathy
What is another name for IgA nephropathy?
dermatitis herpitaformis
What is the likely complication if Ig A nephropathy spreads to the vasculature?
Henoch Shonlein Purpura
What is the morphology of Berger's disease?
focal proliferation of mesangial cells
what is another name for Ig A nephropathy?
Berger's disease
Pt presents with upper respiratory tract infection, rash on body and hematurea, what is ur diagnosis
Ig A nephropathy
What is another name for IgA nephropathy that affects the skin?
dermatitis herpitiformis
What is the likely complication if Ig A nephropathy spreads to the vasculature?
Henoch Shonlein Purpura
What is the morphology of Berger's disease?
focal proliferation of mesangial cells
what is another name for Ig A nephropathy?
Berger's disease
What is another name of henoch shonlein purpura?
leukocytoclastic reaction- produces neutrophils- hypersenstivity allergic vasculitis
what are the symptoms of henoch shonlein purpura?
-skin purpuric rash
- abdominal pain
- arthritis
- kidney change
What is the etiology of membranoproliferative GN 1?
-HBV
-HCV
-HIV, SLE
-Chronic liver disease, bacterial infection
What is the morphology of membranoproliferative GN 1?
LM: hypercellular glomeruli but NO PMN'S and thick GBM, silver stain track- BM splitting of type 4 collagen
-IF:- granular deposit
-serum: low complement
what is the hypersensitivity involved in Membranoproliferative GN1?
Type 3 hypersensitivity
What is the syndrome associated with Membranoproliferative GN 2 disease?
Hematurea and Chronic Renal Failure (40% progress to ESRF)
What is the morphology of Membranoproliferative GN2
IF: dense deposit in GBM- C3 in capillary walls and mesangium
Serum: C3Nef Autoantibody
Which disease in the kidney does not allow any formation of complements in the glomerulus?
MPGN2- C3NeF autoantibody
What is the syndrome associated with Focal segmental GN?
Nephrotic Syndrome
What causes destruction of podocytes in minimal change disesase
pt has an infection (respiratory), increasing no. of T-cell lymphokines
What are the clinical presentations of Focal segmental Glomerulosclerosis?
Children or HIV+ adult
Develop non-selective proteinurea(albumin, fibrinogen, globulin)
What is the etiology of Focal segmental Glomerulosclerosis?
- HIV infection(IV drug abusers)
- Inherited congenital disease
- start as a primary disease
What is the morphology of FSGS?
Sclerosis of some glomeruli with partial involvement
What is the stain used to identigy FSGS?
Trichrome blue
Pt. presents with hematurea, HTN, poor response to steroids, and progression to CRF( 50% develop ESRF within 10 years?
FSGS
How do you differentiate FSGS from membranoproliferative GN/
Trichrome stain is positive in FSGS
What are the kidney disease affected by SLE?
- crescenteric GN
-Membranous like- Wire loop
-Hyaline arterolosclerosis
- Normarl glomerulous (Minimal change disease)
-focal segmental disease (NS)
What are the findings of SLE?
dsDNA+, Nephrotic syndrome and C1q deposit, and decrease in C3.
Lungs get pruritis
What is the d/d of SLE in Minimal change disease?
Mesangial cell GN (NS)
What is the cause of Diabetic Kidney
complex of glucose and subendothelial collagen deposit
What is the stain used to determine there is diabetic kidney?
PAS +ve
what disease is associated with diabetic kidney
-Kiemesteil Wilson's disease
-Nodular glomerulosclerosis
What happens in amyloidosis of the kidney?
paraprotein deposit in mesangium
What are the Gross ft's of amyloidosis of the kidney?
waxy pale surface
What is the LM ft s of amyloidosis of kidney
pink hyaline deposits in mesangium
What do you use to stain amyloid in the kidney?
Congo red
What are the cf of scleroderma?
Thickening of skin, which is shiny, increase in dermal collagen, HTN, proteinurea +2
why do you have HTN in scleroderma?
fibrosis around blood vessels therefore see malignant type
What is the D/D of Scleroderma
CREST
What must you connect scleroderma with in the GIT?
GERD
interlobular arteries showing intimal thickening
Scleroderma of kidney
What kind of necrosis is seen in scleroderma
fibrinoid necrosis
What syndrome is associated with Alport syndrome?
Recurrant hematurea
What is the genetic predisposition of Alport syndrome?
X linked- dominant or recessive
What are they c/f of Alport syndrome?
Family hx of CRF
-deafness and cataracts, lens dislocation, corneal dystrophy
What is the etiology of alport syndrome
defective gene (alfa5) produce abnormal collagen
What re the lab findings of alport syndrome?
LM: irregular thickenings of glomeruli and foamy cells in tubules (mucopolysaccharide in macrophages)
What is the etiology of chronic glomerulonephritis?
Membraneous GN or focal segmental GS
Pt. presents with increase in BUN and creatinine ( uremia : increase in K+, and PTH, decrease in CA2+ and erythropoietin). Has HTN also
Chronic Glomerulonephritis
What is the prognosis of chronic glomerulonephritis?
30-50% will require renal transplantation
What is the Morphology of Chronic Glomerulonephritis?
Gross: cortical atrophy, coarse granular surface
LM: thickening of small and medium arteries= HTN (hyalinized glomeruli
ESRD: GFR less than 5% and ALL GLOMERULI SCLEROSED
What is the benign tumor of the renal system called?
Angiolmyolipoma
What are the clinical ft s of angiomyolipoma?
cardiac rhabdomyoma (LV near outflow), child, and tuberuerosclerosis (in brain)
What type of tumor is RCC?
adenocarcinoma
What are the risks of renal cell carcinoma?
smoking, cadmium
-acquired cystic disease, VHL (chromosome 3)
What is the morphology of renal cell carcinoma?
Gross: yellow, circumscribed mass in UPPER pole (contains lipid and glycogen)
What usually gets invaded by RCC?
- Renal vein- ivc and reach right heart(metastasis) -hematogenesis
- invade adrenal- addison's disease
What are the micro findings or Renal Cell Carcinoma?
- clear cell carcinoma- big cells w/clear cytoplasm due to glycogen
- papillary necrosis
- chromophobe carcinoma
What is the means of metastasis of RCC
to the lung and the bone (hematogenic metastasis)
- osteolytic lesion- pathological fracture
- increase in alkaline phosphatase increase in Ca2+ (kidney stone)
What are the c/f of RCC?
Hematurea+ flank mass+ pain/fever
What are the paraneoplastic syndromes associated with RCC?
-secondary polycythemia vera ( erythropoietin)- Hct greate than 60%)
-Hypercalcemia (PTH- like hormone)
- Cushing Syndrome (ACTH- increase in GC)
- Feminization/ Masculinization ( increase in Gonadotropin)
What are the clinical ft s f Nephroblastoma ( wilm's tumor)
child age 2 to 5 y/old
- Round smooth abdominal mass extended into the pelvis- paravertebral
- Right kidney
What genes are associated with Nephroblastoma?
WT1 (deletion of Ch11p13) leading to loss of tumor suppresor gene WT
What are the syndromes associated with Nephroblastoma?
-WAGR: Wilm's tumor, Aniridia, Genital abdnormality, mental Retardation.
- Deny's Drash Syndrome: gonadal dysgenesis and renal abnormality
- Beckwith Weidmann Syndrome: enlargement of tongue, mental retardation hemihypertrophy of body segments, enlargement of adrenal cortex.
What is the gross morphology of Nephroblastoma?
Lobulated, tan mass
What is the micro morphology of nephroblastoma
-blastemal + epithelial elements+ stroma(mixed tumor)
-small, blue blastemal cells, abortive glomeruli and tubule formation
What is another name for transitional cell carcinoma of the pelvis?
urothelial neoplasm
What are the causes of transitional cell carcinoma of renal pelvis
Acetominophen poisoning.
What is affected in transitional cell carcinoma of the renal pelvis
papillary growth, irregular cauliflower like surface + interstitial nephrosis
What are the characteristics that are important in transitional cell carcinoma of the ureter/bladder
multifocal (multiple locations)
- most are papillary tumors, some invasive
What are the complications of TCC of ureter/bladder?
Hydronephrosis+ chronic pyelonephritis- CRF-ESKD
What are the risks of TCC of Ureter/bladder?
-smoking
- beta naphthylamine
- chronic cystitis
- aniline dye
What is the clinical presentation usually for TCC of ureter/bladder
male patient 40-60 y/old
What is the genetic association with TCC
there are two different genes involved, both with chromosome 9 deletions, p53, and FGFR3
where does Ig A deposit in the skin?
Dermis= producing dermatitis herpitaformis
what is the similary between good pasture's syndrome and Type 3 GN
-Hematurea and hemoptysis
- they are both cresencteric.

To d/f you get sinusitis nose bleed in wegner's (type 3)
Where is Sq cell carcinoma of the bladder common?
Middle east
What is the etiology of Sq cell carcinoma of the bladder?
long standing cystitis due to Shistoma hematobium- chronic irritation lead to sq metaplasia of bladder
What are the clinical ft s of SCC of bladder?
painless hematurea
- atypical cells
- prognosis depends on grade and stag
What determines if there is a bad prognosis of SCC?
Anaplasia and invasion
what are the ft s of cystitis?
leukocyte, nitrite +ve, NO WBC casts
What is complications of SCC of bladder?
- reoccurance post surgery
What is another name for a childhood bladder tumor?
Embryonal Rhabomyosarcoma
What type of tumor is the embryonal rhabdomyosarcoma?
Mesenchymal metaplasia- smooth muscle to striated muscle
What marker is positive for embryonal rhabdomyosarcoma?
desmin - mass from bladder wall
Age 4, painless hematurea?
Post strep GN
Age 70 painless hematurea?
- Membraneous GN
-TCC/urothelial neoplasm
-RCC