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158 Cards in this Set
- Front
- Back
what do you see in Type 3 hypersensitivity?
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it is deposition of a soluble antigen-antibody complex in glomeruli
Also known as circulating immune complex nephritis IF:- granular depostion (C3, C5) |
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What do you see in a Type 2 hypersensitivy?
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Anti- GBM antibody, i.e. an antibody is formed against and antigen in the glomerulus.
IF:- smooth, linear deposit |
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What do you see in a Type 4 hypersensitivity?
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Lymphocte cell mediated immune nephritis
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Examples of Type 3 hypersensitivity?
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SLE, streptococci, HBV Treponema pallidum, Malaria
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Examples of Type 2 hypersensitivity?
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Good Pasture's syndrome
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hematuria and jaundice
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Hemolytic anemia
its actually myoglobinurea not hematurea |
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hematurea and edema in adults
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acute non post strep GN
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Hematurea and colicky pain (loin to gorin)
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renal stones
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hematurea and adults, past h/x of lung and GIT infection
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Ig A nephropathy
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Hematurea and edema in children
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Post strep GN
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Hematurea, painless and adult
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Bladder tumor:
-TCC -SCC |
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Nephrotic Syndrome
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- 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 |
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Acute Nephritic Syndrome
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very similar to ARF
- Hematurea - HTN +Azotemia - Anuria/ Oligurea - Onset- weeks/months |
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Pathogenesis of Nephrotic Syndrome
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- 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) |
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Complications of Nephrotic Syndrome
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- increased chances of infections
- increase in cholesterol= ATH- MI or Angina -Blood clotting: increased viscosity of blood leads to venous thrombosis |
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Recurrent Hematuria
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- family H/x of CRF and hematurea
- "on and off" blood in the urine - fresh/ smoky urine - fresh blood on the microscope RED CASTS |
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Rapidly Progressive GN
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it has the same symptoms as ANS only a much quicker onset. i.e a couple of days
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Minimal Change Disease is mostly seen in what age group and is assoc with what
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Children (2-6 years)
Nephrotic Syndrome |
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What is another name of Minimal Change Disease
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lipoid nephrosis
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What is the pathogenesis of minimal change disease
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Loss of normal charge barrier in GBM
Lymphocyte production by T cells (type 4 hypersensitivity) |
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What is unique about minimal change disease
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there is +4 proteinurea ( ONLY ALBUMIN)
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What is the morphology of Minimal Change Disease?
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LM: no change
IF: no change EM: effacement of podocytes (foot processes), appearance of fusion of epithelial cell. |
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What is the urine analysis of Minimal Change Disease?
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Lipoid Nephrosis (increase tubular reabsorption of lipoproteins)
- yellow appearance, slightly cloudy 4+proteinurea, oval fat body |
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What are the lab findings in Minimal Change Disease?
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(N) complements
increase in cholestrol |
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What is the treatment for minimal change disease?
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Steroids
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what is the definition of diffuse in terms of GN
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all the glomeruli are involved
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What is the definition of focal in relation to GN
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It is a some of the GN
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What is the definition of segmental in relation to GN
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it is a part of the GN
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What is the definition of proliferative GN
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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
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What is the definition of membraneous GN
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increased thickness of capillary walls e.g. diabetes. Due to Type 3 hypersensitivity causing it to get thicker- deposition of complements
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When do you see Membraneous GN and what are the main associations
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Most Common Cause of Nephrotic Syndrome in adults
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What are the etiologies of Membraneous Glomerulonephritis?
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-idiopathic or genetic
-Drug induced -Penicillamine - Renal transplantation, Heymann Nephritis - SLE, DM - Adenocarcinoma |
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What is the antigen in adenocarcinoma that causes MGN?
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Mucin
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What is the age group you are most likely to associate with Membraneous Glomerulonephritis?
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Adult
|
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If a patient has Wilson's disease, What drug is given to them, and what the ADR's of this drug
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Penicillamine
-Membraneous Glomerulonephritis |
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What is the morphology of Membraneous Glomerulonephritis?
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-LM: diffuse thickening of the capillary walls, silver stain spikes
- IF: granular deposits of IgG and C3 -EM: Subepithelial deposits along GBM |
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What are the lab findings of Membraneous Glomerulonephritis?
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- Proteinurea (+4) WBC 2/hpf
- Low complement values - may loose some RBC casts |
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What are the clinical ft s of Membraneous Glomerulonephritis?
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-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 |
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Can you treat Membranous Glomerulonephritis with drugs?
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No- has to have a transplant
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What are the unique fts of Membraneous that make it very different
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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
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What is another name for acute post-strep GN?
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Proliferative GN, Post infection GN
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What is the etiology for acute post strep GN
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Beta hemolytic group A streptoccocal infection of throat and skin
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Child btw 2-4y/o suffering of throat and skin infection. Develops hematurea. What is the kidney disease they may be suffering of?
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Acute Post strep GN
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What is the pathogenesis behind Acute post strep GN?
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complement-mediated tissue damage with help of PMN
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What type of hypersenstivity is involved in acute post strep GN?
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Type 3
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What are the clinical ft.s of acute post strep GN?
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Acute nephritic syndrome
- proliferative (greater than 100 neutrophils) - decrease in GFR due to decrease in BS's= ARF (oligurea, azotemia, HTN, hematurea)W |
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What is the diagnostic test one can do to determine if it is a strep infection?
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ASO test
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What is the morphology of acute post strep GN?
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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 |
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What are the lab findings of acute post strep GN?
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Urine: smoky (acid hematin) and dysmorphic RBC
- decrease in serum complement |
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What are the unique ft s of urine in acute post strep GN?
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Dysmorphic RBC- the RBC's will squeeze through the walls
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Pt. presents with past hx of sore throat or impetigo(skin infection) followed by hematurea, and they have fever and nausea that occurs suddenly
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Acute post Strep GN
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If an adult suffers of acute post strep GN what can it progress to?
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crescentic GN or chronic GN
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What is the etiology of non-strep acute GN?
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- HBC,HCV
- pneumococcal pneumona - SLE, PAN - Malaria |
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How do you differentiate between acute post strep and non-post acute strep GN?
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ASO titer
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What is unique about Crescentic GN?
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Rapidly progressing GN- acute nephritic syndrome - develops ARF within days
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What are the cells that are more prominent in crescentic GN?
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Proliferation:
- epithelial cells - monocytes - fibrin - visceral cells |
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What are the c/f of crescentic GN
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ANS/RPGN to ARF
prognosis depends on no. of crescent of kidney= greater than 50% crescents but bad prognosis |
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What is the stain used to see crescentic GN
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PAS +ve
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What were the ft s of Type 1 Crescentic GN
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- presence of anti-GBM antibody in serum- reacts with alveolar capillary also
- pulmonary alveolar hemorrhage= hemoptysis -hematurea |
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what are the IF findings of type 1 Crescentic GN?
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Smooth, linear deposit of Ig G or C3 on GBM (type 2 hypersensitivity)
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What is another name of type 1 GN?
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Good Pasture syndrome
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What are the causes of type 2 GN?
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SLE (arthritis, anemia, butterfly rash, photosensitivity)
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What are the IF findings in type 2 GN?
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granular deposition of Ig G and C3 in sub epithelial spaces (type 3 hypersensitivity)
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What are the serum findings of Type 2 GN?
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ANA(+), antismith antibody, dsDNA
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What is another name for type 3 Crescentic GN?
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Pauci-immune
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What are the diseases' associated with type 3 GN?
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Wegner's granulomatosis (c-ANCA) and Polyarteritis Nodosa
|
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What kind of necrosis is seen in Type 3 GN associated with PAN
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patchy fibrinoid necrosis
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What are the serum findings of type 3 GN?
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(N) complements- absence of immune reaction
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What are the LM findings of type 3 GN?
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glomerular crescent + vasculitis= hematurea, hemoptysis, ARF
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What is the etiology of Ig A nephropathy?
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Hx of RTI or diarrhea- release of Ig A
-children or young adult |
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What syndrome is associated with Ig A nephropathy?
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Recurrent hematurea
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Pt presents with upper respiratory tract infection, rash on body and hematurea, what is ur diagnosis
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Ig A nephropathy
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What is another name for IgA nephropathy?
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dermatitis herpitaformis
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What is the likely complication if Ig A nephropathy spreads to the vasculature?
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Henoch Shonlein Purpura
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What is the morphology of Berger's disease?
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focal proliferation of mesangial cells
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what is another name for Ig A nephropathy?
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Berger's disease
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Pt presents with upper respiratory tract infection, rash on body and hematurea, what is ur diagnosis
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Ig A nephropathy
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What is another name for IgA nephropathy that affects the skin?
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dermatitis herpitiformis
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What is the likely complication if Ig A nephropathy spreads to the vasculature?
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Henoch Shonlein Purpura
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What is the morphology of Berger's disease?
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focal proliferation of mesangial cells
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what is another name for Ig A nephropathy?
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Berger's disease
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What is another name of henoch shonlein purpura?
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leukocytoclastic reaction- produces neutrophils- hypersenstivity allergic vasculitis
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what are the symptoms of henoch shonlein purpura?
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-skin purpuric rash
- abdominal pain - arthritis - kidney change |
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What is the etiology of membranoproliferative GN 1?
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-HBV
-HCV -HIV, SLE -Chronic liver disease, bacterial infection |
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What is the morphology of membranoproliferative GN 1?
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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 |
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what is the hypersensitivity involved in Membranoproliferative GN1?
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Type 3 hypersensitivity
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What is the syndrome associated with Membranoproliferative GN 2 disease?
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Hematurea and Chronic Renal Failure (40% progress to ESRF)
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What is the morphology of Membranoproliferative GN2
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IF: dense deposit in GBM- C3 in capillary walls and mesangium
Serum: C3Nef Autoantibody |
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Which disease in the kidney does not allow any formation of complements in the glomerulus?
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MPGN2- C3NeF autoantibody
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What is the syndrome associated with Focal segmental GN?
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Nephrotic Syndrome
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What causes destruction of podocytes in minimal change disesase
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pt has an infection (respiratory), increasing no. of T-cell lymphokines
|
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What are the clinical presentations of Focal segmental Glomerulosclerosis?
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Children or HIV+ adult
Develop non-selective proteinurea(albumin, fibrinogen, globulin) |
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What is the etiology of Focal segmental Glomerulosclerosis?
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- HIV infection(IV drug abusers)
- Inherited congenital disease - start as a primary disease |
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What is the morphology of FSGS?
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Sclerosis of some glomeruli with partial involvement
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What is the stain used to identigy FSGS?
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Trichrome blue
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Pt. presents with hematurea, HTN, poor response to steroids, and progression to CRF( 50% develop ESRF within 10 years?
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FSGS
|
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How do you differentiate FSGS from membranoproliferative GN/
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Trichrome stain is positive in FSGS
|
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What are the kidney disease affected by SLE?
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- crescenteric GN
-Membranous like- Wire loop -Hyaline arterolosclerosis - Normarl glomerulous (Minimal change disease) -focal segmental disease (NS) |
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What are the findings of SLE?
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dsDNA+, Nephrotic syndrome and C1q deposit, and decrease in C3.
Lungs get pruritis |
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What is the d/d of SLE in Minimal change disease?
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Mesangial cell GN (NS)
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What is the cause of Diabetic Kidney
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complex of glucose and subendothelial collagen deposit
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What is the stain used to determine there is diabetic kidney?
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PAS +ve
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what disease is associated with diabetic kidney
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-Kiemesteil Wilson's disease
-Nodular glomerulosclerosis |
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What happens in amyloidosis of the kidney?
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paraprotein deposit in mesangium
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What are the Gross ft's of amyloidosis of the kidney?
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waxy pale surface
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What is the LM ft s of amyloidosis of kidney
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pink hyaline deposits in mesangium
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What do you use to stain amyloid in the kidney?
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Congo red
|
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What are the cf of scleroderma?
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Thickening of skin, which is shiny, increase in dermal collagen, HTN, proteinurea +2
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why do you have HTN in scleroderma?
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fibrosis around blood vessels therefore see malignant type
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What is the D/D of Scleroderma
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CREST
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What must you connect scleroderma with in the GIT?
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GERD
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interlobular arteries showing intimal thickening
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Scleroderma of kidney
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What kind of necrosis is seen in scleroderma
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fibrinoid necrosis
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What syndrome is associated with Alport syndrome?
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Recurrant hematurea
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What is the genetic predisposition of Alport syndrome?
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X linked- dominant or recessive
|
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What are they c/f of Alport syndrome?
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Family hx of CRF
-deafness and cataracts, lens dislocation, corneal dystrophy |
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What is the etiology of alport syndrome
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defective gene (alfa5) produce abnormal collagen
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What re the lab findings of alport syndrome?
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LM: irregular thickenings of glomeruli and foamy cells in tubules (mucopolysaccharide in macrophages)
|
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What is the etiology of chronic glomerulonephritis?
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Membraneous GN or focal segmental GS
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Pt. presents with increase in BUN and creatinine ( uremia : increase in K+, and PTH, decrease in CA2+ and erythropoietin). Has HTN also
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Chronic Glomerulonephritis
|
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What is the prognosis of chronic glomerulonephritis?
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30-50% will require renal transplantation
|
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What is the Morphology of Chronic Glomerulonephritis?
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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 |
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What is the benign tumor of the renal system called?
|
Angiolmyolipoma
|
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What are the clinical ft s of angiomyolipoma?
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cardiac rhabdomyoma (LV near outflow), child, and tuberuerosclerosis (in brain)
|
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What type of tumor is RCC?
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adenocarcinoma
|
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What are the risks of renal cell carcinoma?
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smoking, cadmium
-acquired cystic disease, VHL (chromosome 3) |
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What is the morphology of renal cell carcinoma?
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Gross: yellow, circumscribed mass in UPPER pole (contains lipid and glycogen)
|
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What usually gets invaded by RCC?
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- Renal vein- ivc and reach right heart(metastasis) -hematogenesis
- invade adrenal- addison's disease |
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What are the micro findings or Renal Cell Carcinoma?
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- clear cell carcinoma- big cells w/clear cytoplasm due to glycogen
- papillary necrosis - chromophobe carcinoma |
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What is the means of metastasis of RCC
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to the lung and the bone (hematogenic metastasis)
- osteolytic lesion- pathological fracture - increase in alkaline phosphatase increase in Ca2+ (kidney stone) |
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What are the c/f of RCC?
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Hematurea+ flank mass+ pain/fever
|
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What are the paraneoplastic syndromes associated with RCC?
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-secondary polycythemia vera ( erythropoietin)- Hct greate than 60%)
-Hypercalcemia (PTH- like hormone) - Cushing Syndrome (ACTH- increase in GC) - Feminization/ Masculinization ( increase in Gonadotropin) |
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What are the clinical ft s f Nephroblastoma ( wilm's tumor)
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child age 2 to 5 y/old
- Round smooth abdominal mass extended into the pelvis- paravertebral - Right kidney |
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What genes are associated with Nephroblastoma?
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WT1 (deletion of Ch11p13) leading to loss of tumor suppresor gene WT
|
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What are the syndromes associated with Nephroblastoma?
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-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. |
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What is the gross morphology of Nephroblastoma?
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Lobulated, tan mass
|
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What is the micro morphology of nephroblastoma
|
-blastemal + epithelial elements+ stroma(mixed tumor)
-small, blue blastemal cells, abortive glomeruli and tubule formation |
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What is another name for transitional cell carcinoma of the pelvis?
|
urothelial neoplasm
|
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What are the causes of transitional cell carcinoma of renal pelvis
|
Acetominophen poisoning.
|
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What is affected in transitional cell carcinoma of the renal pelvis
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papillary growth, irregular cauliflower like surface + interstitial nephrosis
|
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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
|
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What are the risks of TCC of Ureter/bladder?
|
-smoking
- beta naphthylamine - chronic cystitis - aniline dye |
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What is the clinical presentation usually for TCC of ureter/bladder
|
male patient 40-60 y/old
|
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What is the genetic association with TCC
|
there are two different genes involved, both with chromosome 9 deletions, p53, and FGFR3
|
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where does Ig A deposit in the skin?
|
Dermis= producing dermatitis herpitaformis
|
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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
|
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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
|
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what are the ft s of cystitis?
|
leukocyte, nitrite +ve, NO WBC casts
|
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What is complications of SCC of bladder?
|
- reoccurance post surgery
|
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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
|
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Age 4, painless hematurea?
|
Post strep GN
|
|
Age 70 painless hematurea?
|
- Membraneous GN
-TCC/urothelial neoplasm -RCC |