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140 Cards in this Set
- Front
- Back
Granular IF
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Type 3 rxn.
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Linear, smooth IF
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Anti-GBM Ab
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Minimal change disease LM & IF
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normal glomeruli
lipid droplet in proximal tubular epithelium no deposit |
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Nephrotic syndrome in children 2-6 yrs.
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minimal change disease
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Test for minimal change disease
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steroids
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Minimal change disease EM
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effacement of epithelial (podocytes) foot processes
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Minimal change disease C/F & lab
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proteinuria of albumin 4+
slightly cloudy urine yellow urine oval fat body Lab: high serum cholesterol normal complement levels |
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Nephrotic syndrome in adults
& etiology |
membranous nephropathy
Etiology: idiopathic or genetic Drug (penicillamine), renal transplantation, Heymann nephritis SLE, DM, amyloidosis Adenocarcinoma of lung and colon |
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Morphology of MGN
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LM: H& E Stain--> diffuse thickening of capillary wall
Silver stain--> spikes IF: granular deposits of IgG and C3 EM: Sub epithelial deposit along BM |
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Urinalysis and Lab of MGN
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urine:
protein 4+ WBC/hpf = <2/hpf Lab: low complements |
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MGN C/F
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HTN
hematuria may progress to renal failure |
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Types of Acute glomerulonephritis
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acute post streptococcal glomerulonephritis
non-streptococcal causes |
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Acute nephritic syndrome in a child
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Acute post-streptococcal glomerulonephritis
ASO titer: high Age: 2-4 years |
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Acute post-streptococcal glomerulonephritis
Etiology--> Path--> Hypersensitivity rxn--> |
beta hemolytic group A strep infection of THROAT & SKIN
complement mediated tissue damage w/ the help of PMN Type 3 (circulating IC) |
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Non-streptococcal glomerulonephritis
Causes--> D/D--> Test--> |
pneumococcal pneumonia
Hep B, C SLE, PAN Malaria Acute post-strep ASO titre |
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Morpho Acute post-strep glomerulonephritis
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LM: hypercellular, large glomeruli contain neutrophils
Tubules: RBC cast IF: granular deposit of IgG, IgM, C3 in all glomerulous EM: subepithelial humps |
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Urinalysis, C/F
Acute post-strep glomerulonephritis |
Urine: smoky b/c of acid hemetin (due to hematuria), dysmorphic RBC (b/c RBC squeezing through glomerulus)
Serum complement low (C3 & C5). Complements used up by IC ARF = acute nephritic syndrome C/F: abrupt onset, malaise, slight fever, nausea |
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Past hx. of post strep glomerulonephritis?
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pharyngitis, dermatitis, impetigo (honey-crusted lesions)
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Complication of post strep glomerulonephritis
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may progress to crescentric GN
may progress to chronic GN |
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rapidly occurring acute nephritic syndrome?
LM--> C/F--> |
CrGN
all types show glomerular crescent hematuria and in 2-3 days ARF + uremia |
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Composition of crescent in CrGN
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epithelial cells (podocytes)
fibrin monocytes |
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Type 1 CrGN
C/F D/D |
1. AKA Anti-GBM DISEASE
2. AKA: Good pasture syndrome. - Presence of Anti GBM antibody in serum: this react with alveolar capillary → pulmonary alveolar hemorrhage. - Present as hematuria and hemoptysis 3. IF: Linear and smooth deposit of IgG, and C3 on GBM. C/F--> decrease C3 D/D--> wegener (+c-anca) |
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Ab to which antigens in CrGN
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lung and GBM
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Which type of hypersensitivity rxn is type 1 CrGN?
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Type 2
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Type 2 CrGN
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Etiology: mainly SLE
IF: Granular deposit Clinical : progress to renal failure. Serum: ANA present Type 3 hypersensitivity |
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C/F of SLE
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butterfly rash over bridge of nose
photosensitivity anemia anti-smith Ab, dsDNA Ab |
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Type 3 CrGN
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Aka: Pauci-immune (no immune reaction)
Disease association: Wagner Granulomatosis, polyarteritis Nodosa Serum: Normal complements Positive ANCA (c or p) LM: glomerular crescent IF and EM: no deposit |
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C/F PAN
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malaise, HTN, fever, purpura in skin (ulcer)
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C/F Type 3 CrGN
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acute onset of hematuria, hemoptysis,
RPGN--> ARF, uremia |
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Prognosis of crescentric glomerulonephritis
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depends upon the number of crescent in kidney: so biopsy is indicated
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Causes of IgA elevation
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longstanding-->
lung infection GI disease (celiac sprue) |
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IgA elevation leads to deposits in...
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kidney
blood vessels --> HSP dermis--> dermetitis herpetiformis |
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recurrent hematuria in children and young adults
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Berger's (IgA nephropathy)
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Berger's
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Syndrome: recurrent hematuria
This hematuria occur 1-2 days after upper respiratory tract infection. May progress to Chronic renal Failure(25%-50%). IgA deposit in skin- dermatitis herpetiformis. Asso: Gluten enteropathy. LM: focal proliferation of mesangial cells IF: IgA is deposited mainly in mesangium. |
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Henoch-Schonlein Purpura
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It is associated with
1.Skin purpuric Rash 2.Abdominal Pain 3.Arthritis 4.And Kidney change Q: What is the similarity? A: Both are caused by IgA deposition in Mesangium and skin deposit of IgA. |
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nephrotic syndrome in adults
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MPGN type 1
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MPGN Type 1
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Etiology: Hepatitis B and C, HIV, SLE, chronic liver diseases, chronic Bacterial Infection.
LM: H&E: hyper cellular glumeruli ( but no PMNs) and thick GBM. Silver stain : Tram track IF: Granular deposit. Serum: low complements ( particularly C3) |
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Tram-tracking
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double basement membranes b/c of basement membrane splitting
MPGN 1 |
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Syndrome: Hematuria/ chronic renal failure-->
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MPGN 2
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MPGN 2
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40% progress to end stage renal failure
IF: Dense deposit in GBM . Aka dense deposit diseases. Serum: C3NeF (C3 Nephritic Factor) autoantibody is Present. |
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Where are the dense deposits in MPGN 2?
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C3 deposits in capillary walls and mesangium
--> bad prognosis. Why? deposition on mesangium is inescapable b/c of lack of blood supply Granular |
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podocyte effacement
minimal change disease |
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MGN
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MGN
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hypercellular glomeruli
acute post-strep glomerulonephritis |
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subepithelial humps
acute post-strep glomerulonephritis |
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CrGN
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Focal proliferation of mesangial cells
Berger's |
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IgA deposit in mesangium
Berger's |
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tram-tracking
MPGN 1 |
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Proliferative
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anasarca
nephrotic syndrome |
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granular
circulating immune complex |
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linear, smooth
anti-GBM |
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FSGS
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Trichrome stain demonstrating blue, collagen deposits
FSGN |
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Non-selective proteinuria of nephrotic range
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FSGS
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nephrotic syndrome in child and adult
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FSGN
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proteins in non-selective proteinuria
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albumin
fibrinogen globulin |
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Morpho
FSGN |
H&E: sclerosis of some glomeruli, w/ partial involvement
Trichrome: blue |
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Urinalysis of FSGS
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frothy b/c of albumin
thick b/c of fibrinogen |
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D/x of FSGS
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biopsy
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FSGS
clinical |
Clinical:
A. Poor response to corticosteroid B. Hematuria, Hypertension C. Progression to chronic renal failure D. 50% develop End stage Renal failure within 10 years. |
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D/D of FSGS from minimal change disease
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poor response to steroids
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Secondary glomerulonephritis
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Diabetes Mellitus
Systemic Lupus Erythematosus. Amyloidosis Goodpasture Syndrome Wagner granulomatosis Henoch-Schönlein Purpura Bacterial Endocarditis |
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Complications of nephrotic syndrome
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1. MI
2. repeated infection 3. DVT |
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An adult comes in with nephrotic syndrome, what is the order of possible causes you will suspect?
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1. MGN
2. DM 3. SLE 4. Amyloid |
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Test for
DM? SLE? amyloidosis? |
DM--> PAS positive, deposit composition
SLE --> anti-smith Ab, dsDNA Ab amyloidosis--> congo red |
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D/x for lupus nephritis?
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decrease in C1q
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Which diseases have a normal looking glomerulus?
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minimal change disease & (FSGS?) Type 3 CrGN?
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LM of SLE
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wire loop
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IF of lupus nephritis
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C1q deposits
Circulating IC nephritis (Type 3) granular |
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All Type 3 hypersensitivity rxns (nephrotic) have anasarca, proteinuria, and lipiduria, but if oliguria is present also what will you suspect?
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CrGN
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1. CrGN= RPGN
2. Focal proliferative GN = ANS 3. Wire loop= NS 4. Mesangial lupus GN = NS 5. Normal glomerulus = NS |
Lupus nephritis
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Etiology of scleroderma and kidney
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interlobular arteries of the kidney show intimal thickening (deposits on and w/in intima).
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C/F of scleroderma and the kidney
nephritic or nephrotic? |
shiny taught skin, increased dermal collagen, HTN, proteinuria +2 (non-nephrotic)
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What is difference between CREST and Scleroderma?
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CREST--> focal
Scleroderma--> diffuse, HTN, increased collagen |
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Composition of deposits inside glomerulus of diabetic kidney?
Nephrotic or nephritic? |
increased glucose & subendothelial collagen
nephrotic |
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A diabetic kidney is at risk of....
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infection
MI thrombosis (DVT |
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What vascular change does a diabetic kidney undergo?
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hyaline arteriosclerosis
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Which test will not be useful in diagnosing a diabetic kidney?
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IF
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Amyloidosis of Kidney
Gross? |
waxy pale surface
Pale b/c of lack of blood vessels |
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LM of amyloidosis of kidney
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pink hyaline like deposit in mesangium
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What are the diagnosing features of primary amyloidosis of the kidney?
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Primary: (multiple myeloma)
M spike Bence-Jones protein in urine (amyloid light chain) |
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Where do you expect to find amyloid in the kidney?
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acellular paraprotein deposits in the extracellular space causing atrophy
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What is the secondary (reactive- in rheumatoid arthritis) type of amyloid?
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AA
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C/F of secondary type of amyloid of the kidney?
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symmetrical joint pain in the morning, morning stiffness.
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Hereditary recurrent hematuria in children?
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Alport syndrome
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Which organs are involved in Alport syndrome and what is the resulting effect?
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ears--> nerve deafness
eye--> cataract, lens dislocation, corneal dystrophy |
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Sign of Alport syndrome?
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family history of chronic renal failure
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Inheritance of Alport syndrome?
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X-linked autosomal recessive or dominant
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What do we find in the tubules?
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deposition of foamy cells (macrophages) containing mucopolysaccharide (MPS)
Seen on LM Simulates a storage disease |
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What is the pathogenesis of Alport syndrome?
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defective gene (alpha5) produces abnormal collagen type IV
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Differential for alport syndrome and is there a genetic history with this differential disease?
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thin membrane disease
No genetic hx. like Alport does. |
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What is found in the glomeruli of Alport syndrome under a light microscope?
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irregular thickening b/c of collagen
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Where do we find recurrent hematuria?
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IgA nephropathy (Berger's disease)- kidney only
Henoch-Schonlein Purpura- systemic ( joint, abdomen, GIT, skin) Alport syndrome (+ family hx. of hematuria) |
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Where do we find RPGN?
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CrGN
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What are the associates of CrGN?
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1. Good pasture syndrome--> lungs
2. Wegner granulomatosis--> lungs 3. PAN--> kidney, skin 4. SLE--> multi organ (crescent, wire-looped, normal?) |
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C/F chronic glomerulos nephritis?
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increasing BUN and creatinine, uremia, HTN, CRF, specific gravity= 1010, occasional polyuria unless in terminal stage
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How long does it usually take to achieve CGN?
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10 years
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Which nephrotic syndromes are most likely to convert from acute Gn to chronic GN in 10years?
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MGN & FSGS
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Rx. for CGN?
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30%-50% of all patients need hemodialysis and renal transplant
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Gross of CGN?
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cortical atrophy
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LM of CGN?
Does this allow us to find the source of the problem? |
non-specific findings (biopsy not useful)
Scarring of Glomeruli, bowmen’s space Hyalinization of glomeruli. Interstitial fibrosis. Tubular atrophy Thickening of the small and medium sized arteries. NO! |
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GFR of ESKD?
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less than 5% of the normal
Use GFR to tell when the kidney reaches the end stage |
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What is the appearance of the glomeruli in CGN?
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all sclerosed
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Test for proteinuria?
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24 hour urine collection
dipstick |
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What is the cause of nephritic syndrome in an adult?
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infections:
1. Hepatitis 2. malaria 3. SLE |
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What is the cause of nephritic syndrome in a child?
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post-strep
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What is the name of type 2 hypersensitivity reaction?
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in-situ IC nephritis
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What is the cause of hyperlipiduria and lipiduria?
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decreased albumin in blood triggers lipoprotein synthesis
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How does lipiduria present?
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oval fat body in urine
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What does hyperlipiduria do in the kidney?
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steatosis of tubular epithelial cells causing increased permeability of GBM to lipoproteins
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C/F of acute nephritic syndrome
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anuria or oliguria
proteinuria 2+ 3+ hematuria azotemia HTN |
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Onset of nephritic syndrome
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weeks
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Diseases where circulating immune complex nephritis (Type 3- B-cell activation) is seen?
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1. SLE
2. Strep 3. Hep B 4. Treponema pallidum (syphillis) 5. Malaria IF--> granular deposits |
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Antibody reacting to in-situ antigen in glomeruli (Type 2)?
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Anti-GBM Ab
Ex. Good pasture syndrome (CrGn type 1) IF--> linear, smooth deposits |
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Primary glomerular diseases
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Minimal change disease
Membranous glomerulonephritis Acute glomerulonephritis Crescentic glomerulonephritis Berger's disease (IgA nephropathy) Membrenoproloferative GN Alport syndrome |
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What stain is useful for minimal change disease?
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Oil O-red stain, b/c of fat in tubular epithelium hence the name lipoid nephrosis
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Mucin deposits from which complication is one of the causes of MGN?
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adenocarcinoma of lung and colon
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Acute nephritic syndrome is hand in hand with....
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ARF b/c of the decrease in GFR caused by the proliferation/ hypercellularity
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What is responsible for the proliferation in acute glomerulonephritis?
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neutrophils
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How would you distinguish post-strep GN from non-strep GN?
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ASO titer
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D/D for post-strep GN?
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pyelonephritis, but WBC casts will signify pyelonephritis
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Humpy-dumpy lesions- large IC
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Acute post-strep GN
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c-anca (+)
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Wegner's
Type 3 CrGN |
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p-anca (+)
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PAN
Type 3 CrGN |
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Where would IgA deposit in the skin (dermatitis herpetiformis)?
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papillary dermis
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Treatment for IgA nephropathy or elevation?
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stop primary source of infection
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SLE (lupus nephritis)
LM: wire loop |
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Diabetic Kidney
LM: nodular hyaline deposit PAS positive Kimmelstiel-Wilson disease or Nodular glomerulosclerosis |
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DM kidney
hyaline arteriosclerosis |
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Amyloidosis of kidney
LM: pink hyaline like deposit in mesangium |
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CGN
"contracted kidney" coarse granular surface |
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CGN
hyalinized glomeruli |
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IF
amyloidosis |
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amyloidosis
LM: congo red stain- brick red |
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LM
normal glomerulus |
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MPGN type 2
Dense deposit These bright deposits are of C3 in capillary walls and in the mesangium. |
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SLE
lupus nephritis IF: C1q deposits everywhere granular, Type 3 rxn. |