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Renal and Urinary 1 > Nephritic Syndromes > Flashcards

Nephritic Syndromes Flashcards

1
Q

Neprhitic

Nephrotic

A

Hemauturia, aotemia, variable proteinurea, oliguria, edema, and HTN

Over 3.5 g/proteinuria, hypoalbulinemia, hyperlipidemia, lipidurisa

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2
Q

RPGN
AKI
CKD

A

Acute neprhtisi, protienuria, and acute renal failure

Renal dysfunction with unexpected elevation of creatinninae and BUN…may be reversible or irreversible

CKD - renal scarring, irreversible, uremia

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3
Q

3 studies

A

Light microscopy…good view of the overall architecture
IF…complement and Igs, fibrinogen
Electron microscopy

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4
Q

H and E
PAS
Trichrome
Jones

A

Overall

Tubular basement membranes

Collagen - chornic change

Best for glomerular basement membrane

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5
Q

Granular vs linear IF

A

Grnaular - immune complex

Linear - type 2 HS

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6
Q

Focal
Diffuse
Segmental
Global

A

Focal - some but not all glomeruli
Diffuse - all or almost all glomeruli

Segmental - part but not all of glomerulus
Global - entire glomerulus

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7
Q

IgAN

A

Most common worldwide

Macroscopic heamture 1-2 days after URI in young adults and children

Older adults - microscopic with proteinuria (subnephrotic) or HTN alone or in combo

Limited to renal - IgA neprhopathy…systeimc - HSP

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8
Q

IgAN pathogenesis

A

Galatose def IgA1

Anti-glycan IgG or IgA1

More dimeric ABs in circulation

Immune complexes in mesangial area and result in loss of RBCs and other downstream pathology

Segmental glomeruloscleoriss, mesangial proliferation, endothelial cell proliferation

Sometimes seoncdary to liver dz

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9
Q

IgA nephropathy light microscopy/IF/electron

A

Light - Mesangial hypercellularity

Look for more than 3 nuclei clustered together***

IF - granular mesangial IgA and C3 deposits

Electron - mesangial immune complexes

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10
Q

IgA Prognosis

A

Poor - impair GFR…Sustained HTN, substantial proteinuria

Mesangial hypercellularity, endocapillary hypercellularity, segmental glomerulosceloriss, tubular atrophy and IS fibrosis

MEST

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11
Q

IgAN tx

A

No proven tx

General managment of glomerular dz - BP control and angiotensin 2 blockade

Fish oil?

L-T CSs

Renal transplantation for ESRD…may recur

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12
Q

HSP

A

IgA induced small vessel leukocytoclastic vasculitis

Systemic - purpura, arthralgia, ab pain and bloody diarrhea, nephritic syndrome in kdiney

Mostly in children (3-15)

1/2 of cases preceded by a URI

Self-limited process in majority of cases

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13
Q

Infection associated glomeruloneprhitis

A

IMmunological response ot microorgansim

Strep (28%) and Staph (24%)

Upper resp tract, skin, lung and HV

Comorbodities like diabetes, alcoholism, drug abuse

Decreased inicdnece due ot early detectio nand tx

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14
Q

APSGN

A

Acute post-strep glomeruloneprhitis

Children 2-14…more in males

Acute neprhtiic syndrome - gross hematuira, proteinura (subneprhotic), edema, HTN, oliguria

Onset 1-2 weeks after recovery from sore throat or longer with skin impetigo

POsitive step culture and/or increased antistreptolysin ), anti-DNAse B or antihyaluronidase tities

Mostly do NOT need biospy

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15
Q

APSGN pathogenesis

A

Nephritogenic strains of GAS, Beta hemolytic

Strep pyogenic exotoxin - antigenic determinant

In situ immune complex foromation in glomeruli…subepithalil humps…subendothelial and mesangial

Activation of alt complement (low C3 and normal C4)

Glomerulate infiltration by PMNs and monocytes

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16
Q

APSCGN light microscopy/IF/electron

A

Acute porliferative GN
Exudative GN
Can see PMNs in the glomeruli
Can see entire glumerulus is involved

Granular staining for IgG and C3

Subepithelial humps

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17
Q

Prognosis of APSCGN

A

Children - self-limited and excellent

Adults - increased incidence of decreased (mild) renal function with persistent HTN and mild proteinuria/hematuria

18
Q

Tx of APSGN

A

Early penicillin
CSs in refractory

Tx of nephritic syndrome - BP control, Na restriction, loop diuretics

19
Q

Endocariditis associated

A

S. aureus/epidermidis…strep viridans/pyogenes…enterococcus faecalis

Most common in rheumatic HD or IVDU

Fever, arthralgias, anemia and purpura, septic embolization
New heart murmur or change
Neprhitic
Pos blood culture
Low C3 AND C4 (classical)
20
Q

Patholgoy and tx of endocarditis associated

A

LM - diffuse proli GN…sometime iwht cap thrombi…SBE tends to be more focal prolif GN

IF - IgG and C3

EM - mesangium, subendothelial and sbuepithelial immune complexes

IV ABs for 4-6 weeks

21
Q

Membranoproliferative pattern with Ddx

A

AKA mesangiocapillary

Neprhitic—-nephrotic

Light micrscopie - hypercellular glomeruli with lobulation…GMB thickened with double contours (tram tracks)

Immune complex GN
Decreased C3 and C4
Thrombotic microangiopathies
Monoclonal depostion dz
Chronic transplant glomerulopathy
22
Q

Membranoproliferative pattern IF

A

If IgG and C3 staining…think infections (Hep C), AI or rehum, or monoclonal gammopathy

If only granular C3 and NO IgG, then raise concern for complement dysregulation

23
Q

Dense deposit dz…type of MPGN

A

C3 convertase is depleted and causes the abnormality and leads to MPGN

24
Q

Type 1 vs. type 2

A

Type 1 - subendothelial immune complexes

2 - Dense deposit disease…very long intramembranous immune complexes

25
Q

MPGN Type 1 and 2 on IF

A

1 - IgG and C3…can see along the capillary loops

2 - C3 only…see very long segments

26
Q

Cryoglobulinemic glomeruloneprhitis

A

Hyalien thrombi (cryglobulins) at arrows

Associated with HepC

27
Q

Tx of MPGN

A

1 - treat infeciton…general managment…may use CSs..trnaslpant may help

DDD - replacing factor H with plasam infusion or eculizumab (AB to C5)…recurrence more often after trnasplant

Type 3 - no tx

28
Q

SLE

A

15-45 y/o…more in females and non-white

Sun exposure, exacerbation during or after pregnancy

B cell hyperactivtion and defective T cell autoregulation

Wide range of autoantibodies - many against nucleic acids

Type 2 HS - hemolytic anemia (direct cell damage)

Type 3 HS - (immune complexes) - glomeruloneprhitis and arthritis

Classical pathway activation - low C3 and C4

29
Q

SLE main renal and extrarenal manifestations

A 
Protienuria - may be nephrotic
Hematuria - dysmorphic with casts
RPGN in 10-20
HTN
Tubular abnormalities - RTA or hyperkalemia (out of proporion to decreased GFR)

Labs - anemia, thrombocytopenia, positive ANA, dsDNA, low C3 and C4

30
Q

IF og lupus

A

POsitive for IgG, A, M, C3 and C1q

“full house”

31
Q

6 stages

A 
Minimal mesangial
Mesangial prolif
Focal
DIfufse
Membranous
Advanced scleorising
32
Q

Active proliferative LN tx

A

Induction - high dose CSs and cyclophosphamide…high dose CS and mycophenolate mofetil

Maintencne - low dose CSs with quarterly IV cyclophosphamide X 2 years OR oral azathiprine or MMF

Membranous lesion with neophrotic syndrome - in addition to genrral managment, CSs and cyclosporine or MMF

33
Q

RPGN

A

Clinical dx…nephiritic with rapidly declinign kidney function…increased creatinine and decreased GFR >50% in 3 mos

Necrotizing GN with glomerular crescents in over 50% of glomeruli…aka crescentic glomerulonephritis

34
Q

type of RPGN

A

1 - anti-GBM/Goodpasture
2 - immune complex…infection, SLE, HSP
Type 3 - pauci-immune - ANCA assoicated (GPA/MPA)
4 - double-antibody positive dz (1 and 3 combined)

35
Q

Anti-GBM

A

Renal limited
Nephritic syndrome with dark urine and oliguria
Anti-GBM ABs

36
Q

Goodpasture

A

Pulm-renal syndrome
Neprhitic syndrome with dark urine and oliguria
Hemoptysis
Ant-GBM
Teens and 20s , male predominance…could be triggered

37
Q

Pathog of anti-GBM and Goodpasture

A

ABs at peptide within non-collagnous portion of the alpha 3 chain of type 3 collagen

LInear staining of GBM IgG and C 3

38
Q

Anti-GBM prognosis and tx

A

If creatinine over 5, unlikely recovery

Prgression msot rapid

Plasmapheresis

Cycophosphamide

High dose CSs

Renal transplant after “burned out”

39
Q

Type 2 pathology

A

Histology of underlying glomeruloneprhitis plus crescent formation

Granular IF

PLN, IgA, endocarditis associated, Hep C-associated, post strep GN

40
Q

MPA vs PGA

A

MIcroscopic polyangitis - P-ANCA (MPO) positive with leukocytoclastic vasculitis

POlyangitis with granulomatosis…C-ANCA (PR3) pos with necrotizing vasculitis and ncrotizing granulomas

No linear/granular IF staining
No immune complexes on electron micros

41
Q

GPA

A

Necrotizing granulomas in boht URT and LRT

Necrotizing or granulomatious vasculitis (lungs and upper airways)

Glomeruloneprhtisi - focal necrotizing or crescentic

42
Q

Type 3 tx

A

2 phases…induction - CS and cyclophos
Maintencne - azathiprine or methotrexate

PLsma exchane considered in pts iwth pulm hemorrhage or require dialysis at presentation

Renal and Urinary 1 (14 decks)

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