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Renal > SM_209a: Nephritis > Flashcards

SM_209a: Nephritis Flashcards

1
Q

Describe characteristics of nephritic syndromes

A

Nephritic syndromes

  • Key feature: hematuria
  • Other features: oliguria, azotemia, hypertension
  • Common histomorphologic feature: proliferation of cells within the glomerulus and damage to GBM
  • Result: RBC escape into urine
  • Three most common entities: acute post-streptococcal glomerulonephritis, crescentic GN (rapidly progressive GN) types 1/2/3, IgA nephropathy
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2
Q

Key feature of nephritic syndromes is ______

A

Key feature of nephritic syndromes is hematuria

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3
Q

Describe acute post-streptococcal glomerulonephritis

A

Acute post-streptococcal nephritis

  • Kids
  • 1-6 weeks after a Group A (beta-hemolytic) strep infection
  • Cola colored urine and low serum compliment compliment levels
  • less severe cases self resolve and may not be biopsied
  • LM: neutrophils and lymphocytes in glomerulus, fibrin thrombi
  • FM: granular IgG and compliment in starry-sky pattern
  • EM: dense subepithelial humps
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4
Q

_____ occurs 1-6 weeks after a Group A (beta-hemolytic) strep infection

A

Acute post-streptococcal glomerulonephritis occurs 1-6 weeks after a Group A (beta-hemolytic) strep infection

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5
Q

Acute post-streptococcal glomerulonephritis is characterized by _____ urine and low _____ levels

A

Acute post-streptococcal glomerulonephritis is characterized by Cola-colored urine and low serum compliment levels

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6
Q

On FM, acute post-streptococcocal glomerulonephritis manifests as _____

A

On FM, acute post-streptococcocal glomerulonephritis manifests as granular IgG and compliment in starry-sky pattern

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7
Q

On EM, acute post-streptococcocal glomerulonephritis manifests as ______

A

On EM, acute post-streptococcocal glomerulonephritis manifests as dense subepithelial humps

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8
Q

Describe rapidly progressive glomerulonephritis / crescentic glomerulonephritis

A

Rapidly progressive glomerulonephritis / crescentic glomerulonephritis

  • Adults
  • Rapid loss of renal function, rapidly proceeding to need for dialysis
  • RPGN: clinical syndrome
  • Crescentic GN: most common pathologic picture
  • LM: crescents in most glomeruli - proliferation of parietal eptihelial cells of Bowman’s capsule
  • FM and EM for further classification
  • 3 types
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9
Q

RPGN is a ______, while crescentic GN is ______

A

RPGN is a clinical syndrome, while crescentic GN is the most common pathologic picture

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10
Q

RPGN manifests as ______ on LM due to ______

A

RPGN manifests as crescents in most glomeruli on LM due to proliferation of parietal epithelial cells of Bowman’s capsule

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11
Q

Describe RPGN type I

A

RPGN type I

  • Anti-GBM disease: antibody recognizes collagen IV
  • FM: linear IgG deposits with or without C3
  • EM: no distinct deposits, antibody evenly distributed throughout GBM
  • In many patients IgG also recognizes pulmonary basement membrane (Goodpasture syndrome): hematuria, hemoptysis
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12
Q

RPGN type I manifests as _____ on FM

A

RPGN type I manifests as linear IgG deposits with or without C3

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13
Q

RPGN type 1 in which the IgG also recognizes the pulmonary basement membrane is ______

A

RPGN type 1 in which the IgG also recognizes the pulmonary basement membrane is Goodpasture syndrome

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14
Q

Describe RPGN type II

A

RPGN type II

  • Immune complex disease
  • Complication of any autoimmune disorders which generate immune complexes (SLE, post-streptococcal glomerulonephritis, Henoch Schonlein purpura)
  • FM: granular immune complex deposits with C3 and IgG or IgA
  • EM: lumpy-bumpy appearance
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15
Q

RPGN type II manifests as ______ on FM

A

RPGN type II manifests as granular deposits of C3 with IgG or IgA on FM

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16
Q

Describe RPGN type III

A

RPGN type III (pauci-immune)

  • 50% of crescentic GN cases
  • FM: negative
  • EM: no deposits
  • 80% have ANCAs: anti-neutrophil cytoplasmic antibodies: myeloperoxidase (MPO, p-ANCA), proteinase-3 (PR3, c-ANCA), atypical ANCA (X-ANCA)
17
Q

Describe RPGN type III with ANCA

A

RPGN type III with ANCA

  • ANCA seen in vasculitides
  • Systemic manifestations: purpura, ulcers, pulmonary hemorrhage, peripheral neuropathies, may have fever
  • MPA (microscopic polyangiitis): vasculitis with neutrophols
  • GPA (granulomatosis with polyangiitis): formerly called Wegener’s, vasculitis with neutrophils and granulomas
  • EGPA (eosinophilic granulomatosis with polyangiitis): formerly called Churg-Strauss, vasculitis with neutrophils and granulomas with addition of asthma and eosinophilia
18
Q

RPGN III is a _____ involving _____, _____, and _____

A

RPGN III is a necrotizing vasculitis involving fibrinoid necrosis, neutrophils, and thrombi

(scarred so will not regain function)

19
Q

Describe IgA nephropathy (Berger disease)

A

IgA nephropathy (Berger disease)

  • Kids and young adults, recurrent episodes of hematuri, first episode occurs after infection
  • Abnormal IgA deposits at GBM
  • Serum IgA increased, including immune complexes
  • Pathogenesis: immune complexes lodge in kidney, activate complement, damage GBM, and cause RBC leakage into filtrate
  • LM: mesangial proliferation, mesangial deposits, or crescents
  • FM: granular IgA and C3 deposts in mesangium
  • EM: mesangial deposits
20
Q

IgA nephropathy (Berger disease) is characterized by ______

A

IgA nephropathy (Berger disease) is characterized by abnormal IgA deposits at GBM

21
Q

IgA nephropathy manifests as ______ on FM

A

IgA nephropathy manifests as granular IgA and C3 deposits in mesangium on FM

22
Q

Describe lupus nephritis

A

Describe lupus nephritis

  • Classified into 6 classes which directly affect treatment: minimal changes -> active changes -> chronic changes with somewhat parallel increases in severity
  • Full house on FM (including IgG, IgM, and IgA with C3 and C1q)
  • Active lesions show hypercellularity, endocapillary fibrin, and apoptotic bodies
  • Chronic changes: sclerosis (scarring) and crescents
23
Q

Amyloid is _____

A

Amyloid is deposit of certain proteins capable of forming beta-pleated sheet polymers resistant to degradation that interferes with normal functions of the organ

24
Q

Lupus nephritis manifests as ______ on FM

A

Lupus nephritis manifests as full house (IgG, IgM, IgA and C3 and C1q) on FM

25
Q

Describe the classification system of lupus nephritis

A

Lupus nephritis classification system

26
Q

Describe amyloidosis

A

Amyloidosis

  • Clinical syndrome occurring as a result of amyloid (proteins capable of forming beta-pleated sheet polymers resistant to degradation) in various organ systems
  • Deposition interferes with normal function of organ - filtration (glomerulus), reabsorption (GI), vascular compliance (vasculature), barrier function (bladder, skin)
  • Currently classified according to protein type
27
Q

Amyloid protein AA stands for _____ and occurs in cases of _____

A

Amyloid protein AA stands for Amyloid of Serum Amyloid A (SAA) and occurs in cases of chronic inflammation

(infectious or autoimmune)

28
Q

Amyloid protein AL stands for _____ and usually occurs in _____

A

Amyloid protein AL stands for amyloid of light chains and usually occurs in lymphoid neoplasms

29
Q

Amyloid protein AH stands for ______ and usually occurs in ______

A

Amyloid protein AH stands for amyloid of heavy chains and usually occurs in lymphoid neoplasms

30
Q

Amyloid protein Abeta2M stands for ______ and is seen in ______ patients

A

Amyloid protein Abeta2M stands for amyloid of beta2-microglobulin and is seen in chronic hemodialysis patients

31
Q

Amyloid protein ATTR stands for ______, is seen in ______, and has a propensity for the ______

A

Amyloid protein ATTR stands for amyloid of transthyretin, is seen in elderly or familial if mutated transthyretin, and has a propensity for the heart

32
Q

Amyloid exhibits _______ on polarized light

A

Amyloid exhibits apple-green birefringence on polarized light

Renal (31 decks)

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