Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

X Clin Med II Exam 3 > Glomerulonephritis and PKD > Flashcards

Glomerulonephritis and PKD Flashcards

1
Q

What can cause glomerulonephritis?

A 
IgA nephropathy
Poststreptococcal GN
Anti-GBM antibody disease
Lupus nephritis
IgA vasculitis (HSP)
Pauci-immune GN
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is an early sign of kidney disease?

A

Proteinuria (due to increased permeability and the filtration not working right)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is glomerular disease?

A

Damage to the major components of the glomerulus (podocyte, glomerular basement membrane, capillary endothelium or mesangium)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Primary vs secondary glomerular disease

A

Primary: glomerular injury limited to kidney
Secondary: renal abnormalities result from a systemic disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is glomerulonephritis?

A

Group of diseases that present in nephritic spectrum and usually signifies inflammatory process in glomerulus causing renal dysfunction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Cause of glomerulonephritis

A

Deposition of immune complexes of glomerulus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Major clinical findings of nephritic syndrome

A 
Hematuria (dysmorphic RBCs and RBCs casts)-cola urine
Proteinuria (subnephrotic <3 g/day)
Elevated creatinine (or decrease in GFR)
Oliguria
Edema (periorbital, peripheral)
HTN
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is rapidly progressive glomerulonephritis?

A

Most severe and urgent end of nephritic spectrum

Progressive loss of renal function over short period of time

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What characterizes rapidly progressive glomerulonephritis on a biopsy?

A

Crescent formation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the crescent formation?

A

Nonspecific response to severe injury to glomerular capillary wall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Common causes of hematuria

A

UTI, stones, cancer, BPH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

How do you distinguish extraglomerular from glomerular hematuria?

A

Extraglomerular: red/pink, may have clots!, normal RBCs
Glomerular: cola-colored, no clots, may have proteinuria, dysmorphic RBCs, maybe RBC casts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Serologic testing for GN

A

Creatinine, ANA, anti-ds DNA, complement, ANCA, anti-GBM antibodies, antistreptolysin O titer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Antiproteinuric therapy of GN

A

ACE-I or ARB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What warrants immediate hospitalization in GN?

A

Acute nephritic syndrome or RPGN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Most common cause of primary GN in world

A

IgA nephropathy (Berger’s disease)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Which type of IgA nephropathy is most common?

A

Primary (renal-limited)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

When is IgA nephropathy seen in the most?

A

2nd and 3rd decades of life

Males more

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Pathogenesis of IgA nephropathy

A

IgA deposition in the glomerular mesangium due to an inflammatory response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Most common presentation of IgA nephropathy

A

An episode of gross hematuria usually following a URI (often 1-2 days after illness)
On nephritis spectrum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

How do you confirm IgA nephropathy

A

Kidney biopsy (more severe or progressive disease)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Tx of IgA nephropathy

A

1/3 is spontaneous clinical remission
Tailor to risk of progression-ACE-I or ARB
Glucocorticoids +/- immunosuppressive agents

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Who is at higher risk of progressing to end stage renal disease?

A

Proteinuria > 1 g/d
Decreased GFR
HTN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Cause of poststreptococcal GN

A

Infection with group A beta-hemolytic strep infections (usually pharyngitis or impetigo)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

When is poststreptococcal GN seen more?

A

In kids

Seen more in males

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Pathophysiology of post-streptococcal GN

A

Immune complex with antigen deposited into glomerulus and triggers complement activation and inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Presentation of post-streptococcal GN

A

Varies across nephritic spectrum

Sxs 1-2 wks after infection!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Lab findings of post-streptococcal GN

A

Elevated streptococcal antibodies (ASO titers)

Low complement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Tx of post-streptococcal GN

A

Usually see resolution within first 2 wks
Supportive
Children more likely to recover

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is anti-GBM disease?

A

Circulating antibodies directed against glomerular basement membrane

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Where else is target antigen seen in anti-GBM disease?

A

Alveolar basement membrane (acute renal pulmonary glomerulonephritis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Who tends to have anti-GBM disease?

A

First is about 30 (male predominance and lung involvement)

Second about 60 (with female predominance)

33
Q

Difference between anti-GBM disease and goodpasture syndrome

A

Anti-GBM: anti-GBM and GN (just kidney)

Goodpasture: GN and pulmonary hemorrage

34
Q

Presentation of anti-GBM disease

A

Usually idiopathic (HLA predisposition)–pulm infection, tobacco use, hydrocarbon exposure
Renal involvement: nephritic spectrum (RPGN)
Pulm involvement: cough, dyspnea, overt hemoptysis

35
Q

How to diagnose anti-GBM disease

A

See anti-GBM antibodies in serum or kidney biopsy

Test for ANCA

36
Q

Tx of anti-GBM disease

A

Plasmapheresis (remove antibodies from circulation)

Immunosuppressive agents

37
Q

What is lupus nephritis?

A

Immune complex mediated glomerular disease due to anti-ds DNA antibodies
Many patterns of injury (usually nephritis spectrum)
Nonwhites

38
Q

When do you usually suspect lupus nephritis?

A

By abnormal urinalysis and/or elevation of serum creatinine

Confirm with renal biopsy

39
Q

What is IgA vasculitis (HSP) caused by?

A

Immune mediated vasculitis with tissue deposition of IgA containing immune complexes

40
Q

When is HSP usually seen?

A

Mostly in kids (more males)
Unknown cause
Often post-URI

41
Q

Classic tetrad of HSP

A

Rash (palpable purpura), arthralgias, abdominal pain and renal disease

42
Q

Renal disease associated with HSP

A

Adults have increased risk of progressive renal disease

Involvement usually after rash (nephritis or nephrotic)

43
Q

Tx of HSP

A

Usually self limited

Support, symptomatic care and targeted therapy for complications

44
Q

What causes Pauci-immune GN?

A

Systemic ANCA-associated vasculitides:

  • granulomatosis with polyangiitis (GPA)
  • microscopic polyangiitis (MPA)
  • eosinophilic granulomatosis with polyangiitis (EGPA)
45
Q

What is ANCA?

A

Antibodies that produce vascular and tissue damage (P-ANCA and C-ANCA)

46
Q

What is seen on renal biopsy in pauci-immune GN?

A

Absence/paucity of immune deposits

47
Q

How does pauci-immune GN present?

A

RPGN

48
Q

What is granulomatosis with polyangiitis?

A

ANCA-associated vasculitis
Rare, complex multisystem autoimmune disease
-C-ANCA

49
Q

Hallmark feature of GPA

A

Necrotizing granulomatous inflammation (pauci-iimune vasculitis of small-medium vessels)

50
Q

Where does GPA usually occur?

A

Upper and lower respiratory tracts or kidneys

51
Q

Presentation of GPA

A

Upper and lower airway: sinusitis, saddle nose, otitis media, ocular involvement, cough, dyspnea
Renal: GN (crescent necrotizing GN-cap wall collapses in on self)
Fever, malaise, weight loss, arthritis, skin, polyneuropathy

52
Q

What is microscopic polyangiitis?

A

ANCA-associated vasculitis of small-medium vessels

-P-ANCA

53
Q

MPA and GPA

A

Present similarly with GN and lung involvement

but in MPA there is absence of granuloma formation and sparing of upper respiratory tract (no sinusitis etc)

54
Q

What is eosinophilic granulomatosis with polyangiitis?

A

Churg-strauss syndrome
ANCA associated vasculitis (lung, skin, heart, renal, GI, neuro-peripheral neuropathy)
-P-ANCA

55
Q

What is EGPA associated with?

A

Asthma and eosinophilia

56
Q

3 phases of EGPA

A

Prodrome: allergic disease (asthma or allergic rhinitis)
Eosinophilic/tissue infiltrative phase
Vasculitis: necrotizing vasculitis of small-medium vessels

57
Q

Tx for pauci-immune GN

A

Immunosuppressants (corticosteroids and cytotoxic agents)

Without tx, poor prognosis

58
Q

When are simple cysts usually found?

A

Incidentally on ultrasound

59
Q

What are the benign features associated with a simple cyst?

A

Smooth thin walls that are sharply demarcated and do not enhance with contrast

60
Q

Standard of care for benign cyst

A

Periodic reevaluation

61
Q

What is polycystic kidney disease?

A

Inherited disease that causes irreversible decline in kidney function (dominant or recessive)

62
Q

What do pts with PKD usually need?

A

Renal replacement therapy (dialysis or kidney transplant)

63
Q

Most common genetic cause of CKD

A

Autosomal dominant PKD

+FH in most

64
Q

Genes associated with most cases of ADPKD

A

PKD1 or PKD2

65
Q

What characterizes ADPKD?

A

Massive kidney enlargement (bilateral)

Progressive decline in renal function (GFR)–renal function remains intact until 4th decade (usually ESRD by 60)

66
Q

Pathogenesis of ADPKD

A

Cysts accumulate fluid
Enlarge
Compress neighboring renal parenchyma
Progressively compromising renal function

67
Q

What is hyperfiltration of the kidneys?

A

When some nephrons lose function, others will begin to compensate

68
Q

Why is decreased GFR seen in ADPKD?

A

Increasing kidney size and cyst volume and reduction in renal blood flow

69
Q

Manifestations of ADPKD

A

Renal: HTN and abdominal/flank pain (palpable kidneys, hematuria, proteinuria, UTIs and nephrolithiasis)
Extrarenal: hepatic cysts (intracranial aneurysms, CV disease, other cysts)

70
Q

Initial modality to diagnose ADPKD

A

U/s

71
Q

Typical findings on diagnostics of ADPKD

A

Large kidneys and extensive cysts scattered throughout both kidneys

72
Q

Definitive diagnosis of ADPKD

A

Genetic testing

73
Q

Tx of ADPKD

A

Strict BP control, low salt diet, statins
Avoid caffeine
Supportive therapy
Complications (infected renal cyst, cyst hemorrhage, stones)
Meds: Tolvaptan (vasopressin receptor antagonist)
Dialysis or kidney transplant

74
Q

When is autosomal recessive PKD seen more?

A

Infants and kids (due to mutation of PKHD1 gene)

75
Q

2 primary systems affected in ARPKD

A

Kidneys and hepatobiliary tract (so more limited)

76
Q

What characterizes ARPKD?

A

Bilateral markedly enlarged kidneys and congenital hepatic fibrosis
(if neonates have ESRD then maybe respiratory distress)

77
Q

Manifestations of ARPKD

A

Varies
Progressive renal impairment or HTN
Portal HTN
May see feeding difficulties and growth impairment (b/c they compress diaphragm and cause satiety)

78
Q

How to detect ARPKD

A

Routine antenatal u/s after 24 wks gestation

79
Q

Tx of ARPKD

A

Supportive therapy with multidisciplinary team

X Clin Med II Exam 3 (6 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions