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Block 9 Week 5 > Fung > renal path > Flashcards

Fung > renal path Flashcards

1
Q

what are the 3 metabolic fxns of the kidney?

A
  1. excrete ions (water, Na, Ca, P)
  2. maintain acid-base balance
  3. excrete toxic metabolic waste pdts
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2
Q

what are the 2 endocrine fxns of the kidney?

A
  1. secrete EPO & PGs

2. regulate vitamin D metabolism

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3
Q

how does the kidney regulate BP?

A

secretes renin

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4
Q

what are the 3 main renal fxns?

A
  1. metabolism
  2. endocrine
  3. regulate BP
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5
Q

if you have a histo slide w/ glomeruli, what part of the kidney are you in?

A

cortex

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6
Q

what is the endothelium of the renal cortex?

A

fenestrated endothelial cells

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7
Q

where is the endothelium of the renal cortex located?

A

adjacent to the lamina rara interna of the GBM

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8
Q

what is another name for visceral epithelium?

A

podocytes

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9
Q

where are the podocytes?

A

adjacent to the lamina rara externa of the GBM (externa > epi)

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10
Q

what is unique about podocytes?

A

foot processes separated by filtration slits

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11
Q

how many layers does the GBM have?

A

3

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12
Q

what are the layers of the GBM?

A

lamina rara interna
lamina densa
lamina rara externa

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13
Q

the GBM is composed of what 6 things?

A
  1. type IV collagen
  2. laminin
  3. heparan sulfate
  4. fibronectin
  5. entactin
  6. glycoproteins
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14
Q

what does the mesangium do?

A

supports the glomerular tuft

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15
Q

where is the mesangium?

A

btwn capillaries in the cortex

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16
Q

what does the mesangium contain?

A

matrix similar to the basement membrane

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17
Q

what 4 things are special about mesangial cells?

A
  1. contractile
  2. phagocytic
  3. secrete mediators
  4. lay down matrix
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18
Q

what are mesangial cells similar to?

A

vascular smooth muscle cells & pericytes

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19
Q

how can you tell the DCT apart from the PCT?

A

the DCT has a wider lumen

the PCT has fatter cells and is cloudier in the lumen

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20
Q

what structures are in the renal medulla (think histo)?

A

collecting ducts & tubules

NO GLOMERULI

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21
Q

T/F: renal disease is always caused by primary reasons

A

FALSE

tons of diverse causes

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22
Q

what are the 4 basic patterns of rxns in response to glomerular injury?

A
  1. hypercellularity
  2. BM thickening
  3. hyalinosis
  4. sclerosis
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23
Q

define diffuse

A

involving ALL glomeruli

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24
Q

define focal

A

involving SOME glomeruli

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25
define global
involving a WHOLE glomerulus
26
define segmental
involving PART of a glomerulus
27
what is most glomerular injury d/t (generally)?
immunologic causes
28
what are the 3 ways immunologic causes can injury a glomerulus?
1. ab rxn in situ w/i glomerulus 2. circulating ab deposits w/i glomerulus 3. cytotoxic ab directed against the glomerulus
29
when cytotoxic ab are directed against the glomerulus, what pathway is activated?
complement
30
when circulating ab gets deposited in the glomerulus, what types of ag are involved (generally)?
endogenous AND exogenous
31
when ab reacts in situ w/i the glomerulus, what types of ag are involved?
intrinsic glomerular ag AND ag planted w/i the glomerulus
32
what is the biggest difference btwn CIRCULATING ab & IN SITU ab deposits?
the circulating deposits are actually immune COMPLEXES w/ ab & ag (in situ means that the ag is already IN the glomerulus, so the ab just comes in and reacts w/ that)
33
what clinical presentations can glomerular injury lead to?
``` nephrotic syndrome nephritic syndrome rapidly progressive glomerulonephritis chronic renal failure asymptomatic hematuria ```
34
define azotemia
BUN or Cr elevation, either prerenal, intrinsic, or postrenal
35
what is acute renal failure?
rapid decline in GFR
36
how long is "rapid" in acute renal failure?
hours to days
37
what are the 3 characteristics of acute renal failure?
1. azotemia 2. fluid & electrolyte imbalance 3. oliguria or anuria
38
what types of fluid & electrolyte imbalances can be found w/ acute renal failure?
``` hyponatremia hyperkalemia hyperphosphatemia hypocalcemia metabolic acidosis ```
39
what are the 4 causes of acute renal failure?
1. glomerular injury 2. interstitial injury 3. vascular injury 4. tubular injury
40
what are the 4 sx of acute renal failure?
1. decreased/absent urine output 2. lethargy 3. fatigue 4. nausea
41
what 5 categories can you use to distinguish btwn prerenal and renal acute renal failure?
1. FENa+ 2. BUN/Cr 3. Urine Na+ 4. Urine osmolality 5. Specific gravity
42
how does FENa+ differ btwn prerenal and renal acute renal failure?
prerenal: 1% renal: >1%
43
how does BUN/Cr differ btwn prerenal and renal acute renal failure?
prerenal: >20:1 renal: <20:1
44
how does Urine Na+ differ btwn prerenal and renal acute renal failure?
prerenal: 20mEq/L
45
how does urine osmolality differ btwn prerenal and renal acute renal failure?
prerenal: >500 mOsm/kg renal: <400 mOsm/kg
46
how does specific gravity differ btwn prerenal and renal acute renal failure?
prerenal: >1.020 renal: <1.020
47
what is chronic renal failure?
diminished GFR for at least 3 months
48
what constitutes "diminshed" GFR in chronic renal failure?
<60 mL/minute/1.73m^2
49
what are the 3 characteristics of chronic renal failure?
1. azotemia 2. fluid & electrolyte imbalance 3. uremia (1 & 2 are the same as acute)
50
what is uremia?
a syndrome assoc w/ fluid, electrolyte, & hormonal imbalances + metabolic abnormalities
51
what are the sx of uremia?
``` N/V fatigue anorexia weight loss muscle cramps pruritis mental status change visual disturbances inc thirst ```
52
what are the 3 main causes of chronic renal failure?
1. diabetes 2. HTN 3. renal parenchymal disease
53
how bad does GFR get w/ chronic renal failure?
<20-25% of normal
54
what is the difference btwn chronic renal failure & end stage renal disease in terms of GFR?
chronic renal failure is <5% of normal
55
what are the 4 sx of nephritic syndrome?
1. hematuria (RBC casts) 2. azotemia 3. oliguria 4. slight proteinuria
56
what characterizes nephritic syndrome?
INFLAMMATION of glomeruli | the i in nephritic means inflammation
57
what are the sx of acute proliferative glomerulonephritis?
1. URI or skin infection 1-6 wks prior 2. gross hematuria 3. oliguria 4. RBC casts 5. peripheral edema 6. HTN 7. azotemia 8. mild proteinuria (2, 3, 4, & 8 = nephritic syndrome)
58
what causes acute proliferative glomerulonephritis?
beta-hemolytic group A strep | Lancefield M types 1, 2, 4, 12, 47, 49, 57
59
what is another name for acute proliferative glomerulonephritis?
poststreptococcal glomerulonephritis (PSGN) (pathoma)
60
what is the pathogenesis of acute proliferative glomerulonephritis?
circulating immune complexes are deposited in the glomerulus (subepithelial HUMPS)
61
what findings will you see for acute proliferative glomerulonephritis on IF & EM?
IF: granular d/t immune complexes EM: subepithelial humps d/t immune complexes
62
what does acute proliferative glomerulonephritis look like on H&E?
hypercellular | inflamed glomeruli
63
name the nephritides according to pathoma
1. post-strep glomerulonephritis (acute proliferative) 2. rapidly progressive glomerulonephritis 3. IgA nephropathy (Berger) 4. Alport syndrome
64
what is the difference btwn the clinical course of acute proliferative glomerulonephritis in adults vs kids?
KIDS: 95% recover totally ADULTS: only 60% recover
65
what is the therapy for acute proliferative glomerulonephritis in kids?
maintain Na & water balance (i.e. supportive therapy bc most kids recover)
66
what can acute proliferative glomerulonephritis progress to?
rapidly progressive glomerulonephritis
67
what characterizes rapidly progressive glomerulonephritis?
1. rapid & progressive loss of renal fxn 2. oliguria 3. nephritic sx
68
what can you see on H&E & IF w/ rapidly progressive glomerulonephritis?
crescents in Bowman's space made of FIBRIN & MACS
69
what are the 3 types of rapidly progressive glomerulonephritis?
1. Type I > anti-GBM ab 2. Type II > immune complex 3. Type III > pauci-immune
70
what characterizes type I rapidly progressive glomerulonephritis?
LINEAR deposits of IgG/C3
71
what is the treatment for type I rapidly progressive glomerulonephritis?
plasmapharesis | immunosuppression
72
what are the causes of type II rapidly progressive glomerulonephritis?
1. complication of post-infectious glomerulonephritis 2. lupus nephritis complication 3. IgA nephropathy 4. idiopathic
73
what is the treatment for type II rapidly progressive glomerulonephritis?
treat the UNDERLYING DISEASE! | DO NOT DO PLASMAPHARESIS
74
what is the diff btwn type I & type II rapidly progressive glomerulonephritis on IF?
type I is LINEAR | type II is GRANULAR
75
what kind of ab do you have in Type III rapidly progressive glomerulonephritis?
circulating ANCA! (pauci-ANCA = paul anka)
76
what ANCA is assoc w/ Wegener?
PR3-ANCA | c-ANCA
77
what ANCA is assoc w/ microscopic polyangiitis?
MPO-ANCA | p-ANCA
78
what ANCA is assoc w/ Churg-Strauss?
p-ANCA
79
what causes most cases of type III rapidly progressive glomerulonephritis?
most cases are idiopathic
80
what is the treatment for type III rapidly progressive glomerulonephritis?
steroids | cytotoxic agents
81
what are the 4 things you need to remember about nephrOtic syndrome?
1. massive proteinuria 2. hypoalbuminemia (& hypogammaglobulinemia) 3. edema 4. hyperlipidemia / hypercholesterolemia (Pathoma says also loss of ATIII)
82
T/F: proteinuria in nephrotic syndromes can be selective
TRUE
83
what are the 2 MAIN/broad causes of nephrotic syndromes?
primary glomerular disease AND systemic disease
84
what are the 5 primary glomerular diseases that can be nephrotic?
1. MCD 2. FSGS 3. MN 4. MPGN 5. DM 6. SA
85
what is the most frequent cause of nephrotic syndrome in kids?
MCD
86
what does MCD look like on H&E?
normal glomeruli
87
what does MCD look like on IF?
negative/normal (no immune complexes)
88
what does MCD look like on EM?
effacement of foot processes of podocytes
89
what do MCD & FSGS have in common?
no immune complex deposition so negative IF | effacement of foot processes on EM
90
what is the biggest diff btwn MCD & FSGS?
FSGS has focal segmental glomerular sclerosis on H&E (hence the name)
91
what is the pathophysiology of MCD?
immune dysfxn > cytokine > damage to visceral epithelial cells > proteinuria
92
what is MCD assoc w/?
respiratory illness post-immunization atopic disorders
93
what nephrotic syndrome can you give steroids to treat?
MCD only!
94
what cancer is MCD assoc w/?
Hodgkin lymphoma
95
T/F: MCD has highly selective proteinuria
TRUE | selective for albumin
96
T/F: MCD is assoc w/ HTN & hematuria
FALSE MCD is nephrOtic HTN & hematuria are nephritic sx
97
what age group is affected by membranous nephropathy?
adults
98
which 2 nephrotic syndromes are immune complex mediated?
membranous nephropathy AND membranoproliferative glomerulonephritis
99
T/F: there is only primary membranous nephropathy
FALSE | primary & secondary
100
what characterizes membranous nephropathy?
diffuse thickening of the glomerular capillary wall | thickened glomerular BM
101
does membranous nephropathy have Ig deposits?
YES | subepithelial > spike & dome
102
what does membranous nephropathy look like on H&E?
thickened glomerular BM
103
what does membranous nephropathy look like on IF?
granular d/t immune complexes
104
what does membranous nephropathy look like on EM?
spike & dome d/t subepithelial deposits
105
what are the 3 clinical features of membranous nephropathy?
1. insidious onset 2. mild HTN & hematuria possible 3. progression leads to glomerular sclerosis
106
what is the treatment for membranous nephropathy?
treat underlying condition | does NOT respond to steroids
107
what is FSGS characterized by?
focal & segmental sclerosis of the capillary tuft
108
what are the 4 possible causes of FSGS?
1. idiopathic 2. secondary 3. complication of focal glomerulonephritis 4. adaptive response to loss of renal tissue
109
what is FSGS thought to be a part of clinically & why?
thought to be a phase in MCD accentuated diffuse epithelial cell change of MCD cytokine-mediated or defects in slit diaphragm proteins
110
T/F: you get hyalinosis & sclerosis w/ FSGS
TRUE | d/t entrapment of plasma proteins
111
what does FSGS look like on H&E?
focal segmental sclerosis
112
what does FSGS look like on EM?
effacement of foot processes
113
what does FSGS look like on IF?
negative/normal (bc no immune complexes)
114
T/F: idiopathic FSGS spontaneously remits
FALSE | "little tendency"
115
does FSGS respond to steroids?
variable (poor)
116
what are the sx of MPGN (generally)?
1. nephrotic sx 2. nephritic sx 3. nephritic-nephrotic sx 4. asymptomatic hematuria
117
what characterizes MPGN?
1. altered GBM 2. proliferation of glomerular cells/mesangium 3. leukocyte proliferation
118
T/F: there is only one type of MPGN
FALSE | there are 2!
119
what is type I MPGN?
immune complex deposition + activation of classical & alt complement pathways
120
what can cause type I MPGN?
planted exogenous ag (HBV & HCV) | circulating immune complexes
121
where are the deposits in type I MPGN?
subendothelial
122
where are the deposits in type II MPGN?
intramembranous
123
is type I or type II MPGN more common?
type I
124
what is type II MPGN?
dysregulation of alt complement pathway
125
what is the pathophysiology of type II MPGN?
auto-ab (C3 nephritic factor) that stabilizes C3 convertase > overactivates complement > low circulating C3 + inflammation
126
what does MPGN look like on H&E?
thickened glomerular BM w/ tram-track appearance
127
T/F: most cases of MPGN spontaneously remit
FALSE | few spontaneous remissions w/ a slowly progressive & unremitting course
128
T/F: steroids & immunosuppressives are the RX for MPGN
FALSE
129
what is the most common type of glomerulonephritis worldwide?
IgA nephropathy
130
how do IgA nephropathy pts present?
w/ recurrent episodic gross or microscopic hematuria (episodes coincide w/ mucosal [resp or GI] infections)
131
what is the systemic form of IgA nephropathy?
Henoch-Schonlein purpura
132
where does the IgA immune complex deposit in IgA nephropathy?
mesangium
133
what form of IgA increases in IgA nephropathy?
polymeric forms of IgA1
134
what causes IgA nephropathy?
genetic or acquired abnormality of immune regulation upon exposure to env agents in lungs & GI tract
135
how does IgA nephropathy cause glomerular injury?
IgA & immune complexes get trapped in the mesangium > activate complement > injury
136
what does IgA nephropathy look like on IF?
granular d/t IgA immune complex deposition
137
T/F: IgA nephropathy pts may retain renal fxn for decades
TRUE!
138
what can diabetic nephropathy cause?
kidney failure (one of the leading causes)
139
what 3 glomerular syndromes is diabetic nephropathy assoc w/?
1. non-nephrotic proteinuria 2. nephrotic syndrome 3. chronic renal failure
140
what are the 3 manifestations of diabetic nephropathy?
1. hyalinizing arteriolar sclerosis 2. pyelonephritis 3. tubular lesions
141
what does diabetic nephropathy look like on H&E?
Kimmelstiel-Wilson nodules (sclerosis) = pathognomonic
142
what is Alport syndrome?
X-linked inherited defect in type IV collagen
143
what 3 things characterize Alport syndrome?
1. hematuria progressing to renal failure 2. sensory hearing loss 3. ocular disorders
144
what is familial renal disease (Alport) d/t?
defects in type IV collagen
145
how does familial renal disease (Alport) clinically manifest?
familial asymptomatic hematuria
146
what is the prognosis for familial renal disease (Alport)?
normal renal fxn | excellent prognosis
147
what is the kidney lesion in familial renal disease (Alport)?
thinned & split glomerular BM

Block 9 Week 5 (3 decks)

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