HY - inflammation + renal Flashcards
(127 cards)
1
Q
catalase + organisms in CGD
A
pseudamonas cepacia, s marcescens, nocardia, aspergillus, s aureus
2
Q
first step of oxidative burst
A
O2 to superoxide by NADPH reductase; if intact then + nitro blue tetrazolium test
3
Q
second step of oxidative burst
A
superoxide to hydrogen peroxide by SOD
4
Q
third step of oxidative burst
A
hydrogen peroxide to HOCL by myeloperoxidase; normal nitro blue tetrazolium test; + risk of candida infections
5
Q
opsonins
A
IgG and C3b
6
Q
failure of phagolysosome fusion
A
chediak higashi; big granules, albinism, prone to infections
7
Q
IL8
A
macrophages calling in neutrophils for continued acute inflammation
8
Q
2nd activation signal of CD4+ T cell
A
B7 on APC binds CD28 on CD4
9
Q
Two cell types that CD4 helps
A
B and CD8
10
Q
Th1 cytokines - helps CD8
A
IL2 (2nd signal of T cell activation) and IFN y
11
Q
Th2 cytokines - helps B cells
A
IL4, IL5, and IL10
12
Q
modes of cell killing
A
cytochrome c, FasL, CD8 dumping of granzyme
13
Q
2nd activation signal of B cells
A
CD40 R on B cells bind CD40L on helper T cell
14
Q
IL4 and IL5 released by Helper T cells
A
mediate B cell isotype switch, hypermutatino and maturation to plasma cells
15
Q
epithelioid histiocytes
A
Th1 secrete IFNy induce; aggregation (macrophages with abundant pink cytoplasm) = granuloma
16
Q
non-caseating granuloma
A
sarcoidosis, beryllium exposure, crohn disease, cat scratch disease, freaction to foreign material (breast implant etc)
17
Q
crypt abscesses + non-caseating granuloma
A
crohn disease
18
Q
stellate shaped granuloma
A
cat scratch disease
19
Q
caseating granuloma
A
TB (AFB stain) and funcus (silver/GMS stain)
20
Q
DiGeorge (22q11)
A
T cell deficiency (no thymus), hypocalcemia (no parathyroid), abnormalities of heart, great vessels, and face
21
Q
SCID
A
adenosine deaminase deficiency (accumulation of toxic material kills lymphocytes) = NO cell mediated and humoral immune cells making susceptible to ALL kinds of infections
22
Q
X-linked agammaglobinemia
A
mutation Bruton Tyrosine Kinase; X-linked; surfaces at 6 mo when mom’s Ab leave system; no maturation of B cells; recurrent bacterial, enterovirus, giarida; AVOID live vaccines
23
Q
Elevated IgM
A
mutated CD40 or CD40L = cytokines necessary for Ig class switching will not be produced
24
Q
Wiskott-Aldrich
A
thrombocytopenia, eczema, recurrent of infections, mutations in x-linked WASP mutation
25
C5-9 deficiency
increased risk of neisseria
26
C1 inhibitor deficiency
hereditary angioedema with peiorbital edema + mucosal surfaces
27
negative selection
removal of self-reactive lymphocytes
28
AIRE mutation
necessary for expressino of self-antigens in thymus for negative slection; if mutated = autoimmune polyendocrine syndrome of HYPOPARATHYROIDISM, ADRENAL INSUFFICIENCY and CANDIDA INFECTIONS
29
peripheral tolerance
anergy and expression of FASL
30
HLA B27
Ankylosing Spondylitis
31
loss of self tolerance + environmental trigger
auto-immne disease
32
SLE
Type 3 hypersensitivity + decreased CH50, C4 and C4 because complement proteins are being used against tissues + antibodies against host nuclear material
33
SLE sx
fever, weight loss, fatigue, raynauds, discoid rash, malar rash worse with sun, oral/nasopharyngeal ulcers, arthritis, serositis
34
SLE clinical sx
renal damage (diffuse proliferative glomerulonephritis, membranous glomerulonephritis), Ab against cells (anemia, thrombocytpoenia, leukopenia due to T2 hyper-sensitivity), libman-sacks endocarditis (vegetations on both sides of valve)
35
Anti-nuclear (sensitive) + Anti-dsDNA + Anti-Sm ANTIBODIES (specific)
SLE
36
Anti-phospholipid Ab
SLE; false positive for syphilis, paradoxical elevated PTT; risk of spontaneous abortions and thrombosis requiring life-long anti-coagulation
37
Anti-histone Ab
Drug induced lupus: Hydralazine, Procainamide, Isoniazide
38
SLE COD
renal failure, infection, accelerated coronary atherosclerosis
39
dry mouth, dry eyes, recurrent dental caries, type 4 hypersensitivity
Sjogrens Syndrome, often with RA (presence of RF)
40
Anti-SSA + Anti-SSB
anti-ribonucleoproteins in Sjogrens; can cross placental resulting in neonatal lupus with congenital heart block
41
Screen pregnant SLE patient for what AB?
Anti-SSA and Anti-SSB
42
Salivary gland biopsy DDx
Sjogrens, Amyloidosis, Sarcoidosis
43
Salivary gland biopsy Sjogrens
lymphocyte invasion
44
Lymphoma risk in Sjogrens
B cell lymphoma; unilateral partodi gland swelling
45
Anti-Centromere antibodies
Systemic Sclerosis
46
CREST
Systemic Sclerosis: Calcinosis, anti-Centromere, Raynaud, Esophageal dysmotility with GERD, Sclerodactyly (loss of wrinkles, thick and hard), Telangiectasia
47
anti-DNA topoisomerase antibodies
diffuse type scleroderma
48
mixed connective tissue disease
SLE + systemic sclerosis + polymyositis overlapping symptoms + ANA + antibodies-U1 ribonucleoprotein
49
anti-U1 ribonucleoprotin
mixed connective tissue disease
50
scleroderma COD
pulmonary fibrosis
51
labile tissues - constantly cycling and able to regenerate
bowel, skin, bone marrow
52
bowl stem cells
mucosal crypts
53
skin stem cells
basal layer
54
CD34+
marker for hematopoietic stem cells
55
stable tissues - quiescent but able to reenter
liver, proximal renal tubular cells following ATN
56
permanent tissue - lack regenerative potential
myocardium, skeletal muscle, neurons; repair with fibrous scar
57
fibroblasts + capillaries + myofibroblasts
granulation tissue
58
what type of collagen do fibroblasts deposit during repair?
type 3 (very pliable)
59
Which type of collagen replaces type 3 laid down by fibroblasts?
Type 1 - seen in bone, tendon STRONG structures
60
FGF + VEGF
angiogenesis
61
healing by primary intention
wound edges brought together with minimal scar formation
62
healing by secondary intention
edges NOT brought together = granulation tissue fills in defect
63
vitamin C for wound healing
hydroxylation of pro-collagen
64
main AA in collagen
Glycine
65
copper for wound healing
unable to crosslink collagen
66
zinc for wound healing
co-factor for removal of type 3 collagen so can replace with type 1
67
dehiscence
rupture/re-opening of wound
68
keloid
excess type III collagen + ear lobes + african american + WAYYY out of proprotion to insult, ie ear piercing
69
which type of collagen seen in keloids?
type III
70
bilateral enlarged kidneys with cysts in renal cortex and medullar
PKD, inherited
71
causes of Pottery Sequence
ARPKD (kidneys essentially non-functinoal) and renal agenesis
72
baby with signs of portal hypertension
ARPKD
73
ADPKD COD
berry aneurysm
74
cysts in medullary collecting ducts + shrunken kidneys
medullary chronic kidney disease
75
azotemia + oliguria
cute renal failure, either pre-renal (decrease blood into) intra-renal and post-renal (obstrcut flow from leaving)
76
Bun:Crea in pre-renal ARF
INCREASED; Bun:Crea > 15 due to low kidney blood flow triggering of RAAS increasing RBF and thus reabsorption of Bun
77
long-standing post-renal damage
TUBULAR DAMAGE with 1) DECREASE BUN:Crea (damage to cells so no reabosrption can happen) 2) DECREASE FENa 3) INABIliTY to concentrate urine
78
brown, granular casts
ATN
79
tubular epithelial cells die and plug tubules causing decrease GFR
ATN, will see brown, granular casts in urine (cells that died in tubules)
80
causes of intra-renal ARF
ATN and Acute Interstitial Nephritis
81
causes of ATN
aminoglycosides, crush injury, ehtylene glycol, radiocontrast dye, tumor lysis syndrome
82
causes of Acute Interstitial Nephritis
NSAIDs, penicillin, diuretics - hyper-sensitivity reaction + inflammatory infiltrate + eosinophils
83
risk of Acute Interstitial Nephritis
renal papillary necrosis with gross hematuria and flank pain
84
general findings of nephrotic syndrome
hypoalbuminemia + hypogammaglobulinemia + hypercoagulable state + hyperlipidemia + hypercholesterolemia
85
Nephrotic syndrome associated with Hodgkin Lymphoma (cytokines knocking out podocytes)
MCD
86
use of IF
identify immune complex deposition
87
which nephrotic syndrome responds super well to steroids because largely mediated by T cells?
MCD
88
nephrotic syndrome in someone with HIV, heroin use, SCD
FSGS
89
two nephrotic syndromes that cause podocyte effacement
MCD + FSGS
90
immune complex deposition on FSGS or MCD?
NO
91
destiny of FSGS patients?
chronic renal failure
92
nephrotic syndrome in someone with HBV, HCV, SLE or using NSAIDS or penicillamine
Membranous nephropathy
93
SLE + nephrotic syndrome
membranous glomerulopathy
94
thick GBM + immune complex deposition (granular IF; spoke + dome EM)
membranous glomerulopathy
95
membrano in the name = ?
thickening of membranes
96
what is shared by membranous glomerulopathy and membranoproliferative glomerulonephritis?
ICD and membrane thickening
97
tram-track appearance + granular IF (ICD) + thick membranes
membranoproliferative glomerulonephritis
98
Type I MPGN
HBV/HCV + tram-track
99
Type 2 MPGN
C3 nephritic factor stabilizes C3 so over-activation of complement
100
sub-epithelium ICD
membranous glomerulopathy
101
ICD nephrotic syndromes
membranous glomverolapth + Type 1 and Type 2 membranoproliferative glomerulonephritis
102
non-enzymatic glycosylation + hyaline arteriolosclerosis, preferentially efferent with microalbuminuria
DM
103
Kimmelstein-Wilson nodules seen in which neprhotic syndrome?
DM
104
Most common organ affected in systemic amyloidosis
Kidney + apple-green bi-refringence seen in polarized light
105
oliguria + azotemia + hyper-natremia + peri-orbital edema + HTN + RBC casts and dysmorphic RBCs in urine
nephritic syndrome
106
hypercellular, inflamed glomeruli in nephritic or nephrotic syndrome?
nephritic
107
What increases risk of PSGN?
M protein virulence factors
108
ICD (granular IF) + subepithelial humps on EM
PSGN
109
RPGN following PSGN?
If adults, because normally self-limited in children as immune complex move out
110
RPGN
fibrin and inflammatory cell crescnets
111
RPGN + linear immunofluroescene
Goodpasture syndrome (respiratory symptoms as well)
112
RPGN + granular immunofluroescne
PSGN (will progress in adults) or diffuse proliferative glomerulonephritis
113
most common renal disease in SLE
diffuse proliferative glomerulonephritis due to diffuse antigen-antibody complex deposition
114
RPGN + normal IF, what next?
ANCA to determine if Wegeners (c-ANCA) or Churgg Strauss or microscopic polyangiitis (p-ANCA)
115
lung (hemoptysis) + kidney (hematuria) + nose (sinusitis) + c-ANCA
Wegener's
116
c-ANCA
Wegener's
117
p-ANCA
Churgg Strauss or microscopic polyangiitis
118
hematuria with mucosal infections
Bergers, IgA nephropathy
119
isolated hematuria + vision problems + hearing loss
X-linked Alport Syndrome
120
Tuberous Sclerosis associated tumor
Angiomyolipoma
121
kidney sx + fever + weight loss + paraneoplastic syndrome (EPO, renin, PTHrP, ACTH)
renal cell carcinoma
122
kidney disease + left sided varicocele
blocking renal vein so L gonadal vein cannot drain into renal vein
123
kidney disease due to loss of VHL
renal cell carcinoma
124
risk factor for sporadic development of RCC
smoking
125
chromosome 11 + kidney mass in child + primitive kidney tissue
Wilms tumor
126
Wilms tumor + neonatal hypoglycemia + muscular hemihypertrophy + organomegaly
Becwith-Widedmann Syndrome
127
recurrent skin infections without pus + delayed detachment of the umbilical cord
LAD type 1 = absence of CD18 so no integrin formation and no tight adhesion of leukocytes necssary for transmigration
