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neuro > HY - inflammation + renal > Flashcards

HY - inflammation + renal Flashcards

1
Q

catalase + organisms in CGD

A

pseudamonas cepacia, s marcescens, nocardia, aspergillus, s aureus

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2
Q

first step of oxidative burst

A

O2 to superoxide by NADPH reductase; if intact then + nitro blue tetrazolium test

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3
Q

second step of oxidative burst

A

superoxide to hydrogen peroxide by SOD

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4
Q

third step of oxidative burst

A

hydrogen peroxide to HOCL by myeloperoxidase; normal nitro blue tetrazolium test; + risk of candida infections

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5
Q

opsonins

A

IgG and C3b

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6
Q

failure of phagolysosome fusion

A

chediak higashi; big granules, albinism, prone to infections

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7
Q

IL8

A

macrophages calling in neutrophils for continued acute inflammation

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8
Q

2nd activation signal of CD4+ T cell

A

B7 on APC binds CD28 on CD4

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9
Q

Two cell types that CD4 helps

A

B and CD8

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10
Q

Th1 cytokines - helps CD8

A

IL2 (2nd signal of T cell activation) and IFN y

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11
Q

Th2 cytokines - helps B cells

A

IL4, IL5, and IL10

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12
Q

modes of cell killing

A

cytochrome c, FasL, CD8 dumping of granzyme

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13
Q

2nd activation signal of B cells

A

CD40 R on B cells bind CD40L on helper T cell

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14
Q

IL4 and IL5 released by Helper T cells

A

mediate B cell isotype switch, hypermutatino and maturation to plasma cells

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15
Q

epithelioid histiocytes

A

Th1 secrete IFNy induce; aggregation (macrophages with abundant pink cytoplasm) = granuloma

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16
Q

non-caseating granuloma

A

sarcoidosis, beryllium exposure, crohn disease, cat scratch disease, freaction to foreign material (breast implant etc)

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17
Q

crypt abscesses + non-caseating granuloma

A

crohn disease

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18
Q

stellate shaped granuloma

A

cat scratch disease

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19
Q

caseating granuloma

A

TB (AFB stain) and funcus (silver/GMS stain)

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20
Q

DiGeorge (22q11)

A

T cell deficiency (no thymus), hypocalcemia (no parathyroid), abnormalities of heart, great vessels, and face

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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

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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

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23
Q

Elevated IgM

A

mutated CD40 or CD40L = cytokines necessary for Ig class switching will not be produced

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24
Q

Wiskott-Aldrich

A

thrombocytopenia, eczema, recurrent of infections, mutations in x-linked WASP mutation

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25
Q

C5-9 deficiency

A

increased risk of neisseria

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26
Q

C1 inhibitor deficiency

A

hereditary angioedema with peiorbital edema + mucosal surfaces

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27
Q

negative selection

A

removal of self-reactive lymphocytes

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28
Q

AIRE mutation

A

necessary for expressino of self-antigens in thymus for negative slection; if mutated = autoimmune polyendocrine syndrome of HYPOPARATHYROIDISM, ADRENAL INSUFFICIENCY and CANDIDA INFECTIONS

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29
Q

peripheral tolerance

A

anergy and expression of FASL

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30
Q

HLA B27

A

Ankylosing Spondylitis

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31
Q

loss of self tolerance + environmental trigger

A

auto-immne disease

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32
Q

SLE

A

Type 3 hypersensitivity + decreased CH50, C4 and C4 because complement proteins are being used against tissues + antibodies against host nuclear material

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33
Q

SLE sx

A

fever, weight loss, fatigue, raynauds, discoid rash, malar rash worse with sun, oral/nasopharyngeal ulcers, arthritis, serositis

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34
Q

SLE clinical sx

A

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)

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35
Q

Anti-nuclear (sensitive) + Anti-dsDNA + Anti-Sm ANTIBODIES (specific)

A

SLE

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36
Q

Anti-phospholipid Ab

A

SLE; false positive for syphilis, paradoxical elevated PTT; risk of spontaneous abortions and thrombosis requiring life-long anti-coagulation

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37
Q

Anti-histone Ab

A

Drug induced lupus: Hydralazine, Procainamide, Isoniazide

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38
Q

SLE COD

A

renal failure, infection, accelerated coronary atherosclerosis

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39
Q

dry mouth, dry eyes, recurrent dental caries, type 4 hypersensitivity

A

Sjogrens Syndrome, often with RA (presence of RF)

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40
Q

Anti-SSA + Anti-SSB

A

anti-ribonucleoproteins in Sjogrens; can cross placental resulting in neonatal lupus with congenital heart block

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41
Q

Screen pregnant SLE patient for what AB?

A

Anti-SSA and Anti-SSB

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42
Q

Salivary gland biopsy DDx

A

Sjogrens, Amyloidosis, Sarcoidosis

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43
Q

Salivary gland biopsy Sjogrens

A

lymphocyte invasion

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44
Q

Lymphoma risk in Sjogrens

A

B cell lymphoma; unilateral partodi gland swelling

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45
Q

Anti-Centromere antibodies

A

Systemic Sclerosis

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46
Q

CREST

A

Systemic Sclerosis: Calcinosis, anti-Centromere, Raynaud, Esophageal dysmotility with GERD, Sclerodactyly (loss of wrinkles, thick and hard), Telangiectasia

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47
Q

anti-DNA topoisomerase antibodies

A

diffuse type scleroderma

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48
Q

mixed connective tissue disease

A

SLE + systemic sclerosis + polymyositis overlapping symptoms + ANA + antibodies-U1 ribonucleoprotein

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49
Q

anti-U1 ribonucleoprotin

A

mixed connective tissue disease

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50
Q

scleroderma COD

A

pulmonary fibrosis

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51
Q

labile tissues - constantly cycling and able to regenerate

A

bowel, skin, bone marrow

52
Q

bowl stem cells

A

mucosal crypts

53
Q

skin stem cells

A

basal layer

54
Q

CD34+

A

marker for hematopoietic stem cells

55
Q

stable tissues - quiescent but able to reenter

A

liver, proximal renal tubular cells following ATN

56
Q

permanent tissue - lack regenerative potential

A

myocardium, skeletal muscle, neurons; repair with fibrous scar

57
Q

fibroblasts + capillaries + myofibroblasts

A

granulation tissue

58
Q

what type of collagen do fibroblasts deposit during repair?

A

type 3 (very pliable)

59
Q

Which type of collagen replaces type 3 laid down by fibroblasts?

A

Type 1 - seen in bone, tendon STRONG structures

60
Q

FGF + VEGF

A

angiogenesis

61
Q

healing by primary intention

A

wound edges brought together with minimal scar formation

62
Q

healing by secondary intention

A

edges NOT brought together = granulation tissue fills in defect

63
Q

vitamin C for wound healing

A

hydroxylation of pro-collagen

64
Q

main AA in collagen

A

Glycine

65
Q

copper for wound healing

A

unable to crosslink collagen

66
Q

zinc for wound healing

A

co-factor for removal of type 3 collagen so can replace with type 1

67
Q

dehiscence

A

rupture/re-opening of wound

68
Q

keloid

A

excess type III collagen + ear lobes + african american + WAYYY out of proprotion to insult, ie ear piercing

69
Q

which type of collagen seen in keloids?

A

type III

70
Q

bilateral enlarged kidneys with cysts in renal cortex and medullar

A

PKD, inherited

71
Q

causes of Pottery Sequence

A

ARPKD (kidneys essentially non-functinoal) and renal agenesis

72
Q

baby with signs of portal hypertension

A

ARPKD

73
Q

ADPKD COD

A

berry aneurysm

74
Q

cysts in medullary collecting ducts + shrunken kidneys

A

medullary chronic kidney disease

75
Q

azotemia + oliguria

A

cute renal failure, either pre-renal (decrease blood into) intra-renal and post-renal (obstrcut flow from leaving)

76
Q

Bun:Crea in pre-renal ARF

A

INCREASED; Bun:Crea > 15 due to low kidney blood flow triggering of RAAS increasing RBF and thus reabsorption of Bun

77
Q

long-standing post-renal damage

A

TUBULAR DAMAGE with 1) DECREASE BUN:Crea (damage to cells so no reabosrption can happen) 2) DECREASE FENa 3) INABIliTY to concentrate urine

78
Q

brown, granular casts

A

ATN

79
Q

tubular epithelial cells die and plug tubules causing decrease GFR

A

ATN, will see brown, granular casts in urine (cells that died in tubules)

80
Q

causes of intra-renal ARF

A

ATN and Acute Interstitial Nephritis

81
Q

causes of ATN

A

aminoglycosides, crush injury, ehtylene glycol, radiocontrast dye, tumor lysis syndrome

82
Q

causes of Acute Interstitial Nephritis

A

NSAIDs, penicillin, diuretics - hyper-sensitivity reaction + inflammatory infiltrate + eosinophils

83
Q

risk of Acute Interstitial Nephritis

A

renal papillary necrosis with gross hematuria and flank pain

84
Q

general findings of nephrotic syndrome

A

hypoalbuminemia + hypogammaglobulinemia + hypercoagulable state + hyperlipidemia + hypercholesterolemia

85
Q

Nephrotic syndrome associated with Hodgkin Lymphoma (cytokines knocking out podocytes)

A

MCD

86
Q

use of IF

A

identify immune complex deposition

87
Q

which nephrotic syndrome responds super well to steroids because largely mediated by T cells?

A

MCD

88
Q

nephrotic syndrome in someone with HIV, heroin use, SCD

A

FSGS

89
Q

two nephrotic syndromes that cause podocyte effacement

A

MCD + FSGS

90
Q

immune complex deposition on FSGS or MCD?

A

NO

91
Q

destiny of FSGS patients?

A

chronic renal failure

92
Q

nephrotic syndrome in someone with HBV, HCV, SLE or using NSAIDS or penicillamine

A

Membranous nephropathy

93
Q

SLE + nephrotic syndrome

A

membranous glomerulopathy

94
Q

thick GBM + immune complex deposition (granular IF; spoke + dome EM)

A

membranous glomerulopathy

95
Q

membrano in the name = ?

A

thickening of membranes

96
Q

what is shared by membranous glomerulopathy and membranoproliferative glomerulonephritis?

A

ICD and membrane thickening

97
Q

tram-track appearance + granular IF (ICD) + thick membranes

A

membranoproliferative glomerulonephritis

98
Q

Type I MPGN

A

HBV/HCV + tram-track

99
Q

Type 2 MPGN

A

C3 nephritic factor stabilizes C3 so over-activation of complement

100
Q

sub-epithelium ICD

A

membranous glomerulopathy

101
Q

ICD nephrotic syndromes

A

membranous glomverolapth + Type 1 and Type 2 membranoproliferative glomerulonephritis

102
Q

non-enzymatic glycosylation + hyaline arteriolosclerosis, preferentially efferent with microalbuminuria

A

DM

103
Q

Kimmelstein-Wilson nodules seen in which neprhotic syndrome?

A

DM

104
Q

Most common organ affected in systemic amyloidosis

A

Kidney + apple-green bi-refringence seen in polarized light

105
Q

oliguria + azotemia + hyper-natremia + peri-orbital edema + HTN + RBC casts and dysmorphic RBCs in urine

A

nephritic syndrome

106
Q

hypercellular, inflamed glomeruli in nephritic or nephrotic syndrome?

A

nephritic

107
Q

What increases risk of PSGN?

A

M protein virulence factors

108
Q

ICD (granular IF) + subepithelial humps on EM

A

PSGN

109
Q

RPGN following PSGN?

A

If adults, because normally self-limited in children as immune complex move out

110
Q

RPGN

A

fibrin and inflammatory cell crescnets

111
Q

RPGN + linear immunofluroescene

A

Goodpasture syndrome (respiratory symptoms as well)

112
Q

RPGN + granular immunofluroescne

A

PSGN (will progress in adults) or diffuse proliferative glomerulonephritis

113
Q

most common renal disease in SLE

A

diffuse proliferative glomerulonephritis due to diffuse antigen-antibody complex deposition

114
Q

RPGN + normal IF, what next?

A

ANCA to determine if Wegeners (c-ANCA) or Churgg Strauss or microscopic polyangiitis (p-ANCA)

115
Q

lung (hemoptysis) + kidney (hematuria) + nose (sinusitis) + c-ANCA

A

Wegener’s

116
Q

c-ANCA

A

Wegener’s

117
Q

p-ANCA

A

Churgg Strauss or microscopic polyangiitis

118
Q

hematuria with mucosal infections

A

Bergers, IgA nephropathy

119
Q

isolated hematuria + vision problems + hearing loss

A

X-linked Alport Syndrome

120
Q

Tuberous Sclerosis associated tumor

A

Angiomyolipoma

121
Q

kidney sx + fever + weight loss + paraneoplastic syndrome (EPO, renin, PTHrP, ACTH)

A

renal cell carcinoma

122
Q

kidney disease + left sided varicocele

A

blocking renal vein so L gonadal vein cannot drain into renal vein

123
Q

kidney disease due to loss of VHL

A

renal cell carcinoma

124
Q

risk factor for sporadic development of RCC

A

smoking

125
Q

chromosome 11 + kidney mass in child + primitive kidney tissue

A

Wilms tumor

126
Q

Wilms tumor + neonatal hypoglycemia + muscular hemihypertrophy + organomegaly

A

Becwith-Widedmann Syndrome

127
Q

recurrent skin infections without pus + delayed detachment of the umbilical cord

A

LAD type 1 = absence of CD18 so no integrin formation and no tight adhesion of leukocytes necssary for transmigration

neuro (34 decks)

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