Sherry Flashcards
(99 cards)
1
Q
4-8 yrs, sclerotic margins
A
Fibrous Cortical Defect (Nonossifying Fibroma)
2
Q
low T1 & T2 evidence of hemosiderin
A
Pigmented villonodular synovitis
overexpressin of CSF1
3
Q
most common soft tissue sarcoma
5% of pediatric cancer
age 5, painless
A
Rhabdomyosarcoma
4
Q
Expansile +/- pain
Metaphysis of proximal humerus or femur
A
Unicameral bone cyst
5
Q
Adolescent girls, expansile, pain
Lack distinct margins, fluid levels
Recur 60%
A
Aneurysmal bone cyst
6
Q
Bone mineral
A
70% hydroxyapatite
7
Q
Bone organic component
A
98% matrix, mostly type 1 collagen
8
Q
Distal 1/3 of radius %
A
95% cortex, 5% trabecular
9
Q
Vertebral bodies %
A
5% cortex, 95% trabecular
10
Q
Bone formation markers
A
Alk phos, osteocalcin
11
Q
Bone resorption markers
A
TRAP, hydroxyproline
12
Q
RANKL
A
made by osteoBlasts
When bind to RANK on osteoClasts – chew up bone
Blocked by Osteoprotegerin
Induced by T cell activation
13
Q
blocks RANK-RANKL which causes osteoporosis, DECREASED in menopause
A
Osteoprotegerin
14
Q
Q angle 15-17⁰
A
Patella-femoral syndrome
15
Q
Activity related MEDIAL knee pain, snapping, catching
A
plica (ultrasound)
16
Q
Activity related LATERAL
Knee pain
A
Iliotibial band syndrome
17
Q
Osteochondritis desiccans
A
MEDIAL femoral condyle
18
Q
Tennis elbow
A
LATERAL epicondylitis
19
Q
Golfer elbow
A
MEDIAL epicondylitis
20
Q
de Quervain tenosynovitis
A
abductor pollicis longus & extensor pollicis brevis
21
Q
diskitis
A
toddlers, L4-5, MRI
22
Q
Schurmann
A
juvenile kyphosis, adolescent boys, T7-9, Schmorl nodes, pain with activity
23
Q
Kienböck
A
Lunate [?post trauma –Karate] avascular necrosis
24
Q
Kohler
A
Tarsal navicular avascular necrosis
25
Frieberg
Metatarsal head (2nd, 3rd) avascular necrosis
26
Legg-Calvé-Perthes
proximal femur avascular necrosis, 4-10, no prolonged bracing
27
Slipped capital femoral epiphysis
puberty, obese, bilateral 30%, thyroid, growth hormone, AVN
28
Carpal Tunnel Syndrome
Mucopolysaccharidoses
Mucolipidosis
29
90% bone matrix collagen
Type I collagen
30
articular cartilage, vitreous, discs collagen
type 2 collagen
31
Intramembranous ossification
clavicle, skull, face
32
Th17 effects
bone resorption
↑ IL-1, IL-6, IL-17, RANKL
33
Tregs
bone formation
↓ inflammation IL-4, IL-10, TGF-β
34
Tidemark
in cartilage between deep zone and calcified zone
35
Fibrocartilage
triangular fibrocartilage wrist, TMJ, iliac side SI joint, labrum, menisci
36
chondroitin sulfate, keratan sulfate, heparan sulfate
Proteoglycans
37
Metalloproteinases stabilized by _ , activated by _
stabilized by zinc, activated by calcium
38
Most abundant proteoglycan
Decorin – cross-linking between collagen fibers in tendon
39
insert into bone (entheses, ligaments, capsule, tendons, periosteum)
Sharpey fibers
40
functional unit of muscle
Sarcomere
41
slow, red, thick z bands, lots of mitochondria, oxidative enzymes
Muscle type I fibers
42
fast, white, rich in glycogen, phosphorylases, Atrophic with steroid myopathy (normal CK)
Muscle type II fibers
43
poly & oligo JIA, encodes lymphoid protein tyrosine kinase, that negatively regulates T cells. Mutation associated with T-cell activation & promotion of autoimmune disease.
PTPN22
44
differentially influence susceptibility to SLE & SLE nephritis
Fc-γIII receptor polymorphisms
45
IgG4 and Complement activation
IgG1,2,3 but not IgG4 activate
Classic pathway (as does IgM, CRP & serum amyloid protein
46
present endogenous antigen via MHCI to CD8+ T Cells
Present phagocytized endogenous antigen via MHCII to CD4+ T Cells
Cross presentation presents EXOGENOUS peptides via MHCI to CD8+ T cells
Dendritic cells
47
subtype of DC that produces large amount of type I interferon (IFN-α & IFN-β)
Plasmacytoid dendritic cells
48
if bind to MHCI – stand down – does not kill. If cell has no MHCI (if infected) will kill
natural killer cells
49
produce type II interferon (IFN-γ)
natural killer cells
50
_ recognize antibody coated cells, make IFN-γ, this stimulates macrophage to make IL-12, which induces _ cells
natural killer cells
51
Lectin C’ activation
mannose binding lectin, pathogen associated molecular patterns, ficolins
52
C3a, C5a actions
chemotaxis, anaphylatoxins C5a critical to anti-phospholipid antibody syndrome
53
C3b, iC3b actions
opsonization, phagocytosis
54
Adhesion sequence
injury, macrophages make TNF, IL-1, chemokines, then selectins slow down leukocytes, integrins more adhesive, then transmigration into tissue
55
bacterial infections, no pus, delay in umbilical cord sloughing
Leukocyte adhesion Deficiency
56
IL-5 action
eosinophils
57
IL-2 action
proliferation and activation of T cells
Increases transcription and expression of Fas Ligand
58
IL-4 action
Th2 differentiation
Isotype switching to IgE in B cells
59
IL-12 action
Th1 differentiation
Synthesis of IFN-γ by T & NK cells
60
IL-18 action
Synthesis of IFN-γ by T & NK cells
61
IL-23 action
Th17 differentiation
62
Pro-inflammatory cytokines
IL-1, IL-1β, TNF-α, IL-6, IL-17,
IL-22
63
Anti-inflammatory cytokines
IL-10, TGF-β
64
IFN-γ action
Th1 differentiation,
Induces MHCI on somatic cells, MHCII on antigen presenting cells [in SLE & JDMS – so more MHCI on muscle cells in JDMS]
65
CD4+ - Th1 differentiation
IL-12, INF-γ
66
CD4+ - Th2 differentiation
IL-4
67
Plasmacytoid dendritic cell action
makes up to 1000 times more type I INF (IFN-α & IFN-β) than any other cell
68
Type I INF (IFN-α & IFN-β) action
activate NK cells, antiviral
69
Positive selection (1st)
thymic medulla, double positive T cells (CD4+CD8+) are positively selected if they recognize MCH I or MHCII (turn into CD4+ OR CD8+) [most die here]
70
Negative selection (2nd)
thymic cortex, the CD4+ or CD8+ cells meet thymic epithelioid cells who present self peptides – if bind to these self peptides, they die – too autoreactive.
71
Memory T cells
cells that, after presented with an antigen, rather than differentiate into effector (active) helper or cytotoxic cells, form memory T cells (either CD4+ or CD8+) that survive in an inactive stage until re-encounter same antigen
72
Regulatory T cells
mediate tolerance, restrict the adaptive immune responses, make IL-10, express FOXP3
73
Th1
CD4+ (helper) makes IFN-γ (differentiation driven by IL-12 & IFN-γ)
74
Th2
CD4+ (helper) makes IL-4, IL-5, IL-13 (differentiation driven by IL-4)
75
Th17
CD4+ (helper) makes IL-17, IL-22, TNF, GM-CSF, (differentiation driven by IL-23, IL-1, IL-6)
76
T independent antigens
elicit plasma cell differentiation and antibody production without T-cell help – make low affinity IgM
[lipopolysaccharide, poly-immune complexes, polysaccharides]
77
B effector type 1
primed by Th1 – make IFN-γ, IL-12, TNF-α, IL-6
78
B effector type 2
primed by Th2, make IL-2, IL-4, IL-13, lymphotoxin
79
B regs
make antiinflammatory cytokines, IL-10, TGF-β, IL-35
80
Hypogammaglobulinemia
bacterial infections
X-linked – at birth
CVID – gradually lose IgG
81
OmEnn syndrome
Erythroderma (like GVHD)
IgE high
Eosinophilia
Low T cells, No B cells,
Mutant RAG genes
[smells bad – wrapped in RAGs]
82
X-linked SCID
most common form
IL-2r gene
Low T cells, no NK cells, non-functioning B cells
83
Bare lymphocyte syndrome
Type 1 – MHCI not expressed
Type 2 – MHCII not expressed
84
Mutation in JAK3
leads to SCID
85
family history discoid lupus
measure superoxide anion generation (respiratory burst) in neutrophils
Staph, Serratia, Nocardia
CGD
86
Herpes, HLH, SCID
NK cell deficiency
87
XIAP X-linked inhibitor
Of apoptosis protein
HLH with EBV
88
Wiskott-Aldrich syndrome
thrombocytopenia, eczema, ENT infections
Low CD8+, high IgA, low IgM
x-linked WASP [Wiskott-Aldrich syndrome protein]
89
low IgG, mutations in NFκB pathway, ITP 15%, lung disease if haplosufficiency of CTLA4
CVID
90
mutation in Fas mediated apoptotic pathway
Double negative T cells (no CD4+, CD8+). Hypergammaglobulinemia, high B12, sFASL,
Use MMF or mTOR inhibitor sirolimus (rapamycin) or rituximab
ALPS
91
Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dysplasia (APECED)
half Sjögren syndrome
HSCT not indicated
AIRE mutation
92
FOXP3 mutation
Immunodysregulation Polyendocrinopathy Enteropathy X-linked Syndrome (IPEX), Day 1 of life enteropathy – diabetes, rash, cytopenia
93
HLH
inefficient CD8 and NK cells
High IFN-γ, IL-18, IL-1, IL-6, IL-33, TNF (cytokine storm)
94
IL-10 deficiency
very early IBD, arthritis, lymphoma
95
CTLA4 deficiency
enteropathy, adenopathy, hepatosplenomegaly, diabetes, arthritis, lung disease. Treat with abatacept
96
Positive Schirmer
≤ 5 mm in 5 minutes, without anesthesia
97
in localized scleroderma but not systemic scleroderma
Papillary dermis sclerosis
98
Diseases associated with microchimerism
limited scleroderma, neonatal lupus, JDMS
99
Generalized morphea
≥ 4 individual lesions
