Quiz 4 Flashcards
(130 cards)
1
Q
which collagen disorder with a bluish tinge to the sclera
A
osteogenesis imperfecta
deficiency of normal type-1 collagen
2
Q
this is characterized by idiopathic avascular osteonecrosis of the epiphysis of the femoral head, interrupting the blood supply
A
Legg-Calve-Perthes syndrome
another name may be aseptic necrosis..
3
Q
what is among the most frequent childhood diseases in many developing countries? and what is the predominant cause?
A
Rickets
- vitamin D deficiency is the predominant cause
- lack of calcium in the diet may also lead to rickets
4
Q
what is the term used for the bowing of the legs in Rickets?
A
genu varum
5
Q
older children with rickets may present with?
A
genu valgum (knock knees)
or windswept knees
6
Q
prominent knobs of bone at the costochodral joints of cricket’s patients are known as
A
rachitic rosary
or
beading of the ribs
7
Q
this is a disease characterized by low bone mass and structural deterioration of bone tissue
A
osteoporosis
8
Q
Most significant type of fracture for someone in their 60s or older with osteoporosis are..
A
hip fractures (devastating prognosis)
spine
wrist
9
Q
secondary osteoporosis implies
A
decreased bone mass due to other medical conditions - medications, malabsorption, immobilization
far and away - steroid, MC cause!!
10
Q
this condition is characterized by a hormone dependent acceleration of bone loss primarily due to estrogen deficiency
A
postmenopausal osteoporosis (secondary)
decreased estrogen increases production of
IL-1
IL-6
TNF-alpha
11
Q
increased levels of cytokines are in large part responsible for what?
A
increased osteoclast activity
12
Q
any ectopic production of what can result in osteoporosis?
A
PTHrP
or
ACTH
13
Q
this is commonly seen in patients with osteoporosis due to compression fractures of the vertebrae
A
kyphosis
14
Q
this condition is characterized by high bone turn over due to accelerated osteoCLAST AND osteoBLAST activity
A
PAGET’S DISEASE
also referred to as
osteitis deformans
15
Q
which condition presents with markedly elevated serum alkaline phosphatase?
A
Paget’s disease
16
Q
which condition presents with a histological hallmark termed the mosaic pattern of lamellar bone - looks like a jigsaw puzzle
A
Paget’s disease of bone
woven bone is created AFTER fracture in paget’s disease
17
Q
which condition commonly develops in the 4th decade of life, as opposed to which condition that presents at birth?
A
Paget’s disease - 4th decade, common in caucasians/males
osteopetrosis - birth - increased hardening of the bone
18
Q
patient’s w. Paget’s disease have an increased risk for?
A
development of
MALIGNANT NEOPLASMS
ex: osteosarcoma
19
Q
which condition presents with cotton ball appearance on x-ray?
A
Paget’s disease
MRI will show marked thickening of the calvarium
20
Q
what may be the first warning sign of Paget’s disease?
A
pain from a PATHOLOGIC FRACTURE
- head can visible swell, HL is common, legs may bow out.
21
Q
widening bone with course trabecular marking is characteristic of which condition?
A
Paget’s disease
22
Q
this is a rare inherited disorder whereby the bones harden and become more dense
A
osteopetrosis
23
Q
with this condition - th most common bones affected in children are the long bones, for adults its the vertebrae and pelvis that are most commonly affected
A
osteomyelitis
24
Q
which bug is most commonly isolated in all forms of osteomyelitis?
A
STAPHYLOCOCCUS aureus
**kiddos tend to present more acutely, while adults tend to be due to a pre-existing chronic condition
25
what term is used to describe collectively, all of the skeletal change of chronic renal disease?
renal osteodystrophy
26
children with this condition are at an increased risk for rickets
renal osteodystrophy
27
fractures are classified as what when the overlying tissue is intact? and as what when the fracture site communicates with the skin surface?
simple/closed
-no soft tissue injury
compound/open
-soft tissue injury associated
28
when a fractured bone is splintered or crushed, what is it called?
comminuted
29
these cysts appear predominantly in the distal ends of long bones such as the humerus and femur
solitary bone cysts
30
these are most common in young males in their first and second decades of life
solitary bone cysts
31
x-ray reveals cystic changes with a smooth, thin cortex in close proximity to the epiphysis
solitary bone cyst
often symmetrical in appearance
32
what is it called when you scrape the sides of a cyst
curettage
33
these cysts occur most often in the metaphysics of the long bones and are found in vertebrae
aneurysmal bone cyst
34
these are more common in females in the 2nd and 3rd decades
aneurysmal bone cyst
gross pathology - distortion of the bone, less symmetrical
35
these cysts may have honeycomb appearance on x-ray
aneurysmal bone cyst
36
this is a benign, bone-forming neoplasm characterized by a small area of neoplastic tissue surrounded by a wide zone of mature, reactive bone
osteoid osteoma
37
multiple osteoid esteems are seen in patient's with this condition?
Gardner's syndrome
| - aka familial colorectal polyposis
38
this is a protein matrix which mineralizes to become bone
osteoid
primarily composed of type 1 collagen
39
this is viewed as a small, well circumscribed lesion which is round/oval located in or near the CORTEX, surrounded by densely sclerotic bone
osteoid osteoma
40
gross examination of this condition is characterized by an area of gritty, cherry red tissue, surrounded by dense sclerotic bone
osteoid osteoma
41
microscopically, this condition has narrow trabecular or osteoid or newly formed bone produced by osteoblasts in richly vascularized parenchyma surrounded by wide trabeculae and sclerotic bone
osteoid osteoma
42
what is the primary malignant tumor of bone?
osteosarcoma
| most pts
43
what is the third most common malignancy in adolescents after leukemia and lymphoma?
osteosarcoma
usually seen in metaphysis of long bones
44
what is more common in elderly patients who have an underlying condition of Paget's disease
osteosarcoma
45
on x-ray, variable bone destruction/formation is noted along with adjacent soft tissue showing spicules of calcium
osteosarcoma
46
this condition presents classically with aggressive, rapid growth at high risk of local spread that may classically metastasize as "skip" lesions within the bone
osteosarcoma
47
early pulmonary metastasis is common with which condition?
osteosarcoma
48
this condition presents histologically with atypical cells, large nuclei, small cytoplasm with osteoid formation
osteosarcoma
49
this is uncommon, but usually found around the knee in either the distal femur &/ proximal tibia
fibrosarcoma
**produces a collagen matrix but does not produce osteoid or chondroid
50
sheets of spindle shaped cells is characteristic for..
fibrosarcoma
51
this is an aggressive tumor composed of oval tumor cells and multinucleate tumor cells - uniformly distributed throughout the lesion
giant cell tumor
52
an x-ray of this tumor shows a destructive radiolucent lesion that is usually oval shaped and centered in the epiphysis
giant cell tumor
53
this generally causes cortical erosion and thinning - expanding the bone contours WITHOUT a border of NEW BONE formation and WITHOUT sclerosis
giant cell tumor
54
this condition is composed of uniform, densely packed, tumor cells with a single round or oval nucleus, indistinct cellular borders with a size and appearance somewhat resembling lymphocytes
ewing sarcoma
55
x-ray of this condition shows a mottled "moth eaten" destructive lesion - and it commonly infiltrates the medullary cavity
ewing sarcoma
56
these tumors often extend beyond the bone into the surrounding soft tissue
ewing sarcoma
57
which condition presents with characteristic lytic lesions?
multiple myeloma
58
more than 75% of skeletal metasasis in adults originate from cancers of?
prostate
breast
kidney
lung
59
in children - boney metastasis most commonly originate from?
neuroblastoma
wilm's tumor
bone tumor - osteosarcoma/ewing
60
skeletal metastasis are typically
multifocal
61
the ivory vertebrae sign is seen in either?
metastatic cancer
or
Paget's disease
62
this is a malignant disease of plasma cells in the bone marrow - monoclonal overgrowth of one clone of plasma cells occurs also producing these?
multiple myeloma
produces:
- monoclonal immunoglobulin
- paraprotein
63
what is hyaline cartilage composed of
type II collagen
water
proteoglycans
chondrocytes
64
what cytokines trigger degradation processes of OA and what do they inhibit?
IL-1, IL-6 & TNF
inhibit - synthesis of type II collagen and proteoglycans
65
what is the most common type of joint disease?
osteoarthritis
66
this condition is characterized by the progressive erosion of articular cartilage
osteoarthritis
| - an intrinsic disease of cartilage
67
which systemic conditions can cause secondary osteoarthritis?
Diabetes
Hemachromatosis
Wilson's dz
68
what are typically observed on X-ray for OA?
joint space narrowing
marginal osteophyte formation (bone spurs)
subchondral bone cysts
sclerosis
69
Which node is which? Differentiate location for Heberden and Bouchard's nodes
Heberdon - Distal
| Bouchard's - Proximal
70
this condition has a hallmark appearance of fibrous-lined cysts in the subchondral bone on histology..
osteoarthritis
71
this condition is characterized by polyarthritis which is often progressive and deforming, with exacerbations and remissions
rheumatoid arthritis
- BL, symmetrical, fusiform swelling and redness of the fingers at the PIPs -- characteristic
72
what autoantibodies are associated with RA and what class II antigen?
anti-IgG autoantibodies (rheumatoid factors)
association with class II antigen: HLA-DR4
73
in regards to rheumatoid arthritis, joint involvement is typically..
polyarticular (3+ jts)
BL
symmetrical
74
which joints are commonly affected in RA?
MCP
PIP
MTP
wrist, elbow, knee, ankle
75
this condition presents histologically with multiple layers of proliferated synoviocytes with lymphocytic infiltration
rheumatoid arthritis
76
are subcutaneous rheumatoid nodules common? who generally gets them? and what do they look/feel like?
~25% of patients with RA have these nodules
- generally seropositive disease
- up to 2 cm diameter, firm, NT and generally over bone prominence
77
what is the name of the clinical triad for RA consisting of: RA, splenomegaly, hyperspelnism
Felty's syndrome
78
Is rheumatoid factor specific for RA?
NO
79
which test for RA has high specificity and is used clinically for dx?
anti-citrullinated protein antibodies
| ACPAs or anti-CCP
80
what is the big difference between juvenile RA vs RA?
more frequently, JRA presents with:
high fever
rash
lymphadenopathy
81
what is the most common form of arthritis in children and adolescents?
Juvenile Idiopathic Arthritis
AKA - JRA, Still's dz
82
this group of joint disease is characterized by inflammatory arthritis with inflammatory involvement of the SI joints, posterior intervertebral articulations and adjacent soft tissues resulting in: sacroiliitis, spondylitis and other abn's
seronegative spondyloarthropathies
STRONG association with HLA-B27
Ankylosing spondylitis
Reactive arthritis
enteropathic arthritis
psoriatic arthritis
83
what are associated complications of ankylosing spondylitis?
uveitis
aortitis
amyloidosis
84
this immunogenetic phenotype may predispose to the development of autoantibodies direct at joints after certain infections
HLA-B27 +
| 90% of AS pts have this
85
what is the classic radiographic characteristic of ankylosing spondylitis
bamboo spine
86
this condition is defined by a triad of arthritis, non-gonococcal urethritis and conjunctivitis
Reactive arthritis
87
what is the characteristic rash of reactive arthritis?
keratoderma blenorrhagica
88
what is the most common cause of reactive arthritis
chlamydia
89
for reactive arthritis - synovitis of digital tendons results in ..
swollen digits AKA
SAUSAGE FINGERS/TOES
90
what are the most commonly affected joints of enteropathic arthritis?
knees and ankles
- LPS response following bowel infxn by:
shigella, salmonella, yersina, campylobacter
91
DIP joints are generally the first affected in this condition
psoriatic arthritis
- inflammation of the tendon sheaths can result in sausage appearance
92
what is a distinctive feature in some cases of PsA?
pitting of the nails
93
this term refers to the detachment of the nail from the nail bed.. what condition most commonly causes this?
onycholysis
often the 4th/5th digit**
most commonly caused by psoriasis
94
what does the presence of onycholysis make the nail more vulnerable to?
bacterial infection
- ex: pseudomonas
*melanoma can also be found under the nail
95
what are the 2 most common causative organisms in bacterial arthritis?
staph - MOST COMMON IN ADULTS
strep - 2nd MC
96
what is the most common causative organism for KIDDOS in bacterial arthritis? what if it's from a puncture wound?
h. influenzae
- now less common due to vaccination
* * suffered a puncture wound? think pseudomonas
97
multiple macules or vesicles seen of the trunk is pathognomonic for this causative organism of bacterial arthritis
neisseria gonorrhoea
98
patients with sickle cell are at a greater risk for what infecting their joints?
salmonella
99
what is the most common cause of bacterial arthritis in young adults
neisseria gonorrhoea
100
what is the most common cause for a single joint?
bacterial arthritis - if non-gonococcal arthritis
101
with a bacterial infection - which wbcs are increase most?
neutrophils
102
which joint is most commonly affected in tuberculous arthritis?
hips
then:
knees, ankles
103
what is tuberculosis of the spine called? and what portions of the vertebrae are commonly affected?
Pott's disease
commonly affects:
thoracic
lumbar
104
what is the name for the vector of Lyme disease
Ixodes scapularis - deer tick
* MC in NE coastal region of the states
105
what spirochete is responsible for Lyme disease?
Borrelia burgdorferi
106
what joints are most commonly involved in Lyme?
knees, ankles, shoulders, elbows
107
what is the characteristic rash of Lyme?
erythema migrans
108
what are some of the viruses that can cause viral arthritis?
parvo B19
rubella
hep C
HIV
109
gout is due to the deposition of?
monosodium urate crystals
110
pseudogout is due to the deposition of?
calcium pyrophosphate crystals
111
what type of toxicity leads to increased risk for gout?
lead
112
what is the term use do gout developing as a consequence of ingesting lead contaminated alcohol (moonshine)
saturnine gout
113
what is the pathognomonic lesion for gout?
tophus
| - contain uric acid crystals
114
rhomboidal, intracellular crystals are characteristics of
pseudogout
115
what is the most common tumor affecting joints?
ganglion
116
what is it called when synovial fluid herniates at the popliteal fossa?
Baker's cyst
117
what are the most common forms of villonodular synovitis (benign neoplasms) - what is the distinguishing factor between the two?
pigmented villonodular synovitis (PVNS)
**hemosiderin laden macrophages**
diffuse-type giant cell tumor of tendon sheaths (GCTTS)
118
what is the most common form of muscular dystrophy? what is it due to?
Duchenne's
defective gene on X chromosome
119
which protein is not produced in Duchennes?
dystrophin
- in it's absence, calcium XS
starts in the pelvis - moves to the shoulder
120
what is it called when muscle breaks down, but there is a build up of fat making the tissue actually appear larger?
pseudohypertrophy
121
what do circulating antibodies do that are responsible for the weakness experienced in myasthenia gravis?
block acetylcholine receptors
POST-SYNAPTICALLY
weakness worse activity, better rest
122
for myasthenia gravis there is a strong association with thymic abnormalities.. what is most common for women/men?
thymic hyperplasia
-women
thymoma
-men
123
this condition presents microscopically with large collections of lymphocytes, necrotic muscle fibers appearing pale and atrophic
myasthenia gravis
124
what would you test for in Myasthenia gravis?
serum anti-acetylcholine receptor ABs
125
what is lambert-eaton syndrome associated with and what are it's antibodies directed against?
small cell lung cancer
antibodies against:
PRESYNAPTIC calcium channels
126
this condition is classically microscope with mixed b- and t- cell perivascular inflammatroy infiltrate (lymphocytic infill) and perifasicular muscle fiber atrophy
dermatomyositis
- characteristic periorbital heliotrope rash
- Gottron's sign - MCP erythema
127
what invades microscopically for polymyositis?
macrophages
128
this condition is characterized by focal areas of white blood cells and other inflammatory cells, vacuoles in muscle, deposits of AMYLOID related proteins
inclusion body myositis
129
these are benign tumors of striate muscle
rhabdomyomas
130
what is the most common soft tissue tumor in children
rhabdomyosarcoma
- spindle shaped cells, elongated hyper-chromatic nuclei
- MC in orbit
