Diseases Of The Bone

Front 1

Deficiency in the synthesis of type-1 collagen?

Back 1

Osteogenesis Imperfecta

Front 2

Is Osteogenesis Imperfecta considered a type of osteoporosis?

Back 2

yes

Front 3

what is the basic disorder of Osteogenesis Imperfecta?

Back 3

Osteoporosis in the form of bone thinning and weakness leading to fragility and increased breakage

Front 4

What are the 4 subtypes of OI?

Back 4

OI:1 Post-natal fracture w/ Blue Sclera
OI: 2 Perinatal Lethal in Utero
OI: 3 Progressive Deforming
OI: 4 Post-natal fracture w/ normal sclera

Front 5

Which of the OI subclasses is not compatible with survival?

Back 5

OI 2 : Perinatal Lethal

Front 6

Which of the 4 subtypes of OI results in short stature?

Back 6

OI 4: Postnatal Fracture w/ normal sclera

Front 7

Normal stature
Skeletal fragility
Dentinogenesis Imperfecta
Hearing impairment
Joint laxity
Blue Sclera

Back 7

OI : 1 PNF w/ blue sclera

Front 8

Growth retardation
Multiple fractures
Progressive kyphoscoliosis
Blue sclera @ birth --> white
Hearing impairment
Dentinogenesis imperfecta

Back 8

OI : 3 Progressive Deforming

Front 9

Which OI subtypes may lead to Dentinogenesis Imperfecta?

Back 9

OI: 1, 3, & 4

Front 10

What is the presentation of teeth for OI?

Back 10

opalescent teeth

Front 11

'Marble bone / Alber-Schonberg Disease'

Back 11

Osteopetrosis

Front 12

In osteopetrosis, osteoclasts decrease in number thus causing reduced bone resorption? T/F

Back 12

False:
reduced activity, but numbers stay the same or even are higher

Front 13

reduced osteoclastic bone resorption due resulting in diffuse symmetrical skeletal sclerosis

Back 13

Osteopetrosis

Front 14

carbonic anhydrase II deficiency is a variant of what pathology?

Back 14

Osteoporosis

Front 15

What type of Osteopetrosis would exhibit:
-fractures
-anemia
- hydrocephaly
- optic atrophy
- deafness
- paralysis
-repeated infections

Back 15

Infantile Malignant Osteopetrosis

Front 16

What is the reason for repeated infections of patients with Osteopetrosis?

Back 16

decreased bone marrow --> (WBC production)

Front 17

_____ bone is laid down haphazardly thus decreasing durability of bone associated with ______?

Back 17

woven
Osteopetrosis

Front 18

presents with:
- repeated fractures in an X ray
- mild cranial deficits
- anemia
- smaller foramina

Back 18

Osteopetrosis: Autosomal Benign type

Front 19

What is a treatment for Osteopetrosis to desist increase osteoclastic activity and reverse some of the skeletal defects?

Back 19

bone marrow transplant

Front 20

X ray exhibits :
- bony enlargements
- bilateral sclerosis of bone
- lessening of vasculature to bony site

Back 20

Osteopetrosis

Front 21

characterized by increasesed porosity of the skeleton resulting from reduced bone mass which predisposes the bone to fracture

Back 21

Osteoporosis

Front 22

Apart from Primary Osteoporosis due to PM women, senile or idiopathic, what are the secondary categories for cause?

Back 22

1) Endocrine disorders (hypothyroidism/diabetes I, etc..)
2) Neoplasia (Multiple Myeloma)
3) Gastrointestinal ( malnutrition)
4) Rheumatologic disease drugs (DMARDS)
5) Miscellaneous (OI, anemia etc..)

Hint 22

5

Front 23

What are the most common forms of Osteoporosis?

Back 23

Senile
Post-menopausal

Front 24

what genetic factor contributes to the density of bone and is a factor in Osteoporosis cases?

Back 24

Vitamin D receptor

Front 25

What is the rate of bone loss at the point that one has met their peak/

Back 25

0.7%/ year

Front 26

What 5 factors contribute to the onset of osteoporosis?

Back 26

1) Age - related (low turnover variant)
2) Reduced Physical Activity (remod)
3) Genetic factors (Vit D receptors)
4) Calcium homeostasis (diet)
5) Hormonal influences (estrogen)

Hint 26

charg

Front 27

How do estrogen levels contribute to osteoporosis?

Back 27

In PM women, an estrogen deficiency takes place that leads to the increased secretion of cytokines ( IL-1, IL-6, TNF) which stimulate osteoclastic activity recruitment and activity that exceed osteoblastic deposition

Front 28

Increased expression of _____ and _____ will lead to the increase the activity of osteoclasts

Back 28

RANK (receptor) and RANKL (ligand)

Front 29

clinical manifestations of Osteoporosis would be : (5)

Back 29

1) vertebral fractures
2) lumbar lordosis
3) kyphoscoliosis
4) pulmonary embolism
5) pneumonia

Front 30

What may cause the clinical manifestation of pneumonia in Osteoporosis?

Back 30

fractures of:
-neck of femur
-pelvis
-spine

Front 31

bisphosphonates and recombinant PTH are used for what?

Back 31

tx of Osteoporosis

Front 32

2 pathologies that result in a defect in bone matrix mineralization?

Back 32

1) Rickets
2) Osteomalacia

Front 33

What cohort does Rickets affect?

Back 33

children

Front 34

What cohort does Osteomalacia affect?

Back 34

adults

Front 35

Children with deranged bone growth producing skeletal deformities such as:
-craniotabes
-frontal bossing
-square head
-lumbar lordosis
-bowing of legs

Back 35

Rickets

Front 36

In an adult that exhibits a matrix mineralization defect resulting in osteopenia and due bone fragility

Back 36

Osteomalacia

Front 37

What are 5 etiologies of matrix mineralization deficiency?

Back 37

1) Inadequate synthesis or dietary of Vit D
2) Decreased absorption of Vit D
3) Deranged Vit D metabolism
4) End Organ Vit D resistance
5) Phosphate depletion

Front 38

'pigeon breast' deformity is associated with _____?

Back 38

Rickets

Front 39

inflammation of bone and its marrow

Back 39

Osteomyelitis

Front 40

In the US what 2 bacteria are common to Osteomyelitic infections?

Back 40

1) pyogenic
2) myocobacteria

Front 41

Fever
Leukocytosis
Lymphadenopathy
Tissue sensitivity/swelling
all lasting less than a month is the course of _______?

Back 41

Acute Osteomyelitis

Front 42

Pain
Swelling
Sinus formation
Purulent discharge
Sequestrum formation
Xray: patchy/ragged radiolucency
sequestrum radiopacity

Back 42

Chronic Osteomyelitis

Front 43

What are the 3 routes of infection for Pyogenic Osteomyelitis?

Back 43

1) hematognous (most common)
2) extension from contigous site
3) direct implantation

Front 44

What are the most common infection sites for Pyogenic Osteomyelitis?

Back 44

Long bones
Vertebral bodies

Front 45

What pathogen is most common to Pyogenic Osteomyelitis

Back 45

S. aureus

Front 46

Who are the patients that contract the following infectious agents:
1) E. coli; Klebsiella; Pseudomonas
2) H. influenzae; B Streptococci
3) Salmonella
4) Mixed infection

Back 46

1) Urogenital Infection pts.
2) Neonatal pts.
3) Sickle cell pts.
4) Surgery pts.

Front 47

What % of acute infections of Osteomyelitis progress to the Chronic form?

Back 47

5-25%

Front 48

1-3% of those with TB (pulmonary/ or extrapulmonary) may manifest what infection?

Back 48

Tuberculous Osteomyelitis

Front 49

Pott's disease (vertebrae destruction) causing skeletal deformities and neurological deficits is associated with _____?

Back 49

Tuberculous Osteomyelitis

Front 50

____ is characterized by:
-localized, frenzied osteoclastic activity with bone resorption followed by exuberant bone formation with a net gain of unsound bone.

Back 50

Paget's Disease (Osteitis Deformans)

Front 51

What are the 3 phases of Paget's Disease?

Back 51

1) initial phase: osteoclastic activity, hypervascularity, and bone loss

2) mixed osteoclastic / osteoblastic activity

3) osteosclerotic phase (late)

Front 52

Basophilic, 'resting lines' characterize the bony mosaic in ______?

Back 52

Paget's Disease

Front 53

Paramyxovirus
Vitamin D dysfunction
RANKL disorder
-all may be culprits to what disease?

Back 53

Paget's

Front 54

Bone pain is the most common problem in what disorder?

Back 54

Paget's Disease

Front 55

-Pain (bone)
-Headache
-Hearing problems
-Enlargement of head (leontiasis ossea)
-Bowing/chalkstick fractures of leg
-Tumors

Back 55

Paget's Disease

Front 56

What % of Paget's Disease pts. may develop cancer?

Back 56

1-3%

Front 57

benign lesions of bone that usually are developmental growths composed of woven lamellar bone

Back 57

Osteoma

Front 58

What is the most common location for development of Osteomas?

Back 58

Facial bones
Skull

Front 59

What is the cohort for Osteomas

Back 59

Middle aged (40-50 yo)

Front 60

exophytic growths that may arise within bone?

Back 60

osteomas

Front 61

Multiple Osteomas may be present in what disease, accompanied with oral problems and other malignancies?

Back 61

Gardner Syndrome

Front 62

Osteomas often undergo malignant change that may result in fast-growing manifestations. T/F

Back 62

False
Slow growing; non-malignant

Front 63

malignant mesenchymal tumor whereby cancerous cells produce bone matrix

Back 63

Osteosarcoma

Front 64

Excluding myelomas and lymphomas, what is the most common primary malignant tumor of bone?

Back 64

Osteosarcoma

Front 65

What cohort incurs Osteosarcomas?

Back 65

<20 yo = 75%
Older are those with predispositions (Paget's disease)

Front 66

Most osteosarcomas of the jaw arise in pts. between what age?

Back 66

20-30yo

Front 67

What gender is most affected by osteomas?

Back 67

Males
1.6:1

Front 68

Osteosarcomas arise in the metaphyseal region of long bones while ____% arise in the ______

Back 68

50-60%
knee

Front 69

10-20% of pts. with Osteosarcomas already have ________

Back 69

pulmonary metastases

Front 70

The _____ yr survival rate of Osteosarcoma pts. has increased to 60-70%

Back 70

5

Front 71

What may be the tumorous factor of Osteosarcomas?

Back 71

Osteoblasts

Front 72

benign tumor like lesion that is arrested , immature developmental tissue in the medulla of bones

Back 72

Fibrous Dysplasia

Front 73

Fibrous CT from Fibrous Dysplasia pts. that is within bone medulla is replaced eventually by _______

Back 73

non-maturing bone

Front 74

What are the 3 presentations of Fibrous Dysplasia lesions?

Back 74

1) monostotic (single bone involved)
2) polystotic (multiple)
3) polystotic w/ cafe au lait skin and endocrine abnormalities

Front 75

Accounts for 70% of Fibrous Dysplasia cases

Back 75

Monostotic form

Front 76

When in a patient's life does the asymptomatic Monostotic form of FD desist in growth?

Back 76

adolescence

Front 77

Polystotic FD w/o endocrine involvement represents ___ % of the cases and about ___% of these patients have craniofacial involvement.

Back 77

27%
50%

Front 78

When does Polystotic FD present in a patients life?

Back 78

early age

Front 79

have propensity to develop:
-shoulder + pelvic problems
-crippling
-Shepard-Crook deformities
-fractures

Back 79

Polystotic FD w/o endocrine dysfunction

Front 80

Polystotic FD with Skin pigmentation and Endrocrine dysfunction is also called?

Back 80

McCune Albright syndrome

Front 81

Polystotic FD pts. are often _____ in gender and present with:

Back 81

girls
-cafe au lait spots
-endocrine abnormalities: sexual precocity, hyperthyroidism, pituatary adenomas, etc.

Front 82

Monostotic FD usually presents with ____ complications while Polystotic may be associated with ______ diseases

Back 82

-minimal
-progressive

Front 83

'ground glass' appearance in radiographs

Back 83

Fibrous Dysplasia

Front 84

benign tumors of hyaline cartilage found in hands and feet

Back 84

Chondroma

Front 85

what is the cohort for Chrondromas

Back 85

20-40 yo (young)

Front 86

Multiple chondromas (enchondromas) are seen in _________

Back 86

Ollier Disease

Front 87

Chrondromas develop from _______ often limiting growth potential

Back 87

rests of growth plates

Front 88

how might chondromas become malignant?

Back 88

enchondromas (multiple)

Front 89

chondromas are usually painful? T/F

Back 89

False
-usually not but can be at times

Front 90

what is the tx of choice for chondromas?

Back 90

surgical excision

Front 91

malignant neoplasms populated by mesenchymal cells that produce a cartilagenous matrix

Back 91

Chondrosarcomas

Front 92

What is the cohort and incidence of Chondrosarcomas?

Back 92

Males X2
Osteosarcomas x2 in comparison
Older pts. > 40 yo

Front 93

What might be the result of an enlarging, painful mass in the shoulder, pelvis, femur or ribs and is made of cartilage?

Back 93

Chondrosarcoma

Front 94

Chondrosarcomas often metastisize thru the ______ route usually with the ____ as the most common site

Back 94

hemagenous
lungs

Front 95

5 year survival rate of those with Chondrosarcomas in grades:
1 -
2 -
3 -

Back 95

90%
81%
43%