Musculoskeletal Bone Pathology

Front 1

What condition is also known as brittle bone disease?

Back 1

Osteogenesis Imperfecta (OI)

Front 2

In OI what is primarily affected?

Back 2

Type 1 collagen synthesis problems

Front 3

What body parts can be affected by OI?

Back 3

Trabeculae of bone, structures rich in type 1 collagen like joints, eyes, ears, skin and teeth (blue sclera can also be seen)

Front 4

How many subtypes of OI are there?

Back 4

IV
(involve pro-alpha chains)

Front 5

What term is used when the only thing affected in the dentin of the teeth?

Back 5

Dentinogenesis imperfecta

Front 6

What term is used when dentin of the teeth is affected as well as osteogenesis imperfecta?

Back 6

Opalescent Teeth

Front 7

What is Marble Bone or Albers-Schönberg Disease?

Back 7

Osteopetrosis

Front 8

T/F
In Osteopetrosis the defect is attributed to a reduction in number of osteoclasts.

Back 8

FALSE**
Osteopetrosis is due to dysfunction of the osteoclasts

Front 9

What bone disease presents as woven bone (stone-like) due to reduced osteoclastic bone resorption resulting in a diffuse symmetric skeletal sclerosis?

Back 9

Osteopetrosis

Front 10

What are 2 of the 4 types of osteopetrosis?

Back 10

Infantile Malignant Osteopetrosis (IMOp)
Autosomal Dominant Benign Adult Form (ADBAFOp)

Front 11

What type of osteopetrosis is:
1) evident in utero or soon after birth
2) reduced bone marrow space leads to decreased hematopoiesis
3) anemia
4) reduced WBC (more susceptible to infection)
5) smaller foramen lead to facial paralysis due to pressure upon cranial nerves

Back 11

Infantile malignant osteopetrosis IMOp

Front 12

What type of osteopetrosis is:
1) Not detected until adolescent or adult (x-ray due to repeated fractures)
2) milder cranial nerve deficits and anemia
3) compatible with life
4) Bone marrow transplant can provide patient w/osteoprogenitors that can produce normally functioning osteoclasts (so some of skeletal defects may be reversed)

Back 12

Autosomal Dominant Benign Adult Form Osteopetrosis

Front 13

What bone disease is characterized by increased porosity of the skeleton resulting from reduced bone mass, which predisposes the bone to fracture?

Back 13

Osteoporosis

(porosity...think the link)

Front 14

What may be primary or secondary to a large variety of conditions such as endocrine disorders?

Back 14

Osteoporosis

Front 15

When is peak bone mass achieved?

Back 15

during Young Adulthood**

Front 16

What is the best way to help reduce the risk for osteoporosis?

Back 16

Generate as much bone mass as possible during young adulthood.

Front 17

What are some hypotheses that could attribute to osteoporosis?

Back 17

1) Age-related Changes
2) Reduced physical activity
3) Genetic Factors
4) Calcium nutritional state
5) Hormonal influences

Front 18

What bone disease can present clinically as:
1) vertebral fractures
2) lumbar lordosis (anterior curvature of spine)
3) kyphoscoliosis (lateral and posterior curvature of spine)

Back 18

Osteoporosis

Front 19

What are the treatments for osteoporosis?

Back 19

1) exercise
2) Calcium and Vit D
3) Estrogen replacing therapy, bisphosphonates and recombinant PTH

Front 20

What is osteomalacia also known as?

Back 20

Rickets

Front 21

What disease is characterized by a defect in Matrix Mineralization in Adults?

Back 21

Osteomalacia

Front 22

What disease is characterized by a defect in Matrix Mineralization in Children?

Back 22

Rickets

Front 23

What are these predisposing conditions characterizing?
1) lack of Vit D
2) lack of Vit D Absorption
3) Derangements in Vit D metabolism
4) phosphate depletion

Back 23

Rickets and Osteomalacia

Front 24

What bone disease denotes inflammation of the bone and marrow and implies Infection?

Back 24

Osteomyelitis

Front 25

What bone disease presents as:
a) signs of acute inflammation that has been less than 1 mo long
b) feverm leukocytosis, lymphadenopathy
c) radiographs NOT helpful (ill-defined radiolucency)

Back 25

Acute Osteomyelitis

Front 26

What bone disease presents as:
a) may develop de novo or with quiescence of an acute infection
b) pain, some swelling, sinus formation, purulent discharge, sequestrum formation
3) Radiographs reveal patchy, ragged and ill-defined radiolucency containing radiopaque sequestra

Back 26

Chronic Osteomyelitis

(sequestrum is a piece of dead bone that can migrate away from the healthy bone)

Front 27

What bone disease is characterized by woven bone, episodes of localized, frenzied osteoclastic activity with bone resorption followed by exuberant bone formation with a net effect of a gain in bone mass?

Back 27

Paget Disease (Osteitis Deformans)

Front 28

What is the most common indicator of Pagets Disease?

Back 28

Bone Pain*

Front 29

What are the 3 phases of Paget's Disease?

Back 29

Osteolytic Phase
Mixed Phase
Osteosclerotic Phase

Front 30

What are benign lesions of bone that in many cases represent developmental growth rather than true neoplasms & are composed of woven and lamellar bone?

Back 30

Osteoma

Front 31

What specific form of multiple osteomas manifests in the mandible, leads to supernumerary teeth, intestinal polyps that undergo malignant change with a high frequency?

Back 31

Gardner Syndrome
(form of osteoma)

Front 32

What is a malignancy of connective tissue?

Back 32

Sarcoma

Front 33

What is a malignant mesenchymal tumor in which the cancerous cells produce bone matrix?

Back 33

Osteosarcoma

Front 34

What is the most common primary malignant tumor of bone, exclusive of myeloma and lymphoma?

Back 34

Osteosarcoma

Front 35

What bone disease is a benign tumor-like lesion of bone in which normal medullary bone is replaced by an abnormal fibrous connective tissue proliferation in which new non-maturing bone is formed?

Back 35

Fibrous dysplasia

Front 36

When fibrous dysplasia is involved in a single bone it is referred to as?

Back 36

Monostotic

Front 37

When fibrous dysplasia is involved in multiple, but not all, bones it is referred to as?

Back 37

Polystotic

Front 38

What is polystotic disease associated with?

Back 38

Cafe au Lait skin pigmentation and endocrine abnormalities

Front 39

What type of Fibrous Dysplasia is associated with:
*Accounts for approximately 70% of cases of fibrous dysplasia.
*Usually occurs in early adolescence and affects male and females about equally.
*Generally, stops growing at the time of growth plate closure.
*Most commonly affects ribs, femur, tibia, jaws, and skull
*Usually develops as an asymptomatic swelling that can cause marked enlargement of the affected bone.

Back 39

Monostotic Fibrous Dysplasia

Front 40

What type of dysplasia:
*Represents about 27% of the cases.
*It appears at an early age and progresses into adulthood.
*Around 50% of these patients have craniofacial involvement.
*All such cases have a propensity to develop shoulder and pelvic girdle problems, which may result from crippling (shepherd-crook) deformities and fractures.
*Most have craniofacial problems.

Back 40

Polystotic Fibrous Dysplasia w/o Endocrine Dysfunction

Front 41

What type of fibrous dysplasia has:
*This form of fibrous dysplasia is also known as McCune-Albright syndrome.
*These patients typically manifest:
1) polyostotic fibrous dysplasia
2) café au lait spots
3) endocrine abnormalities, which may include:
a) sexual precocity, (early development)
b) hyperthyroidism,
c) pituitary adenomas producing GH
d) primary adrenal hyperplasia
*This entity is recognized more frequently in girls.

Back 41

Polystotic Fibrous Dysplasia w/skin pigmentation and endocrinopathy

Front 42

What are benign tumors of hyaline cartilage with most arising in the small cones of the hand and feet of people in the 20-40 year age range?

Back 42

Chondromas

Front 43

What are malignant neoplasms populated by mesenchymal cells that produce a cartilaginous matrix?

Back 43

Chondrosarcoma