Musculoskeletal System Flashcards

1
Q

Uniformly fatal variant of osteogenesis imperfecta

A

Type II OI

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2
Q

The underlying etiology in achondroplasia is a point mutation in the ___ that results in its constitutive activation and inhibits chondrocyte proliferation.

A

Fibroblast growth factor 3

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3
Q

A variant of osteopetrosis is characterized by deficiency in ____ necessary for osteoclast hydrogen ion secretion and bone acidification.

A

Carbonic anhydrase II

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4
Q

Complications of osteopetrosis

A

Fractures
Cranial nerve deficits (from compression of surrounding bone)
Recurrent infections
Hepatosplenomegaly (extramedullary hematopoiesis)

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5
Q

Which is more effective in increasing bone mass?
A. Resistance exercises (weight training)
B. Endurance activities (jogging)

A

A. The magnitude of skeletal loading is more important than the number of load cycles.

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6
Q

What are two factors that conspire to convert macrophages into osteoclasts?

A

RANK (RANK-R in macrophages; RANK expressed by stromal cells)
M-CSF (secreted by osteoblasts)

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7
Q

Pathognomonic histologic feature of Paget disease

A

Mosaic pattern of lamellar bone

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8
Q

Paget disease is associated with ___ infection.

A

Paramyxovirus

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9
Q

Cytokines produced in large amounts in pagetic bone.

A

IL-1

M-CSF

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10
Q

Collections of osteoclasts, reactive giant cells, and hemorrhagic debris form a distinct mass termed ___.

A

Brown tumor of hyperparathyroidism

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11
Q

Osteonecrosis Quiz
A. Subchondral infarct
B. Medullary infarct

  1. Pain during physical activity
  2. Stable
A
  1. A

2. B

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12
Q

Favored bony sites of hematogenous spread of TB.

A

Long bones

Vertebrae

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13
Q

Tumors of Bone Quiz

Cortical tumors characterized by pain; histologically interlacing trabeculae of woven bone

Commonly located in the metaphysis of femur and tibia

A

Osteoid osteoma

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14
Q

Tumors of Bone Quiz

Arise in vertebral transverse and spinous process

A

Osteoblastoma

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15
Q

Tumors of Bone Quiz

Bony excrescences with a cartilaginous cap

A

Osteochondroma

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16
Q

Tumors of Bone Quiz

Osteochondroma is associated with inactivation of this gene which encodes ___ essential for polymerization of heparan sulfate

A

EXT

Glycosyltransferase

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17
Q

Tumors of Bone Quiz

Arise within the medullary cavity and erode cortex; microscopically well-differentiated cartilage-like or anaplastic

A

Chrondrosarcoma

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18
Q

Tumors of Bone Quiz

Arise in medullary cavity; microscopically, sheets of small round cells that contain glycogen

A

Ewing tumor

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19
Q

Tumors of Bone Quiz

Developmental defects; arise in the metaphysis of distal femur or proximal tibia. Microscopically, fibroblasts and activated macrophages in a storiform pattern

A

Fibrous cortical defect

20
Q

Tumors of Bone Quiz

Benign tumor in which all components of normal bone are present but they fail to differentiate into mature structures.

A

Fibrous dysplasia

21
Q

Most common location of fibrous dysplasia

A

Ribs

22
Q

Tumors of Bone Quiz

Well-circumscribed intramedullay lesions. Microscopically, it exhibits curved trabeculae of woven bone (mimicking Chinese characters) surrounded by fibroblastic proliferation

A

Fibrous dysplasia

23
Q

Tumors of Bone Quiz

Painful enlarging masses in the diaphyses of long tubular bones (femur); X-ray shows destructive lytic tumor with extension into surrounding tissue. Characteristic periosteal reaction depositing bone in an onionskin fashion

A

Ewing Sarcoma

24
Q

Tumors of Bone Quiz

Sheets of uniform small, round cells with scant glycogen-rich cytoplasm. Tumor cells circled around a central fibrillary space indicates neural differentiation.

A

Ewing sarcoma

with Homer-Wright rosettes

25
Q

Most common etiologic agents of suppurative arthritis:

  1. Children under 2
  2. Older children and adults
  3. Adolescents, young adulthood
  4. Sickle cell patients
A
  1. Haemophilus influenzae
  2. Staphylococcus aureus
  3. Neisseria gonorrhea
  4. Salmonella cholerasuis
26
Q

Most common site of nongonococcal suppurative arthritis

A

Knee

27
Q

Ganglion cyst is a true cyst. T/F.

A

False. It lacks a true cell lining because they arise from cystic degeneration of connective tissue.

28
Q

Most common soft tissue tumor of the hand; arises as a painless tumor of wrist and finger tendon sheats

A

Giant Cell Tumor of Tendon Sheath

29
Q

Presenting as monoarticular arthritis. Joint synovium becomes a contorted mass of red-brown folds, finger like projections and nodules

A

Pigmented villonodular tenosynovitis

30
Q

Myotonic dystrophy is inherited as an autosomal dominant trait associated with triple repeats of ___.

A

CTG; affects synthesis of an intracellular (dystrophila myotonia) protein kinase

31
Q

Muscle biopsy reveals irregular fiber with subsarcolemmal collections of mitochondria that stain red with Gomori trichome stain

A

Mitochondrial myopathy;

“ragged red fibers”

32
Q

This agent can cause proximal myopathy. The most prominent finding is myocyte vacuolization, and with progression, myocyte necrosis.

A

Chloroquine

33
Q

In MG,
Thymic hyperplasia is seen in ___%.
Thymoma in ___%.

A

Thymic hyperplasia in 65%; thymoma in 15%.

34
Q

Name three important differences between MG and Lambert-Eaton Myasthenic Syndrome.

A
  1. Anticholinestrase does NOT improve sx.
  2. Autonomic function affected.
  3. Repeated stimulation elicits INCREASING muscle strength.
35
Q

Malignancy which characteristically causes Lambert-Eaton syndrome

A

Small cell lung Ca

36
Q

Which are the target of autoantibodies produced in Lambert-Eaton syndrome

A

Antibodies recognize PRE-synaptic Ca channels; leading to fewer vesicles than normal released in response to action potential.

37
Q

Most common soft tissue sarcoma of childhood

A

Rhabdomyosarcoma

38
Q

Rhabdomyosarcoma is characterized by which genetic abnormality

A

Formation of chimeric PAX3-FKHR protein which control skeletal muscle differentiation and tumor development

39
Q

Several week history of a solitary, rapidly growing mass on the volar aspect of forearm. There is positive history of trauma. Histology reveals richly cellular, plump, randomly arranged immature fibroblasts in an abundant myxoid stroma. Numerous mitotic figures are seen.

A

Nodular fasciitis

Not a true neoplasm but a reactive proliferation.

40
Q

Athletic adolescent noted swollen, painful proximal muscles after trauma. It then evolved into a painless, hard, well-demarcated mass.

A

Myositis ossificans

41
Q

Histologically, fibromatoses are composed of plump cels in broad sweeping fascicles that invade adjacent tissues. These cells are derived from:

A

Myofibroblasts

42
Q

What are desmoid tumors?

A

Aka deep fibromatoses

Fibroblastic proliferations that arise in the abdominal wall and muscles of the trunk and extremities

43
Q

Soft tissue mass arising in the retroperitoneum; with soft areas of hemorrhage and necrosis. Histology reveals malignant spindle cells in a herringbone pattern

A

Fibrosarcoma

44
Q

Soft tissue mass which on histology demonstrates bizarre multinucleate cells and storiform architecture.

A

Malignant fibrous histiocytoma

45
Q

Well-dermarcated mass which on histology reveals interlacing spindle cells admixed with foamy, lipid-rich histiocyte-like cells

A

Benign fibrous histiocytoma

Dermatofibroma

46
Q

Soft tissue mass which on histology reveals spindle cells with cigar-shaped nuclei in interweaving fascicles.

A

Leiomyosarcoma

47
Q

Soft tissue tumor histology reveals two population of cells: epithelial cuboidal cells forming glands and spindle cells arranged in fascicles

A

Synovial sarcoma