Sweep 1.1

Question 1

Duchenne muscular dystrophy is an ——— disorder caused by the absence of a structural protein termed ———- and is seen in about 1/3500 live male births.

Answer 1

X-linked recessive

dystrophin

Question 2

Dystrophin attaches portions of the muscle fibers to the ———, thus playing an important role in the .

Answer 2

cell membrane

structural and functional integrity of the myocyte

Question 3

Myasthenia gravis is an ———- which results in destruction of ———— and is manifested by weakness and prominent fatigability of voluntary muscles.

Answer 3

autoimmune disease

acetylcholine receptors in the neuromuscular junction

Question 4

Myasthenia gravis

The most active muscles generally are

Answer 4

the most severely affected, such as the muscles involved in eye movement, facial expression, chewing, swallowing and respiration.

Question 5

Myasthenia gravis

The natural course of the condition is improved by ——— ——– and removal of the tumor in those with a ——–. In addition, medications such as ——– have been proven to be beneficial in many cases.

Answer 5

thymectomy in cases of thymus hyperplasia

thymoma

cholinesterase inhibitors and corticosteroids

Question 6

Myasthenia gravis

Some cases of ———— have been reported. In the past, ———— was a major cause of death; due to improved therapies, the current 5-year survival is 95%.

Answer 6

spontaneous remission

respiratory compromise

Question 7

Gout: After the joints, the ——– is the most frequently affected organ.

Answer 7

kidney

Question 8

1. Oligodendroglioma.

a. Patients often have several years of

Answer 8

neurologic symptoms including seizures.

Question 9

1. Oligodendroglioma.

b. Mostly

Answer 9

cerebral hemisphere.

Question 10

1. Oligodendroglioma.

c. Well ———-, often ——–. Cells with uniform, round nuclei and perinuclear ———–

Answer 10

circumscribed

calcified

halo (fried egg look)

Question 11

1. Oligodendroglioma.

. Generally better prognosis than .

Answer 11

astrocytoma

Question 12

1. Ependymoma.

a. May manifest with ———- secondary to ——– of the ventricle. May also have ———–.

Answer 12

hydrocephalus

obstruction

CSF dissemination

Question 13

1. Ependymoma.
   b. ———- lesions, arising from ———–. Elongated cells with processes radiating around ————- (perivascular pseudorosettes)

Answer 13

Well-circumscribed

ventricular wall

blood vessels

Question 14

Hypothalamus

• posterior lobe: composed of

Answer 14

modified glial cells and axonal processes extending from hypothalamic neurons

Question 15

A. Hyperfunction (hyperpituitarism)

o almost always associated with a

Answer 15

pituitary adenoma

Question 16

B. Hypofunction (hypopituitarism)

• causes:

Answer 16

o nonfunctional pituitary adenoma
o Ischemic necrosis, most commonly from Sheehan’s syndrome (postpartum infarct). Need over 75% of anterior lobe to be destroyed for clinically significant effects.
o Ablation of pituitary by surgery or radiation
o destruction by adjacent tumor

Question 17

I. Normal Thyroid
• embryology

o develops from an invagination of ——— which arises at the base of the tongue, in the region of the ———

Answer 17

endoderm

foramen cecum

Question 18

I. Normal Thyroid
• embryology

migrates

Answer 18

caudually to its location anterior and inferior to the thyroid cartilage

Question 19

Graves’ Disease

• —————, significant genetic component
o Autoantibodies to

Answer 19

autoimmune

TSH receptor; constantly stimulated

Question 20

Hashimoto Thyroiditis

o Most common cause of ———– where ———– is sufficient

Answer 20

hypothyroidism

dietary iodine

Question 21

Hashimoto Thyroiditis

o Autoimmune; progressive destruction of ———– with ———-

Answer 21

parenchyma

inflammatory infiltrates

Question 22

Hashimoto Thyroiditis

o Involves———–cells

Answer 22

CD4+, CD8+, and NK

Question 23

Hashimoto Thyroiditis

o initially ———-, with progression of disease most patients will become ———–

Answer 23

euthyroid

hypothyroid

Question 24

Hashimoto Thyroiditis

o Painless thyroid enlargement with

Answer 24

hypothyroidism

Question 25

Follicular Adenoma o -------------, 3-5 cm in diameter

Answer 25

solitary nodules

Question 26

Follicular Adenoma o grossly separated from the ------------ by a ----------

Answer 26

normal thyroid thin, discrete capsule

Question 27

Follicular Adenoma o microscopically composed of --------- with varying amounts of ----------

Answer 27

follicles colloid

Question 28

Papillary Thyroid Carcinoma o accounts for >-------of thyroid cancers

Answer 28

85%

Question 29

Papillary Thyroid Carcinoma | o 60-70% are -------

Answer 29

multifocal

Question 30

Papillary Thyroid Carcinoma o some cases related to

Answer 30

radiation exposure

Question 31

Papillary Thyroid Carcinoma o Many have mutations in the --------------

Answer 31

RET proto-oncogene

Question 32

Follicular Carcinoma o accounts for--------- of thyroid cancers

Answer 32

5-15%

Question 33

Follicular Carcinoma o Older age than --------; areas with -----------

Answer 33

papillary dietary iodine deficiency

Question 34

Follicular Carcinoma o grossly may resemble an ---------- with a ---------

Answer 34

adenoma discrete capsule

Question 35

Follicular Carcinoma o Must see invasion through the

Answer 35

capsule or into the blood vessels

Question 36

Anaplastic Thyroid Carcinoma o Rare <5%; typically presents as o --------- prognosis

Answer 36

rapid enlargement in a long standing goiter extremely poor

Question 37

Chronic gouty arthritis arises secondary to --------.

Answer 37

repeated episodes of acute attacks

Question 38

Chronic gouty arthritis Crystals become encrusted on the articular surface and even penetrate deeply into -------------- Each deposit is known as a ------------- and is diagnostic of gout.

Answer 38

surrounding bone and soft tissue. tophus

Question 39

Acute gouty arthritis arises upon precipitation of -------- in the synovial fluid and membranes. These crystals elicit an acute inflammatory response until ----------.

Answer 39

urate crystals the crystals resolubilize

Question 40

Fragments of dislodged bone and cartilage often float free in the joint space and form ----------

Answer 40

“joint mice”.

Question 41

Eventually, the entire surface of the bone becomes exposed, thickened and polished to an ivory-like appearance. At the margins of the joint, small bony outgrowths termed ---------- arise and help to further inflame the surrounding synovial membrane.

Answer 41

osteophytes

Question 42

Osteosarcoma: The radiographic appearance is generally that of a -----------

Answer 42

large, destructive, mixed radiolucent-radiopaque lesion that may present with a “sunburst” appearance.

Question 43

Osteosarcoma: A characteristic feature is the formation of an acute angle between the ------------

Answer 43

neoplastic bone and the cortex (Codman’s triangle).

Question 44

Osteosarcoma: Diagnosis requires

Answer 44

histologic evidence of direct production of osteoid by malignant mesenchymal cells.

Question 45

Histopathologic examination of early lesions reveals a mixture of ----------- intermixed with elongated trabeculae of immature woven bone and this pattern has been termed Chinese characters. When radiographically examined with film capable of high detail (dental films), the affected area will appear as an ill-defined area of ----------------

Answer 45

cellular fibrous connective tissue increased radiopacity described as ground glass.

Question 46

i. Fibrous Dysplasia is characterized by --------------- This may reflect ---------- maturation with arrest at an -------------- due to a specific mutation.

Answer 46

progressive replacement of normal bone by fibrous tissue intermixed with unorganized, woven bone. disordered bone immature stage of woven bone

Question 47

Fibrous dysplasia | Three clinical patterns are seen:

Answer 47

Monostotic fibrous dysplasia Polyostotic fibrous dysplasia without endocrine involvement (Jaffe-Lichtenstein Syndrome) Polyostotic fibrous dysplasia in association with endocrinopathies (McCune-Albright syndrome)

Question 48

Hematogenous osteomyelitis is caused most frequently by -----------; while mixed infections, often with anaerobes, are seen in those secondary to ---------------

Answer 48

Staphylococcus aureus trauma or dental problems.

Question 49

Osteomyelitis refers to an infection spreading through the -----------.

Answer 49

medullary spaces of a bone

Question 50

Osteomyelitis | Bacteria can colonize the spaces within bone, normally through one of three pathways.

Answer 50

1. Hematogenous spread 2. Contiguous infection (i.e. odontogenic infections) 3. Direct traumatic introduction (i.e. compound fractures, surgical procedures)