Sweep 1.2

Question 1

Medullary Thyroid Carcinoma
o uncommon, accounts for —– of thyroid cancer
o derived from the parafollicular (C) cells
o may be sporadic or familial (component of MEN syndromes)
o All have mutation in the RET proto-oncogene
o Increased serum calcitonin

Answer 1

5%

Question 2

Medullary thyroid carcinoma

o derived from the

Answer 2

parafollicular (C) cells

Question 3

Medullary thyroid carcinoma

o may be ————-

Answer 3

sporadic or familial (component of MEN syndromes)

Question 4

Medullary thyroid carcinoma

o All have mutation in the ———
o Increased ———

Answer 4

RET proto-oncogene

serum calcitonin

Question 5

II. Hyperparathyroidism

• An important cause of

Answer 5

hypercalcemia

Question 6

II. Hyperparathyroidism

• Treatment: Surgical removal of ———-

Answer 6

hyperplastic parathyroid glands.

Question 7

Hyperparathyroidism

Kidney transplant may be beneficial in patients with

Answer 7

hyperparathyroidism secondary to renal failure.

Question 8

III. Hypoparathyroidism

• Clinical manifestations

Answer 8

o Hypocalcemia
o Increased neuromuscular excitability
o Cardiac arrhythmias
o Increased intracranial pressure and seizures

Question 9

o Glucagon:

Answer 9

mobilizes carbohydrates (stored in the liver) into circulation when the body needs them. Promotes glycogenolysis and gluconeogenesis in fasting states.

Question 10

o Insulin:

Answer 10

Major anabolic hormone, many synthetic and growth-promoting effects - allows glucose to be transported and stored in cells within the body after meals

Question 11

Presenting symptoms of DM

• Type 1

Answer 11

o Ketoacidosis
• With fat as primary energy source: excess ketones in blood, low blood pH
• Can lead to diabetic coma

Question 12

Primary hyperparathyroidism is one of the most common endocrine disorders, and it is an important cause of ———–. In more than 95% of cases, primary hyperparathyroidism is caused by ———-

Answer 12

hypercalcemia

parathyroid adenomas or hyperplasia (less commonly by parathyroid carcinoma, but more severe).

Question 13

Paget disease

a fairly common bone disorder characterized by repetitive episodes of ——— followed by exuberant ———- (mixed osteoclastic-osteoblastic stage), and finally by an apparent exhaustion of ——-

Answer 13

frenzied, regional osteoclastic activity and bone resorption (osteolytic stage),

osteoblastic activity and bone formation

cellular activity (osteosclerotic stage).

Question 14

Paget disease

——— (lion face, characterized by an overgrowth of the facial and cranial bones) and a slight increased risk (1 % lifetime) for development of ——–

Answer 14

leontiasis ossea

sarcomas (particularly osteosarcoma).

Question 15

Paget disease

The diagnosis is made secondary to the ———- combined with demonstration of elevated levels of ———–

Answer 15

radiographic and microscopic appearances

urinary hydroxyproline (⇑bone resorption) and serum alkaline phosphatase (⇑bone apposition).

Question 16

Paget disease

Most patients have mild symptoms which can be controlled by

Answer 16

calcitonin or bisphosphonates and analgesics.

Question 17

1. Monostotic fibrous dysplasia (single bone): 70% of all cases. It usually manifests in ——– and often becomes stationary at puberty.

Answer 17

childhood

Question 18

Monostotic fibrous dysplasia

The most common sites of involvement in descending order of frequency are the

Answer 18

ribs, femur, tibia, jaws, calvarium, and humerus.

Question 19

ACQUIRED DISEASES OF BONE DEVELOPMENT

Although all bones are affected, those containing abundant ——— bone such as the spine and femoral head demonstrate the most severe involvement.

Answer 19

cancellous

Question 20

Polyostotic fibrous dysplasia without endocrine involvement (Jaffe-Lichtenstein Syndrome): ———– are affected, and the involvement often tends to be ———.

Answer 20

Multiple bones

unilateral

Question 21

Polyostotic fibrous dysplasia without endocrine involvement

This pattern accounts for about 25% of the cases, often arises at an ———, and can continue to cause problems during adulthood.

Answer 21

earlier age

Question 22

Polyostotic fibrous dysplasia without endocrine involvement

Frequently affected bones are

Answer 22

craniofacial bones (including jaws), femur, tibia, humerus, and pelvis.

Question 23

Polyostotic fibrous dysplasia without endocrine involvement

———— are variable but not rare; related endocrine abnormalities are absent.

Answer 23

Café-au-lait pigmentations

Question 24

3. Polyostotic fibrous dysplasia in association with endocrinopathies (McCune-Albright syndrome): This pattern is the ———, represents around 3% of all cases, and typically is accompanied by ———- and ———–.

Answer 24

least common

melanin pigmentation of the skin (café-au-lait spots)

endocrine abnormalities

Question 25

3. Polyostotic fibrous dysplasia in association with endocrinopathies (McCune-Albright syndrome): Although precocious sexual development is the most common endocrine abnormality, other disorders such as ------------- may be seen. Females are affected much more than males. The process often is --------- and affects the ------------- on the altered side.

Answer 25

hyperthyroidism, excess growth hormone, and primary adrenal hyperplasia unilateral majority of the bones

Question 26

Osteopetrosis (marble bone disease) is a group of rare hereditary disorders characterized by --------- with resultant defective ----------.

Answer 26

deficient osteoclastic activity bone remodeling

Question 27

Osteopetrosis Both -------- forms are seen with an associated variable presentation and severity.

Answer 27

autosomal dominant and autosomal recessive

Question 28

Manifestations of DM • Vasculopathy o Responsible for

Answer 28

80% of DM-related deaths

Question 29

Manifestations of DM • Vasculopathy o --------- is severe and accelerated ----------- seen

Answer 29

Atherosclerosis o Myocardial infarction and stroke

Question 30

Manifestations of DM • Vasculopathy o ----------- of lower extremities is 100-fold increased over normal population

Answer 30

Gangrene

Question 31

Manifestations of DM • Vasculopathy o Thickened -----------, especially around small blood vessels (microangiopathy)

Answer 31

basement membrane

Question 32

Manifestations of DM • Kidneys (diabetic nephropathy) o --------- behind vascular diseases, leads to HTN and ESRD

Answer 32

2nd leading cause b

Question 33

Manifestations of DM Kidneys o Glomerular lesions • Diffuse ------------ • 90% of diabetics within 10 years; not specific to diabetics • --------- around --------- and deposition of matrix

Answer 33

glomerulosclerosis Microangiopathy glomerular capillaries

Question 34

Manifestations of DM Kidneys Glomerular lesions See:

Answer 34

• Proteinuria, total renal failure

Question 35

Manifestations of DM Kidneys o Nodular glomerulosclerosis

Answer 35

* 15-30% of long-term diabetics; specific to diabetics * Ball-like deposition of matrix at the periphery of the glomerulus * Total renal failure * Renal atherosclerosis * Pyelonephritis

Question 36

Manifestations of DM • Eye (retinopathy) o 4th leading cause of ---------

Answer 36

blindness

Question 37

Manifestations of DM • Eye (retinopathy) See:

Answer 37

o Microangiopathy and microaneurysms | o Retinal detachment and vision loss

Question 38

* Gastrinoma | * Zollinger-Ellison Syndrome

Answer 38

III. Islet Cell Tumors

Question 39

III. Islet Cell Tumors • --------------- • Most are from the --------- • may be ------------

Answer 39

Uncommon exocrine pancreas functional or nonfunctional

Question 40

* Gastrinoma | * Arise in ------------------

Answer 40

duodenum, peripancreatic tissues, or pancreas

Question 41

Gastrinoma see • ---------- hypersecretion

Answer 41

gastric acid • 90-95% of recalcitrant peptic ulcers

Question 42

* Zollinger-Ellison Syndrome | * Pancreatic islet cell tumor, hypersecretion of----------

Answer 42

gastric acid, severe peptic ulcers

Question 43

• Zollinger-Ellison Syndrome Most are -------------, require ------------

Answer 43

malignant (60%) surgical resection

Question 44

B. Pancreatic Neoplasms 1. Cystic e.g.

Answer 44

serous cystadenomas, females over males, 2:1, usually seventh decade

Question 45

2. Pancreatic Cancers “infiltrating ----------- of the pancreas”

Answer 45

ductal carcinomas

Question 46

B. Pancreatic cancers • Precursor lesion is -------------- • Often remain silent until ------------- Pain is usually the first sign, but typically too late. • Trousseau sign: Migratory thrombophlebitis-formation of platelet aggregation factors and procoagulants from tumor or its necrotic products.

Answer 46

PanINS (pancreatic intraepithelial neoplasias) invade into adjacent structures.

Question 47

B. Pancreatic cancers | ------------ is associated with tumors at the --------------

Answer 47

Obstructive jaundice pancreatic head.

Question 48

Pancreatic cancers | • Trousseau sign:

Answer 48

Migratory thrombophlebitis-formation of platelet aggregation factors and procoagulants from tumor or its necrotic products.

Question 49

2. Chronic pancreatitis • Develop ------------, with ensuing ---------- and eventual destruction of the ------------.

Answer 49

irreversible destruction exocrine pancreas fibrosis endocrine parenchyma

Question 50

2. Chronic pancreatitis | • Etiology unclear:

Answer 50

Alcoholism, biliary disease, hypercalcemia, hyperlipidemia, oxidative stress, idiopathic? genetic?

Question 51

2. Chronic pancreatitis • Morphology:

Answer 51

Reduced acini, chronic inflammation, fibrosis, obstruction ducts, spare islets

Question 52

2. Chronic pancreatitis • Diagnosis-requires a high degree of

Answer 52

suspicion-may have already destroyed acinar cells, so may not see an increase in serum amylase.