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Gen Path Final > Sweep 1.2 > Flashcards

Sweep 1.2 Flashcards

1
Q

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

A

5%

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2
Q

Medullary thyroid carcinoma

o derived from the

A

parafollicular (C) cells

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3
Q

Medullary thyroid carcinoma

o may be ————-

A

sporadic or familial (component of MEN syndromes)

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4
Q

Medullary thyroid carcinoma

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

A

RET proto-oncogene

serum calcitonin

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5
Q

II. Hyperparathyroidism

• An important cause of

A

hypercalcemia

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6
Q

II. Hyperparathyroidism

• Treatment: Surgical removal of ———-

A

hyperplastic parathyroid glands.

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7
Q

Hyperparathyroidism

Kidney transplant may be beneficial in patients with

A

hyperparathyroidism secondary to renal failure.

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8
Q

III. Hypoparathyroidism

• Clinical manifestations

A

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

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9
Q

o Glucagon:

A

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

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10
Q

o Insulin:

A

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

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11
Q

Presenting symptoms of DM

• Type 1

A

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

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12
Q

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 ———-

A

hypercalcemia

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

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13
Q

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 ——-

A

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

osteoblastic activity and bone formation

cellular activity (osteosclerotic stage).

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14
Q

Paget disease

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

A

leontiasis ossea

sarcomas (particularly osteosarcoma).

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15
Q

Paget disease

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

A

radiographic and microscopic appearances

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

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16
Q

Paget disease

Most patients have mild symptoms which can be controlled by

A

calcitonin or bisphosphonates and analgesics.

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17
Q
  1. Monostotic fibrous dysplasia (single bone): 70% of all cases. It usually manifests in ——– and often becomes stationary at puberty.
A

childhood

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18
Q

Monostotic fibrous dysplasia

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

A

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

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19
Q

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.

A

cancellous

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20
Q

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

A

Multiple bones

unilateral

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21
Q

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.

A

earlier age

22
Q

Polyostotic fibrous dysplasia without endocrine involvement

Frequently affected bones are

A

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

23
Q

Polyostotic fibrous dysplasia without endocrine involvement

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

A

Café-au-lait pigmentations

24
Q
  1. 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 ———–.
A

least common

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

endocrine abnormalities

25
Q
  1. 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.

A

hyperthyroidism, excess growth hormone, and primary adrenal hyperplasia

unilateral

majority of the bones

26
Q

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

A

deficient osteoclastic activity

bone remodeling

27
Q

Osteopetrosis

Both ——– forms are seen with an associated variable presentation and severity.

A

autosomal dominant and autosomal recessive

28
Q

Manifestations of DM
• Vasculopathy
o Responsible for

A

80% of DM-related deaths

29
Q

Manifestations of DM
• Vasculopathy
o ——— is severe and accelerated
———– seen

A

Atherosclerosis

o Myocardial infarction and stroke

30
Q

Manifestations of DM
• Vasculopathy

o ———– of lower extremities is 100-fold increased over normal population

A

Gangrene

31
Q

Manifestations of DM
• Vasculopathy

o Thickened ———–, especially around small blood vessels (microangiopathy)

A

basement membrane

32
Q

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

A

2nd leading cause b

33
Q

Manifestations of DM
Kidneys

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

A

glomerulosclerosis

Microangiopathy

glomerular capillaries

34
Q

Manifestations of DM
Kidneys
Glomerular lesions
See:

A

• Proteinuria, total renal failure

35
Q

Manifestations of DM
Kidneys
o Nodular glomerulosclerosis

A
  • 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
36
Q

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

A

blindness

37
Q

Manifestations of DM
• Eye (retinopathy)
See:

A

o Microangiopathy and microaneurysms

o Retinal detachment and vision loss

38
Q
  • Gastrinoma

* Zollinger-Ellison Syndrome

A

III. Islet Cell Tumors

39
Q

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

A

Uncommon

exocrine pancreas

functional or nonfunctional

40
Q
  • Gastrinoma

* Arise in ——————

A

duodenum, peripancreatic tissues, or pancreas

41
Q

Gastrinoma
see
• ———- hypersecretion

A

gastric acid

• 90-95% of recalcitrant peptic ulcers

42
Q
  • Zollinger-Ellison Syndrome

* Pancreatic islet cell tumor, hypersecretion of———-

A

gastric acid, severe peptic ulcers

43
Q

• Zollinger-Ellison Syndrome

Most are -------------, require  ------------
A

malignant (60%)

surgical resection

44
Q

B. Pancreatic Neoplasms

  1. Cystic e.g.
A

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

45
Q
  1. Pancreatic Cancers “infiltrating ———– of the pancreas”
A

ductal carcinomas

46
Q

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.

A

PanINS (pancreatic intraepithelial neoplasias)

invade into adjacent structures.

47
Q

B. Pancreatic cancers

———— is associated with tumors at the ————–

A

Obstructive jaundice

pancreatic head.

48
Q

Pancreatic cancers

• Trousseau sign:

A

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

49
Q
  1. Chronic pancreatitis

• Develop ————, with ensuing ———- and eventual destruction of the ————.

A

irreversible destruction exocrine pancreas

fibrosis

endocrine parenchyma

50
Q
  1. Chronic pancreatitis

• Etiology unclear:

A

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

51
Q
  1. Chronic pancreatitis

• Morphology:

A

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

52
Q
  1. Chronic pancreatitis

• Diagnosis-requires a high degree of

A

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

Gen Path Final (18 decks)

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