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12. CP - Diseases of the Hepatobiliary system and Pancreas > (60) Disease of the pancreas > Flashcards

(60) Disease of the pancreas Flashcards

1
Q

Describe the exocrine function of the pancreas

A

Most of pancreas (85%)

  • Secrets digestive enzymes (trypsin, lipase, phospholipase, elastase, amylase) which require activation
  • secretes from glands through ducts to duodenum
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2
Q

Which digestive enzyme secreted by the pancreas is a marker of pancreatic inflammation?

A

Amylase

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3
Q

Describe the endocrine function of the pancreas

A
  • islets of Langerhans

- secrete peptide hormones into the blood eg. insulin and glucagon

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4
Q

Which cells secrete insulin and glucagon?

A

Islets of langerhans

glucagon = alpha cells
insulin = beta cells
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5
Q

What is pancreatitis?

A

Inflammation of the pancreas (can be acute or chronic)

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6
Q

What are the clinical features of acute pancreatitis?

A
  • sudden onset of severe abdo pain radiating to back
  • nausea and vomiting
  • may be mild (recovery within 5-7 days) but can be serious with high mortality
  • raised serum amylase/lipase (over 3x normal)
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7
Q

Serum levels of what are raised in acute pancreatitis?

A

Amylase/lipase

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8
Q

What causes 50% of cases of acute pancreatitis?

A

Gallstones

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9
Q

What causes 25% of cases of acute pancreatitis?

A

Alcohol

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10
Q

What are the more rare causes of acute pancreatitis?

A
  • vascular insufficiency
  • viral infections eg. mumps, coxsackie B
  • hypercalcaemia
  • ERCP
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11
Q

How many cases of acute pancreatitis are idiopathic?

A

10%

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12
Q

List the causes of acute pancreatitis

A
  • gallstones
  • alcohol
  • vascular insufficiency
  • viral infections (mumps, coxsackie B)
  • hypercalcaemia
  • ERCP
  • idiopathic
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13
Q

Why do you get increased serum amylase in acute pancreatitis?

A

There is leakage and activation of pancreatic enzymes - amylase released into the blood

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14
Q

Describe the pancreas in mild acute pancreatitis

A

Swollen gland with fat necrosis

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15
Q

Describe then pancreas in severe acute pancreatitis

A

Swollen, necrotic gland with fat necrosis and haemorrhage

Grey Turner’s sign and Cullen’s sign

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16
Q

What do you get in fat necrosis?

A

Chalky white material containing calcium salts that have been freed up by lipase-mediated cleavage of fatty acids

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17
Q

What is Grey Turner’s sign? (seen in severe acute pancreatitis)

A

Haemorrhage into the subcutaneous tissues of the flank

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18
Q

What is Cullen’s sign?

A

Superficial oedema and bruising in the subcutaneous fatty tissue around the umbilicus

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19
Q

Other than swelling, necrosis and haemorrhage, what else do you get in acute pancreatitis?

A
  • hypocalcaemia (fatty acids bind calcium ions)
  • hyperglycaemia
  • abscess formation
  • pseudocysts
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20
Q

What are the complications associated with acute pancreatitis?

A
  • shock
  • intravascular coagulopathy
  • haemorrhage
  • pseudocysts
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21
Q

What are pseudocysts in acute pancreatitis?

A

Collections of pancreatic juice secondary to duct rupture

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22
Q

What is chronic pancreatitis?

A

Progressive inflammatory disorder in which parenchyma of the pancreas is destroyed and replaced by fibrous tissue
- irreversible destruction of the exocrine tissue, followed by destruction of the endocrine tissue

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23
Q

What 2 conditions does chronic pancreatitis lead to?

A

Malnutrition and diabetes

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24
Q

Describe the ways in which acute and chronic pancreatitis are not two distinct disease but rather a continuum

A
  • recurrent acute can develop chronic pancreatitis
  • overlapping causative factors
  • both genetic and environmental
  • experimental protocols can be modified to induce each condition
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25
Q

Most cases of chronic pancreatitis are due to what?

A

Alcohol or idiopathic (when exclusion cystic fibrosis)

26
Q

Other than alcohol, what else is a strong independent risk factor for pancreatitis?

A

Cigarette smoking

27
Q

What genetic mutations can cause chronic pancreatitis?

A
  • CFTR
  • PRSS1
  • SPINK 1
28
Q

What are the toxic causes of chronic pancreatitis?

A
  • alcohol
  • smoking
  • drugs
  • hypercalcaemia
  • hyperparathyroidism
  • infections
29
Q

List the causes of chronic pancreatitis

A
  • toxic (alcohol, smoking, hypercalcaemia etc)
  • genetic
  • obstruction of main duct (cancer, scarring)
  • recurrent acute pancreatitis
  • autoimmune
  • idiopathic
30
Q

Describe the clinical features of chronic pancreatitis

A
  • repeated episodes of acute pancreatitis
  • intermittent abdo pain, back pain and weight loss
  • fibrosis of exocrine tissue - can mimic carcinoma macroscopically and microscopically
31
Q

What are the complications associated with chronic pancreatitis?

A
  • malabsorption of fat (lack of lipases)
  • diabetes (late feature)
  • pseudocysts
  • stenosis of common bile duct/duodenum
  • mortality of 50% within 20-25 years
32
Q

Chronic pancreatitis leads to malabsorption of fat due to lack of lipases. What does this cause?

A
  • steatorrhoea
  • impairment of fat soluble vitamins absorption (vitamins A, D, E and K)
  • diarrhoea, weight loss, and cachexia
33
Q

Which are the fat-soluble vitamins?

A

Vitamins A, D, E and K

34
Q

What is the mortality rate of chronic pancreatitis?

A

Nearly 50% within 20-25 years of disease onset

35
Q

What is seen on a scan in chronic pancreatitis?

A
  • localised, irregular involvement of the gland early on, later global atrophy
  • dilated and distorted ducts
  • calculi (especially she alcohol-induced)
  • fatty replacement
  • pseudocyst formation
36
Q

What is the most common type of pancreatic cancer? (90% of all pancreatic neoplasms)

A

Pancreatic adenocarcinoma (ductal adenocarcinoma) - PDAC

37
Q

Who gets pancreatic adenocarcinoma?

A

1.3 males: 1 female

60-80 years, rare before 40

38
Q

What is the prognosis of pancreatic adenocarcinoma?

A
  • 5 year survival = 4%
  • Mean survival untreated = 3-5 months/10-20 months with surgery
  • only 10-20% resectable at time of diagnosis
  • almost universally fatal
39
Q

What are the risk factors for pancreatic adenocarcinoma?

A
  • CIGARETTE SMOKING
  • heavy alcohol intake
  • diet rich in red meats
  • obesity
  • hereditary cancer syndromes (10%)
  • chronic pancreatitis
40
Q

Where is the pancreas does pancreatic adenocarcinoma affect?

A
  • head of pancreas (60-70%)
  • tail (10-15%)
  • body (5-15%)
  • diffuse involvement (5-15%)
41
Q

What are the signs and symptoms of pancreatic adenocarcinoma?

A
  • epigastric pain, radiating to back
  • weight loss
  • painless jaundice
  • pruritus
  • nausea
  • Trousseau’s syndrome
  • Courvoisier’s sign
  • diabetes
42
Q

What is pruritus? (symptom of pancreatic adenocarcinoma)

A

Severe itching of the skin

43
Q

What is Trousseau’s sign of malignancy? (sign of pancreatic adenocarcinoma)

A

Thrombophlebitis which is recurrent or appearing in different locations over time = migratory thrombophlebitis

44
Q

What is Courvoisier’s law?

A

States that in the presence of a palpably enlarged gallbladder which is nontender and accompanied with mild painless jaundice, the cause is unlikely to be gallstones (palpable gallbladder without pain = sign of pancreatic adenocarcinoma)

45
Q

In pancreatic adenocarcinoma, there may be distant metastases where?

A
  • liver
  • peritoneum
  • lung
46
Q

What are the poor prognostic factors of pancreatic adenocarcinoma?

A
  • extension of tumour outside of the pancreas
  • metastatic spread to local lymph nodes
  • vascular and perineural invasion
  • grade of differentiation
47
Q

What hereditary cancer syndromes are associated with 10-15% of pancreatic adenocarcinomas?

A
  • HNPCC
  • FAMMM
  • familial breast cancer
  • ataxia-telangiectasis
  • von hippel-lindau
  • familial pancreatitis
  • peutz-jeghers
48
Q

Name another type of pancreatic cancer

A

Pancreatic neuroendocrine tumours (less than 3% of all pancreatic neoplasms)

49
Q

What is the causes of recent increasing incidence of pancreatic neuroendocrine tumours?

A

Improvements in imaging and increased awareness (so increased diagnosis)

50
Q

Who gets pancreatic neuroendocrine tumours?

A

20-60 years

M=F

51
Q

There is an increased risk of pancreatic neuroendocrine tumours in which syndromes?

A
  • MEN1

- von Hippel Lindau

52
Q

What are the features of well-differentiated neuroendocrine tumours?

A
  • uncommon
  • any age, rare in children
  • 7-13% multiple (MEN-1)
  • 15-35% non-functioning
  • single tumours often produce multiple hormones but usually single hyper-functional syndrome
53
Q

What are the features of poorly differentiated neuroendocrine carcinomas?

A
  • rare
  • males more than females
  • 40-75 years
  • advanced disease at presentation
  • prognosis without treatment = 1-2 months
  • prognosis with chemotherapy = up to 50 months
54
Q

Name 6 types of functioning PENs

A
  • insulinoma
  • glucagonoma
  • gastrinoma
  • somatostatinoma
  • VIPoma
  • PP-cell PEN
55
Q

What cell type do each of the functioning PENs form in?

A
  • insulinoma = B cell
  • glucagonoma = a cell
  • gastrinoma = G cell
  • somatostatinoma = delta cell
  • VIPoma = unknown
  • PP-cell PEN = PP cells
56
Q

What syndrome occurs in insulinoma and what are the clinical findings?

A

Insulinoma syndrome

- hypoglycaemia

57
Q

What syndrome occurs in glucagonoma and what are the clinical findings?

A

Glucagonoma syndrome

  • stomatitis
  • rash
  • diabetes
  • weight loss
  • hyperglycaemia
58
Q

What syndrome occurs in gastrinoma and what are the clinical findings?

A

Zollinger-Ellison syndrome

  • peptic ulcer
  • diarrhoea

(gastrin produces stomach acid)

59
Q

What syndrome occurs in stomatostatin and what are the clinical findings?

A

Somatostatinoma syndrome

  • diabetes
  • cholelithiasis
  • hypochlorhydria
60
Q

What syndrome occurs in VIPoma and what are the clinical findings?

A

Verner-Morrison syndrome

  • diarrhoea
  • hypokalaemia
  • achlorhydria
61
Q

What syndrome occurs in PP-cell PEN and what are the clinical findings?

A

None

62
Q

Describe the macroscopic features of pancreatic neuroendocrine tumours?

A
  • usually well-circumscribed, sometimes encapsulated
  • solid
  • 10- over 50mm
  • occur anywhere in pancreas
  • can be multiple

12. CP - Diseases of the Hepatobiliary system and Pancreas (5 decks)

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