Pancreas Pathology- Congenital Anomalies

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| By Agambino

Agambino

Community Contributor

Quizzes Created: 19 | Total Attempts: 14,601

Questions: 15 | Attempts: 342 | Updated: Mar 21, 2023

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Questions and Answers

1.

_________ ________ is an inherited disease of the exocrine glands beginning in infancy; characterized by viscous _________ and _________ of multiple glands including the pancreas.

Explanation
Cystic fibrosis is an inherited disease of the exocrine glands that typically starts in infancy. It is characterized by the production of thick and sticky secretions, which leads to dysfunction in multiple glands, including the pancreas.

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2.

Cystic fibrosis can lead to pancreatic ________ with majority of patients showing evidence of exocrine pancreatic __________.

Explanation
Cystic fibrosis is a genetic disorder that affects various organs in the body, including the pancreas. The condition can cause pancreatic insufficiency, which means that the pancreas is unable to produce enough digestive enzymes to properly break down food. This leads to difficulties in absorbing nutrients and can result in malnutrition. Additionally, cystic fibrosis can cause dysfunction in the exocrine function of the pancreas, further impairing its ability to secrete enzymes. Therefore, the majority of patients with cystic fibrosis show evidence of both pancreatic insufficiency and dysfunction.

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3.

True pancreatic cysts sonographically have the same characteristics of all "true" cysts.
- A.
  
  True
- B.
  
  False
Correct Answer
A. True

Explanation
True pancreatic cysts are characterized by having a well-defined wall, a fluid-filled cavity, and no solid components. These cysts are typically benign and can be detected through ultrasound imaging. The statement suggests that true pancreatic cysts share the same characteristics as other true cysts found in the body. Therefore, the answer "True" is correct.

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4.

True pancreatic cysts are usually found _________ with the pancreatic duct and are most commonly in the ______.

Correct Answer
continuous, head

Explanation
True pancreatic cysts are usually found in a continuous relationship with the pancreatic duct, meaning that they are connected to the duct and the fluid can flow freely between them. These cysts are most commonly located in the head of the pancreas, which is the part of the organ that is closest to the duodenum. This location is significant because cysts in the head of the pancreas can potentially cause obstruction or other complications due to their proximity to the bile duct and the duodenum.

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5.

True pancreatic cysts are associated with Von ______________ Syndrome.

Correct Answer
Hippel-Lindau

Explanation
True pancreatic cysts are associated with Von Hippel-Lindau Syndrome. This syndrome is a genetic disorder that causes the growth of tumors and cysts in various parts of the body, including the pancreas. These cysts are typically benign and filled with fluid. Von Hippel-Lindau Syndrome is caused by mutations in the VHL gene, which regulates the growth of blood vessels. Individuals with this syndrome have an increased risk of developing tumors and cysts in multiple organs, including the pancreas. Therefore, the correct answer is Hippel-Lindau.

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6.

Von Hippel-Lindau is a _________ acquired syndrome, associated with true _________ cysts, and is usually seen in _______ and __________.

Correct Answer
genetically, pancreatic, infancy, childhood

Explanation
Von Hippel-Lindau is a genetically acquired syndrome, meaning it is caused by genetic mutations that are passed down from parents to their children. It is associated with true pancreatic cysts, which are fluid-filled sacs that develop in the pancreas. This syndrome is usually seen in infancy and childhood, suggesting that symptoms and complications associated with Von Hippel-Lindau tend to appear early in life.

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7.

True pancreatic cysts are ___________ lined, true cysts of the pancreas, which are ______ and believed to be __________ in origin.

Correct Answer
epithelial, rare, congenital

Explanation
True pancreatic cysts are lined with epithelial cells, making them true cysts of the pancreas. These cysts are rare and are believed to be congenital in origin, meaning they are present from birth.

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8.

Ture pancreatic cysts represent anomalous development of the pancreatic ducts.
- A.
  
  True
- B.
  
  False
Correct Answer
A. True

Explanation
Pancreatic cysts are abnormal fluid-filled sacs that develop in the pancreas. True pancreatic cysts are considered to be a result of anomalous development of the pancreatic ducts. This means that there is a problem with the normal growth and formation of the ducts in the pancreas, leading to the development of cysts. Therefore, the statement "True pancreatic cysts represent anomalous development of the pancreatic ducts" is correct.

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9.

Multiple congenital cysts are associated with cystic disease of the ________, _________, _______, and __________. (alphabetically)

Correct Answer
kidneys, liver, pancreas, spleen

Explanation
Multiple congenital cysts are associated with cystic disease of the kidneys, liver, pancreas, and spleen. This means that individuals with multiple congenital cysts may have cystic abnormalities or diseases affecting these organs. These cystic diseases can cause various complications and impair the normal functioning of these organs.

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10.

Check all that apply to patient presentation of cystic fibrosis:
- A.
  
  Pancreatitis
- B.
  
  Most patients DO NOT live over 10
- C.
  
  Diagnosed late in life
- D.
  
  Most patients DO NOT live over 30
- E.
  
  Diagnosed early in life
Correct Answer(s)
A. Pancreatitis
D. Most patients DO NOT live over 30
E. Diagnosed early in life

Explanation
Cystic fibrosis is a genetic disorder that affects various organs in the body, including the pancreas. Pancreatitis, inflammation of the pancreas, is a common complication of cystic fibrosis. Additionally, most patients with cystic fibrosis do not live beyond the age of 30 due to the progressive nature of the disease. Diagnosis of cystic fibrosis typically occurs early in life, as it is often identified through newborn screening or when symptoms manifest in infancy or childhood.

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11.

Check all that apply to cystic fibrosis:
- A.
  
  Exocrine cells increase secretion of highly viscous mucus
- B.
  
  Decreased echogenicity
- C.
  
  Secretions coagulate and obstruct pancreatic ducts leading to degeneration, fibrosis, fatty replacement, and atrophy
- D.
  
  Increased echogenicity caused by fibrosis and fatty replacement-->atrophy
- E.
  
  Breakdown of pancreatic architecture
- F.
  
  Proximal distention of ducts and acini, leading to degeneration and replacement by small cysts
Correct Answer(s)
A. Exocrine cells increase secretion of highly viscous mucus
C. Secretions coagulate and obstruct pancreatic ducts leading to degeneration, fibrosis, fatty replacement, and atropHy
D. Increased echogenicity caused by fibrosis and fatty replacement-->atropHy
F. Proximal distention of ducts and acini, leading to degeneration and replacement by small cysts

Explanation
Cystic fibrosis is a genetic disorder that affects exocrine cells, causing them to increase the secretion of highly viscous mucus. This thick mucus can coagulate and obstruct the pancreatic ducts, leading to degeneration, fibrosis, fatty replacement, and atrophy of the pancreas. The increased echogenicity seen on imaging is caused by the fibrosis and fatty replacement, ultimately leading to atrophy. Additionally, the proximal distention of ducts and acini can occur, resulting in degeneration and replacement by small cysts.

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12.

When someone is severely affected with cystic fibrosis the pancreas is: shrunken with marked ________, has _______ replacement, and has cysts __________ to ductal obstruction.

Correct Answer(s)
fibrosis, fatty, secondary

Explanation
When someone is severely affected with cystic fibrosis, the pancreas undergoes fibrosis, which is the formation of excess fibrous connective tissue. This causes the pancreas to become shrunken. Additionally, there is fatty replacement, meaning that the normal pancreatic tissue is replaced by fatty tissue. This can further impair the function of the pancreas. Finally, the cysts in the pancreas are secondary to ductal obstruction, meaning that they occur as a result of blockages in the pancreatic ducts.

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13.

This is the MC congenital defect. It is a lack of fusion of dorsal and ventral buds and occurs in 10% of the population.

Correct Answer(s)
pancreatic divisum

Explanation
Pancreatic divisum is a congenital defect where there is a lack of fusion between the dorsal and ventral buds of the pancreas. This condition is relatively common, occurring in approximately 10% of the population. It can lead to complications such as recurrent pancreatitis and abdominal pain.

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14.

Drainage of the entire _________ pancreas is through the minor ________ with only the _______ part draining through the major papilla (ampulla of vater)

Correct Answer(s)
dorsal, papilla, ventral

Explanation
The drainage of the entire dorsal pancreas is through the minor papilla, while only the ventral part of the pancreas drains through the major papilla (ampulla of Vater). This means that the dorsal pancreas has its own separate drainage pathway through the minor papilla, while the ventral pancreas shares its drainage pathway with the common bile duct through the major papilla.

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15.

Because much of the drainage of the pancreas is through the _____ papilla, there is predisposition, in patients with ________ _________ to pancreatitis.

Correct Answer(s)
minor, pancreatic divisum

Explanation
The pancreas drains through the minor papilla, and patients with pancreatic divisum have a higher risk of developing pancreatitis. Pancreatic divisum is a condition where the ducts in the pancreas do not properly fuse during development, leading to impaired drainage and increased susceptibility to inflammation of the pancreas. Therefore, the combination of the minor papilla and pancreatic divisum increases the likelihood of pancreatitis in patients.

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Current Version
Mar 21, 2023

Quiz Edited by
ProProfs Editorial Team
Jan 26, 2011

Quiz Created by
Agambino

Pancreas Pathology- Congenital Anomalies

_____ is an inherited disease of the exocrine glands beginning in infancy; characterized by viscous _ and _____ of multiple glands including the pancreas.

Cystic fibrosis can lead to pancreatic with majority of patients showing evidence of exocrine pancreatic __.

True pancreatic cysts sonographically have the same characteristics of all "true" cysts.

True pancreatic cysts are usually found ___ with the pancreatic duct and are most commonly in the .

True pancreatic cysts are associated with Von ______________ Syndrome.

Von Hippel-Lindau is a _____ acquired syndrome, associated with true _ cysts, and is usually seen in _ and ________.

True pancreatic cysts are _ lined, true cysts of the pancreas, which are and believed to be __ in origin.

Ture pancreatic cysts represent anomalous development of the pancreatic ducts.

Multiple congenital cysts are associated with cystic disease of the ____, _, _, and ________. (alphabetically)

Check all that apply to patient presentation of cystic fibrosis:

Check all that apply to cystic fibrosis:

When someone is severely affected with cystic fibrosis the pancreas is: shrunken with marked ____, has _ replacement, and has cysts ________ to ductal obstruction.

This is the MC congenital defect. It is a lack of fusion of dorsal and ventral buds and occurs in 10% of the population.

Drainage of the entire ___ pancreas is through the minor with only the _ part draining through the major papilla (ampulla of vater)

Because much of the drainage of the pancreas is through the _ papilla, there is predisposition, in patients with _____ to pancreatitis.

Pancreas Pathology- Congenital Anomalies

_________ ________ is an inherited disease of the exocrine glands beginning in infancy; characterized by viscous _________ and _________ of multiple glands including the pancreas.

Cystic fibrosis can lead to pancreatic ________ with majority of patients showing evidence of exocrine pancreatic __________.

True pancreatic cysts sonographically have the same characteristics of all "true" cysts.

True pancreatic cysts are usually found _________ with the pancreatic duct and are most commonly in the ______.

True pancreatic cysts are associated with Von ______________ Syndrome.

Von Hippel-Lindau is a _________ acquired syndrome, associated with true _________ cysts, and is usually seen in _______ and __________.

True pancreatic cysts are ___________ lined, true cysts of the pancreas, which are ______ and believed to be __________ in origin.

Ture pancreatic cysts represent anomalous development of the pancreatic ducts.

Multiple congenital cysts are associated with cystic disease of the ________, _________, _______, and __________. (alphabetically)

Check all that apply to patient presentation of cystic fibrosis:

Check all that apply to cystic fibrosis:

When someone is severely affected with cystic fibrosis the pancreas is: shrunken with marked ________, has _______ replacement, and has cysts __________ to ductal obstruction.

This is the MC congenital defect. It is a lack of fusion of dorsal and ventral buds and occurs in 10% of the population.

Drainage of the entire _________ pancreas is through the minor ________ with only the _______ part draining through the major papilla (ampulla of vater)

Because much of the drainage of the pancreas is through the _____ papilla, there is predisposition, in patients with ________ _________ to pancreatitis.

_____ is an inherited disease of the exocrine glands beginning in infancy; characterized by viscous _ and _____ of multiple glands including the pancreas.

Cystic fibrosis can lead to pancreatic with majority of patients showing evidence of exocrine pancreatic __.

True pancreatic cysts are usually found ___ with the pancreatic duct and are most commonly in the .

Von Hippel-Lindau is a _____ acquired syndrome, associated with true _ cysts, and is usually seen in _ and ________.

True pancreatic cysts are _ lined, true cysts of the pancreas, which are and believed to be __ in origin.

Multiple congenital cysts are associated with cystic disease of the ____, _, _, and ________. (alphabetically)

When someone is severely affected with cystic fibrosis the pancreas is: shrunken with marked ____, has _ replacement, and has cysts ________ to ductal obstruction.

Drainage of the entire ___ pancreas is through the minor with only the _ part draining through the major papilla (ampulla of vater)

Because much of the drainage of the pancreas is through the _ papilla, there is predisposition, in patients with _____ to pancreatitis.