Internal Medicine - Disorders of Pancreas Flashcards
Which enzyme gene mutation causes hereditary pancreatitis?
A) carbonic anhydrase
B) cationic trypsin
C) phospholipase
D) kallikrein
E) hexokinase
B) cationic trypsin
EXPLANATION
Cationic trypsin mutations are responsible for two-thirds of hereditary pancreatitis, mutations in other enzymes do not cause pancreatitis. Bile flow is blocked by the obstructed and compressed choledochus by a pancreas head cancer. The cholecyst is intact, stretching, but it cannot compensate to the obstructive icterus. In the other diseases the cholecyst does not expand, because it is inflamed and ill, or there is no distal choledochus obstruction.
The main functions of pancreatic ductal cells, except:
A) to guarantee a shell for th acinic cells
B) to excrete a liquid rich in bicarbonate
C) to bring the enzymes produced by the acini into the duodenum
D) to neutralize the protons produced by acini through bicarbonate secretion
E) to excrete a liquid rich in sodium and chloride ions
E) to excrete a liquid rich in sodium and chloride ions
EXPLANATION
The main functions of pancreatic ductal cells are to guarantee a shell for the acinic cells and to excrete a bicarbonate rich liquid, which neutralize the protons produced by acini and keep enzymes in an inactive state and bring them from the acinic cells into the duodenum. Sodium and chlorine-rich liquids are produced by acini.
It is true for the enzymes produced by pancreas:
A) Three different enzymes are produced by acini: amylase, trypsin and lipase
B) The enzymes are activated under physiological conditions in the pancreatic duct.
C) Lipase is activated by the enzyme enterokinase in the duodenum.
D) Enzymes are produced in inactive state in acini and under physiological conditions they only become activ in duodenum.
E) Most of the enzymes are produced by pancreatic ductal cells.
D) Enzymes are produced in inactive state in acini and under physiological conditions they only become activ in duodenum.
EXPLANATION
Pancreatic acini produce more than 20 types of enzymes, which get into the pancreatic duct in an inactive state. Under physiological conditions, enterokinase secreted by the duodenum activates trypsinogen, which turns into active trypsin. Activated trypsin then activates the other enzymes.
It is true for acute pancreatitis, except:
A) Antibiotic therapy should always be used due to the risk of infection.
B) The lipase level increases in the blood.
C) Usually it is induced by gallstone or excessive alcohol consumption.
D) It leads to abdominal pain.
E) The amylase level increases in the blood.
F) The inflammation can also spread to other organs in severe cases.
A)
Antibiotic therapy should always be used due to the risk of infection.
EXPLANATION
Acute pancreatitis develops mainly due to gallstones or excessive alcohol consumption. Usually it is accompanied by abdominal pain, amylase and lipase elevations in the blood. In severe cases, the disease may affect several organs. It is clear, however, that antibiotic therapy should not be initiated in all cases.
In case of acute pancreatitis, the first thing to do is:
A) performing a CT scan
B) performing an ERCP test
C) starting fluid replacement
D) surgical console
E) performing an MRCP test
C)
starting fluid replacement
EXPLANATION
In case of acute pancreatitis, the most important to do is to start fluid replacement immediately.
It is true for the distant complications of acute pancreatitis, except:
A) Lungs, kidneys and liver are the most common affected.
B) They are not related to the severity of the disease.
C) Migratory leukocytes, cytokines and free radicals are primarily responsible for their development.
D) The inflammation of organs may also lead to dysfunction and may cause multiple organ failure (MOF).
E) In severe cases, intensive therapy may be also needed.
B) They are not related to the severity of the disease.
EXPLANATION
During the progress of the inflammation, from the localized pancreatitis may develop a generalized multiple organ inflammation, which always refers to the severity of the inflammation. Lungs, kidneys and liver are the most common affected. Migratory leukocytes, cytokines and free radicals are primarily responsible for the spread of this inflammation to the whole body. The inflammation of organs may also lead to dysfunction and may cause multiple organ failure (MOF).
It refer to the severity of the acute necrotizing pancreatitits, except:
A) Ranson’s prognostic criteria
B) Glasgow prognostic criteria
C) Apache II. prognostic criteria
D) amylase elevation
E) markers of the inflammatory reaction (TNF-α, IL-6, CRP etc.)
F) CT findings (Balthazar-score) with contrast agent enhancement
G) clinical picture
H) infected necrosis
D) amylase elevation
EXPLANATION
Amylase elevation is not proportional to the severity of pancreatitis, in case of an extensive necrosis, enzyme levels may be low, because there is no more enzyme in the acinic cells, however, very high values can be found in case of mild pancreatitis with papillitis.
It is characteristic for the SPINK1 gene:
A) It is located on chromosome 8.
B) It encodes a protein called pancreatic secretory trypsin inhibitor.
C) The production of the protein that it encodes is essential for the activation of trypsin.
D) Its mutation leads in all cases to pancreatitis.
E) It often occurs in pancreatitis with hereditary or familial accumulation.
B) It encodes a protein called pancreatic secretory trypsin inhibitor.
EXPLANATION
The human SPINK1 gene is located on chromosome 5, it is 7.1 kilobases long, it contains 4 exons and it encodes a protein of 6.3 kilodaltons, a physiological inhibitor of trypsin named pancreatic secretory trypsin inhibitor. This genetic alteration is present in 16% of the idiopathic patients, but it rarely occurre in cases with hereditary or family accumulation. Unlike PRSS1 gene mutations, that can not be found in healthy people.
The most sensitive and specific imaging method is for the diagnosis of chronic pancreatitis:
A) transabdominal ultrasound
B) abdominal CT
C) endoscopic ultrasound
D) ERCP
E) native abdominal X-ray
F) irrigoscopy
D) ERCP
EXPLANATION
Endoscopic retrograd cholangiopancreatography is still the most sensitive and most specific of the listed imaging techniques in chronic pancreatitis, but it can be soon replaced by dynamic MRCP in this diagnostic function.
In the case of chronic pseudocysts, primarily selectable procedures are, excluded:
A) percutaneous drainage
B) endoscopic cystogastrostomy or cystoduodenostomy
C) transpapillar drainage of the pseudocyst
D) surgical cystogastrostomy posterior or cystoduodenostomy
E) surgical cystowirsungogastrostomy or cystowirsungo-jejunostomy
A) percutaneous drainage
EXPLANATION
Percutaneous drainage should not be selected for chronic pseudocysts, because the cyst comes back, since the cavity is usually connected with the pancreatic duct.
Mutations in the CFTR molecule in the pancreas can cause the following diseases or increase their risks, except:
A) cystic fibrosis
B) chronic pancreatitis
C) acute pancreatitis
D) pancreatic carcinoma
E) pancreatic division
E) pancreatic division
EXPLANATION
Mutations in the CFTR molecule can cause or increase the risk of pancreatitis or cystic fibrosis. The pancreatic division is an anatomical disorder, that is not related to the mutation of the CFTR gene.
n case of cystic fibrosis, the most endangering for the patient’s life is:
A) pancreatic insufficiency
B) gallstones
C) colonic stricture
D) pulmonary complications
E) meconium ileus
F) complications of diabetes mellitus
G) biliary cirrhosis
D) pulmonary complications
EXPLANATION
Lung complications endanger the life expectancy of a patient with mucoviscidosis the longest and most intense, the other complications cause the patient’s death rarely and only at a certain age
It is valid for the endoscopic retrograde cholangiopancreatography (ERCP):
1) It is an unlimited repeatable, uncomplicated procedure.
2) The obstruction of the choledochus and the pancreas line can be solved by an additional intervention.
3) It is always diagnostic in mild chronic pancreatitis as well.
4) It has a high sensitivity and specificity in case of pancreatic head tumors.
A) the 1., 2. and 3. answers are correct
B) the 1. and 3. answers are correct
C) the 2. and 4. answers are correct
D) only the 4. answer is correct
E) all of the answers are correct
C) the 2. and 4. answers are correct
EXPLANATION
The great advantage of ERCP is that in case of blockage of pancreatic and / or bile duct, a practised endoscopic specialist can solve the stricture with drainage, expansion or other operative endoscopic procedures at the same time as the diagnosis. However, the diagnostic value of this examination is also outstanding in case of pancreatic head tumors, because the so-called. double channel signal (combined local stricture of the choledochus and the pancreatic duct) refers to cancer with 95% safety.
It is characteristic of cystic fibrosis:
1) CFTR gene mutations with autosomal recessive inheritance cause cystic fibrosis disease
2) CFTR protein is a cationic channel located on pancreatic acinic cells
3) mutations reduce the amount or inhibit the function of CFTR protein
4) Cystic fibrosis is the rarest anomaly with recessive inheritance in the Caucasian population
A) the 1., 2. and 3. answers are correct
B) the 1. and 3. answers are correct
C) the 2. and 4. answers are correct
D) only the 4. answer is correct
E) all of the answers are correct
B) the 1. and 3. answers are correct
EXPLANATION
The CFTR gene encodes a membrane protein called cystic fibrosis transmembrane conductance regulator with size of 168 kilodaltons, that catalyzes the transport of chloride ions in the apical membrane of the exocrine glands. The gene is located on the long arm of chromosome 7, it is 250 kilobases long and contains 27 exons. Mutations of the CFTR gene with autosomal recessive inheritance cause mucoviscidosis or cystic fibrosis. Mutations reduce the amount or inhibit the function of CFTR protein, and thereby lead to the thickening of the glandular secretion, that may cause first of all disturbances in the lungs, pancreas, skin, reproductive organs, intestines and liver. Cystic fibrosis is the most common recessive inherited disorder in the Caucasian population, there is one case for every 2500 births.
It is valid for transabdominal pancreatic ultrasound diagnostics:
1) unlimited repeatable, relatively cheap
2) suitable for detecting fluid collections and pseudocysts
3) meteorism is not an obstacle
4) the accompanying bile disease can be well proven
5) edema is well separated from necrosis
6) obesity is not an obstacle
7) ductal diatation can be well recognized
A) the 1., 2. and 3. answers are correct
B) the 4., 5., 6. and 7. answers are correct
C) the 1., 2., 3. and 4. answers are correct
D) the 1., 2., 4. and 7. answers are correct
E) all of the answers are correct
D)
the 1., 2., 4. and 7. answers are correct
EXPLANATION
Ultrasound examination is not invasive, many patients can be examined in more shifts with one device. At the border of the liquid, the echo is sharply reflected, so the pseudocyst can be well defined, cholecysts can be examined, and a fluid-filled, expanded duct can be represented. However, meteorism and obesity make testing difficult, edema can not be confidently separated from necrosis.
It is valid for pancreatic computer tomography (CT):
1) unlimited repeatable, relatively cheap
2) suitable for detecting fluid collections and pseudocysts
3) obesity and meteorism are not obstacles
4) it also detects small parapapillary tumors
5) edema is well separated from necrosis
6) calcification and ductal diatation can be well recognized
A) the 1., 2., 3. and 6. answers are correct
B) the 3., 4. and 5. answers are correct
C) the 2., 3., 5. and 6. answers are correct
D) all of the answers are correct
C) the 2., 3., 5. and 6. answers are correct
EXPLANATION
CT also shows fluid collections well based on density differences. Obesity and meteorism do not disturb the contrast of the image. After administration of an intravenous contrast agent, the density of the live parenchyma increases, the „enhancement” of the non-living tissue is missing. The calcification is particularly piercingly visible, but the line expansion is also well represented. In contrast, it causes radiation strain and is more expensive than ultrasound, in addition, small papillary tumors are difficult to detect near the duodenum.
It is characteristic of pancreatic tumors:
1) the five-year survival rate is below 5%
2) it usually begins with jaundice and severe spastic abdominal pain
3) it mainly origins from acinic cells
4) it never causes pancreatitis
5) it responds well to cytostatics
A) the 1., 2. and 3. answers are correct
B) the 1., 2. and 4. answers are correct
C) the 1., 2. and 5. answers are correct
D) only the 1. answer is correct
E) the 3. and 4. answers are correct
D)
only the 1. answer is correct
EXPLANATION
Pancreatic tumors mainly origin from the ductal cells, their prognosis is not good at all. The five-year survival rate is below 5%. They usually develop silently and asymptomatic. The first symptom is often the jaundice of the patient. Cytostatic therapy can lengthen the survival time only minimal. In case of severe stricture of the pancreatic duct pancreatitis may be induced.
