Bile Acid Metabolism Quiz

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Questions and Answers

Which lesion primarily affects the metabolism of bile acids?

  • Canalicular membrane
  • Inflamed bile canaliculi
  • Microsomal biotransfer system (correct)
  • Hepatocyte membrane

What is the primary clinical manifestation of bile secretion and excretion disturbance?

  • Cirrhosis
  • Cholestasis
  • Acute hepatitis
  • Jaundice (correct)

The alteration of which bilirubin stage is rarely isolated?

  • Pigment excretion
  • Conjugation
  • Capture (correct)
  • Excretion

Gilbert syndrome is characterized by a deficiency in what activity?

<p>Conjugation of bilirubin (B)</p> Signup and view all the answers

What is a common cause of acquired deficiency in glucuronide conjugation?

<p>Toxic hepatocyte lesions (A)</p> Signup and view all the answers

Which syndrome is NOT associated with inherited bilirubin excretion defects?

<p>Acute hepatitis (C)</p> Signup and view all the answers

What is the primary effect of excess bile salts on hepatocytes?

<p>Damage to the hepatocytic membrane (C)</p> Signup and view all the answers

Mixed hyperbilirubinemia primarily involves which fraction of bilirubin?

<p>Direct (conjugated) bilirubin (A)</p> Signup and view all the answers

What primarily contributes to the hypocoagulability observed in chronic liver diseases?

<p>Decreased synthesis of coagulation factors (D)</p> Signup and view all the answers

Which of the following factors is least likely to be low in advanced liver cirrhosis?

<p>Factor VIII (C)</p> Signup and view all the answers

What is a common cause of thrombocytopenia in chronic liver diseases?

<p>Hypersplenism (C)</p> Signup and view all the answers

Which of the following accurately describes a outcome of disseminated intravascular coagulation (DIC) in liver disease?

<p>Excessive fibrinolysis (B)</p> Signup and view all the answers

What describes the role of plasminogen in liver disease-related coagulation?

<p>Serves as a substrate for plasmin (B)</p> Signup and view all the answers

Which coagulation factor is primarily affected by vitamin K deficiency in liver diseases?

<p>Factor II (D)</p> Signup and view all the answers

What is the consequence of hypersplenism in chronic liver disease?

<p>Decreased platelet count (B)</p> Signup and view all the answers

How does fibrinolysis typically behave in hepatopathies?

<p>Excessive in most cirrhotics (B)</p> Signup and view all the answers

What is the more frequently involved mechanism in the pathogenesis of hepatogenic diabetes?

<p>Resistance to exogenous insulin (C)</p> Signup and view all the answers

What happens when ketonemia exceeds 70 mg/dl?

<p>Hyperketonemia occurs (A)</p> Signup and view all the answers

In chronic hepatopathy, which factor contributes to the decrease in albumin concentration?

<p>Increased catabolism of albumins (D)</p> Signup and view all the answers

What role does the liver have in the synthesis of globulins during chronic alpha and beta hepatitis?

<p>Increase in globulin synthesis (B)</p> Signup and view all the answers

What metabolic alteration occurs when the liver is unable to utilize amino acids effectively?

<p>Increase in blood amino acid levels (B)</p> Signup and view all the answers

What effect do ethanol and prednisone have on lipid metabolism?

<p>Increased hepatic steatosis (B)</p> Signup and view all the answers

In mechanical jaundice, what is the primary change observed in the blood concentration?

<p>Increased concentration of bile salts (B)</p> Signup and view all the answers

What is a consequence of the destruction of the hepatocyte mass?

<p>Altered lipid and protein metabolism (B)</p> Signup and view all the answers

Which of the following factors synthesized exclusively in the liver does not directly relate to amino acid degradation?

<p>Serum cholinesterase (C)</p> Signup and view all the answers

What happens to blood ammonia levels in the case of parenchymal insufficiency of the liver?

<p>They increase. (B)</p> Signup and view all the answers

Which metabolism is least explored in the context of liver function despite its importance?

<p>Carbohydrate metabolism (C)</p> Signup and view all the answers

Which of the following substances is released into the blood as a result of liver cell cytolysis during acute hepatitis?

<p>Iron (B)</p> Signup and view all the answers

In the case of cholestasis, which of the following changes in serum levels is typically observed?

<p>Increased serum cholesterol and bile salts (D)</p> Signup and view all the answers

Which of the following is not a typical consequence of extensive liver cytolysis?

<p>Normalization of substance values (C)</p> Signup and view all the answers

In the context of liver inflammation due to infectious hepatitis, which test is specifically useful for assessing immune reactions?

<p>Protein immunoelectrophoresis (D)</p> Signup and view all the answers

What is the effect of liver cirrhosis on liver enzyme values?

<p>Normal or slightly elevated enzyme values without necrosis (B)</p> Signup and view all the answers

What is the primary enzyme deficiency associated with Cori type I glycogenosis?

<p>Glucose-6-phosphatase (C)</p> Signup and view all the answers

What characterizes the storage of glycogen in Cori type II / Pompe disease?

<p>In lysosomes (B)</p> Signup and view all the answers

Which of the following conditions is NOT associated with reduced glycogen content in cells?

<p>Cori type I disease (B)</p> Signup and view all the answers

What is the nature of hyaline as described in the content?

<p>Homogeneous, translucent, and eosinophilic substance (C)</p> Signup and view all the answers

Fibrinoid degeneration exhibits which of the following characteristics?

<p>Acidophilic and homogeneous composition (A)</p> Signup and view all the answers

What is a common cause of the deposition of Mallory hyaline bodies in liver cells?

<p>Chronic alcoholism (C)</p> Signup and view all the answers

Amyloid degeneration typically involves the deposition of proteins characterized by:

<p>Extracellular storage with common morpho-tinctorial traits (D)</p> Signup and view all the answers

Which organ is primarily affected by fibrinoid dystrophy, particularly in systemic lupus erythematosus (SLE)?

<p>Liver (B)</p> Signup and view all the answers

Which serological manifestation is associated with the autoimmune effects of viral hepatitis (VH) infection?

<p>Anti-smooth muscle antibodies (B), Rheumatoid factor (D)</p> Signup and view all the answers

What is the role of antiHBc – IgM in diagnosing active HBV infection?

<p>Indicates active infection (D)</p> Signup and view all the answers

Which of the following is NOT a renal manifestation associated with hepatitis viral infections?

<p>Diabetic nephropathy (D)</p> Signup and view all the answers

Which autoimmune manifestation is linked to HCV infection in the neurologic context?

<p>Peripheral neuropathy (A)</p> Signup and view all the answers

What type of autoimmune effect can be caused by the interferon treatments in patients with HCV?

<p>Alopecia (D)</p> Signup and view all the answers

Which lymphoproliferative manifestation is associated with VH infection?

<p>Monoclonal gammopathy (B)</p> Signup and view all the answers

What autoimmune condition may arise due to hepatitis C infection affecting the gastrointestinal system?

<p>Ischemic colitis (D)</p> Signup and view all the answers

Which autoimmune condition is NOT included among the manifestations of VH infection?

<p>Vitamin D deficiency (C)</p> Signup and view all the answers

Flashcards

Hepatogenic Diabetes

A condition where the liver cannot produce enough insulin or properly use insulin produced by the pancreas. This leads to high blood sugar levels.

Increased Growth Hormone (STH) in Hepatogenic Diabetes

Increased levels of growth hormone in the blood, which can contribute to insulin resistance and high blood sugar.

Increased Glucagon in Hepatogenic Diabetes

Excess glucagon levels in the blood, contributing to insulin resistance and increased blood sugar. This can be due to overproduction or decreased breakdown of glucagon.

Decreased Albumin Production in Liver Disease

The liver’s inability to produce enough albumin, which is crucial for maintaining blood volume and pressure.

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Hepatic Steatosis

An abnormally high level of triglycerides in the liver, leading to fatty liver disease. This can result from various metabolic deficiencies.

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Hyperketonemia in Liver Disease

The build-up of ketone bodies in the blood, a result of increased fat breakdown. It can occur when the body cannot use ketone bodies for energy, often due to severe liver dysfunction.

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Increased Alpha & Beta Globulins in Liver Disease

An overproduction of alpha and beta globulins, often seen in chronic liver disease. This can lead to hypergammaglobulinemia.

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Increased Amino Acid Levels in Liver Disease

A condition where the body accumulates excess amino acids in the blood, leading to their excretion in urine. This occurs when the liver cannot properly process amino acids.

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Hypocoagulability

A state of reduced blood clotting ability, often found in people with chronic liver disease.

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Hepatocellular Insufficiency (Hepatoprivy Syndrome)

A primary cause of hypocoagulability in chronic liver diseases: the liver's inability to produce essential clotting factors like fibrinogen and prothrombin complex.

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Vitamin K Deficiency

Another cause of hypocoagulability in chronic liver disease. The liver cannot absorb fat-soluble vitamins like Vitamin K, which are crucial for blood clotting.

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Hypersplenism

A condition where the spleen removes too many platelets from circulation, leading to lower platelet count and potential clotting issues.

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Fibrinolysis

The breakdown of fibrin clots, which can occur in chronic liver diseases, contributing to hypocoagulability.

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Disseminated Intravascular Coagulation (DIC)

A process where small blood clots form throughout the bloodstream, leading to consumption of clotting factors, platelet depletion, and potential bleeding problems.

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Hepatic Clearance Mechanism Insufficiency

The liver's inability to clear harmful substances from the bloodstream, leading to accumulation and potential clotting issues.

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Thrombasthenia

A condition where platelets cannot clump together properly, impairing clot formation and contributing to bleeding.

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What are the locations that can be affected in hepatopathies?

A process where various parts of the liver can be damaged, affecting the secretion and excretion of bile.

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What is Gilbert syndrome?

An enzyme deficiency that results in an inability to conjugate bilirubin, leading to high levels of unconjugated bilirubin in the blood.

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What is an alteration of bilirubin conjugation?

The inability to conjugate bilirubin, leading to high levels of unconjugated bilirubin in the blood.

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What is an alteration of bilirubin excretion?

This condition is caused by an impaired ability to excrete conjugated bilirubin into the bile, leading to high levels of conjugated bilirubin in the blood.

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What is Dubin-Johnson syndrome?

An inherited disorder that affects the excretion of bilirubin, leading to elevated levels of conjugated bilirubin.

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What is Hyperbilirubinemia?

Elevated levels of bilirubin in the blood, which can be caused by problems with capturing, conjugating, or excreting bilirubin.

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What is Mixed Hyperbilirubinemia?

This condition is characterized by increased levels of conjugated bilirubin, suggesting a problem with the excretion of bilirubin.

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What are 5-nucleotidase, GGT, and LAP?

A group of enzymes that are involved in the breakdown of nucleotides, which can be elevated in cases of liver damage.

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Localized Glycogenoses

A metabolic disorder characterized by excessive glycogen storage in the cytoplasm and sometimes the nucleus of cells, often associated with inflammation, anemia, or leukemia.

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Generalized Glycogenoses

Group of inherited conditions caused by deficiencies in enzymes involved in glycogen metabolism, leading to abnormal glycogen accumulation in various organs.

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Cori Type I

A type of generalized glycogenoses characterized by deficiency in glucose-6-phosphatase enzyme, resulting in impaired glucose production and hypoglycemia.

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Cori Type II (Pompe Disease)

A type of generalized glycogenoses caused by deficiency of lysosomal acid alpha-glycosidase, leading to glycogen accumulation in lysosomes, primarily affecting muscle and heart.

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Hyaline

A structural protein or glycoprotein that appears homogeneous, translucent, and eosinophilic under a microscope. It's often found in tissues, particularly extracellularly.

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Mallory Hyaline Bodies

A type of hyaline deposition found in the cytoplasm of liver cells, particularly in conditions like viral hepatitis or chronic alcoholism.

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Fibrinoid

An acellular substance characterized by its affinity for acid dyes, often granular in appearance, and associated with inflammation and tissue damage.

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Amyloid

A group of proteins that deposit extracellularly in tissues, often forming amyloid plaques. They have characteristic staining properties and a complex fibrillar structure.

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Protein Catabolism

The process of breaking down proteins into amino acids within the body.

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Protein Synthesis

The process of creating new proteins from individual amino acids.

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Urea

A substance produced in the liver that plays a role in removing ammonia from the blood.

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Ureogenesis

The process by which the liver converts ammonia into urea to be excreted from the body.

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Albumin

A type of blood protein primarily made by the liver. Its levels are often tested to assess liver health.

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Serum Cholinesterase

An enzyme produced in the liver, its levels can be altered in liver disease.

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Liver Enzymes (GPT, GOT, OCT, GGT, LDH)

Enzymes released into the bloodstream when liver cells are damaged.

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Fibrinogen

A type of protein that helps with blood clotting. Levels can decrease in liver disease.

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HBsAg blocked in immune complexes

Immune complexes block circulating HBsAg, causing severe hepatitis. This can lead to fulminant hepatitis, which may reappear later after corticosteroid therapy.

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Mutant HBsAg

HBsAg can mutate, making it unrecognizable by standard antibody tests.

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Anti-HBc IgM and active infection

In cases of mutated HBsAg, anti-HBc IgM is the primary indicator of active infection.

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Autoimmune manifestations in viral hepatitis

The presence of antinuclear antibodies (ANAs) and other autoantibodies is associated with various autoimmune manifestations in patients with viral hepatitis.

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Autoimmune disorders associated with viral hepatitis

Viral hepatitis can lead to a spectrum of autoimmune disorders, including musculoskeletal, lymphoproliferative and glandular complications.

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Antiphospholipid syndrome and platelet disorders

Antiphospholipid syndrome, a disorder involving antibodies that target phospholipids, can occur in association with viral hepatitis and contribute to platelet abnormalities.

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Multi-organ complications of viral hepatitis

Viral hepatitis, particularly HCV, can manifest in various ways, including renal, endocrine and dermatological complications.

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Autoimmune reactions to interferon therapy

In patients with HCV, interferon therapy can trigger autoimmune manifestations, including endocrine and dermatological issues.

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Study Notes

Biochemical Exploration of the Liver

  • The liver is sensitive to metabolic, toxic, microbial, circulatory, and neoplastic aggressions. Liver damage can be primary (e.g., viral hepatitis, hepatocellular carcinoma) or secondary (e.g., heart failure, cancer, alcoholism, extrahepatic infections).
  • Liver function reserve is high, so early liver damage often shows few clinical symptoms.
  • However, as the pathological process progresses, functional imbalance can lead to life-threatening consequences.

Alteration of Carbohydrate Metabolism

  • Severe liver disease affects glucose production and utilization.
  • Mechanisms affected include glycogenolysis, gluconeogenesis, glycogenogenesis, and triglyceride synthesis.
  • These changes can cause hypoglycemia, hyperglycemia, or glucose intolerance.
  • Cirrhosis and alcoholism decrease glycogen levels in liver cells and reduce glucose uptake.

Alteration of Lipid Metabolism

  • In metabolic deficiencies, lipids affect the liver, leading to steatosis (fatty liver).
  • Excess fatty acids are converted into ketone bodies.
  • Increased ketone levels can overwhelm tissues like skeletal and cardiac muscle.
  • Severe liver failure reduces cholesterol synthesis, increasing cholesterol levels (hypercholesterolemia). This is especially seen in cholestasis (reduced bile flow).
  • Reduced synthesis and/or excretions of certain bile acids occurs as well.
  • Elevated bile salts in the blood may lead to hypocalcemia and coagulopathy.

Alteration of Protein Metabolism

  • Chronic liver disease, especially cirrhosis, causes a reduction in albumin concentration.
  • This reduction can be due to decreased synthesis, altered distribution, and increased breakdown.
  • The majority of broken down albumins move to the gastrointestinal tract.
  • The liver is important for synthesizing globulins, particularly alpha and beta fractions.
  • Persistent or aggressive forms of chronic hepatitis and cirrhosis can lead to an abnormally high amount of gamma globulins (hypergammaglobulinemia), often associated with a lympho-plasmacytic infiltration.

Disturbance of the Coagulation Mechanism

  • Chronic liver diseases lead to hypocoagulability due to decreased synthesis of coagulation factors (II, VII, IX, X).
  • Factors contributing to this effect include hepatocellular insufficiency.
  • A level of coagulation factors can be increased in some cases, however this does not appear to affect the coagulation deficit.
  • There may also be an increased consumption of coagulation factors.
  • The production of modified coagulation factors also plays a role in the coagulation problems.
  • There may be vitamin K deficiency, also affecting the process.
  • Platelet numbers may be decreased, resulting in altered clotting processes.
  • Fibrinolysis (breakdown of fibrin) may also be increased in some cases.

Disturbance of Secretion and Excretion of Bile

  • Liver lesions affect bile secretion and excretion at various locations.
  • These include the microsomal biotransfer system, hepatocyte membrane, desmosomes, canalicular membrane, and canaliculi.
  • Damage may lead to increased bilirubin, bile acids, and impairment by enzymes like ALP, 5-nucleotidase, GGT, and LAP.
  • The lesions that affect the structures result in an alteration of bile metabolism, and excretion.

Alterations in Bilirubin Metabolism

  • Disorders in bilirubin capture, conjugation, or excretion stages alter serum bilirubin levels.
  • These problems often relate to liver cell damage, and can also have other causes, such as conjugation deficiency (e.g in Gilbert syndrome).

Disturbances in Clearance and Detoxification Function

  • Liver function includes detoxification of endogenous substances (such as hormones and ammonia) and exogenous substances (drugs and toxins).
  • Pathological changes in the liver parenchyma and mesenchyme affect the liver's detoxification and clearance capacities leading to toxic syndromes.

Disturbance of Hydro-Electrolytic and Acid-Base Balance

  • Liver damage can affect the balance of electrolytes, especially potassium and sodium.
  • Potassium loss from hepatocytes and increased sodium intake result in hypokalemia, and secondary alkalosis.

Phospho-calcium Balance

  • Low calcium levels (hypocalcemia) are seen in some liver conditions.
  • This is due to issues in calcium transport, intake, absorption, and conversion of vitamin D3 into cholecalciferol .
  • Hypocalcemia is often associated with conditions like anorexia, severe hepatopathies.

Morphological Models of Hepatic Injury

  • The liver has limited capacity to respond to various aggressions.
  • Depending on the assault, possible consequences include degeneration, inflammation, regeneration, fibrosis, and necrosis.
  • These changes lead to various morphological models.

Pathological Consequences

  • Fatty degeneration, Hyaline degeneration, Amyloid degeneration
  • Alcoholic hepatitis, Focal necrosis, Massive necrosis, Granulomatous necrosis, Liver cirrhosis.

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