Conjugated Hyperbilirubinemia Overview
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Questions and Answers

What is the primary consequence of drug-induced liver injury?

The primary consequence is hyperbilirubinemia along with elevated liver enzymes.

List two factors that may contribute to total parenteral nutrition (TPN)-induced cholestasis.

Excessive calories and micronutrient deficiencies are two contributing factors.

How does TPN potentially lead to cholestasis?

TPN can lead to cholestasis through excessive caloric intake and possible bacterial translocation from the gut.

What is one major difference between benign recurrent intrahepatic cholestasis (BRIC) and drug-induced liver injury?

<p>BRIC resolves spontaneously without significant liver damage, unlike drug-induced liver injury.</p> Signup and view all the answers

What treatment is commonly used for Wilson disease, which is related to liver injury?

<p>Cupper chelation is commonly used for treating Wilson disease.</p> Signup and view all the answers

What is the typical age of fatality for untreated type I bilirubin metabolic disorder?

<p>About age two years.</p> Signup and view all the answers

In the context of liver injury, what role do drugs play in causing hyperbilirubinemia?

<p>Drugs can cause liver injury leading to hyperbilirubinemia and elevated liver enzymes.</p> Signup and view all the answers

How does phenobarbital affect patients with type II bilirubin metabolism issues?

<p>It can induce the expression of UGT.</p> Signup and view all the answers

Describe the initial symptoms associated with primary biliary cirrhosis.

<p>Initial symptoms typically include pruritus, fatigue, and jaundice.</p> Signup and view all the answers

What infection is commonly associated with AIDS cholangiopathy?

<p><em>Cryptosporidium parvum</em> is the organism most commonly associated with AIDS cholangiopathy.</p> Signup and view all the answers

Identify the primary management strategy for Gilbert syndrome.

<p>Management is centered on reassurance.</p> Signup and view all the answers

What condition is associated with conjugated hyperbilirubinemia in the context of hepatitis?

<p>Hepatitis (viral, alcoholic, autoimmune).</p> Signup and view all the answers

What is the possible outcome of biliary obstruction, and name one cause?

<p>Can lead to conjugated hyperbilirubinemia; one cause is infections.</p> Signup and view all the answers

Describe the genetic mutation characteristic of Dubin-Johnson syndrome (DJS).

<p>It involves a mutation in the gene responsible for MRP2 protein.</p> Signup and view all the answers

What is a significant risk associated with progressive scarring of bile ducts in Primary Sclerosing Cholangitis (PSC)?

<p>Increased risk for cholangiocarcinoma.</p> Signup and view all the answers

What distinguishes Rotor syndrome from Dubin-Johnson syndrome?

<p>Rotor syndrome is similar but has a different genetic basis.</p> Signup and view all the answers

What are the potential effects of drugs such as rifamycin on bilirubin levels, and how can this effect be reversed?

<p>Drugs like rifamycin inhibit bilirubin uptake by the liver, leading to increased levels of unconjugated bilirubin; this effect can be reversed upon cessation of the drug.</p> Signup and view all the answers

Describe how congestive heart failure can lead to unconjugated hyperbilirubinemia.

<p>Congestive heart failure impairs the delivery of bilirubin to the liver, resulting in decreased hepatic uptake and leading to increased unconjugated bilirubin levels.</p> Signup and view all the answers

Discuss the mechanism through which ineffective erythropoiesis increases unconjugated bilirubin production.

<p>Ineffective erythropoiesis leads to rapid hemoglobin turnover and destruction of developing erythroid cells, increasing the production of unconjugated bilirubin up to 70%.</p> Signup and view all the answers

What is Crigler-Najjar syndrome and how does it lead to unconjugated hyperbilirubinemia?

<p>Crigler-Najjar syndrome is an autosomal-recessive disorder caused by a genetic alteration affecting bilirubin-UGT, leading to a nearly complete loss of bilirubin conjugation and high levels of unconjugated bilirubin.</p> Signup and view all the answers

How can phototherapy be used in the context of Crigler-Najjar syndrome type I?

<p>Phototherapy is used to reduce high levels of unconjugated bilirubin in individuals with Crigler-Najjar syndrome type I, often following emergent plasma exchange for kernicterus.</p> Signup and view all the answers

Identify the role of underlying conditions in the development of unconjugated hyperbilirubinemia.

<p>Underlying conditions like cirrhosis or portosystemic shunts can impair bilirubin uptake by the liver, contributing to unconjugated hyperbilirubinemia.</p> Signup and view all the answers

Explain the implications of gallstone formation in patients with prolonged unconjugated hyperbilirubinemia.

<p>Prolonged unconjugated hyperbilirubinemia can lead to the precipitation of bilirubin salts, resulting in the formation of gallstones.</p> Signup and view all the answers

What role does hepatic clearance play in managing unconjugated hyperbilirubinemia?

<p>Decreased hepatic clearance due to conditions like cirrhosis can exacerbate unconjugated hyperbilirubinemia, necessitating management of the hepatic condition to improve bilirubin clearance.</p> Signup and view all the answers

Study Notes

Conjugated Hyperbilirubinemia

  • Primary biliary cirrhosis (PBC): autoimmune disease affecting small intrahepatic ducts
  • Benign recurrent intrahepatic cholestasis (BRIC): rare, autosomal-recessive or sporadic disorder with recurrent episodes of intense pruritus and jaundice
  • AIDS cholangiopathy: syndrome of biliary obstruction caused by infection-induced strictures of the biliary tract
  • Total parenteral nutrition(TPN)-induced cholestasis: multifactorial, likely caused by excessive calories with deficiencies in micronutrients and possible bacterial translocation from the gut
  • Wilson disease: autosomal-recessive disease involving copper deposition in multiple tissues, including the brain and liver
  • Drugs: many drugs can cause liver injury resulting in hyperbilirubinemia associated with elevated liver enzymes
  • Chronic hepatitis: can also contribute to unconjugated hyperbilirubinemia
  • Hepatitis: (viral, alcoholic, autoimmune) associated with conjugated hyperbilirubinemia

Liver Infiltration

  • Can lead to conjugated hyperbilirubinemia

Biliary Obstruction

  • Caused by:
    • Gallstones
    • Carcinoma
    • Choledocholithiasis
    • Strictures
    • Benign tumors

Primary Sclerosing Cholangitis (PSC)

  • Progressive inflammation and scarring of bile ducts, often associated with inflammatory bowel disease
  • Treatment is mainly supportive
  • Associated with increased risk for cholangiocarcinoma
  • Liver transplant used for end-stage liver disease

Congential cystic dilations of the bile duct

  • Typically associated with intermittent abdominal pain, jaundice, and right upper quadrant mass
  • Risk of malignancy
  • Treatment usually surgical

Infections

  • Associated with conjugated hyperbilirubinemia:
    • Cryptosporidium parvum
    • Chlamydia trachomatis
    • Ascaris lumbricoides
    • Clonorchis sinensis

Inherited Disorders

  • DJS: autosomal-recessive disease with a mutation in the gene responsible for the human canalicular multispecific organic anion transporter (cMOAT) protein, also known as the multidrug resistance protein 2 (MRP2)
  • Rotor syndrome: very similar to DJS, but with high urinary levels of coproporphyrin

Unconjugated Hyperbilirubinemia

Increased bilirubin production via hemolysis and dyserythropoiesis

  • Hemolysis: increased destruction of red blood cells
  • Dyserythropoiesis: ineffective erythropoiesis
  • Causes: thalassemia major, megaloblastic anemia, congenital erythropoietic porphyria, lead poisoning

Decreased Hepatic Clearance

  • Causes:
    • Congestive heart failure
    • Cirrhosis/portosystemic shunts
    • Certain drugs

Defective bilirubin conjugation

  • Inherited Disorders:
    • Crigler-Najjar syndrome types I and II: autosomal-recessive disorder caused by an alteration of the coding region of the gene responsible for producing bilirubin-UGT
    • Gilbert syndrome: decreased UGT activity, benign, no medical therapy needed

Other Causes for Defective Bilirubin Conjugation

  • Ethinyl estradiol
  • Hyperthyroidism

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Introduction to Laboratory Diagnostics PDF

Description

Explore the various conditions associated with conjugated hyperbilirubinemia, including primary biliary cirrhosis, benign recurrent intrahepatic cholestasis, and AIDS cholangiopathy. This quiz covers the mechanisms, pathophysiology, and related drug-induced liver injuries. Test your knowledge on these serious liver conditions and their implications.

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