Internal Medicine
Hepatology
Jaundice

Jaundice

Introduction

Jaundice is a crucial topic for MCCQE1 preparation, particularly in the context of Canadian healthcare. This comprehensive guide will cover the essential aspects of jaundice, focusing on its relevance to the Canadian medical licensing exam and clinical practice in Canada.

💡

Jaundice is the yellowish discoloration of the skin, sclera, and mucous membranes due to hyperbilirubinemia (serum bilirubin >50 μmol/L).

Epidemiology in the Canadian Context

  • Jaundice affects approximately 60% of term and 80% of preterm newborns in Canada.
  • In adults, the prevalence varies depending on the underlying cause.
  • Certain Indigenous populations in Canada have a higher incidence of Gilbert's syndrome, affecting jaundice presentation.

Pathophysiology

Understanding the pathophysiology of jaundice is crucial for MCCQE1 success. Let's break it down into key steps:

Step 1: Bilirubin Production

  • Bilirubin is primarily produced from the breakdown of heme in senescent red blood cells.
  • Normal production rate: 250-300 mg/day in adults.

Step 2: Bilirubin Transport

  • Unconjugated bilirubin is bound to albumin in the blood.
  • It's transported to the liver for conjugation.

Step 3: Hepatic Processing

  • Bilirubin is taken up by hepatocytes.
  • Conjugation occurs via UDP-glucuronosyltransferase (UGT) enzyme.

Step 4: Excretion

  • Conjugated bilirubin is excreted into bile.
  • It's then eliminated in feces as urobilinogen.

Classification of Jaundice

Jaundice can be classified based on the stage of bilirubin metabolism affected:

Increased bilirubin production (e.g., hemolysis)

Clinical Presentation

Canadian medical students should be familiar with the following clinical features for MCCQE1 preparation:

  1. Yellowing of skin and sclera
  2. Dark urine (in conjugated hyperbilirubinemia)
  3. Pale stools (in obstructive jaundice)
  4. Pruritus (more common in cholestatic jaundice)
  5. Associated symptoms depending on underlying cause (e.g., abdominal pain, fever, weight loss)
🇨🇦

In the Canadian healthcare system, primary care physicians play a crucial role in the initial assessment of jaundice. Familiarity with the CanMEDS framework, particularly the "Medical Expert" and "Communicator" roles, is essential for effective patient management.

Diagnostic Approach

For MCCQE1 success, remember this systematic approach to jaundice diagnosis:

  1. History:

    • Onset and duration of jaundice
    • Associated symptoms
    • Risk factors (e.g., alcohol use, hepatitis exposure)
    • Medication history
    • Travel history (relevant for certain infectious causes)
  2. Physical Examination:

    • Assess extent and distribution of jaundice
    • Look for signs of chronic liver disease
    • Check for hepatosplenomegaly
    • Evaluate for signs of infection
  3. Laboratory Tests:

    • Liver function tests (ALT, AST, ALP, GGT)
    • Bilirubin (total and direct)
    • Complete blood count
    • Coagulation profile
  4. Imaging:

    • Ultrasound (first-line in Canada for biliary obstruction)
    • CT or MRI/MRCP (if ultrasound inconclusive)
  5. Additional Tests (as indicated):

    • Hepatitis serology
    • Autoimmune markers
    • Genetic testing (e.g., for Gilbert's syndrome)

Canadian Guidelines for Jaundice Management

The Canadian Association for the Study of the Liver (CASL) provides guidelines for managing various liver conditions that can cause jaundice. Key points include:

  1. Neonatal Jaundice:

    • Universal bilirubin screening for all newborns
    • Use of the Canadian Paediatric Society's guidelines for phototherapy initiation
  2. Viral Hepatitis:

    • Screening high-risk populations for Hepatitis B and C
    • Timely referral to a hepatologist for antiviral therapy
  3. Alcoholic Liver Disease:

    • Emphasis on alcohol cessation counseling
    • Nutritional support and consideration of corticosteroids in severe cases
  4. Drug-Induced Liver Injury:

    • Prompt discontinuation of offending agent
    • Careful monitoring and supportive care

Treatment Approaches

Treatment of jaundice in Canada focuses on addressing the underlying cause:

  1. Hemolytic Jaundice:

    • Treat underlying condition (e.g., autoimmune hemolytic anemia)
    • Consider splenectomy in hereditary spherocytosis
  2. Hepatocellular Jaundice:

    • Supportive care
    • Specific treatments for viral hepatitis, autoimmune hepatitis, etc.
  3. Obstructive Jaundice:

    • ERCP or surgical intervention for stone removal or stent placement
    • Treatment of underlying malignancy if present

MCCQE1 High-Yield Topic

Pay special attention to the management of acute cholangitis, a potentially life-threatening complication of obstructive jaundice. The Tokyo Guidelines, widely used in Canada, provide a severity assessment and management algorithm.

Key Points to Remember for MCCQE1

  1. Differentiate between conjugated and unconjugated hyperbilirubinemia
  2. Recognize the importance of fractionated bilirubin in diagnosis
  3. Understand the role of ultrasound as the first-line imaging modality in Canada
  4. Be familiar with Canadian guidelines for managing common causes of jaundice
  5. Know the indications for specialist referral in the Canadian healthcare system
  6. Understand the unique aspects of jaundice management in Indigenous populations

Sample MCCQE1-Style Question

# Sample Question

A 28-year-old woman presents to her family physician with a 2-week history of jaundice and pruritus. She denies alcohol use and has no history of liver disease. Physical examination reveals yellowing of the sclera and skin, along with multiple excoriations. Laboratory tests show:

- Total bilirubin: 120 μmol/L (normal <17 μmol/L)
- Direct bilirubin: 90 μmol/L (normal <5 μmol/L)
- ALT: 65 U/L (normal <35 U/L)
- AST: 60 U/L (normal <35 U/L)
- ALP: 450 U/L (normal 30-120 U/L)
- GGT: 380 U/L (normal <40 U/L)

Which one of the following is the most appropriate next step in management?

- [ ] A. Order hepatitis B and C serology
- [ ] B. Perform liver biopsy
- [ ] C. Start ursodeoxycholic acid
- [ ] D. Arrange abdominal ultrasound
- [ ] E. Refer for ERCP

Explanation

The correct answer is:

  • D. Arrange abdominal ultrasound

This patient presents with obstructive jaundice, as evidenced by the predominantly conjugated hyperbilirubinemia (direct bilirubin 90 μmol/L) and markedly elevated ALP and GGT. In the Canadian healthcare system, abdominal ultrasound is the first-line imaging modality for evaluating biliary obstruction. It's non-invasive, readily available, and can identify common causes such as gallstones or biliary dilation.

Option A (hepatitis serology) is less likely given the predominantly obstructive picture. Option B (liver biopsy) is too invasive as an initial step. Option C (ursodeoxycholic acid) may be used in certain cholestatic conditions but is premature without a diagnosis. Option E (ERCP) is therapeutic and would only be considered after diagnostic imaging.

This question tests the candidate's ability to interpret liver function tests, recognize the pattern of obstructive jaundice, and apply Canadian guidelines for appropriate initial management.


References

  1. Canadian Association for the Study of the Liver. (2018). Clinical Practice Guidelines for the Management of Adult Patients with Acute and Chronic Liver Diseases. Canadian Liver Journal, 1(2), 47-59.

  2. Canadian Paediatric Society. (2021). Guidelines for detection, management and prevention of hyperbilirubinemia in term and late preterm newborn infants. Paediatrics & Child Health, 26(1), 13-14.

  3. Friedman, L. S. (2021). Approach to the patient with abnormal liver biochemical and function tests. UpToDate. Retrieved from https://www.uptodate.com/contents/approach-to-the-patient-with-abnormal-liver-biochemical-and-function-tests (opens in a new tab)

  4. Moyer, V., Freese, D. K., Whitington, P. F., et al. (2004). Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology and Nutrition, 39(2), 115-128.

  5. Stapelbroek, J. M., van Erpecum, K. J., Klomp, L. W., & Houwen, R. H. (2010). Liver disease associated with canalicular transport defects: current and future therapies. Journal of Hepatology, 52(2), 258-271.