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Patient case: Secondary Biliary Cirrhosis

Discussion

Name: E.F

Age: 48 years old

Gender: Female

Occupation: Office manager

Medical History: 

History of cholelithiasis (gallstones)

Previous cholecystectomy (gallbladder removal) 5 years ago

Hypertension, managed with medication

No significant family history of liver disease

Presenting Complaints:

E.F presents to the clinic with complaints of progressive fatigue, unintentional weight loss of 8 kg over the past 3 months, and generalized itching (pruritus) that has worsened over the past month. She also reports yellowing of her skin and eyes (jaundice). There has been no recent history of alcohol consumption or medication changes.

She also complains of intermittent upper abdominal discomfort, particularly after meals, but denies severe pain. E.F states that she has been feeling more fatigued lately, which has affected her work and daily activities.

Clinical Examination:

General Appearance: Jaundiced sclera and mild yellowing of the skin.

Vital Signs: 

Blood pressure: 130/85 mmHg

Pulse: 88 bpm

Temperature: 37.2°C

Respiratory rate: 18 breaths per minute

Abdominal Examination: 

Tenderness in the upper right quadrant, but no rebound tenderness.

No palpable masses.

No signs of ascites.

Laboratory Tests:

Liver function tests:

Elevated bilirubin (total: 3.5 mg/dL, direct: 2.2 mg/dL)

Elevated alkaline phosphatase (ALP: 450 U/L)

Elevated gamma-glutamyl transferase (GGT: 320 U/L)

Normal AST and ALT levels.

Hepatitis panel: Negative for hepatitis A, B, and C.

Ultrasound:

No gallstones noted.

Evidence of intrahepatic biliary dilatation suggestive of obstructed bile ducts.

No signs of cirrhosis at this stage, but suspicious of bile duct injury or obstruction.

Diagnosis:

The clinical presentation, history of gallstones, and imaging findings suggest a diagnosis of secondary biliary cirrhosis, likely due to prior biliary tract obstruction, possibly related to a common bile duct injury or stenosis following cholecystectomy.

Plan:

1. Referral to gastroenterology/hepatology for further evaluation.

2. Magnetic resonance cholangiopancreatography (MRCP) to assess the biliary tree for strictures or stones.

3. Management of pruritus with cholestyramine or ursodeoxycholic acid.

4. Vitamin supplementation (vitamin A, D, E, and K) due to fat malabsorption.

5. Monitor liver function tests regularly to assess for progression of liver disease.

6. Consider liver biopsy if there is concern for advancing cirrhosis.

7. Discussion of potential liver transplant if cirrhosis progresses.

Questions
1. What is the most likely cause of secondary biliary cirrhosis in this patient?

A) Chronic alcohol use

B) Hepatitis B infection

C) Previous cholelithiasis and cholecystectomy

D) Autoimmune hepatitis

2. Which of the following lab results is most consistent with secondary biliary cirrhosis in this case?

A) Elevated ALT and AST, normal bilirubin

B) Elevated bilirubin, alkaline phosphatase, and gamma-glutamyl transferase

C) Low albumin, low bilirubin, high AST

D) Normal liver function tests and bilirubin

3. Which imaging modality would be most helpful in further evaluating this patient’s condition?

A) Chest X-ray

B) Ultrasound of the abdomen

C) CT scan of the abdomen

D) Magnetic Resonance Cholangiopancreatography (MRCP)

4. Which of the following is a key management strategy for secondary biliary cirrhosis in this patient?

A) Immediate liver transplantation

B) Referral to cardiology for evaluation

C) Treating pruritus and monitoring liver function

D) High-dose corticosteroids

Reveal answers

Answers

1. C) Previous cholelithiasis and cholecystectomy

• Secondary biliary cirrhosis often occurs after bile duct obstruction, which can be caused by previous gallbladder disease or surgery, as in this patient.

2. B) Elevated bilirubin, alkaline phosphatase, and gamma-glutamyl transferase

• These are the key lab findings typically seen in biliary tract obstruction and cholestasis, consistent with secondary biliary cirrhosis.

3. D) Magnetic Resonance Cholangiopancreatography (MRCP)

• MRCP is the preferred non-invasive imaging method to evaluate biliary tract abnormalities, including intrahepatic bile duct dilation and obstructions.

4. C) Treating pruritus and monitoring liver function

• The management of secondary biliary cirrhosis includes treating symptoms like pruritus and regular monitoring of liver function, as well as managing complications that arise from biliary obstruction.