1. Identify salient historical information that my have a bearing to this patient's chief complaint. Elaborate on the significance of each.

• Emergency aorto-coronary bypass surgery

• Intraoperative hypotension and bleeding

• Gilbert's syndrome

• Post-op 2 days Jaundice

• Jaundice worsens in 8 days

• Appears well with no symptoms

• Chemistry values

• Ultrasound gall bladder

He has two stages to his illness one following surgery and another 4 years later

2. List salient findings in first ER visit  Elaborate on the significance of each.

• Severe epigastric pain with nausea and vomiting

• Chemistry

• 20 pound weight loss

3. List salient findings in his second  ER visit in 2 months  Elaborate on the significance of each.

• No follow-up with primary care Physician

• Recurrent severe epigastric pain

• Tenderness and guarding in RUQ

• diminished bowel sounds

• Liver function tests

• Alkaline phosphatase

• WBC count

4. What is Gilbert’s syndrome?  What would the  fractionation  of bilirubin show in patients with Gilbert’s?

• Gilbert’s syndrome is a genetic enzymatic defect in the liver resulting in mild, unconjugated hyerbilirubinemia.  

• Deficiency of bilirubin UDP-glucuronyltransferase activity.  

• Inherited as autosomal recessive.  5% incidence with male: female=4:1. 

• Total bilirubin <5 mg/dl.  Transient increase with fasting, intercurrent infections, ETOH, and physical activity. 

• Should rule out a chronic hemolysis such as spherocytosis.    

• Fractionation of bilirubin would show increased indirect fraction.  

5. Explain the pathophysiology behind Gilbert’s syndrome.

• This disorder is a defect of the uptake enzymes in the hepatocyte. 

• There is decreased uptake of circulating indirect bilirubin due to decrease UDP-glucoronyltransferase activity. 

• Also decreased hepatic transport resulting in an indirect hyperbilirubinemia seen in the laboratory.

6. Is Gilbert’s syndrome associated with disease?  What harm can result from chronically elevated Bilirubin?

• No associated  liver disease.  

• Bilrubin per se has no patho-physiologic consequence.

7. What is the most likely etiology for his Jaundice in post op hospitalization period?

The diagnosis is pot-operative jaundice .

It is usually self limited and is a result of the surgery (15% open heart cases) 

Multifactorial:  

• overproduction of bilirubin from hemolysis

• blood transfusions

• drugs

• anesthesia 

• ischemia

• hypoxia

• sepsis  

8. During the patient’s pot-op hospitalization, what additional tests should be performed?

• The laboratory picture suggests an obstructive process.  

• The patient feels well and is eating. 

• The only test indicated at this point in time is an ultrasound examination of the liver and biliary tree.

9. What is the significance of the ultrasound findings?

• There is sludge in the gallbladder but the ductal system of the liver and gallbladder are normal.  

• No further steps need to be taken for this GB sludge since patient is doing well and there is another explanation for the jaundice and mild liver abnormalities. 

10. What is sludge?

Embryonic stage of gallstones (cholesterol monohydrate crystals and calcium bilirubinate granules embedded in mucus gel).

11. What is the significance of the history of Gilbert's disease. Is it a factor in his Jaundice?

• Also in this patient, the Gilbert’s would be a factor.

12. Discuss a differential diagnosis for this patient when he visits the ER 4 years later for the first time.

• Patient presents with epigastric pain,  epigastric tenderness, nausea and vomiting.  

• Laboratory is normal but he lost 20# on a rapid weight loss diet. 

• The differential diagnosis includes:  cholecystitis, cholelithiasis, choledocholithiasis, pancreatitis, and peptic ulcer disease.  

• The rapid weight loss is very important and biliary tract disease is most likely in this patient.

13. What additional tests would you do as a result of this first ER visit?

The patient should have had an ultrasound exam of the liver and biliary tree.

14.  Discuss the laboratory result obtained during the second ER visit.

• On the second visit the patient presents with severe epigastric pain, fever, and RUQ guarding. 

• The liver panel is that of obstructive disease.  Amylase is normal.  

• There has been additional weight loss from his diet.  

• The clinical picture is that of cholecystitis/cholangitis with possible common duct stone.  

• He needs and emergent ultrasound exam to again evaluate the GB and ductal system.  He likely needs a surgical intervention at this point. 

15. What comments would you make regarding the rapid weight loss liquid protein diet?

These diets are problematic in that they promote gallbladder stasis and resultant in sludge/stone formation.

16. What definitive recommendations would you make for this patient at this time?  

The patient needs a cholecystectomy.  

• If he had pancreatitis and evidence of a common duct stone, an ERCP with papillotomy could be done initially with cholecystectomy at a later date.

• In this patient, the amylase is normal and if the US does not show dilated ducts, then one should proceed directly to cholecystectomy.

• Options for medical therapy with chenodeoxycholic acid or shock wave lithotripsy.