Primary Sclerosing Cholangitis

Presentation:
M.W., a 15yo male presents with jaundice and the following abnormal labs: Alk Phos- 497 unit/L, AST- 88 unit/L, ALT- 62 unit/L, Total bilirubin- 4.6 m/dL, Direct bilirubin- 3.4 mg/dL, GGT- 163 unit/L, WBC- 16 k/uL. Patient’s family reports a history of slowly increasing liver function tests and a recent history of fever. Patient’s family reports “yellow coloring has increased recently” and patient is slightly itchy.

Differential List:
Primary Sclerosing Cholangitis, Liver cancer

Diagnosis:
A CT and ultrasound of the abdomen were performed and no tumors were noted. A magnetic resonance cholangiopancreatography was performed next. The MRCP showed narrowing of several small branches of the bile ducts. Patient was then scheduled for an endoscopic retrograde cholangiopancreatography. Patient was diagnosed with PSC and acute cholangitis secondary to the PSC.

Treatment:
Patient was treated with a week course of IV Zosyn® for the cholangitis. During the ERCP two stents were placed to open the narrowed areas, and external bile drains were placed. Patient was also started on Questran® to reduce the itching secondary to hypercholesterolemia.

Outcome:
Patient’s acute cholangitis resolved following the course of IV antibiotics. Patient’s itching improved with the addition of Questran®. Patient is being followed in the outpatient clinic. Patient’s liver function tests are monitored closely on the outpatient level.

Case created by Melissa Walker, 2011.