Internal Medicine

0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:19

Primary Sclerosing Cholangitis Prion Disorders 1217

■cholangiocarcinoma: in approximately 30% of patients; more preva-

lent with long-standing PSC; no proven therapy although OLT

increases survival in specific cases

■biliary obstruction: antibiotics either for acute episodes of cholan-

gitis secondary to sludge/stones or strictures, or prior to endoscopic

intervention; chronic antibiotic therapy may be indicated in certain

patients with intrahepatic disease

■therapeutic ERCP and dilatation with/without temporary stenting

of dominant bile duct strictures

■rule out cholangiocarcinoma with biliary brushings and biopsies as

indicated (yield is <50%); CA 19–9 not validated as a screening marker

for cholangiocarcinoma

■pruritus: cholestyramine, 4 grams QID spaced 4 hours apart from

other oral medications

■rifampin 150 mg BID/TID

■naltrexone 50 mg daily, for resistant cases

■liver transplantation for uncontrollable pruritus

■osteoporosis: calcium 1500 mg daily supplementation, vitamin D

1000 IU daily; bone mineral density annually; regular exercise

■calcium and or vitamin D: side effects encountered when hypercal-

cemia occurs

■absolute contraindications: hypercalcemia, hypervitaminosis D

Prognosis

■8-year survival of asymptomatic patients 80%

■8-yr survival symptomatic patient 50%

■cirrhosis diagnosed at initial presentation has associated poor sur-

vival

■survival after OLT excellent (≥90% at one year without cholangiocar-

cinoma), although disease recurrence has been reported to occur in

up to 25% of patients

PRION DISORDERS (CREUTZFELDT-JAKOB DISEASE)

CHAD CHRISTINE, MD

history & physical

Sporadic Form: May Present W/Diffuse or Localized Neurologic

Dysfunction

■Onset at any age

■Dementia develops in all cases