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cholestasis
Absence or reduction of bile flow from the liver.
Etiology:
1) biliary tract obstruction
a) choledocholithiasis
b) biliary stricture
c) pancreatic carcinoma
d) neoplasm in region of ampulla of Vater
e) pancreatitis
f) biliary atresia
g) biliary cyst
2) drug-induced cholestasis
a) androgens
b) estrogens
c) carbamazepine
d) clavulanate
e) diazepam
f) disulfiram
g) erythromycin
h) haloperidol
i) oral hypoglycemics
j) penicillamine
k) propoxyphene
l) phenothiazines
3) right-sided heart failure
4) cholestasis of pregnancy
5) hepatobiliary neoplasms
a) primary benign & malignant tumors
b) metastatic tumors
6) primary biliary cirrhosis
7) cholangitis
Genetics:
- defects in ABCB11 may cause chronic intrahepatic cholestasis without obvious familial history of chronic liver disease
- genetic variations in ABCB11 may play a role in drug-induced cholestasis
Clinical manifestations:
1) pruritus may precede biochemical abnormalities
2) jaundice
3) severe cholestasis may result in steatorrhea
Laboratory:
1) liver function tests
a) significantly elevated alkaline phosphatase & gamma-glutamyltransferase
b) mildly abnormal aminotransferases
2) endoscopic retrograde cholangiopancreatography (ERCP)
3) liver biopsy
Radiology:
1) ultrasound imaging of liver & biliary tract
2) computed tomography (CT) can identify mass lesions of the liver & biliary tree
3) cholangiography
Management:
1) vitamin K SC may improve PT in the absence of hepatic dysfunction
2) decompression with relief of extrahepatic obstruction
3) pruritus due to intrahepatic obstruction
a) cholestyramine
b) antihistamine
c) ursodeoxycholic acid or ursodiol (Actigall)
- especially useful in patients with primary biliary cirrhosis
d) phototherapy with UV-B radiation
Related
biliary obstruction
cholangitis
cholestatic jaundice
Specific
benign recurrent intrahepatic cholestasis (BRIC, Summerskill syndrome)
cholestatic hepatitis
familial intrahepatic cholestasis
intrahepatic cholestasis of pregnancy (obstetric cholestasis)
General
sign/symptom
biliary disease
References
- Saunders Manual of Medical Practice, Rakel (ed), WB Saunders,
Philadelphia, 1996, pg 364
- Medical Knowledge Self Assessment Program (MKSAP) 11, American
College of Physicians, Philadelphia 1998