TY - JOUR
T1 - Disorders of bile formation and biliary transport
AU - Tomer, Gitit
AU - Shneider, Benjamin L.
PY - 2003/9
Y1 - 2003/9
N2 - A wide range of cholestatic liver diseases result from various primary defects in bile formation. Clinical features include jaundice, pruritus, failure to thrive, fat malabsorption, cholelithiasis, and variably progressive cirrhosis. Accurate diagnosis of these disorders is essential for determination of prognosis and selection of the most appropriate therapies. Severe genetic defects in canalicular bile acid and phospholipid excretion lead to progressive liver disease that often requires liver transplantation. Defects in bile acid biosynthesis and aminophospholipid transport may be responsive to medical or non-transplant surgical approaches.
AB - A wide range of cholestatic liver diseases result from various primary defects in bile formation. Clinical features include jaundice, pruritus, failure to thrive, fat malabsorption, cholelithiasis, and variably progressive cirrhosis. Accurate diagnosis of these disorders is essential for determination of prognosis and selection of the most appropriate therapies. Severe genetic defects in canalicular bile acid and phospholipid excretion lead to progressive liver disease that often requires liver transplantation. Defects in bile acid biosynthesis and aminophospholipid transport may be responsive to medical or non-transplant surgical approaches.
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U2 - 10.1016/S0889-8553(03)00044-X
DO - 10.1016/S0889-8553(03)00044-X
M3 - Review article
C2 - 14562577
AN - SCOPUS:0141682673
SN - 0889-8553
VL - 32
SP - 839
EP - 855
JO - Gastroenterology clinics of North America
JF - Gastroenterology clinics of North America
IS - 3
ER -