Review
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World J Gastroenterol. Jul 28, 2014; 20(28): 9418-9426
Published online Jul 28, 2014. doi: 10.3748/wjg.v20.i28.9418
Atypical causes of cholestasis
Ken D Nguyen, Vinay Sundaram, Walid S Ayoub
Ken D Nguyen, Department of Internal Medicine, Cedars Sinai Medical Center, Los Angeles, CA 90048, United States
Vinay Sundaram, Walid S Ayoub, Department of Gastroenterology and Hepatology, Multi Organ Transplant Center, Cedars Sinai Medical Center, Los Angeles, CA 90048, United States
Author contributions: Nguyen KD performed the research and wrote the draft of the manuscript; Sundaram V and Ayoub WS co-performed the research with Nguyen KD in addition to designing the outline of the manuscript, editing, and finalizing the final form of the manuscript.
Correspondence to: Walid S Ayoub, MD, Department of Gastroenterology and Hepatology, Multi Organ Transplant Center, Cedars Sinai Medical Center, 8635 West 3rd St, Suite 1060W, Los Angeles, CA 90048, United States. walid.ayoub@cshs.org
Telephone: +1-310-4231971 Fax: +1-310-4232356
Received: October 12, 2013
Revised: March 13, 2014
Accepted: April 5, 2014
Published online: July 28, 2014
Processing time: 286 Days and 19.9 Hours
Abstract

Cholestatic liver disease consists of a variety of disorders. Primary sclerosing cholangitis and primary biliary cirrhosis are the most commonly recognized cholestatic liver disease in the adult population, while biliary atresia and Alagille syndrome are commonly recognized in the pediatric population. In infants, the causes are usually congenital or inherited. Even though jaundice is a hallmark of cholestasis, it is not always seen in adult patients with chronic liver disease. Patients can have “silent” progressive cholestatic liver disease for years prior to development of symptoms such as jaundice and pruritus. In this review, we will discuss some of the atypical causes of cholestatic liver disease such as benign recurrent intrahepatic cholestasis, progressive familial intrahepatic cholestasis, Alagille Syndrome, biliary atresia, total parenteral nutrition induced cholestasis and cholestasis secondary to drug induced liver injury.

Keywords: Cholestasis; Benign recurrent intrahepatic cholestasis; Progressive familial intrahepatic cholestasis; Alagille syndrome; Biliary atresia; Total parenteral nutrition; Drug induced liver injury

Core tip: The approach and management of cholestasis remain an important aspect of the clinical practice. Different causes of cholestasis have been identified. We will review in this paper some atypical causes of cholestasis that clinicians should be aware off and consider in their approach to management of patients with cholestasis.