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World J Hepatol. May 27, 2014; 6(5): 358-362
Published online May 27, 2014. doi: 10.4254/wjh.v6.i5.358
Published online May 27, 2014. doi: 10.4254/wjh.v6.i5.358
Ductal paucity and Warkany syndrome in a patient with congenital extrahepatic portocaval shunt
Vikrant Sood, Rajeev Khanna, Seema Alam, Dinesh Rawat, Shorav Bhatnagar, Archana Rastogi, Departments of Pediatric Hepatology, Radiology and Pathology, Institute of Liver and Biliary Sciences, New Delhi 110070, India
Author contributions: Sood V and Khanna R contributed equally in compiling clinical and laboratory information and writing the full manuscript; Alam S and Rawat D helped in editing and revision; Bhatnagar S provided the radiologic information; Rastogi A provided the pathological details; and Khanna R drafted the article.
Correspondence to: Dr. Rajeev Khanna, MD, Assistant Professor, Departments of Pediatric Hepatology, Radiology and Pathology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi 110070, India. drrajeev_khanna@rediffmail.com
Telephone: +91-96542-46963 Fax: +91-96542-46963
Received: November 14, 2013
Revised: January 9, 2014
Accepted: March 17, 2014
Published online: May 27, 2014
Processing time: 193 Days and 12.3 Hours
Revised: January 9, 2014
Accepted: March 17, 2014
Published online: May 27, 2014
Processing time: 193 Days and 12.3 Hours
Core Tip
Core tip: This study highlights the association between the congenital extrahepatic portocaval shunt with hepatic ductal paucity and trisomy 8 for the first time in the world literature. Although the exact pathophysiology remains uncertain, plausible explanations are proposed.