Case Report
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World J Hepatol. Sep 27, 2014; 6(9): 685-687
Published online Sep 27, 2014. doi: 10.4254/wjh.v6.i9.685
Liver fibrosis in primary intestinal lymphangiectasia: An undervalued topic
Raffaele Licinio, Mariabeatrice Principi, Enzo Ierardi, Alfredo Di Leo
Raffaele Licinio, Mariabeatrice Principi, Enzo Ierardi, Alfredo Di Leo, Gastroenterology Unit, Department of Emergency and Organ Transplantation, University of Bari, Cesare, 70124 Bari, Italy
Author contributions: Ierardi E, Di Leo A, Principi M and Licinio R followed the patient, reviewed the literature, wrote the manuscript and approved the final version.
Correspondence to: Alfredo Di Leo, Professor, Gastroenterology Unit, Department of Emergency and Organ Transplantation, University of Bari, Piazza G, Cesare, 70124 Bari, Italy. alfredo.dileo@uniba.it
Telephone: +39-08-05592577 Fax: +39-08-05593088
Received: April 9, 2014
Revised: July 7, 2014
Accepted: August 27, 2014
Published online: September 27, 2014
Processing time: 193 Days and 3.9 Hours
Abstract

The relationship between primary intestinal lymphangiectasia (PIL) and liver fibrosis is an emerging topic with many obscure aspects due to the rarity of the disorder. A recent paper reported that a six-month low-fat diet improved liver fibrosis. We report the case of a 17-year-old girl affected by PIL whose hepatic fibrosis progressively worsened within one year, despite dietetic support. This and the previous case report describe extraordinary events, which do not allow clear-cut clinical aspects to be established. Nevertheless, both cases suggest that in patients with PIL, it is necessary to closely monitor liver morphology with in-depth investigations including not only ultrasonography, but also elastography.

Keywords: Hepatic transient elastography; Liver fibrosis; Low-fat diet; Primary intestinal lymphangiectasia

Core tip: The relationship between primary intestinal lymphangiectasia and liver fibrosis is an emerging topic with many obscure aspects due to the rarity of the disorder. The fibrosis outcome after a low-fat diet in the patient described in this report is in contrast with other literature reports. We emphasize the need for systematic monitoring of liver fibrosis in primary intestinal lymphangiectasia.