Approach and management considerations in low phospholipid associated cholelithiasis (LPAC) syndrome

Indrajit Suresh; Lokesh E.; Amrit Nanaiah; Soumya Ganesh Nanaiah; Suhas C.; Chandrababu D.

doi:10.18203/2320-6012.ijrms20184452

Authors

Indrajit Suresh Department of Pancreatology and Hepatology, CEG Hospital & Research Institute, Mysore, Karnataka, India http://orcid.org/0000-0002-1127-5283
Lokesh E. Department of Critical Care & Anesthesiology, SLES Hospital, Mysore, Karnataka, India
Amrit Nanaiah Departments of Internal Medicine, Lopamudra Medical Centre, Gonikoppal, Karnataka, India
Soumya Ganesh Nanaiah Departments of Ophthalmology, Lopamudra Medical Centre, Gonikoppal, Karnataka, India
Suhas C. Department of Internal Medicine, CEG Hospital & Research Institute, Mysore, Karnataka, India
Chandrababu D. Department of Pancreatology and Hepatology, CEG Hospital & Research Institute, Mysore, Karnataka, India

DOI:

https://doi.org/10.18203/2320-6012.ijrms20184452

Keywords:

Cholelithiasis, Gallstones, intrahepatic calculi, LPAC, UDCA

Abstract

Low phospholipid associated cholelithiasis (LPAC) syndrome, first described in 2001, could be the causality in a significant number of young patients with cholelithiasis, who have a recurrence of symptoms despite cholecystectomy. A mutation of the ABCB4/MDR3 gene, causes a disruption in the translocation of phosphatidylcholine, resulting in bile acid mediated biliary tract injury. The ABCB4 gene is also implicated in other diseases such as progressive familial intrahepatic cholestasis type 3, which is greater in severity and tools like genotyping can aid the physician in prognostication, as well as determining the response to medical therapy. A few symptomatic patients develop features of biliary obstruction due to intrahepatic calculi, and they require interventions-which may be endoscopic or surgical in nature. Although a majority of patients with LPAC syndrome respond well to ursodeoxycholic acid (UDCA) therapy, close monitoring is warranted to keep a check on disease progression.

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