International Journal of Research in Medical Sciences | November 2018 | Vol 6 | Issue 11 Page 3794 International Journal of Research in Medical Sciences Suresh I et al. Int J Res Med Sci. 2018 Nov;6(11):3794-3800 www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012 Review Article Approach and management considerations in low phospholipid associated cholelithiasis (LPAC) syndrome Indrajit Suresh 1 *, Lokesh E. 2 , Amrit Nanaiah 3 , Soumya Ganesh Nanaiah 4 , Suhas C. 5 , Chandrababu D. 1 INTRODUCTION Recurrent cholelithiasis in young individuals often poses a management conundrum for most physicians. The problem in itself carries significant morbidity, with many patients experiencing episodes of cholecystitis, cholangitis, pancreatitis and rarely, intrahepatic calculi. Low phospholipid associated cholelithiasis (LPAC, OMIM 171060), first described in 2001, is a condition characterized by biliary phospholipid deficiency and cholesterol supersaturation could be responsible for a significant number of obscure cases. 1 Diagnostic criteria have been formulated which have significantly increased the identification rate of this condition (Table 1). The exact prevalence of LPAC remains unknown, but some studies have considered it to be as high as 5% of all symptomatic gall stone disease. 2,3 The disease is more common in young adults, the usual age at the onset of the symptoms is typically lower than 40 years (most commonly during the second and third decade of life). Females are three times more likely to be affected than males. 3 1 Department of Pancreatology and Hepatology, CEG Hospital & Research Institute, Mysore, Karnataka, India 2 Department of Critical Care & Anesthesiology, SLES Hospital, Mysore, Karnataka, India 3 Departments of Internal Medicine, 4 Departments of Ophthalmology, Lopamudra Medical Centre, Gonikoppal, Karnataka, India 5 Department of Internal Medicine, CEG Hospital & Research Institute, Mysore, Karnataka, India Received: 26 August 2018 Accepted: 6 October 2018 *Correspondence: Dr. Indrajit Suresh, E-mail: indrajit.suresh@yahoo.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 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. Keywords: Cholelithiasis, Gallstones, intrahepatic calculi, LPAC, UDCA DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20184452