[Low phospholipidassociated cholelithiasis syndrome]

LOW PHOSPHOLIPIDASSOCIATED CHOLELITHIASIS (LPAC) SYNDROME. LPAC (low phospholipid-associated cholelithiasis) syndrome is a rare genetic form of intrahepatic cholelithiasis, associated in 30% to 50% of cases with a pathogenic variant of the phospholipid transporter MDR3 (multidrug resistance protein 3). Clinical presentation of LPAC syndrome is similar to that of common cholelithiasis, but young adult onset of symptoms (before the age of 40) and recurrence of biliary symptoms after cholecystectomy are highly suggestive of the syndrome. Ultrasound of the liver is key for diagnosis, showing intrahepatic microlithiasis in the form of ductal comet-tail images or microspots. Ursodeoxycholic acid (UDCA), at a dose of 5 to 15 mg/kg/d, is the reference treatment. Endoscopic treatment of lithiasis of the common main bile duct and/ or the main hepatic ducts is sometimes necessary.