Presentation
Patient with no specific pathological history, not obese (BMI 22), presented to the emergency room with generalised mucocutaneous jaundice and right hypochondrial pain. His liver workup showed cytolysis (AST = 92 IU, ALT = 153 IU) and signs of biological cholestasis (ALP = 374 IU, GGT = 284 IU, TB = 94 µmol/l, CB = 82 µmol/l).
Patient Data
Hepatic ultrasound images showing intrahepatic lithiasis with posterior acoustic shadowing.
Hepatic ultrasound images showing intrahepatic lithiasis with posterior acoustic shadowing.
Magnetic resonance cholangiography in 3D reconstructions showing multiple intrahepatic stones and in the common hepatic duct with alithiasic gallbladder.
Magnetic resonance cholangiography in 3D reconstructions showing multiple intrahepatic stones and in the common hepatic duct with alithiasic gallbladder.
Case Discussion
Low phospholipid-associated cholelithiasis syndrome is a new entity described for the first time in 2001 by the team of Raoul Poupon 1. It is a particular form of symptomatic and recurrent cholelithiasis occurring in young adults.
Given the sex, age, personal and family history, absence of obesity, and the presence of symptomatic intrahepatic cholelithiasis without morphological abnormalities of the bile ducts, LPAC syndrome was strongly suspected.
The patient was placed on ursodeoxycholic acid (UDCA) at 10 mg/kg/d with good clinical progress with regression of painful symptoms in a few days.
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