LOW-PHOSPHOLIPID-ASSOCIATED CHOLELITHIASIS SYNDROME (LPAC): WHEN TO SUSPECT? CASE REPORT

Abstract

Introduction: Low-phospholipid-associated cholelithiasis syndrome (LPAC) is a rare form of intrahepatic lithiasis linked to a defect of phospholipid canalicular secretion into bile. LPAC is associated with a phenotype of ABCB4/MDR3 gene mutation and it’s defined by the recurrence of biliary pain, acute cholangitis, or pancreatitis after cholecystectomy. Objectives: Report a case of LPAC to expand the knowledge of this pathology to physicians and health professionals to benefit patients. Case report: Patient, feminine, 39 years old, sought medical attention with pain on the right hypochondrium associated with nausea and vomit that initiated two days ago. The patient reported the first episode of acute pancreatitis in 2019 and six more episodes afterward. In the last episode, on April 2020, cholecystectomy was performed during hospitalization. Three more episodes of acute pancreatitis occurred since the surgery. Elevated lipase levels appeared on laboratory evaluation. Therefore, the diagnosis of acute pancreatitis and LPAC were made based on clinical and laboratory signs and clinical signs respectively.  Conclusion: Althought there is few articles about LPAC syndrome, it must be reminded as a differential diagnosis in cases of recurrence of biliary pain, acute pancreatitis after cholecystectomy, and when these events occur in young patients with no risk factors. The understanding of this disease will happen with the awareness and wide dissemination of diagnostic criteria, which will facilitate the screening and treatment of patients.

Key-words: Cholestasis; Ursodeoxycholic Acid, Cholelithiasis.