Acta Scientific Gastrointestinal Disorders (ASGIS)(ISSN: 2582-1091)

Case Report Volume 6 Issue 12

Diagnosis of Secondary Sclerosing Cholangitis in a Young Patient with LPAC Syndrome Revealed by Gastrointestinal Bleeding

Soumaya Zaouga*, Wided Bouhlel, Ramzi Tababi, Mejda Zakhama, Nabil Ben Chaabane and Leila Safer

Gastroenterology, Fattouma Bourguiba Hospital, Monastir

*Corresponding Author: Soumaya Zaouga, Gastroenterology, Fattouma Bourguiba Hospital, Monastir.

Received: October 06, 2023; Published: November 27, 2023


Low phospholipid-associated cholelithiasis (LPAC) is a rare genetic disorder characterized by the association of an ABCB4 mutation and low biliary phospholipid concentration with recurrent cholelithiasis, responsible for the development of intrahepatic lithiasis in adults.

The patients with the LPAC syndrome present typically with the following main features: age less than 40 years at onset of symptoms, recurrence of biliary symptoms after cholecystectomy, intrahepatic hyperechoic foci or sludge or microlithiasis along the biliary tree.

While the majority of clinical forms are simple, there also exist complicated forms, involving extended intrahepatic lithiasis and its consequences: lithiasis migration, acute cholangitis, intrahepatic abscess. Chronic evolution can lead to secondary sclerosing cholangitis or secondary biliary cirrhosis.

We report a case of a 32-year-old woman, cholecystectomized 10 years ago, who presented with hematemesis, upper gastrointestinal endoscopy revealed: Esophageal varices. Ultrasound and MRI showed the presence of intrahepatic calculi disseminated along the bile duct pathway, dysmorphic liver, and signs of secondary sclerosing cholangitis. Viral serologies and immunology tests were negative and the diagnosis of secondary sclerosing cholangitis complicating LPAC syndrome was retained and the patient was put under medical treatment with ursodeoxycholic acid (UDCA).

Keywords: LPAC Syndrome; Gastrointestinal Bleeding; Secondary Sclerosing Cholangitis; UDCA


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Citation: Soumaya Zaouga.,et al. “Diagnosis of Secondary Sclerosing Cholangitis in a Young Patient with LPAC Syndrome Revealed by Gastrointestinal Bleeding".Acta Scientific Gastrointestinal Disorders 6.12 (2023): 59-61.


Copyright: © 2023 Soumaya Zaouga.,et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


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