Low phospholipid-associated cholelithiasis syndrome:A rare cause of acute pancreatitis that should not be neglected

• 作者: Anne-Laure Pelletier Dimitrios Konstantinou Gaël Goujon Hakim Bécheur Nicolas Gille Pascale Karila-Cohen Samia Rekik
• 来源期刊: 世界肝病学杂志:英文版(电子版)
• 学科: 医学
• 关键词: Low phospholipid-associated cholelithiasis syndrome Acute pancreatitis CHOLELITHIASIS ECHOGRAPHY Ursodeoxycholic acid
• 年，卷（期）: 2020,（6）
• 页码范围: 312-322
• 页数: 11页
• 分类号: R57

摘要

BACKGROUND Low phospholipid-associated cholelithiasis(LPAC)syndrome is a very particular form of biliary lithiasis with no excess of cholesterol secretion into bile,but a decrease in phosphatidylcholine secretion,which is responsible for stones forming not only in the gallbladder,but also in the liver.LPAC syndrome may be underreported due to a lack of testing resulting from insufficient awareness among clinicians.AIM To describe the clinical and radiological characteristics of patients with LPAC syndrome to better identify and diagnose the disease.METHODS We prospectively evaluated all patients aged over 18 years old who were consulted or hospitalized in two hospitals in Paris,France(Bichat University Hospital and Croix-Saint-Simon Hospital)between January 1,2017 and August 31,2018.All patients whose profiles led to a clinical suspicion of LPAC syndrome underwent a liver ultrasound examination performed by an experienced radiologist to confirm the diagnosis of LPAC syndrome.Twenty-four patients were selected.Data about the patients’general characteristics,their medical history,their symptoms,and their blood tests results were collected during both their initial hospitalization and follow-up.Cytolysis and cholestasis were expressed compared to the normal values(N)of serum aspartate and alanine transaminase activities,and to the normal value of alkaline phosphatase level,respectively.The subjects were systematically reevaluated and asked about their symptoms 6 mo after inclusion in the study through an in-person medical appointment or phone call.Genetic testing was not performed systematically,but according to the decision of each physician.RESULTS Most patients were young(median age of 37 years),male(58%),and not overweight(median body mass index was 24).Many had a personal history of acute pancreatitis(54%)or cholecystectomy(42%),and a family history of gallstones in first-degree relatives(30%).LPAC syndrome was identified primarily in patients with recurring biliary pain(88%)or after a new episode of acute pa