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LPAC syndrome is characterized by the appearance of cholelithiasis at an abnormally early age and the persistence of biliary symptoms after cholecystectomy. The diagnosis is usually confirmed by ultrasound, revealing intrahepatic microlithiasis. Recent findings highlight the importance of genetic factors, particularly mutations in the ABCB4 gene, in the syndrome's pathophysiology. Treatment typically involves ursodeoxycholic acid, with endoscopic interventions considered for complicated cases.
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