【摘要】 本文报道1例罕见的DM合并由ABCB11基因突变导致的肝内胆汁淤积症及肝细胞癌病例，经手术及药物治疗后，患者的消化道症状及血糖均得到不同程度控制。DM合并原发性肝内胆汁淤积症目前尚少见报道，介绍本病例的临床表现、基因学特征和治疗经过，拟为临床医生掌握该病的诊治和随访提供参考。更多还原

【Abstract】 This paper reports an early-onset diabetes patient combined with intrahepatic cholestasis and hepatocellular carcinoma caused by a mutation of ABCB11 gene. After surgery and drug treatment,the patient’s digestive tract symptoms and diabetes were alleviated. Diabetes with primary intrahepatic cholestasis has not been reported in the literature. By introducing this case’s clinical manifestations,genetic characteristics and clinical course,we hope to provide a reference for clinicians to understand the diagnosis,treatment and follow-up of this disease.更多还原