CAJ | 학술논문

总结并探讨特发性成人肝内胆管缺失症的发病原因、临床特征、诊治及预后.27岁女性患者,2002-10无明显诱因出现巩膜黄染,皮肤瘙痒,伴尿颜色加深、便灰白色,症状反复出现并进行性加重入住武装警察部队总医院接受治疗.B超提示,肝损害,脾脏轻度肿大.肝穿病理诊断为特发性成人肝内胆管缺失症.给予熊去氧胆酸、肾上腺皮质激素治疗,效果均不明显.于2005-06-24行原位肝移植.肝移植后患者黄疸、瘙痒症状迅速消失,肝功能各项指标恢复正常.2007-03因激素减量出现肝功能异常,出现轻度急性排异反应,调整用药剂量,并辅以保肝治疗,肝功能逐渐降至正常.术后随访51个月,患者及移植物功能均正常,未出现原发病的复发.提示特发性成人肝内胆管缺失症的病因及发病机制尚不明确,属于一种以组织学特征为主的排除诊断性疾病,且目前尚无治疗特发性成人肝内胆管缺失症确切、有效的药物.肝移植是治疗终末期特发性成人肝内胆管缺失症惟一有效的方法.
총결병탐토특발성성인간내담관결실증적발병원인、림상특정、진치급예후.27세녀성환자,2002-10무명현유인출현공막황염,피부소양,반뇨안색가심、편회백색,증상반복출현병진행성가중입주무장경찰부대총의원접수치료.B초제시,간손해,비장경도종대.간천병리진단위특발성성인간내담관결실증.급여웅거양담산、신상선피질격소치료,효과균불명현.우2005-06-24행원위간이식.간이식후환자황달、소양증상신속소실,간공능각항지표회복정상.2007-03인격소감량출현간공능이상,출현경도급성배이반응,조정용약제량,병보이보간치료,간공능축점강지정상.술후수방51개월,환자급이식물공능균정상,미출현원발병적복발.제시특발성성인간내담관결실증적병인급발병궤제상불명학,속우일충이조직학특정위주적배제진단성질병,차목전상무치료특발성성인간내담관결실증학절、유효적약물.간이식시치료종말기특발성성인간내담관결실증유일유효적방법.
To summarize and explore the etiopathogenisis, clinical 'characteristics, diagnosis and treatment and prognosis of idiopathic adulthood ductopenia. We described a 27-year-old Chinese female initially manifested with jaundice, pruritus, dark urine, and pallor of the stools in October, 2002. Symptoms occurred repetitively and severed progressively, and she received therapy at the General Hospital of Chinese People's Armed Police Forces. B-ultrasound demonstrated that hepatic lesion and slightly swelling spleen. Pathological diagnosis of liver biopsy showed that she affected idiopathic adulthood ductopenia. The outcome of treatment of ursodeoxycholic acid and adrenal cortical hormone was not significant. She received orthotopic liver transplantation on June 24~(th), 2005. After liver transplantation, pruritus and jaundice were promptly disappeared. Each index of hepatic function recovered to a normal level. In March 2007, decreased dose of hormone induced an abnormal hepatic function, with mildly acute rejection. Drug dose was regulated, and hepatic function gradually returned to normal. During follow-up 51 months after the operation, she was doing well and was free of symptoms with normal liver function and no evidence of allograft dysfunction. The pathogenesis of idiopathic adulthood ductopenia is still unknown. There are still no precise and effective drugs for treatment of idiopathic adulthood ductopenia. Liver transplantation offers an effective therapy for end-stage patients with idiopathic adulthood ductopenia.