CAJ | 학술논문

目的总结肝脏遗传性出血性毛细血管扩张症(HHHT)的诊断和治疗经验.方法回顾性分析2002年1月至2010年9月山东大学齐鲁医院、山东省临沂市蒙阴县人民医院、山东省聊城市人民医院、河南省人民医院、河北医科大学第二医院、浙江大学医学院附属第一医院收治的15例HHHT患者的临床资料.总结该病的临床表现、影像学及实验室检查,探讨该病的诊断、治疗和预后.结果 HHHT患者早期无特异性临床表现,多表现为上腹痛、气短、贫血、肝区杂音等,但病情可迅速进展并出现门静脉高压症、肝硬化等并发症；彩超、CT检查示肝内毛细血管扩张、动静脉瘘、肝动脉瘤；数字减影血管造影检查对多支肝动脉的HHHT患者,血管显影欠佳,而CT血管造影检查则显示清晰.15例患者根据病变程度和发病阶段被分为无症状型、简单型和复杂型,针对不同患者采用个体化治疗.6例患者采用手术治疗,其中5例接受肝动脉结扎或缩扎手术,随访效果满意.3例患者采用介入治疗,其中1例复杂型患者介入栓塞失败,药物治疗30个月后死亡.6例患者采用内科保守治疗,2例起始无症状,21个月、35个月后分别出现肝功能异常、腹腔积液等,其中1例死亡；4例仅行药物治疗的患者,随访示肝脏病变逐渐加重.结论依据肝内毛细血管扩张、动静脉瘘、肝动脉瘤三大特征,影像学检查具有重要诊断意义；HHHT为进展性疾病,早期、积极的个体化治疗具有较好的临床效果；保留肝周韧带及胆道供血的肝动脉结扎或缩扎手术疗效满意.
목적 총결간장유전성출혈성모세혈관확장증(HHHT)적진단화치료경험.방법 회고성분석2002년1월지2010년9월산동대학제로의원、산동성림기시몽음현인민의원、산동성료성시인민의원、하남성인민의원、하북의과대학제이의원、절강대학의학원부속제일의원수치적15례HHHT환자적림상자료.총결해병적림상표현、영상학급실험실검사,탐토해병적진단、치료화예후.결과 HHHT환자조기무특이성림상표현,다표현위상복통、기단、빈혈、간구잡음등,단병정가신속진전병출현문정맥고압증、간경화등병발증；채초、CT검사시간내모세혈관확장、동정맥루、간동맥류；수자감영혈관조영검사대다지간동맥적HHHT환자,혈관현영흠가,이CT혈관조영검사칙현시청석.15례환자근거병변정도화발병계단피분위무증상형、간단형화복잡형,침대불동환자채용개체화치료.6례환자채용수술치료,기중5례접수간동맥결찰혹축찰수술,수방효과만의.3례환자채용개입치료,기중1례복잡형환자개입전새실패,약물치료30개월후사망.6례환자채용내과보수치료,2례기시무증상,21개월、35개월후분별출현간공능이상、복강적액등,기중1례사망；4례부행약물치료적환자,수방시간장병변축점가중.결론 의거간내모세혈관확장、동정맥루、간동맥류삼대특정,영상학검사구유중요진단의의；HHHT위진전성질병,조기、적겁적개체화치료구유교호적림상효과；보류간주인대급담도공혈적간동맥결찰혹축찰수술료효만의.
Objective To summarize the experiences in the diagnosis and treatment of the hepatic hereditary hemorrhagic telangiectasia (HHHT).Methods The clinical data of 15 HHHT patients who were admitted to the Qilu Hospital,People's Hospital of Mengyin,People's Hospital of Liaocheng,Henan Provincial People's Hospital,the Second Hospital of Hebei Medical University,First Affiliated Hospital of Zhejiang University were retrospectively analyzed.The clinical manifestation,features of imaging and laboratory examination were summarized,and the diagnosis,treatment and prognosis of the disease were investigated.Results HHHT patients had nonspecific symptoms in the early stage,and some patients presented with right upper quadrant discomfort,shortness of breath,anemia and liver bruit.The condition of HHHT patients could be worsened by liver cirrhosis or portal hypertension rapidly.The results of color doppler ultrasound and computed tomography showed intrahepatic telangiectasia,arteriovenous fistula and hepatic artery aneurysm in the 15 patients.Digital subtraction angiography was not clear enough for 2 HHHT patients with more than 1 enlarged hepatic arteries,but computed tomographic angiography was feasible.According to the degree and stages of the HHHT,all the 15 patients were divided into asymptomatic HHHT,simple HHHT and complex HHHT.Among the 6 patients who underwent surgical treatment,5 received ligation or banding of the enlarged hepatic arteries with subsequent disappearance of symptoms.Three patients received interventional treatment,and the treatment for 1 patient with complex HHHT was failed,and the patient died 30 months after medical treatment.Six patients were treated by conservative treatment,2 patients of them had no symptoms at the beginning,then they suffered from hepatic dysfunction and ascites at 21 and 35 months,respectively,and 1 of them died 6 months later.Four patients received medical treatment,and the results of color doppler ultrasound and computed tomography showed the pathological changes were aggravated gradually.Conclusions Telangiectasia,intrahepatic arteriovenous fistula and hepatic artery aneurysm are the main imaging characteristics of HHHT,and imaging diagnosis has significant value in the diagnosis of HHHT.HHHT is a progressive disease,early,active and individualized treatment is beneficial to the patients.The outcome of ligation or banding of the hepatic arteries is satisfactory.