·566·主堡澧丝处型苤盍!!!!生!!旦筮!!鲞筮!塑￡!也』堡g坠垡：旦!!!里!竺!!!!：y!!：!!：盟!：!遗传性出血性毛细血管扩张症的和治疗宋勰徐万峰葛英辉李彩英李红光高海女刘恩宇陈雨信【摘要】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I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。【关键词】肝脏疾病；毛细血管扩张，遗传性出血性；诊断；治疗DiagnosisandtreatmentofhepatichereditaryhemorrhagictelangiectasiaSONGXie+，XUWan-feng，GEYing—hui，LICai—ying，LIftong—gua．ng，GAOHai—hi2，LIUEn—yu，CHENYu—xin．+DepartmentofHepatobiliarySurgery，QiluHospital，ShandongUniversity，Jinan250012，ChinaCorrespondingauthor：CHENYu—xin，Email：yxchen@sdu．edu．cn【Abstract】0bjectiveTosummarizetheexperiencesinthediagnosisandtreatmentofthehepatichereditaryhemorrhagictelangiectasia(HHHT)．MethodsTheclinicaldataof15HHHTpatientswhowereadmittedtotheQiluHospital，People7SHospitalofMengyin，People’SHospitalofLiaocheng，HenanProvincialPeople’SHospital，theSecondHospitalofHebeiMedicalUniversity，FirstAffiliatedHospitalofZhejiangUniversitywereretrospectivelyanalyzed．Theclinicalmanifestation，featuresofimagingandlaboratoryexaminationweresummarized，andthediagnosis，treatmentandprognosisofthediseasewereinvestigated．ResultsHHHTpatientshadnonspecificsymptomsintheearlystage，andsomepatientspresentedwithfightupperquadrantdiscomfort，shortnessofbreath，anemiaandliverbruit．TheconditionofHHHTpatientscouldbeworsenedbylivercirrhosisorportalhypertensionrapidly．Theresultsofcolordopplerultrasoundandcomputedtomographyshowedintrahepatictelangiectasia，arteriovenousfistulaandhepaticarteryaneurysminthe15patients．Digitalsubtractionangiographywasnotclearenoughfor2HHHTpatientswithmorethan1enlargedhepaticarteries，butcomputedtomographicangiographywasfeasible．AccordingtOthedegreeandstagesoftheHHHT，allthe15patientsweredividedintoasymptomaticHHHT，simpleHHHTandcomplexHHHT．Amongthe6patientswhounderwentSU唧caltreatment，5receivedligationorbandingoftheenlargedhepaticarterieswithsubsequentdisappearanceofsymptoms．Threepatientsreceivedinterventionaltreatment．andthetreatmentfor1patientwithcomplexHHHTwasfailed．andthepatientdied30monthsaftermedicaItreatment．Sixpatientsweretreatedbyconservativetreatment，2patientsofthemhadnosymptomsatthebeginning，thentheysufferedfromhepat...
