胆道疾病(jíbìng)长江大学(dàxué)临床医学院外科教研室第一页，共一百一十四页。先天性胆道闭锁(bìsuǒ)•胆道闭锁(bìsuǒ)(biliaryatresia，BA)是新生儿期一种少见的严重黄疸性疾病•先天性畸形(jīxíng)学说•病毒感染学说第二页，共一百一十四页。第三页，共一百一十四页。病理(bìnglǐ)胆道闭锁(bìsuǒ)阻塞性黄疸(huángdǎn)胆汁淤积性肝硬化第四页，共一百一十四页。Bridgingfibrosisintheliverinbiliaryatresia第五页，共一百一十四页。第六页，共一百一十四页。临床表现•黄疸(huángdǎn)•营养及发育不良•肝脾肿大第七页，共一百一十四页。第八页，共一百一十四页。诊断(zhěnduàn)和鉴别诊断(zhěnduàn)特点①进行性梗阻性黄疸、②陶土样粪便、浓茶样尿和③肝脾肿大。有助诊断价值的检查(jiǎnchá)①动态监测血清胆红素持续性生高治疗无效者。②十二指肠液内无胆红素。③B超检查肝外胆道、胆囊缺如或发育不良。④99mT-EHIDA排泄实验：肠内无核素显影。⑤ERCP、MRCP又助诊断。第九页，共一百一十四页。（1）新生儿肝炎(ɡānyán)（2）先天性胆管囊状扩张症（3）新生儿胆汁浓缩第十页，共一百一十四页。治疗唯一有效方法(fāngfǎ)是手术建立胆汁引流通道。宜在出生后6—8周进行，2—3个月不手术则肝硬化再手术效果极差。•尚有肝外部位(bùwèi)胆管者—ROUX-en-Y吻合•肝外胆管完全闭锁者---Kasai手术•胆道完全闭锁者或手术失败者—livertransplantation第十一页，共一百一十四页。Dr.MorioKasaionDecember82008attheageof86.Dr.KasaiwasborninAomoriPrefecture,locatedatthenorthendofthemainislandofJapan,in1922.HegraduatedfromtheTohokuUniversitySchoolofMedicinein1947.In1948hecompletedhisinternship,andjoinedthe2ndDepartmentofSurgery,TohokuUniversitySchoolofMedicine.Dr.Kasai'smostnotableworkwasthedevelopmentofthehepatic-portoenterostomy,forbiliaryatresiain1955.Thescientificprinciplesunderlyingthisoperationweregroundbreaking,andbroughthimgreatrenown.However,heandhisgroupalwaysusedthemedicalterm,“hepatic-portoenterostomy”,ratherthantheeponym“KasaiProcedure”第十二页，共一百一十四页。第十三页，共一百一十四页。Prognosis•Somepatientsproduceadequatebileflow;maynotneedlivertransplant•Anothergrouphasmoderatebileflow,butremainsjaundiced;willneedlivertransplan...
