CAJ | 학술논문

目的：探讨胆道闭锁 Kasai 手术后自体肝的形态及结构改变,分析其与 Kasai 术后肝脏损伤程度及胆管炎之间的关系。方法收集2012年5月至2012年12月间胆道闭锁患儿 Kasai 术后行肝移植手术的自体肝脏标本8例为实验组,其中男7例,女1例,同时收集另外8例胆道闭锁患儿行 Kasai 手术时肝脏活检标本为阳性对照组；收集尸检新生儿肝脏标本2例作为阴性对照组,观察胆道闭锁患儿 Kasai 术后肝脏病理变化特点,并简单分析对胆管炎发生的影响。采用 HE 和免疫组化技术等检测肝脏纤维化程度、胆管及血管的增生情况、肝细胞损伤情况以及炎症细胞的浸润情况,观察 Kasai 术后肝脏的病理改变。结果实验组中生后出现黄疸时间为0~15 d,行 Kasai 手术时间为44~105 d,出现胆管炎的频次为1~6次,肝移植距离 Kasai 手术后时间5~67个月。阳性对照组中肝活检的肝脏标本外观普遍增大,淤胆严重,质地中等；HE 和免疫组化结果显示肝脏纤维化分级介于Ⅱ~Ⅳ级之间,汇管区胆栓情况介于0~Ⅰ级之间,汇管区血管增生情况介于Ⅲ~Ⅳ级之间,胆管增生介于Ⅰ~Ⅲ级之间。实验组中肝脏外观多数呈现部分段、叶萎缩情况,也有部分肝脏标本呈现肥厚改变；肝脏纤维化分级都属于Ⅳ级,汇管区胆栓情况Ⅰ~Ⅱ级之间,汇管区血管增生情况Ⅳ级,胆管增生Ⅲ~Ⅳ级之间。结合临床资料分析,患儿行 Kasai 手术时间越晚,其术后发生胆管炎次数越多,使部分患儿几乎呈现胆管炎持续状态,导致肝脏纤维化和胆管增生速度加快,从而使自体肝生存时间缩短,严重影响预后。反之自体肝生存期较长,预后较好。结论胆道闭锁 Kasai 术后肝病理形态学改变具有特征性。术后肝脏损伤受 Kasai 手术时间影响,并对胆管炎的发生有影响；进一步说明早期Kasai 手术、减少术后胆管炎发生频次是延长胆道闭锁自体肝生存时间的关键因素。目的：探討膽道閉鎖 Kasai 手術後自體肝的形態及結構改變,分析其與 Kasai 術後肝髒損傷程度及膽管炎之間的關繫。方法收集2012年5月至2012年12月間膽道閉鎖患兒 Kasai 術後行肝移植手術的自體肝髒標本8例為實驗組,其中男7例,女1例,同時收集另外8例膽道閉鎖患兒行 Kasai 手術時肝髒活檢標本為暘性對照組；收集尸檢新生兒肝髒標本2例作為陰性對照組,觀察膽道閉鎖患兒 Kasai 術後肝髒病理變化特點,併簡單分析對膽管炎髮生的影響。採用 HE 和免疫組化技術等檢測肝髒纖維化程度、膽管及血管的增生情況、肝細胞損傷情況以及炎癥細胞的浸潤情況,觀察 Kasai 術後肝髒的病理改變。結果實驗組中生後齣現黃疸時間為0~15 d,行 Kasai 手術時間為44~105 d,齣現膽管炎的頻次為1~6次,肝移植距離 Kasai 手術後時間5~67箇月。暘性對照組中肝活檢的肝髒標本外觀普遍增大,淤膽嚴重,質地中等；HE 和免疫組化結果顯示肝髒纖維化分級介于Ⅱ~Ⅳ級之間,彙管區膽栓情況介于0~Ⅰ級之間,彙管區血管增生情況介于Ⅲ~Ⅳ級之間,膽管增生介于Ⅰ~Ⅲ級之間。實驗組中肝髒外觀多數呈現部分段、葉萎縮情況,也有部分肝髒標本呈現肥厚改變；肝髒纖維化分級都屬于Ⅳ級,彙管區膽栓情況Ⅰ~Ⅱ級之間,彙管區血管增生情況Ⅳ級,膽管增生Ⅲ~Ⅳ級之間。結閤臨床資料分析,患兒行 Kasai 手術時間越晚,其術後髮生膽管炎次數越多,使部分患兒幾乎呈現膽管炎持續狀態,導緻肝髒纖維化和膽管增生速度加快,從而使自體肝生存時間縮短,嚴重影響預後。反之自體肝生存期較長,預後較好。結論膽道閉鎖 Kasai 術後肝病理形態學改變具有特徵性。術後肝髒損傷受 Kasai 手術時間影響,併對膽管炎的髮生有影響；進一步說明早期Kasai 手術、減少術後膽管炎髮生頻次是延長膽道閉鎖自體肝生存時間的關鍵因素。
목적：탐토담도폐쇄 Kasai 수술후자체간적형태급결구개변,분석기여 Kasai 술후간장손상정도급담관염지간적관계。방법수집2012년5월지2012년12월간담도폐쇄환인 Kasai 술후행간이식수술적자체간장표본8례위실험조,기중남7례,녀1례,동시수집령외8례담도폐쇄환인행 Kasai 수술시간장활검표본위양성대조조；수집시검신생인간장표본2례작위음성대조조,관찰담도폐쇄환인 Kasai 술후간장병리변화특점,병간단분석대담관염발생적영향。채용 HE 화면역조화기술등검측간장섬유화정도、담관급혈관적증생정황、간세포손상정황이급염증세포적침윤정황,관찰 Kasai 술후간장적병리개변。결과실험조중생후출현황달시간위0~15 d,행 Kasai 수술시간위44~105 d,출현담관염적빈차위1~6차,간이식거리 Kasai 수술후시간5~67개월。양성대조조중간활검적간장표본외관보편증대,어담엄중,질지중등；HE 화면역조화결과현시간장섬유화분급개우Ⅱ~Ⅳ급지간,회관구담전정황개우0~Ⅰ급지간,회관구혈관증생정황개우Ⅲ~Ⅳ급지간,담관증생개우Ⅰ~Ⅲ급지간。실험조중간장외관다수정현부분단、협위축정황,야유부분간장표본정현비후개변；간장섬유화분급도속우Ⅳ급,회관구담전정황Ⅰ~Ⅱ급지간,회관구혈관증생정황Ⅳ급,담관증생Ⅲ~Ⅳ급지간。결합림상자료분석,환인행 Kasai 수술시간월만,기술후발생담관염차수월다,사부분환인궤호정현담관염지속상태,도치간장섬유화화담관증생속도가쾌,종이사자체간생존시간축단,엄중영향예후。반지자체간생존기교장,예후교호。결론담도폐쇄 Kasai 술후간병리형태학개변구유특정성。술후간장손상수 Kasai 수술시간영향,병대담관염적발생유영향；진일보설명조기Kasai 수술、감소술후담관염발생빈차시연장담도폐쇄자체간생존시간적관건인소。
Objective To explore the characteristic changes of hepatic pathology in children with biliary atresia (BA)after Kasai portoenterstomy (KP)and analyze the relation with the degree of hepatic damage and cholangitis.Methods A retrospective study was conducted for 16 consecutive BA patients between May 2012 and December 2012.Eight (7 males,1 females)patients of one group underwent hepatic transplantation and a removal of native liver.And another 8 patients underwent Kasai procedure.And hepatic specimens were collected intra-operatively.The pathology of hepatic tissue from two groups was studied.Another 2 specimens of hepatic tissue were harvested from neonatal autopsy as negative control.Pathological examinations included hematoxylin & eosin and immunohistochemical staining for evaluating grade of hepatic fibrosis,extension of bile duct,vascular proliferation,degree of hepatocellular damage and infiltration of inflammatory cells.Results There were swelling,severe cholestasis and medium texture in the control group.The grading of hepatic fibrosis was between Ⅱ-Ⅳ,portal bile plugs 0-Ⅰ,vascular proliferation Ⅲ-Ⅳ and bile duct proliferation Ⅰ-Ⅲ.Macroscopically,the liver specimens of experimental group had more atrophic areas.Grading of hepatic fibrosis was divided into level Ⅳ,portal bile plugs Ⅰ-Ⅱ,portal vascular proliferation Ⅳ and proliferation of bile duct Ⅲ-Ⅳ.Combined with clinical data,earlier Kasai operation was associated with less frequency of cholangitis.And hypertrophic area was more than atrophic area. Meanwhile,there was severe hepatic fibrosis,but bile duct proliferation was relatively mild.Late Kasai 's procedures had a worse prognosis.And some children showed persistent cholangitis and a short survival period.Conclusions The pathological changes after hepatic removal have characteristic manifestations.Hepatic damage and timing of KP are the risk factors of cholangitis in BA patients after KP.