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先天性肝纤维化
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  congenital hepatic fibrosis
     The Diagnosis and Treatment of Congenital Hepatic Fibrosis
     先天性肝纤维化的诊断与治疗
短句来源
     Methods Liver transplantation was performed in 5 children with biliary atresia, 1 with congenital hepatic fibrosis and 1 with hepatohemoangioendotheliosarcoma in our hospital from November 2001 to December 2002. Their age ranged from 6 months to 12 years.
     方法 我院分别于2 0 0 1年 11月至 2 0 0 2年 12月对 7例患儿 (先天性胆道闭锁 5例 ,先天性肝纤维化 1例和肝脏血管内皮肉瘤 1例 )进行肝移植术 ,患儿年龄 6个月至 12岁。 其中亲体部分肝移植术两例 ,供体分别为患儿的母亲和父亲。
短句来源
     Congenital hepatic fibrosis: a clinicopathologic study of 7 cases
     先天性肝纤维化7例临床病理分析
短句来源
     A CASE REPORT OF CONGENITAL HEPATIC FIBROSIS
     先天性肝纤维化一例报告
短句来源
     Combined operation of distal renal, spleic venous shunt and disconnection for the treatment of congenital hepatic fibrosis in children
     远端脾肾静脉分流加断流术治疗小儿先天性肝纤维化
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  相似匹配句对
     A CASE REPORT OF CONGENITAL HEPATIC FIBROSIS
     先天性纤维化一例报告
短句来源
     The Diagnosis and Treatment of Congenital Hepatic Fibrosis
     先天性纤维化的诊断与治疗
短句来源
     Therapy for liver fibrosis
     纤维化的治疗
短句来源
     Congenital hepatic fibrosis: a clinicopathologic study of 7 cases
     先天性纤维化7例临床病理分析
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     Obesity and liver fibrosis
     肥胖与纤维化
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  congenital hepatic fibrosis
Classic Caroli's disease involves malformations of the biliary tract alone, whereas Caroli's syndrome refers to the presence of associated congenital hepatic fibrosis.
      
Progress in molecular genetics allows better classification of patients with some atypical manifestations, e.g., those with early renal failure or those with congenital hepatic fibrosis.
      
Congenital hepatic fibrosis with saccular dilatation of intrahepatic bile ducts and infantile polycystic kidneys
      
Necropsy findings were consistent with Caroli's disease but the liver also showed evidence of congenital hepatic fibrosis and there was infantile type polycystic disease of the kidneys.
      
Association of congenital hepatic fibrosis with autosomal dominant polycystic kidney disease
      
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Congenital cystic dilatation of the intrahepatic ducts (Caroli' s disease) until recently has been rarely reported in the Chinese literature. 19 patients with this condition are reported from the experience of the surgeons and roentgenologists at the First Affiliated Hospital of the Sun Yat-sen University of Medical Sciences, together with a review of the world literature.

先天性肝内胆管囊性扩张症(Caroli病)在我国至今仅有37例,其中成人型者仅14例。本文报告19例,并作文献复习。Caroli将本病分为两型:①单纯型(Ⅰ型):以反复发作胆道感染为特点;②汇管区周围纤维化型,又称Ⅱ型。Caroli称本病Ⅰ型极少见。我国及本组材料则相反,以Ⅰ型占大多数。本病与肝胆管结石梗阻导致继发性肝内胆管扩张有时难以区别。由于本病累及双侧肝叶较多,故切除病变机会较少。病理学家对本病的病理组织学特点认识也不够。国外对本病Ⅱ型与先天性肝纤维化的关系认识意见亦相当混乱。因此,目前对本病诊断,主要依靠胆管造影所显示的肝内胆管形态。近年文献报告本病有恶性病变倾向,恶变率约为7%。

In this article, 6 cases of Caroli's disease "periportal fibrosis type"were diagnosed by Duplex Doppler Sonography in our hospital. All of patients were confirmed by P. T. C. operation, redographical operating and pathological study. The results show that the male greater than females. The part of patients complicated dilation of renal tunal of sponge kidney. Exept some(patients) presented congenital cystic dilatation of the intrehepatic bile ducts, congenital hepatic fibrosis, cirrbosis and portal hypertension....

In this article, 6 cases of Caroli's disease "periportal fibrosis type"were diagnosed by Duplex Doppler Sonography in our hospital. All of patients were confirmed by P. T. C. operation, redographical operating and pathological study. The results show that the male greater than females. The part of patients complicated dilation of renal tunal of sponge kidney. Exept some(patients) presented congenital cystic dilatation of the intrehepatic bile ducts, congenital hepatic fibrosis, cirrbosis and portal hypertension. About 1/3 patients complicated mild dilation of extrahepatic bile ducts. The change bemodynamic of portal system was observated by Duplex Doppler sonography affter and preoperation. Hyper Volemia was reducted, due to blood flow of splen were cut—off. The blood flow of portal systen were relerantly reducted too, and vasoconsrticted. This study confirms that between the blood flow rate of splen and portal system were correlative. It is reacted change hemodynamic of portal system.

本文总结应用双功能超声诊断“CaroliS病”门脉周围纤维化型”6例,经PTC手术和术中胆道造影及病理证实,男性多见。声像图:除肝内胆管囊状扩张,伴先天性肝纤维化、肝硬化及门脉高压,曾分患者肾小管扩张或呈海绵肾,肝外胆管1/3轻度扩张。脉冲多普勒超声观测脾切除加断流术后,脾血流阻断,门脉血流量相应减少,管径缩小,说明脾静脉与门静脉血流量呈正相关性,二者共同反映门脉系统的血液动力学变化。

Three cases of congenital hepatic fibrosis underwent. splenectomy and cardiofundo- transectomy are reported. The clinical manifestations intcluded hepatosplenomegaly , portal hypertension and upper gastrointestinal hemorrhage. The diagnosis was confirmed by pathologic examination. They were followed-up for 1 to 3 years with normal physical development , hepatic functions , and without GI bleeding. The authors state that patients with hepatosplenomegaly , portal hypertension , multiple dilation of intrahepatic...

Three cases of congenital hepatic fibrosis underwent. splenectomy and cardiofundo- transectomy are reported. The clinical manifestations intcluded hepatosplenomegaly , portal hypertension and upper gastrointestinal hemorrhage. The diagnosis was confirmed by pathologic examination. They were followed-up for 1 to 3 years with normal physical development , hepatic functions , and without GI bleeding. The authors state that patients with hepatosplenomegaly , portal hypertension , multiple dilation of intrahepatic bile ducts but without abnormal liver functions , congenital hepatic fibrosis should be considered. The histologic characteristics include fibrous proliferation of interlobular portal areas , and normal structure of hepatic lobules. Early diagnosis and treatment should be stressed to prevent postoperative complications.

介绍临床以肝脾肿大、门脉高压、上消道出血为主要症状的一种少见的遗传病一先天性肝纤维化的概念、诊断与治疗。1993年以来收治先天性肝纤维化3例,均经临床及病理证实,行脾切除、贲门胃底断流术。术后随访1~3年,患儿发育、营养佳,无再出血,肝功能正常,近期疗效满意。复习文献结合本组病例诊治认为,临床发现肝脾肿大、门脉高压、肝内胆管多发性扩张、肝功能基本正常者应想到该病。肝脏组织学特点为汇管区纤维增生明显,肝小叶结构正常。为预防术后并发症应早期诊断,及时治疗。

 
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