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[先天性肝内和肝外胆管扩张症的手术治疗适应证及局限性]

[The indications for and limits to the surgical treatment of congenital dilatations of the intra- and extrahepatic bile ducts].

作者信息

Andronescu P, Simion S, Gradinaru V, Andronescu C, Mastalier B

机构信息

Clinica de Chirurgie N. Gh. Lupu, Bucureşti.

出版信息

Chirurgia (Bucur). 1998 Jul-Aug;93(4):229-38.

PMID:9755572
Abstract

Congenital cystic dilatations of the biliary ducts are defects in the genetic program which are transmitted in an autosomal recessive way. Todani classified cystic dilatations of extra and intrahepatic ducts in 5 types. In classified cystic dilatations of extra and intrahepatic ducts in 5 types. In "N. Gh. Lupu" Surgical Clinical were treated 9 cases of congenital dilatations of the biliary tree during the last 40 years (1958-1998); five of them were belonging to the type V Todani (Caroli's disease), three to the type I Todani and one case in the type IV a of Todoni's classification. The mean age of the patients was 42.4 years, five of them being men and 4 women. Surgical treatment was particular to each case depending on age, associated diseases, the length of disease's evolution and the general state of the patient. The prognosis of this disease is a reserved one.

摘要

胆管先天性囊状扩张是遗传程序中的缺陷,以常染色体隐性方式遗传。Todani将肝外和肝内胆管的囊状扩张分为5种类型。在“N. Gh. Lupu”外科临床科室,过去40年(1958 - 1998年)共治疗了9例先天性胆管扩张病例;其中5例属于Todani V型(卡罗利病),3例属于Todani I型,1例属于Todoni分类中的IV a型。患者的平均年龄为42.4岁,其中5例为男性,4例为女性。根据年龄、相关疾病、疾病发展时长以及患者的一般状况,每个病例的手术治疗方式都有所不同。这种疾病的预后并不乐观。

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