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一名患有Ib型阿伯内西畸形并伴有多发局灶性结节性增生的香港青春期女孩。

An adolescent girl in Hong Kong with type Ib Abernethy malformation complicated by multiple focal nodular hyperplasia.

作者信息

Yam Max Kai Ho, Cheung Kin On, Sim Shiu Wah, Lee Paul Sing Fun

机构信息

Alice Ho Miu Ling Nethersole Hospital, 11 Chuen On Road, Tai Po, NT., Hong Kong.

North District Hospital, 9 Po Kin Road, Sheung shui, New Territories, Hong Kong.

出版信息

Radiol Case Rep. 2020 Sep 25;15(11):2477-2481. doi: 10.1016/j.radcr.2020.09.016. eCollection 2020 Nov.

Abstract

Congenital portosystemic venous shunts are developmental anomalies. They represent portal communication with the systemic circulation. The pathogenesis is linked to the complexity of the embryological development of the inferior vena cava and portal vein. We reported a case of an asymptomatic 14-year-old Chinese adolescent girl in Hong Kong with a confirmed congenital portosystemic shunt type 1b. The condition can be diagnosed using contrast-enhanced CT scans and MRIs. Early recognition of the condition is important due to elevated risks of developing hepatocellular tumours. Liver transplantation may be considered curative.

摘要

先天性门体静脉分流是一种发育异常。它们表现为门静脉与体循环之间的交通。其发病机制与下腔静脉和门静脉胚胎发育的复杂性有关。我们报告了一例香港14岁无症状中国青少年女孩确诊为1b型先天性门体分流的病例。该病症可通过增强CT扫描和核磁共振成像进行诊断。由于发生肝细胞肿瘤的风险升高,早期识别该病症很重要。肝移植可能被认为是治愈性的。

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