Expert management of congenital portosystemic shunts and their complications.

先天性门体分流及其并发症的专家管理。

• 影响指数: 9.917
• 发表时间: 2024 Jan
• 来源期刊: JHEP Rep
• DOI: 10.1016/j.jhepr.2023.100933
• PMID: 38234409
• 文章类型: Journal Article
• 作者列表: McLin VA,Franchi-Abella S,Brütsch T,Bahadori A,Casotti V,de Ville de Goyet J,Dumery G,Gonzales E,Guérin F,Hascoet S,Heaton N,Kuhlmann B,Lador F,Lambert V,Marra P,Plessier A,Quaglia A,Rougemont AL,Savale L,Sarma MS,Sitbon O,Superina RA,Uchida H,van Albada M,van der Doef HPJ,Vilgrain V,Wacker J,Zwaveling N,Debray D,Wildhaber BE

关键词: adenoma; congenital portosystemic shunt; focal nodular hyperplasia; hepatocellular carcinoma; hyperandrogenism; hyperinsulinism; hypoglycemia; occlusion test; portal pressure; puberty; pulmonary hypertension; β-catenin

Abstract

Congenital portosystemic shunts are often associated with systemic complications, the most challenging of which are liver nodules, pulmonary hypertension, endocrine abnormalities, and neurocognitive dysfunction. In the present paper, we offer expert clinical guidance on the management of liver nodules, pulmonary hypertension, and endocrine abnormalities, and we make recommendations regarding shunt closure and follow-up.

摘要

先天性门体分流常伴有全身并发症,其中最具挑战性的是肝结节，肺动脉高压,内分泌异常,和神经认知功能障碍。在本论文中，我们为肝结节的治疗提供专家临床指导，肺动脉高压,和内分泌异常，我们就分流关闭和随访提出建议。