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婴儿期先天性门腔静脉分流导致肺内血管扩张性低氧血症：病例报告。

Hypoxia due to intrapulmonary vascular dilatation in a toddler with a congenital portacaval shunt: case report.

机构信息

Departments of Pediatrics at Tawam Hospital, Alain City, United Arab Emirates.

KidsHeart Medical Center, Alain City, United Arab Emirates.

出版信息

BMC Pulm Med. 2019 Feb 22;19(1):49. doi: 10.1186/s12890-019-0788-8.

DOI:10.1186/s12890-019-0788-8
PMID:30795758
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6387555/
Abstract

BACKGROUND

The term hepatopulmonary syndrome typically applies to cyanosis that results from "intrapulmonary vascular dilatation" due to advanced liver disease. Similar findings may result from a congenital portosystemic shunt without liver disease. An adverse consequence of such shunts is intrapulmonary vascular dilatation, which affects the microvascular gas exchange units for oxygen.

CASE PRESENTATION

Here, we describe a toddler with chronic cyanosis, exercise intolerance, and finger clubbing due to a malformation shunt between the portal vein and the inferior vena cava. A transcatheter embolization of the shunt resulted in resolution of his findings.

CONCLUSIONS

Congenital portosystemic shunts need to be considered in the differential diagnosis of cyanosis.

摘要

背景

肝肺综合征一词通常适用于由于晚期肝病导致的“肺内血管扩张”引起的发绀。类似的发现也可能是由于先天性门体分流而没有肝病引起的。这种分流的一个不良后果是肺内血管扩张，这会影响氧气的微血管气体交换单位。

病例介绍

在这里，我们描述了一个患有慢性发绀、运动不耐受和杵状指的幼儿，其病因是门静脉和下腔静脉之间的畸形分流。分流的经导管栓塞导致其症状缓解。

结论

在发绀的鉴别诊断中需要考虑先天性门体分流。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/b44355292ccf/12890_2019_788_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/a95f6a41cc9e/12890_2019_788_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/42338e618b4e/12890_2019_788_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/7837eeb50fa7/12890_2019_788_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/7a31710ef4d5/12890_2019_788_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/b44355292ccf/12890_2019_788_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/a95f6a41cc9e/12890_2019_788_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/42338e618b4e/12890_2019_788_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/7837eeb50fa7/12890_2019_788_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/7a31710ef4d5/12890_2019_788_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccdf/6387555/b44355292ccf/12890_2019_788_Fig5_HTML.jpg

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