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血管瘤——阿伯内西综合征的一个提示？

Hemangioma - A pointer to Abernethy syndrome?

作者信息

Kothari Shyam S

机构信息

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Ann Pediatr Cardiol. 2020 Jul-Sep;13(3):269-271. doi: 10.4103/apc.APC_91_20. Epub 2020 Jul 2.

DOI:10.4103/apc.APC_91_20
PMID:32863670
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7437616/
Abstract

Diffuse pulmonary arteriovenous malformations or pulmonary arterial hypertension (PAH) may result from congenital portosystemic venous shunts. Hemangioma as a physical sign of congenital portosystemic shunts (like Abernethy syndrome) has not been described. I report two children (one with severe cyanosis from pulmonary arteriovenous malformations and the other with severe PAH) with cutaneous hemangioma and Abernethy syndrome. Hemangioma may be a clinical pointer to portosystemic shunts even in the absence of obvious liver disease.

摘要

弥漫性肺动静脉畸形或肺动脉高压（PAH）可能由先天性门体静脉分流引起。作为先天性门体分流（如阿伯内西综合征）体征的血管瘤尚未见报道。我报告了两名患有皮肤血管瘤和阿伯内西综合征的儿童（一名因肺动静脉畸形出现严重紫绀，另一名患有严重PAH）。即使在没有明显肝脏疾病的情况下，血管瘤也可能是门体分流的临床指征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/ad281daf0f7f/APC-13-269-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/2e83b9528141/APC-13-269-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/9bb2a535cc0e/APC-13-269-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/afd578bb5bb4/APC-13-269-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/ad281daf0f7f/APC-13-269-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/2e83b9528141/APC-13-269-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/9bb2a535cc0e/APC-13-269-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/afd578bb5bb4/APC-13-269-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c9fc/7437616/ad281daf0f7f/APC-13-269-g004.jpg

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本文引用的文献

1
Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated with surgical shunt ligation: A case report and literature review.手术结扎分流术治疗先天性肝外门体分流（阿伯内西畸形）：病例报告及文献综述
Int J Surg Case Rep. 2020;66:4-7. doi: 10.1016/j.ijscr.2019.11.014. Epub 2019 Nov 20.
2
Congenital portosystemic venous shunt.先天性门体静脉分流。
Eur J Pediatr. 2018 Mar;177(3):285-294. doi: 10.1007/s00431-017-3058-x. Epub 2017 Dec 14.
3
Congenital hemangiomas.先天性血管瘤
Semin Cutan Med Surg. 2016 Sep;35(3):124-7. doi: 10.12788/j.sder.2016.045.
4
Catheter interventional treatment of congenital portosystemic venous shunts in childhood.儿童先天性门体静脉分流的导管介入治疗
Catheter Cardiovasc Interv. 2016 Jun;87(7):1281-92. doi: 10.1002/ccd.26362. Epub 2015 Dec 30.
5
Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report.阿伯内西畸形患者辅助性部分原位肝移植后肝肺综合征的缓解。病例报告。
Liver Transpl. 2007 Dec;13(12):1662-8. doi: 10.1002/lt.21349.
6
Congenital absence of portal vein in the fetus: a case report.胎儿先天性门静脉缺如：一例报告
Ultrasound Obstet Gynecol. 2001 Jan;17(1):71-5. doi: 10.1046/j.1469-0705.2001.00312.x.