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伪装成先天性心脏病的阿伯内西畸形:一名患有紫绀、杵状指和缺氧的男孩。

Abernethy Malformation Masquerading as Congenital Heart Disease: A Boy With Cyanosis, Clubbing, and Hypoxia.

作者信息

Agarwal Rohit, Prasad Durga, Chauhan Gaurav, Verma Abhai

机构信息

Interventional Radiology, Medanta Medicity, Lucknow, IND.

Pediatric Gastroenterology, Medanta Medicity, Lucknow, IND.

出版信息

Cureus. 2023 Jan 8;15(1):e33519. doi: 10.7759/cureus.33519. eCollection 2023 Jan.

DOI:10.7759/cureus.33519
PMID:36779160
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9905039/
Abstract

Abernethy malformation is an extrahepatic congenital portosystemic shunt characterized by the diversion of the portal blood away from the liver through a shunt that drains directly into the inferior vena cava. We present a case of a male child with Abernethy malformation, which was initially diagnosed as cyanotic heart disease due to pulmonary arteriovenous malformation. However, after proper clinical evaluation and investigations, the diagnosis of Abernethy malformation was established. Thereafter, the patient was successfully treated with endovascular embolization. At one year follow-up, marked relief in exertional dyspnea and improvement in physical growth was achieved with no observable complications.

摘要

阿伯内西畸形是一种肝外先天性门体分流,其特征是门静脉血通过直接引流至下腔静脉的分流从肝脏分流。我们报告一例患有阿伯内西畸形的男童病例,该患儿最初因肺动静脉畸形被诊断为青紫型心脏病。然而,经过适当的临床评估和检查后,确诊为阿伯内西畸形。此后,患者通过血管内栓塞术成功治愈。在一年的随访中,劳力性呼吸困难明显缓解,身体生长状况改善,未观察到并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d06d/9905039/5f6178219417/cureus-0015-00000033519-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d06d/9905039/162a43637e85/cureus-0015-00000033519-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d06d/9905039/5f6178219417/cureus-0015-00000033519-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d06d/9905039/162a43637e85/cureus-0015-00000033519-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d06d/9905039/5f6178219417/cureus-0015-00000033519-i02.jpg

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本文引用的文献

1
Congenital portosystemic venous shunt.先天性门体静脉分流。
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Account of Two Instances of Uncommon Formation in the Viscera of the Human Body: From the Philosophical Transactions of the Royal Society of London.人体内脏罕见构造的两个实例记述:源自《伦敦皇家学会哲学学报》
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Complications of congenital portosystemic shunts in children: therapeutic options and outcomes.儿童先天性门体分流的并发症:治疗选择和结果。
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Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting.一名患有阿伯内西畸形、胆道闭锁、室间隔缺损和肺内分流的儿童成功接受活体供肝移植。
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Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies.先天性门静脉缺如:两例报告及门静脉系统血管异常的分类系统建议
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