II型Abernethy畸形合并先天性多指畸形及所有心室增大1例报告

Cheng Zhu, Min Wang, Qian Hao
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引用次数: 1

摘要

先天性门静脉畸形是由门静脉分流异常引起的先天性门静脉系统畸形。它可以与许多其他先天性畸形相结合。自首例患者报告以来,患者数量有限,导致对这种疾病的了解有限。2018年8月,我们收治了1例II型Abernethy畸形合并先天性多指畸形和全心腔肿大的患者,该病例极为罕见,可作为现有病例的补充。在全面充分评估患者病情的基础上,我们给予患者口服水飞蓟宾(70 mg /次,每日3次)治疗,疗程3个月,并建议患者随访。在最近的随访中,我们了解到该患者的健康状况总体上是令人满意的,无论是从实验室检查结果还是日常生活经历来看。虽然阿伯内蒂畸形的主要治疗方法是手术,但本病例提示,对于某些患者,简单的保守治疗和定期随访是合适的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Case Report: Type II Abernethy Malformation Complicated with Congenital Polydactyly and Enlargement of all Cardiac Chambers
Abernethy malformation is a kind of congenital malformation of the portal vein system caused by abnormal portacaval shunts. It can be in combination with many other congenital malformations. There has been a limited number of patients since the first patient was reported, leading to limited knowledge of this kind of disease. In August 2018, we treated a patient diagnosed with type II Abernethy malformation complicated with both congenital polydactyly and enlargement of all cardiac chambers, which is extremely rare and can be supplementary to the existing cases. According to a comprehensive and adequate assessment of patients' condition, we treated him with oral silybin (70 mg every time and 3 times a day) for 3 months, and advised him to make follow-up visits. At the latest follow-up, we knew the health condition of this patient was generally satisfactory, whether in terms of laboratory test results or his daily life experience. Although the major therapy for Abernethy malformation is surgery, this case suggests that simple conservative treatment with regular follow-up visits can be suitable for certain patients.
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