常染色体显性多囊肾病患者的食管动脉假性动脉瘤和高须动脉炎。

Pub Date : 2018-06-01 Epub Date: 2018-06-30 DOI:10.5049/EBP.2018.16.1.11
Hyunsuk Kim, Yeonsil Yu, Kwang Eon Shim, Jin Eop Kim, Junga Koh, Jong-Woo Yoon, Curie Ahn, Yun Kyu Oh
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引用次数: 1

摘要

一名先前被诊断为ADPKD的47岁女性因上腹部和背部突然疼痛而就诊。食管胃十二指肠镜,增强腹部计算机断层扫描(CT)和CT血管造影发现食管动脉假性动脉瘤和食管血肿。进行了紧急血管造影和栓塞。手术后,由于两臂血压差异,进行了CT血管造影和正电子发射断层扫描。患者也被发现患有高须动脉炎,随后接受门诊随访治疗。导致ADPKD患者血管异常的可能机制包括PKD突变引起的多囊蛋白表达异常导致血管完整性受损和结缔组织异常。需要进一步的研究来证实这些机制,并评估ADPKD患者是否存在血管异常。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Esophageal Artery Pseudoaneurysm and Takayasu Arteritis in a Patient with Autosomal Dominant Polycystic Kidney Disease.
A 47-year-old female previously diagnosed with ADPKD visited the hospital due to sudden pain in her upper abdomen and back. Esophagogastroduodenoscopy, contrast-enhanced abdominal computed tomography (CT), and CT angiography identified an esophageal artery pseudoaneurysm and hematoma in the esophagus. Urgent angiography and embolization were performed. After the procedure, CT angiography and positron emission tomography were performed due to differences in blood pressure between the arms. The patient was also found to have Takayasu arteritis and subsequently received outpatient follow-up care. The possible mechanisms that cause vascular abnormalities in ADPKD patients include damaged vascular integrity due to abnormal polycystin expression caused by PKD mutations and connective tissue abnormalities. Further research is needed to confirm these mechanisms, and ADPKD patients should be assessed for vascular abnormalities.
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