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核医学在一例伴有肺部受累的遗传性出血性毛细血管扩张症中的作用。

The role of nuclear medicine in a case of Rendu-Osler-Weber disease with pulmonary involvement.

作者信息

Barral Carlyle Marques, Nunes Isabella Correa Chaves, Nunes Shirleide Santos, Sanches Sandra Monetti Dumont

机构信息

Densitometry and Nuclear Medicine Physician, Head of the Nuclear Medicine Service of Hospital das Clínicas-Federal University of Minas Gerais Clinics (HC-UFMG), Belo Horizonte, Minas Gerais, Brazill.

Resident doctor, Head of the Nuclear Medicine Service of Hospital das Clínicas-Federal University of Minas Gerais Clinics (HC-UFMG), Belo Horizonte, Minas Gerais, Brazill.

出版信息

World J Nucl Med. 2021 Sep 22;20(4):389-391. doi: 10.4103/wjnm.wjnm_48_21. eCollection 2021 Oct-Dec.

Abstract

Rendu-Osler-Weber syndrome or hereditary hemorrhagic telangiectasia (HHT) is a rare systemic disease. Its primary pathogenic expression is multiple arteriovenous malformations (AVM) and severe hypoxia. A case of suspected pulmonary embolism in a 49-year-old male with intestinal, cardiac, and pulmonary HHT affection is reported. Pulmonary AVM could create an apparent mismatch perfusion defect evident upon ventilation and perfusion scan (V/Q scan), leading to misinterpretation. It reinforces the importance between clinics, anatomy, and functional evaluation. Care must be taken when interpreting V/Q scan and the reporting physician must be alert to the possible sources of errors.

摘要

遗传性出血性毛细血管扩张症(HHT),又称伦杜-奥斯勒-韦伯综合征,是一种罕见的全身性疾病。其主要致病表现为多发性动静脉畸形(AVM)和严重缺氧。本文报告了一例49岁男性疑似肺栓塞病例,该患者伴有肠道、心脏和肺部的HHT病变。肺动静脉畸形可在通气灌注扫描(V/Q扫描)时产生明显的不匹配灌注缺损,从而导致误诊。这凸显了临床、解剖学和功能评估之间的重要性。解读V/Q扫描时必须谨慎,报告医生必须警惕可能的误差来源。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ffd0/8686737/1781efee1815/WJNM-20-389-g001.jpg

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