戈谢病合并右向左分流的肝肺综合征：使用锝-99m 标记的大聚合人血清白蛋白进行评估和测量

Hepatopulmonary syndrome in Gaucher disease with right-to-left shunt: evaluation and measurement using Tc-99m MAA.

作者信息

Kim J H, Park C H, Pai M S, Hahn M H, Kim H J

机构信息

Department of Radiology, Ajou University School of Medicine, Suwon, Korea.

出版信息

Clin Nucl Med. 1999 Mar;24(3):164-6. doi: 10.1097/00003072-199903000-00005.

DOI:10.1097/00003072-199903000-00005

PMID:10069725

Abstract

Hepatomegaly is a common manifestation in Gaucher disease. In some patients with the disease, hepatic fibrosis and portal hypertension are observed. A patient with Gaucher disease with the hepatopulmonary syndrome associated with severe cyanosis and hypoxemia was examined for intrapulmonary right-to-left shunt using Tc-99m MAA. Quantitative evaluation revealed an approximately 50% right to left shunt as indicated by Tc-99m MAA activity in the lungs and systemic organs.

摘要

肝肿大是戈谢病的常见表现。在一些患有该疾病的患者中，可观察到肝纤维化和门静脉高压。对一名患有戈谢病且伴有严重紫绀和低氧血症的肝肺综合征患者，使用锝-99m标记的巨聚白蛋白（Tc-99m MAA）检查肺内右向左分流情况。定量评估显示，根据肺和全身器官中Tc-99m MAA的活性，右向左分流约为50%。