系列心脏F-FDG PET-MRI在安德森-法布里病中的作用：一项初步研究。
Role of serial cardiac F-FDG PET-MRI in Anderson-Fabry disease: a pilot study.
作者信息
Nappi Carmela, Ponsiglione Andrea, Pisani Antonio, Riccio Eleonora, Di Risi Teodolinda, Pieroni Maurizio, Klain Michele, Assante Roberta, Acampa Wanda, Nicolai Emanuele, Spinelli Letizia, Cuocolo Alberto, Imbriaco Massimo
机构信息
Department of Advanced Biomedical Sciences, University Federico II, Via Pansini 5, 80131, Naples, Italy.
Department of Public Health, University Federico II, Via Pansini 5, 80131, Naples, Italy.
出版信息
Insights Imaging. 2021 Sep 6;12(1):124. doi: 10.1186/s13244-021-01067-6.
AIM
We investigated the value of serial cardiac F-FDG PET-MRI in Anderson-Fabry disease (AFD) and the potential relationship of imaging results with FASTEX score.
METHODS AND RESULTS
Thirteen AFD patients underwent cardiac F-FDG PET-MRI at baseline and follow-up. Coefficient of variation (COV) of FDG uptake and FASTEX score were assessed. At baseline, 9 patients were enzyme replacement therapy (ERT) naïve and 4 patients were under treatment. Two patients presented a FASTEX score of 0 indicating stable disease and did not show any imaging abnormality at baseline and follow-up PET-MRI. Eleven patients had a FASTEX score > 20% indicating disease worsening. Four of these patients without late gadolinium enhancement (LGE) and with normal COV at baseline and follow-up had a FASTEX score of 35%. Three patients without LGE and with abnormal COV at baseline and follow-up had a FASTEX score ranging from 30 to 70%. Three patients with LGE and abnormal COV at baseline and follow-up had a FASTEX score between 35 and 75%. Finally, one patient with LGE and normal COV had a FASTEX score of 100%. Of the 12 patients on ERT at follow-up, FASTEX score was significantly higher in those 4 showing irreversible cardiac injury at baseline compared to 8 with negative LGE (66 ± 24 vs. 32 ± 21, p = 0.03).
CONCLUSION
F-FDG PET-MRI may be effective to monitor cardiac involvement in AFD.
目的
我们研究了连续心脏F-FDG PET-MRI在安德森-法布里病（AFD）中的价值以及成像结果与FASTEX评分之间的潜在关系。
方法与结果
13例AFD患者在基线期和随访期接受了心脏F-FDG PET-MRI检查。评估了FDG摄取的变异系数（COV）和FASTEX评分。在基线期，9例患者未接受酶替代疗法（ERT），4例患者正在接受治疗。2例患者的FASTEX评分为0，表明疾病稳定，在基线期和随访期的PET-MRI检查中未显示任何成像异常。11例患者的FASTEX评分>20%，表明疾病恶化。其中4例患者在基线期和随访期无延迟钆增强（LGE）且COV正常，其FASTEX评分为35%。3例患者在基线期和随访期无LGE但COV异常，其FASTEX评分在30%至70%之间。3例患者在基线期和随访期有LGE且COV异常，其FASTEX评分在35%至75%之间。最后，1例有LGE且COV正常的患者FASTEX评分为100%。在随访期接受ERT治疗的12例患者中，与8例LGE阴性患者相比，4例在基线期显示不可逆心脏损伤的患者的FASTEX评分显著更高（66±24 vs. 32±21，p = 0.03）。
结论
F-FDG PET-MRI可能对监测AFD患者的心脏受累情况有效。
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