Assessment of Inflammation and Calcification in Pseudoxanthoma Elasticum Arteries and Skin with 18F-FluroDeoxyGlucose and 18F-Sodium Fluoride Positron Emission Tomography/Computed Tomography Imaging: The GOCAPXE Trial.

BACKGROUND: Pseudoxanthoma elasticum (PXE) is an inherited metabolic disease characterized by elastic fiber fragmentation and ectopic calcification. There is growing evidence that vascular calcification is associated with inflammatory status and is enhanced by inflammatory cytokines. Since PXE has never been considered as an inflammatory condition, no incidence of chronic inflammation leading to calcification in PXE has been reported and should be investigated. In atherosclerosis and aortic stenosis, positron emission tomography combined with computed tomographic (PET-CT) imaging has demonstrated a correlation between inflammation and calcification. The purpose of this study was to assess skin/artery inflammation and calcification in PXE patients. Methods: 18F-FluroDeoxyGlucose (18F-FDG) and 18F-Sodium Fluoride (18F-NaF) PET-CT, CT-imaging and Pulse wave velocity (PWV) were used to determine skin/vascular inflammation, tissue calcification, arterial calcium score (CS) and stiffness, respectively. In addition, inorganic pyrophosphate, high-sensitive C-reactive protein and cytokines plasma levels were monitored. RESULTS: In 23 PXE patients, assessment of inflammation revealed significant 18F-FDG uptake in diseased skin areas contrary to normal regions, and exclusively in the proximal aorta contrary to the popliteal arteries. There was no correlation between 18F-FDG uptake and PWV in the aortic wall. Assessment of calcification demonstrated significant 18F-NaF uptake in diseased skin regions and in the proximal aorta and femoral arteries. 18F-NaF wall uptake correlated with CS in the femoral arteries, and aortic wall PWV. Multivariate analysis indicated that aortic wall 18F-NaF uptake is associated with diastolic blood pressure. There was no significant correlation between 18F-FDG and 18F-NaF uptake in any of the artery walls. CONCLUSION: In the present cross-sectional study, inflammation and calcification were not correlated. PXE would appear to more closely resemble a chronic disease model of ectopic calcification than an inflammatory condition. To assess early ectopic calcification in PXE patients, 18F-NaF-PET-CT may be more relevant than CT imaging. It potentially constitutes a biomarker for disease-modifying anti-calcifying drug assessment in PXE.

Are orthopedic surgeons exposed to excessive eye irradiation? A prospective study of lens irradiation in orthopedics and traumatology.

INTRODUCTION: Numerous studies in recent years highlighted an increased risk of pathologies related to ionizing radiation in caregivers. A new French decree was adopted on June 4, 2018, dividing by 7.5 the radiation dose authorized in the lens for exposed workers. HYPOTHESIS: The hypothesis of the present study was that ocular irradiation in orthopedic surgeons was below the new legal threshold. METHOD: The equivalent dose (mSv) received by the lens was prospectively assessed in 10 orthopedic surgeons (5 senior, 5 residents), using 3 passive dosimeters placed at the forehead and either temple. Each intervention of each operator was recorded, with dose per area in the operating room at each use of the fluoroscope. RESULTS: All equivalent doses to the lens at the end of the 4 month study period were well below threshold. Doses were not significantly different between forehead and either temple (p=0.7, p=0.6 for the 2 temples). There was no difference according to side of the head (p=0.3). The dose received in the lens correlated with the dose delivered in the room (p=0.004). There were no significant differences in irradiation according to the surgeon's experience (p=0.2) or trauma activity rate (p=0.4). DISCUSSION: No studies have reported equivalent doses to the lens exceeding the authorized limit. But none previously measured equivalent dose to the lens according to the axis of irradiation in the eyes. The present study showed that orthopedic surgeons received as much eye radiation laterally as frontally. Ocular radiation protection needs therefore to be as effective laterally as frontally. The surgeon's experience did not emerge as a protective factor against ocular irradiation.

Quadri-lineage disease involving nodal B-cell marginal zone lymphoma, high-grade B-cell lymphoma, Kaposi's syndrome, and acute myeloid leukemia.

A patient with a marginal zone lymphoma received RCHOP and obtained PR. He received RDHAP, autograft, and obtained CR. Three months later, he developed Kaposi's sarcoma with spontaneous regression. Two months later, he developed DLBCL treated with R-MIV with CR. Thereafter, he developed AML and died a few days later.