Fatty Acid Accumulation and Resulting PPARα Activation in Fibroblasts due to Trifunctional Protein Deficiency

To examine fatty acid accumulation and its toxic effects in cells, we analyzed skin fibroblasts from six patients with mitochondrial trifunctional protein deficiency, who had abnormalities in the second through fourth reactions in fatty acid β-oxidation system. We found free fatty acid accumulation, enhanced three acyl-CoA dehydrogenases, catalyzing the first reaction in the β-oxidation system and being assumed to have normal activities in these patients, and PPARα activation that was confirmed in the experiments using MK886, a PPARα specific antagonist and fenofibrate, a PPARα specific agonist. These novel findings suggest that the fatty acid accumulation and the resulting PPARα activation are major causes of the increase in the β-oxidation ability as probable compensation for fatty acid metabolism in the patients' fibroblasts, and that enhanced cell proliferation and increased oxidative stress due to the PPARα activation relate to the development of specific clinical features such as hypertrophic cardiomyopathy, slight hepatomegaly, and skeletal myopathy. Additionally, significant suppression of the PPARα activation by means of MK886 treatment is assumed to provide a new method of treating this deficiency

Enlargement of accessory spleen subsequent to splenectomy associated with gastrectomy can mimic a solitary tumor : report of a case

We report a case of a 65-year-old woman with an incidental about 20-mm solitary mass between the lateralsegment of the left lobe of the liver and left kidney 5 years after splenectomy associated with total gastrectomy. The mass wassurgically resected, and histological examination revealed it to be an accessory spleen. Small accessory spleens mostly locatednear the splenic hilus, but large accessory spleens are unusual after total gastrectomy with regional lymph nodes resection. Theremaining accessory splenic tissue would undergo compensatory hypertrophy. Hence, the possibility of accessory spleens mustbe considered when an intra-abdominal mass is identified in a patient with splenectomy associated with gastrectomy

Optimization of iterative reconstruction parameters with attenuation correction, scatter correction and resolution recovery in myocardial perfusion SPECT/CT

Objective: The aim of this study was to characterize the optimal reconstruction parameters for ordered-subset expectation maximization (OSEM) with attenuation correction, scatter correction, and depth-dependent resolution recovery (OSEMACSCRR). We assessed the optimal parameters for OSEMACSCRR in an anthropomorphic torso phantom study, and evaluated the validity of the reconstruction parameters in the groups of normal volunteers and patients with abnormal perfusion. Methods: Images of the anthropomorphic torso phantom, 9 normal volunteers and 7 patients undergoing myocardial perfusion SPECT were acquired with a SPECT/CT scanner. SPECT data comprised a 64 × 64 matrix with an acquisition pixel size of 6.6 mm. A normalized mean square error (NMSE) of the phantom image was calculated to determine both optimal OSEM update and a full width at half maximum (FWHM) of Gaussian filter. We validated the myocardial count, contrast and noise characteristic for clinical subjects derived from OSEMACSCRR processing. OSEM with depth-dependent resolution recovery (OSEMRR) and filtered back projection (FBP) were simultaneously performed to compare OSEMACSCRR. Results: The combination of OSEMACSCRR with 90-120 OSEM updates and Gaussian filter with 13.2-14.85 mm FWHM yielded low NMSE value in the phantom study. When we used OSEMACSCRR with 120 updates and Gaussian filter with 13.2 mm FWHM in the normal volunteers, myocardial contrast showed significantly higher value than that derived from 120 updates and 14.85 mm FWHM. OSEMACSCRR with the combination of 90-120 OSEM updates and 14.85 mm FWHM produced lowest % root mean square (RMS) noise. Regarding the defect contrast of patients with abnormal perfusion, OSEMACSCRR with the combination of 90-120 OSEM updates and 13.2 mm FWHM produced significantly higher value than that derived from 90-120 OSEM updates and 14.85 mm FWHM. OSEMACSCRR was superior to FBP for the % RMS noise (8.52 ± 1.08 vs. 9.55 ± 1.71, p = 0.02) and defect contrast (0.368 ± 0.061 vs. 0.327 ± 0.052, p = 0.01), respectively. Conclusions: Clinically optimized the number of OSEM updates and FWHM of Gaussian filter were (1) 120 updates and 13.2 mm, and (2) 90-120 updates and 14.85 mm on the OSEMACSCRR processing, respectively. Further assessment may be required to determine the optimal iterative reconstruction parameters in a larger patient population. © 2013 The Japanese Society of Nuclear Medicine