ABSTRACT
Autoimmune polyendocrine syndrome type-1 (APS1) is characterized by autoimmune manifestations affecting different organs from early childhood on. Immunological abnormalities, the resulting endocrinopathies, and their treatments may compromise bone health. For the first time in APS1, we analyzed transiliac bone biopsy samples by bone histomorphometry and quantitative backscattered electron imaging in three adult patients (female P1, 38 years; male P2, 47 years; male P3, 25 years). All had biallelic mutations in the autoimmune regulator gene and in addition to endocrinopathies, also significant bone fragility. Histomorphometry showed bone volume in the lower normal range for P1 (BV/TV, - 0.98 SD) and P3 (- 1.34 SD), mainly due to reduced trabecular thickness (TbTh, - 3.63 and - 2.87 SD). In P1, osteoid surface was low (OS/BS, - 0.96 SD); active osteoblasts and double labeling were seen only on cortical bone. P3 showed a largely increased bone turnover rate (BFR/BV, + 4.53 SD) and increased mineralization lag time (Mlt, + 3.40 SD). Increased osteoid surface (OS/BS, + 2.03 and + 4.71 SD for P2 and P3) together with a large proportion of lowly mineralized bone area (Trab CaLow, + 2.22 and + 9.81 SD for P2 and P3) and focal mineralization defects were consistent with abnormal mineralization. In all patients, the density and area of osteocyte lacunae in cortical and trabecular bone were similar to healthy adults. The bone tissue characteristics were variable and included decreased trabecular thickness, increased amount of osteoid, and abnormal mineralization which are likely to contribute to bone fragility in patients with APS1.
Subject(s)
Bone Density , Polyendocrinopathies, Autoimmune , Adult , Humans , Male , Child, Preschool , Female , Polyendocrinopathies, Autoimmune/genetics , Bone and Bones , Cortical Bone , Bone MatrixABSTRACT
Objective:To investigate the effect of the combination of Auto-prescription and ODM full on image quality and radiation dose of chest enhanced CT inorder to explore the feasibility of individualized low radiation dose chest enhanced CT. Methods:The phantoms PH-75 in two sizes were scanned with four scanning modes: group A, 120 kVp + Smart-mA; group B, 120 kVp + Smart-mA+ ODM full; group C, Auto-prescription + Smart-mA; group D, Auto-prescription + Smart-mA+ ODM full. The images of each group were reconstructed with 40%-80% ASIR-V (interval 20%), recorded as A 1-3-D 1-3. Image quality and radiation dose were evaluated in order to obtain optimal scanning modes. Totally 35 cases(from the First Hospital of Dalian Medical University during November 2021 to July 2022) of chest enhancement CT were collected prospectively in the clinical study using the parameters of D 2 group recorded as D patients. Patients in this group received chest enhancement CT scanning for the second time within three months. The CT parameters in first enhanced scanning recorded as A patient were as same as those in group A 1, and the parameters in second scanning recorded as D patient were as same as those in group D 2. The SNRs, CNRs, subjective grading and imaging findings of the lesion in two groups were compared. Results:In phantom study, the radiation dose of each group was statistically significant, and that in D group was the lowest. For two sizes of phantoms, A 1 was uesd as the control group in pairwise comparison. The SDs of B 1, C 1, and D 2 images had no statistical difference with that of A 1 ( P > 0.05), and SD of D 1was higher than that of A 1, and SDs of other groups were lower than that of A 1( F=10.77, 122.50, P<0.05). CNR of B 1 had no statistical difference with that of A 1, and the CNRs of other groups were higher( F=136.20, 30.21, P<0.05). Subjective scores of A 2, C 2, D 2 had no statistical difference with that of A 1 and those of other groups were lower than that of A 1( H=52.89, 43.95, P<0.05). In clinical study, 80 kVp was uesd for 22 cases and 100 kVp was used for 13 cases in D patients group. SNRs and CNRs in the D patients group were all higher than those in the A patients group ( P > 0.05). The consistency of image quality scores for two observers was good ( kappa = 0.754, P < 0.05). The median scores of the two groups were (4, 4). Conclusion:Auto-prescription combined with ODM full in chest enhanced CT imaging can achieve individualized low radiation dose with meeting the requirements of image quality and clinical diagnosis.
ABSTRACT
Objective To detect the impact of Diltiazem on the major adverse cardiac events (MACE) in six months after percutaneous coronary intervention (PCI).Methods A total of 192 patients after PCI with coronary atherosclerotic heart disease were enrolled in this study.The patients were randomly divided into Diltiazem therapy group (101 patients) and non-Diltiazem therapy group (91 patients).The high-sensitivity C-reactive protein (hs-CRP) was assessed before and 24 h after PCI,and the incidence of Major adverse cardiovascular events(MACEs) were assessed at the sixth month after PCI.Results Compared with before PCI,hs-CRP level increased significantly in both group after PCI (P＜0.01),but hs-CRP level was lower in Diltiazem therapy group than in non-Diltiazem therapy group (P＜0.05).Compared with non-Diltiazem therapy group,there was lower incidence of MACEs during six months follow-up in Diltiazem therapy group.Conclusions Diltiazem can decrease the incidence of MACEs during six months after PCI.
