الملخص
Congenital pure red cell aplasia, also known as Diamond-Blackfan anemia (DBA), is a hereditary disease characterized by pure red cell aplasia and congenital malformation. Its main clinical features are anemia, dysplasia, and tumor susceptibility. Ribosomal protein (RP) gene mutation is the main pathogenesis of DBA. The most common type of gene mutation is RPS19 gene mutation. Heterozygous mutations in as many as 19 RP genes and other non-RP genes mutations have been identified in DBA. This review summarized briedfly the latest research advances in the pathogenesis of DBA.
الموضوعات
Humans , Anemia, Diamond-Blackfan , Mutation , Ribosomesالملخص
Objective: To make full usage of resource and turn waste into treasure, the chemical constituents and bioactivity were firstly investigated on Damask rose (Rosa damascena) flower residue (DRFR). Methods: DPPH and ABTS experiments were applied to assess the antioxidant activity of DRFR. Then, column chromatography was used to purify compounds from an antioxidation extract (DRFR-A), and the chemical structure was identified using NMR. The total phenolic acid content was measured by Folin-Ciocalteu colorimetric method, and the content of gallic acid of the indicator ingredient was detected by HPLC. Results: DRFR-A was found to show a high activity both on DPPH (IC50: 2.760 µg/mL) and ABTS (IC50: 2.258 µg/mL) compared to positive control VC. Ten compounds were isolated and identified as quercetin (1), kaempferol (2), gallic acid (3), protocatechuic acid (4), pyrogallic acid (5), 2-phenylethyl 3,4,5-trihydroxybenzoate (6), methyl gallate (7), p-hydroxybenzoic acid (8), p-hydroxyphenethyl alcohol (9) and astragalin (10) from DRFR-A. Among them, pyrogallic acid, 2-phenylethyl-3, 4, 5-trihydroxybenzoate, p-hydroxybenzoic acid and p-hydroxyphenethyl alcohol are obtained from the plant for the first time. The content of total phenolic acids and gallic acid, main ingredient in DRFR-A was determined as 63.73% and 24.67%, respectively. Conclusion: This study provides a reliable data and lays the foundation for the development and utilization of rose residue, and hence for the full utilization of rose resources.
الملخص
OBJECTIVE: Using a pulsating coronary artery phantom at high heart rate settings, we investigated the efficacy of a motion correction algorithm (MCA) to improve the image quality in dual-energy spectral coronary CT angiography (CCTA). MATERIALS AND METHODS: Coronary flow phantoms were scanned at heart rates of 60–100 beats/min at 10-beats/min increments, using dual-energy spectral CT mode. Virtual monochromatic images were reconstructed from 50 to 90 keV at 10-keV increments. Two blinded observers assessed image quality using a 4-point Likert Scale (1 = non-diagnostic, 4 = excellent) and the fraction of interpretable segments using MCA versus conventional algorithm (CA). Comparison of variables was performed with the Wilcoxon rank sum test and McNemar test. RESULTS: At heart rates of 70, 80, 90, and 100 beats/min, images with MCA were rated as higher image scores compared to those with CA on monochromatic levels of 50, 60, and 70 keV (each p < 0.05). Meanwhile, at a heart rate of 90 beats/min, image interpretability was improved by MCA at a monochromatic level of 60 keV (p < 0.05) and 70 keV (p < 0.05). At a heart rate of 100 beats/min, image interpretability was improved by MCA at monochromatic levels of 50 keV (from 69.4% to 86.1%, p < 0.05), 60 keV (from 55.6% to 83.3%, p < 0.05) and 70 keV (from 33.3% to 69.3%, p < 0.05). CONCLUSION: Low-keV monochromatic images combined with MCA improves image quality and image interpretability in CCTAs at high heart rates.
الموضوعات
Angiography , Coronary Vessels , Heart Rate , Heart , Tomography, X-Ray Computedالملخص
OBJECTIVE: The quality and radiation dose of different tube voltage sets for chest digital radiography (DR) were compared in a series of pediatric age groups. MATERIALS AND METHODS: Forty-five hundred children aged 0-14 years (yr) were randomly divided into four groups according to the tube voltage protocols for chest DR: lower kilovoltage potential (kVp) (A), intermediate kVp (B), and higher kVp (C) groups, and the fixed high kVp group (controls). The results were analyzed among five different age groups (0-1 yr, 1-3 yr, 3-7 yr, 7-11 yr and 11-14 yr). The dose area product (DAP) and visual grading analysis score (VGAS) were determined and compared by using one-way analysis of variance. RESULTS: The mean DAP of protocol C was significantly lower as compared with protocols A, B and controls (p < 0.05). DAP was higher in protocol A than the controls (p <0.001), but it was not statistically significantly different between B and the controls (p = 0.976). Mean VGAS was lower in the controls than all three protocols (p < 0.001 for all). Mean VGAS did not differ between protocols A and B (p = 0.334), but was lower in protocol C than A (p = 0.008) and B (p = 0.049). CONCLUSION: Protocol C (higher kVp) may help optimize the trade-off between radiation dose and image quality, and it may be acceptable for use in a pediatric age group from these results.
الموضوعات
Adolescent , Child , Child, Preschool , Humans , Infant , Infant, Newborn , Age Factors , Analysis of Variance , Pediatrics/standards , Prospective Studies , Radiation Dosage , Radiation Protection/standards , Radiographic Image Enhancement/standards , Radiography, Thoracic/standardsالملخص
<p><b>BACKGROUND</b>The computed tomography (CT) findings of invasive pulmonary aspergillosis (IPA) are unclear in non-hematological patients. The present study was a retrospective evaluation of CT images in non-hematological patients with IPA.</p><p><b>METHODS</b>All adult patients who met the 2008 European Organization for Research and Treatment of Cancer/Mycoses Study Group (EORTC/MSG) criteria for proven or probable IPA were included during a 5-year study at our institutions. Initial CT findings in our cohort were retrospectively reviewed by two independent thoracic radiologists blinded to patient demographics and clinical outcomes. The presence, pattern, and distribution of abnormalities were recorded.</p><p><b>RESULTS</b>Twenty-three non-hematological patients with pathologically confirmed IPA were included in our study. Areas of ground-glass opacities were present in 14 patients (61%), which were bilateral in 10 patients and unilateral in four. This pattern mainly involved the middle and upper lung zones. Air-space consolidation was identified in 12 patients (52%), and the areas were distributed along the bronchus or subpleura in most cases. Other findings, including five small nodules (22%), three macronodules (13%), and one halo sign (4%), were less common.</p><p><b>CONCLUSIONS</b>CT findings of IPA in non-hematological patients frequently manifested as acute bronchopneumonia, and ground-glass opacities and air-space consolidations were the most common CT findings of IPA in these patients.</p>
الموضوعات
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Invasive Pulmonary Aspergillosis , Diagnostic Imaging , Pathology , Retrospective Studies , Tomography, X-Ray Computed , Methodsالملخص
<p><b>BACKGROUND</b>Although the computer tomography (CT) or magnetic resonance imaging (MRI) findings of alveolar echinococcosis (AE) have been well documented, the consecutive imaging changes of this disease in each PNM stage (parasite lesion, neighboring organ invasion, metastases) were not described accurately. The aim of this study was to analyze the correlation between imaging type and PNM stage and diameter of AE lesions, and to explore the development features of this disease.</p><p><b>METHODS</b>A total of 87 patients with AE were examined using CT and MRI before medical management. Imaging features including the maximum diameter, calcification pattern, and imaging type of lesion were retrospectively assessed. The correlation of imaging type with PNM stage, diameter and calcification pattern was analyzed.</p><p><b>RESULTS</b>Lesions (n=111) in 87 patients were divided into three types based on imaging characteristics; solid type (33.3%, 37/111, a solid lesion without liquid necrosis or only small patches of necrosis), mixed type (41.4%, 46/111, solid component surrounding large and/or irregular liquid necrosis area), and pseudo-cystic type (25.2%, 28/111, large cyst without visible solid component). Lesion calcification in the alveolar echinococcosis was categorized into three patterns; mild calcification (45.1%, 50/111, i.e. inconspicuous calcification or punctuate scattered calcification), moderate calcification (46.8%, 52/111, coastline calcification located at the periphery of the lesion, with or without the central dot-calcification) and abundant calcification (8.1%, 9/111, large calcified deposits). Significant differences were found between pseudo-cystic type and other two types in PNM stage, maximum diameter and calcification (P <0.05), but there was no significant difference between solid type and mixed type in those mentioned aspects (P >0.05). No correlation was observed between calcification patterns and maximum diameter (P >0.05).</p><p><b>CONCLUSIONS</b>Solid and mixed type lesions showed some similarities during the course of the disease and accounted for the major form of advanced AE. Pseudo-cystic type represented neither earlier nor advanced stage of AE, but a special presentation during AE development.</p>