Objective:To investigate the status and diagnostic reference levels (DRLs) of adult CT radiation dose based on survey result from some hospitals in Beijing.Methods:From September to December 2023, the survey results for 50 hospitals were collected in Beijing, including 47 tertiary hospitals and 3 second grade general hospitals. The CT data sets in total of 20 items including head (sequential scanning), head (spiral scanning), head perfusion, sinus, neck, chest, chest (low dose scanning), abdomen, pelvis, abdomen-pelvis, chest-abdomen-pelvis, lumbar spine, CT urography, coronary CTA (retrospective), coronary CTA (prospective), head CTA, neck CTA, aorta CTA, leg CTA and knee were collected on clinical commonly used CT scanners with annual qualified state inspection. For each item, radiation dose data was collected continuously and randomly for up to 50 cases for every CT scanner. Using the volume CT dose index (CTDI vol) and dose length product (DLP) as dose parameters, the median value of each item in each hospital was obtained. The median CTDI vol and DLP values of all hospitals were arranged, and the local DRL of each item was set as the 75th percentile of the median values. The obtained DRLs were compared with the DRLs issued by domestic and international radiological protection organizations. Results:A total of 26 031 dose values of adult patients were collected and 25 996 dose values were left while 35 invalid values removed.For different CT users, CTDl vol, DLP and scanning phases were different for the same item. The five items with the highest CTDI were head perfusion, head sequential scanning, head spiral scanning, coronary CTA (retrospective mode), and sinus. The five items with the highest DLP were CTU, head perfusion, leg CTA, chest-abdomen-pelvis and aortic CTA. The CTDI vol of CTU was only 16.9 mGy (75th percentile), while the DLP was as high as 2 394.9 mGy·cm (75th percentile). The survey showed that the DRLs of most items in Beijing was lower than the national DRLs of domestic and foreign standards, and the DRLs of a small number of items were slightly higher. Conclusion:The current CT dose level in Beijing is not consistent with national DRLs released at home and abroad, so it is necessary to establish local DRLs according to the clinical status in Beijing.

Objective:To investigate the radiation dose levels of digital radiography (DR) in adult patients and explore the diagnostic reference level (DRL) of radiation dose in Beijing.Methods:Radiation dose data were collected from 39 medical institutions in Beijing on 18 930 DR examinations of chest anterior and lateral view, abdomen anterior and posterior view, abdomen standing view, cervical spine anterior and lateral view, lumbar spine anterior and lateral view, knee joint anterior and lateral view as well as 5 266 digital mammography (CC view and MLO view). For ordinary DR examinations, the incident air kinetic energy ( Ka, i), incident air specific kinetic energy area product ( PKA) and effective dose ( E) to the examined individuals were used for the investigation and estimation of radiation dose. For digital mammography, the average glandular dose (AGD) was calculated. DRLs should be set at the 75 th percentile of median values obtained in each medical institution. Results:The Ka, i, PKA and E of DRL for Chest (PA), Chest (lateral), abdominal(PA), abdominal (AP), Pelvic(AP), Cervical (AP), Cervical (lateral), Lumbar (AP), Lumbar (lateral), Knee joint (AP) and Knee joint (lateral) of conventional DR were 0.14, 0.31, 1.25, 2.18, 1.86, 0.51, 0.14, 2.97, 8.39, 0.37, 0.37 mGy, 159, 259, 1 917, 2 336, 2 867, 312, 301, 3 500, 3 359, 269, 255 mGy·cm 2, 0.03, 0.05, 0.20, 0.43, 0.23, 0.03, 0.02, 0.47, 0.35, <0.001, <0.001 mSv. The DRLs for digital mammography were calculated to be 1.87 mGy (CC view), 1.94 mGy (MLO view) and 3.99 mGy (accumulated for one examination). Conclusions:The radiation dose from DR examinations is relatively low. In clinical practice, the selection of imaging parameters should be further standardized on the basis of the local DRL.

Objective:To investigate devices for radiodiagnosis used in medical institutions in Beijing, China, as well as radiation workers and the devices′ application frequencies, in order to provide data support for determining radiation protection measures and allocating resources for clinical radiodiagnosis.Methods:In October 2023, questionnaires were distributed to the members of the Radiation Technology Branch of the Beijing Medical Association who work in medical institutions at different levels across varying districts of Beijing. The collected data included information about devices for radiodiagnosis and radiation workers, as well as the application frequencies of various medical X-ray imaging devices during 2018-2022, in 38 medical institutions of Level 3 Grade A, Level 3, and Level 2 in 14 municipal districts of Beijing. Then, the collected data were organized using the Microsoft Power BI software to form report models, followed by the statistical analysis of data on the devices, radiation workers, and application frequencies were using the RStudio software.Results:The 38 medical institutions in Beijing possess 591 devices for radiodiagnosis and 2 018 staff in the departments of radiology, including 1 037 radiologic technologists and 738 physicians, with 1.77 technologists and 1.26 physicians arranged for a single device on average. From 2018 to 2022, among all examinations of radiodiagnosis, computed tomography (CT) scans showed the highest frequencies, with total and average annual growth rates of 138.06% and 108.40%, respectively. Specifically, chest CT scans exhibited total and average annual growth rates of 225.20% and 122.50%, respectively. X-ray imaging ranked second in application frequency, with total average annual growth rates of 70.20% and 91.53%, respectively. Notably, chest X-ray imaging showed total and average annual growth rates of 63.89% and 89.41%, respectively.Conclusions:From 2018 to 2022, X-ray imaging examinations displayed significantly fluctuating frequencies with an overall decline trend in the 38 medical institutions. In contrast, the CT scans showed increased frequencies, with chest CT scans displaying an increased frequency and proportion. This necessitates ensuring the justification of CT scans.

Objective:To investigate the status and diagnostic reference levels (DRLs) of adult CT radiation dose based on survey result from some hospitals in Beijing.Methods:From September to December 2023, the survey results for 50 hospitals were collected in Beijing, including 47 tertiary hospitals and 3 second grade general hospitals. The CT data sets in total of 20 items including head (sequential scanning), head (spiral scanning), head perfusion, sinus, neck, chest, chest (low dose scanning), abdomen, pelvis, abdomen-pelvis, chest-abdomen-pelvis, lumbar spine, CT urography, coronary CTA (retrospective), coronary CTA (prospective), head CTA, neck CTA, aorta CTA, leg CTA and knee were collected on clinical commonly used CT scanners with annual qualified state inspection. For each item, radiation dose data was collected continuously and randomly for up to 50 cases for every CT scanner. Using the volume CT dose index (CTDI vol) and dose length product (DLP) as dose parameters, the median value of each item in each hospital was obtained. The median CTDI vol and DLP values of all hospitals were arranged, and the local DRL of each item was set as the 75th percentile of the median values. The obtained DRLs were compared with the DRLs issued by domestic and international radiological protection organizations. Results:A total of 26 031 dose values of adult patients were collected and 25 996 dose values were left while 35 invalid values removed.For different CT users, CTDl vol, DLP and scanning phases were different for the same item. The five items with the highest CTDI were head perfusion, head sequential scanning, head spiral scanning, coronary CTA (retrospective mode), and sinus. The five items with the highest DLP were CTU, head perfusion, leg CTA, chest-abdomen-pelvis and aortic CTA. The CTDI vol of CTU was only 16.9 mGy (75th percentile), while the DLP was as high as 2 394.9 mGy·cm (75th percentile). The survey showed that the DRLs of most items in Beijing was lower than the national DRLs of domestic and foreign standards, and the DRLs of a small number of items were slightly higher. Conclusion:The current CT dose level in Beijing is not consistent with national DRLs released at home and abroad, so it is necessary to establish local DRLs according to the clinical status in Beijing.

Objective:To investigate the radiation dose levels of digital radiography (DR) in adult patients and explore the diagnostic reference level (DRL) of radiation dose in Beijing.Methods:Radiation dose data were collected from 39 medical institutions in Beijing on 18 930 DR examinations of chest anterior and lateral view, abdomen anterior and posterior view, abdomen standing view, cervical spine anterior and lateral view, lumbar spine anterior and lateral view, knee joint anterior and lateral view as well as 5 266 digital mammography (CC view and MLO view). For ordinary DR examinations, the incident air kinetic energy ( Ka, i), incident air specific kinetic energy area product ( PKA) and effective dose ( E) to the examined individuals were used for the investigation and estimation of radiation dose. For digital mammography, the average glandular dose (AGD) was calculated. DRLs should be set at the 75 th percentile of median values obtained in each medical institution. Results:The Ka, i, PKA and E of DRL for Chest (PA), Chest (lateral), abdominal(PA), abdominal (AP), Pelvic(AP), Cervical (AP), Cervical (lateral), Lumbar (AP), Lumbar (lateral), Knee joint (AP) and Knee joint (lateral) of conventional DR were 0.14, 0.31, 1.25, 2.18, 1.86, 0.51, 0.14, 2.97, 8.39, 0.37, 0.37 mGy, 159, 259, 1 917, 2 336, 2 867, 312, 301, 3 500, 3 359, 269, 255 mGy·cm 2, 0.03, 0.05, 0.20, 0.43, 0.23, 0.03, 0.02, 0.47, 0.35, <0.001, <0.001 mSv. The DRLs for digital mammography were calculated to be 1.87 mGy (CC view), 1.94 mGy (MLO view) and 3.99 mGy (accumulated for one examination). Conclusions:The radiation dose from DR examinations is relatively low. In clinical practice, the selection of imaging parameters should be further standardized on the basis of the local DRL.

Objective:To investigate devices for radiodiagnosis used in medical institutions in Beijing, China, as well as radiation workers and the devices′ application frequencies, in order to provide data support for determining radiation protection measures and allocating resources for clinical radiodiagnosis.Methods:In October 2023, questionnaires were distributed to the members of the Radiation Technology Branch of the Beijing Medical Association who work in medical institutions at different levels across varying districts of Beijing. The collected data included information about devices for radiodiagnosis and radiation workers, as well as the application frequencies of various medical X-ray imaging devices during 2018-2022, in 38 medical institutions of Level 3 Grade A, Level 3, and Level 2 in 14 municipal districts of Beijing. Then, the collected data were organized using the Microsoft Power BI software to form report models, followed by the statistical analysis of data on the devices, radiation workers, and application frequencies were using the RStudio software.Results:The 38 medical institutions in Beijing possess 591 devices for radiodiagnosis and 2 018 staff in the departments of radiology, including 1 037 radiologic technologists and 738 physicians, with 1.77 technologists and 1.26 physicians arranged for a single device on average. From 2018 to 2022, among all examinations of radiodiagnosis, computed tomography (CT) scans showed the highest frequencies, with total and average annual growth rates of 138.06% and 108.40%, respectively. Specifically, chest CT scans exhibited total and average annual growth rates of 225.20% and 122.50%, respectively. X-ray imaging ranked second in application frequency, with total average annual growth rates of 70.20% and 91.53%, respectively. Notably, chest X-ray imaging showed total and average annual growth rates of 63.89% and 89.41%, respectively.Conclusions:From 2018 to 2022, X-ray imaging examinations displayed significantly fluctuating frequencies with an overall decline trend in the 38 medical institutions. In contrast, the CT scans showed increased frequencies, with chest CT scans displaying an increased frequency and proportion. This necessitates ensuring the justification of CT scans.

Hereditary gingival fibromatosis (HGF) is a rare inherited condition with fibromatoid hyperplasia of the gingival tissue that exhibits great genetic heterogeneity. Five distinct loci related to non-syndromic HGF have been identified; however, only two disease-causing genes, SOS1 and REST, inducing HGF have been identified at two loci, GINGF1 and GINGF5, respectively. Here, based on a family pedigree with 26 members, including nine patients with HGF, we identified double heterozygous pathogenic mutations in the ZNF513 (c.C748T, p.R250W) and KIF3C (c.G1229A, p.R410H) genes within the GINGF3 locus related to HGF. Functional studies demonstrated that the ZNF513 p.R250W and KIF3C p.R410H variants significantly increased the expression of ZNF513 and KIF3C in vitro and in vivo. ZNF513, a transcription factor, binds to KIF3C exon 1 and participates in the positive regulation of KIF3C expression in gingival fibroblasts. Furthermore, a knock-in mouse model confirmed that heterozygous or homozygous mutations within Zfp513 (p.R250W) or Kif3c (p.R412H) alone do not led to clear phenotypes with gingival fibromatosis, whereas the double mutations led to gingival hyperplasia phenotypes. In addition, we found that ZNF513 binds to the SOS1 promoter and plays an important positive role in regulating the expression of SOS1. Moreover, the KIF3C p.R410H mutation could activate the PI3K and KCNQ1 potassium channels. ZNF513 combined with KIF3C regulates gingival fibroblast proliferation, migration, and fibrosis response via the PI3K/AKT/mTOR and Ras/Raf/MEK/ERK pathways. In summary, these results demonstrate ZNF513 + KIF3C as an important genetic combination in HGF manifestation and suggest that ZNF513 mutation may be a major risk factor for HGF.
Animals ; Humans ; Mice ; Fibromatosis, Gingival/pathology* ; Gingiva ; Kinesins/genetics* ; Mutation/genetics* ; Phosphatidylinositol 3-Kinases/genetics*

Objective:To assess the clinical and imaging features of SMARCB1-deficient sinonasal carcinoma.Methods:Form January 2016 to November 2021, the clinical data and pretreatment imaging findings of 16 cases with pathologically proven SMARCB1-de?cient sinonasal carcinomas were analyzed retrospectively in Beijing Tongren Hospital, Capital Medical University. Immunohistochemistry for SMARCB1 showed loss of the protein in the tumor nuclie. Clinical and imaging features, including tumor location, TNM stage, size, density of CT, bone change, MRI signal intensity, enhancement pattern, type of time-intensity curve (TIC) of dynamic contrast enhanced MRI (DCE-MRI), apparent diffusion coefficient (ADC) value and diffusion weighted imaging (DWI) were evaluated. For 14 cases, correlation of the ADC value and Ki-67 index was subsequently evaluated with Pearson correlation analysis.Results:For the 16 cases SMARCB1-deficient sinonasal carcinomas, clinical stage of T4 was 12 cases and T3 was 4 cases. The location included ethmoid sinus ( n=4), nasal cavity only ( n=1), both nasal cavity and ethmoid ( n=8), ethmoid and maxillary sinus ( n=1), ethmoid and frontal sinus ( n=1), ethmoid and sphenoid sinus ( n=1). The tumor size was (4.5±1.2) cm. Iso-attenuated of CT images was showed in 13 cases and heterogeneous with necrosis was showed in 3 cases. Focal bone erosion was found in 13 cases and extensive bone destruction was found in 3 cases. Compared with adjacent muscles, T 1WI of all 16 cases showed isointense, with focal hypointense in 3 cases. On T 2WI, the tumor was graded as isointense in 9 cases, hyperintense in 7 cases, with lower inner septal in 6 cases. Enhancement was graded as mild in 11 cases, moderate in 5 cases.MRI Enhancement images showed mild enhancement in 11 cases, moderate enhancement in 5 cases, heterogeneous enhancement in 6 cases, and homogeneous enhancement in 10 cases. For DCE-MRI of 14 cases, there were 10 cases of Ⅲ type and 4 cases of Ⅱ type of the TIC. The ADC value of 14 cases was (1.02±0.27)×10 -3 mm 2/s. The Ki-67 index was 48%±21%. No correlation was observed between Ki-67 index and ADC value ( r=-0.38, P=0.183). Conclusions:SMARCB1-deficient carcinomas are mostly centered in the nasal and ethmoid region of anatomic distribution. Tendency to be infiltrative the adjacent bone structure with invasive bone reaction, mild to moderate heterogeneous enhancement, T 2WI with lower inner septal, and Ⅲ types of TIC are certain suggestive imaging features of the entity.

Objective:To assess the clinical and imaging features of NUT gene-related sinonasal carcinomas (NUT midline carcinome).Methods:The clinical data and pretreatment imaging findings of 5 cases with pathologically proven NUT sinonasal carcinomas were analyzed retrospectively in Beijing Tongren Hospital, Capital Medical University from January 2016 to December 2020. Of 5 cases, the tumors affected 4 females and 1 male with an age range of 15 to 48 years (median 19 years). Clinical data of all cases were available before surgery with both CT and MR examination. Tumor location, CT density, boney change, calcification, tumor size, T 1WI, T 2WI and diffusion weighted imaging (DWI) signal intensity, appearance diffusion coefficient (ADC), type of time intensity curve (TIC) of dynamic contrast-enhanced (DCE)-MRI were evaluated. Results:All five cases belonged to T4 stage of the clinic TNM system. The locations were nasal cavity ethmoid, sphenoid and maxillary sinus ( n=1), nasal and maxillary sinus ( n=1), nasal cavity and ethmoid sinus ( n=3). Iso-attenuated in 3 cases, heterogeneous with local necrosis in 2 cases, and heterogeneous with calcification in 3 cases on CT imaging. Bone erosion was found in 4 cases, and bone erosion with destruction in 1 case. The tumor sizes ranged from 4.2 to 4.9 cm (median 4.5 cm) on MR axial imaging. On T 1WI, 5 cases showed isointense compared with adjacent temporal muscles, with focal hypointense in 2 cases. On T 2WI, the tumor was graded as isointense in 3 cases, and hyperintense in 2 cases. Heterogeneous enhancement in all cases with mild in 3 cases, and moderate in 2 cases on postcontrast MR imaging. On DCE-MRI of 5 cases, there were 3 cases of type Ⅲ (washout-shaped curves), and 2 cases of type Ⅱ of the TIC (plateau-shaped curves). The range of ADC values was from 0.63×10 -3 to 1.17×10 -3 mm 2/s, and median ADC value was 0.84×10 -3 mm 2/s, of 5 cases with varying degrees of high signal on DWI. The Ki-67 index ranged from 30% to 80% of the tumor. An immunohistochemical study showed that the tumor cells of 5 cases were all positive for both NUT and INI-1 genes. One case was performed with biopsy and followed by chemotherapy, four cases were performed with surgery, combined with the following chemotherapy, and one also was implemented with radiation therapy. The follow-up time was 7-16 months. Five cases were all alive during the follow-up. Conclusions:The NUT midline sinonasal tract carcinoma is a rare, gene-related solid malignant tumor. The tumor is more commonly seen in young patients, mostly centered in the nasal and ethmoid region with invasive growth, more calcification on CT, and heterogeneous enhancement on MRI. These findings are some characteristics of the tumor.

Objective:To compare the diagnostic value of three quantitative evaluation methods based on three-dimensional rapid fluid attenuation inversion recovery sequence (3D-FLAIR) vein-enhanced labyrinth images in endolymphatic hydrops.Methods:From October 2017 to April 2019, a retrospective study was conducted on 86 patients with unilateral otogenic vertigo who were admitted to Beijing Tongren Hospital, Capital Medical University. MRI was performed 8 h after the single-dose Gd-DTPA intravenously injection in all patients. Three evaluation methods were used to calculate the ratio of the endolymphatic area to the total lymphatic area, the ratio of the saccule to utricle area, and the ratio of the endolymphatic volume to the total lymphatic volume, respectively. The paired t test was used to compare the three ratios between the affected and healthy ears. With clinical diagnosis as the gold standard, the receiver operating characteristic (ROC) curve analysis was used to analyze the efficacy of three methods in diagnosing endolymphatic hydrops. Results:Totally 65 cases were finally diagnosed endolymphatic hydrops clinically. There were statistically significant differences of all the 3 ratios between the affected and healthy ears ( t=9.93, 7.22, 8.20, all P<0.001). The ROC curve showed that the area under the curve (AUC) of endolymph/total lymph area ratio, saccule/utricle area ratio, endolymph/total lymph volume ratio for diagnosis of endolymphatic hydrops were 0.882, 0.768, 0.884 (all P<0.001). And there were no significant differences between each paired AUCs (all P>0.05). Conclusions:All three methods of endolymph/total lymph area ratio, saccule/utricle area ratio, endolymph/total lymph volume ratio can quantitatively evaluate endolymphatic hydrops. The endolymphatic/total lymphatic area ratio method is still the most convenient method at present.