Predictors of delayed function independence in patients with acute vertebrobasilar artery occlusion achieved successful recanalization after endovascular therapy / 国际脑血管病杂志

Objective:To investigate the prevalence and predictors of delayed function independence (DFI) in patients with acute vertebrobasilar artery occlusion (VBAO) achieved successful recanalization after endovascular therapy.Methods:Patients with acute VBAO received endovascular treatment in the Departments of Neurology, the First Affiliated Hospital of University of Science and Technology and General Hospital of Eastern Theater Command, PLA from December 2015 to December 2018 were retrospectively enrolled. The demographic, clinical, laboratory and imaging data were collected. Early functional independence (EFI) was defined as the modified Rankin Scale score 0-2 at discharge, and DFI was defined as the modified Rankin Scale score 0-2 at 90 d after discharge for non-EFI patients. Multivariate logistic regression analysis was used to determine the independent predictors of DFI. Results:A total of 122 patients with acute VBAO were included. Their age was 61.8±11.9 years old and 91 (74.6%) were male. The median Glasgow Coma Scale (GCS) score was 7, the median National Institutes of Health Stroke Scale (NIHSS) score was 26.5, and the median posterior circulation Alberta Stroke Program Early CT Score (pc-ASPECTS) score was 9. Twenty-four patients (20.0%) had EFI; of the 98 patients with non-EFI, 18 (18.4%) had DFI. Multivariate logistic regression analysis showed that male (odds ratio [ OR] 0.038, 95% confidence interval [ CI] 0.002-0.658; P=0.025), cardiogenic embolism ( OR 0.116, 95% CI 0.023-0.579; P=0.009), baseline NIHSS score ( OR 1.136, 95% CI 1.040-1.242; P=0.005) and lung infection ( OR 6.089, 95% CI 1.451-25.562; P=0.014) were the independent predictors of DFI. Conclusions:Nearly 1/5 of the non-EFI patients have DFI. Male, cardiogenic embolism, lower baseline NIHSS score and without pulmonary infection are the independent predictors of DFI.

Clinical observation of early-onset antinuclear antibody-positive juvenile idiopathic arthritis / 中华微生物学和免疫学杂志

Objective:To investigate the clinical features, treatment and follow-up of children with early-onset antinuclear antibody (ANA)-positive juvenile idiopathic arthritis (JIA).Methods:Eighty-six oligoarticular JIA patients with early-onset arthritis (≤6 years old) admitted to the Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2017 to December 2019 were included in this study. According to ANA titer, these patients were divided into two groups: ANA-positive group (44 cases) and ANA-negative group (42 cases). Clinical data including demographic data, clinical features, laboratory testing results, treatment and follow-up data were statistically analyzed.Results:The ratio of male to female was 7∶37 in the ANA-positive group and 15∶27 in the ANA-negative group and there was significant difference between the two groups ( P=0.035). The proportions of patients with increased C-reactive protein and erythrocyte sedimentation rate were higher in the ANA-positive group than in the ANA-negative group [18.18% (8/44) vs 16.67% (7/42) and 29.55% (13/44) vs 19.05% (8/42), both P>0.05]. The most commonly involved joints in the ANA-positive group were knee (95.45%, 42/44), ankle (20.45%, 9/44) and wrist (18.18%, 8/44), and unilateral asymmetric joint involvement accounted for 81.8% (36/44). In the ANA-negative group, the involved joints were knee (85.71%, 36/42), ankle (14.29%, 6/42), wrist (14.29%, 6/42) and hip (11.90%, 5/42), and 27 out of the 42 cases (64.29%) had unilateral asymmetric joint involvement. There was no significant difference in the above indexes between the two groups (all P>0.05). There were seven cases (15.91%) with uveitis in the ANA-positive group and two cases (4.76%) in the ANA-negative group, and the difference between the two groups was significant ( P=0.045). Before treatment, the ANA-positive group had a significantly higher disease activity score (JADAS27) than the ANA-negative group (14.43±2.87 vs 12.09±3.32, P=0.002). After treatment, the JADAS27 score in both groups decreased (both P<0.05). After six months of treatment, the two groups had similar clinical remission rates [70.45% (31/44) vs 76.19% (32/42), P>0.05]. Conclusions:Early-onset ANA-positive JIA was more common in female children, and asymmetric knee joint involvement was the most common clinical manifestation. The incidence of ophthalmic complications was high, and ophthalmological examination should be performed more frequently during follow-up. The prognosis of early-onset ANA-positive JIA was good with early treatment. Positive ANA was not a risk factor for poor prognosis.

The application potential of a modified wireless intelligent capsule cystoscope for dynamic detection of porcine bladder mucosa / 中华泌尿外科杂志

Objective:To develop an improved wireless intelligent capsule cystoscope (WCE)for dynamic detection of bladder mucosa in a pig model.Methods:The WCE was introduced into a healthy experimental pig that under general anesthesia via urethra by applying an improved device. Multi-angle images of the bladder mucosa were then obtained by controlling the position of capsule cystoscope with an external magnetic field system. The shutter speed of the WCE was 2.5 fps and was automatically converted to 1.5 fps 30 minutes after initiation. The Vue software was utilized to download the shoot pictures which were former received by a computer via wireless transmission. The pig was roused and sent to the pigpen, without limitations in moving. The improved WCE was connected with a 2 cm thread. 12 hours later, the dilated sheath was inserted again, and the capsule was removed by a foreign body forceps under observation of a ureteroscopy.Results:The WCE was successfully placed and removed from the pig's bladder with the application of the improved devices. Over 20 thousand images that with 60K pixels of bladder mucosa were captured by the WCE at various angles within 12 hours, which revealed the process of urine filling and excreting in a time-dependent way. No notable adverse effects (bleeding, urinary tract injury, etc) were noted during the process of cystoscope placement, image acquisition, transmission, and removal.Conclusion:This study developed a novel WCE that could dynamically, intelligently and accurately monitor all aspects of the pig bladder mucosa, and has preferable application prospect.

Clinical characteristics of infantile Takayasu arteritis complicated with cardiac involvements / 中华风湿病学杂志

Objective:To analyze the clinical characteristics of infantile Takayasu Arteritis (TAK) complicated with cardiac involvements.Methods:The clinical data and cardiac lesions of infantile TAK were collected retrospectively, and the clinical characteristics of the disease were analyzed and summarized. Mainly using decriptive statistical methods.Results:In these 20 cases, 16 cases (80%) had cardiac involvements, only 2 cases had related symptoms. The common lesions were coronary artery lesion (CAL), valvular disease, and elevated myocardial enzymes, while the rare lesions were arrhythmia, pericardial effusion, hypertensive heart disease, and heart failure. One case had acute heart failure, which was systolic heart failure and was accompanied by hypertensive heart disease. All 14 patients with CAL were found by conventional coronary ultrasound screening. A total of 39 CAL were found, all of which were coronary artery dilation, and the left main coronary artery was involved. Five patients had heart valve disease, all of them were valve insufficiency. The involved valves were mitral and tricuspid valves, and one of them was severe insufficiency. Arrhythmias were found in 2 cases, of which P1 was found to have paroxysmal atrial tachycardia with high atrioventricular block at 3 months. All 20 children survived and were in stable condition after being treat with biological agents and/or glucocorticoids. A case of hypertensive heart disease complicated with heart failure was followed up for 4 years, and the cardiac function and blood pressure returned to normal. Fourteen children with CAL lesions were given oral aspirin disease, the CALs disappeared in 10 cases and retracted in 4 cases. During the follow-up of 5 children with heart valves, insufficiency disappeared in 4 cases and improved in 1. No child underwent valve replacement during the follow-up. One of the children with arrhythmia was treated with antiarrhythmic drugs. After treatment, the arrhythmia disappeared. Now they have been followed up for 5 years without recurrence.Conclusion:Infantile TAK has a high incidence of heart involvement, with extensive lesions but insidious clinical symptoms. CALs are common, and heart failure is rare. It should be evaluated and treated as early as possible.

Application of project-based learning in clinical teaching of radiation physics for radiotherapy residents / 中华放射肿瘤学杂志

Objective:To observe the effect of project-based learning (PBL) in the clinical teaching of radiation physics.Methods:Thirty-two residents specializing in radiotherapy were included in the study. In the experimental group ( n=16), PBL was adopted, while traditional clinical teaching method was employed in the control group ( n=16). After the rotation, the assessment was conducted, as well as a questionnaire survey was performed, including five aspects: overall satisfaction, understanding of radiation physics knowledge, learning motivation, learning burden, and learning efficiency. Results:The assessment score in the experimental group was 86.31±5.41, which was higher than 75.28±5.91 in the control group, and the difference was statistically significant. Residents in the experimental group were satisfied with the effect of PBL.Conclusion:Compared with the traditional teaching method, PBL can improve the learning motivation, efficiency, and performance of radiotherapy residents, which is highly recognized by the residents.

Follow-up and prognosis analysis of rheumatic disease complicated with pulmonary arterial hypertension in children / 中华实用儿科临床杂志

Objective:To explore the clinical features, follow-up characteristics and prognosis of rheumatic disease complicated with pulmonary arterial hypertension (PAH) in children, and to provide support for its clinical diagnosis and treatment.Methods:A retrospective analysis was conducted on the data of rheumatic 24 patients complicated with PAH hospitalized in the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to the Capital Institute of Pediatrics, Department of Rheumatology and Immunology, Jiangxi Children′s Hospital, Department of Pediatrics Ⅰ, the First Affiliated Hospital of Zhengzhou University and Department of Pediatrics, the Affiliated Hospital of Inner Mongolia Medical University from January 2013 to June 2022.The rheumatic patients complicated with PAH were followed up by telephone on June 30, 2022, and their clinical symptoms, treatment, follow-up, and prognosis data were collected.According to different treatment methods, the patients were divided into different clinical subgroups. The change of PAH was analyzed. The t-test was used for comparison between groups. P<0.05 was statistically significant. Results:A total of 24 cases were enrolled, with 7 males and 17 females.The average onset age of PAH was (10.97±3.79) years old.The median duration of PAH was 6.00 (32.20) months.The average pulmonary artery pressure was (51.71±17.66) mmHg(1 mmHg=0.133 kPa). There were 9 cases of systemic lupus erythematosus, 5 cases of Takayasu′s arteritis, 3 cases of juvenile dermatomyositis, 3 cases of undifferentiated connective tissue disease, 2 cases of systemic juvenile idiopathic arthritis, 1 case of Behcet′s disease, and 1 case of Kawasaki disease.Among 24 cases, the common symptoms were fever (14 cases), fatigue (10 cases) and dyspnea (7 cases). Of the 24 cases, 10 cases were complicated with hydropericardium, 9 cases with valve regurgitation, and 5 cases with decreased systolic and/or diastolic function.Lung changes were observed in 17 cases.Eleven cases were tested for B-type natriuretic peptide (BNP), and the BNP levels were all elevated in them (11 cases), with a median BNP of 3 073 (10 645) ng/L.After the first occurrence of PAH, 12 cases were treated with Methylprednisolone therapy, 10 cases received Cyclophosphamide therapy, and 2 cases who were both systemic lupus erythematosus, underwent blood purification.In the treatment of PAH, 11 cases were treated with pulmonary artery pressure reduction, and 7 of the 11 cases took PAH-targeted drugs.The mean decrease of the average pulmonary artery pressure in children receiving the targeted therapy[(44.80±24.08) mmHg] was significant higher than that in children not receiving the targeted therapy [(16.15±17.25) mmHg] ( t=2.661, P=0.016). Twenty children were reexamined and/or followed up, and the average course of PAH at the telephone follow-up was (36.29±26.67) months.The pulmonary arterial hypertension in 6 cases completely recovered, with median recovery time of 8.00 (13.47) months, but 2 of them died after the complete recovery.The pulmonary arterial hypertension improved in 11 children, 1 of whom died and the remaining children were in stable condition.The pulmonary arterial hypertension worsened in 2 children, 1 of them improved previously but aggravated recently, and the other child did not monitor pulmonary artery pressure and died during telephone follow-up. Conclusions:Rheumatic diseases complicated with PAH are rare and most often diagnosed in severe rheumatic children.It can lead to death, and is commonly accompanied by notably elevated BNP levels.The patients who have early PAH detection, intensive treatment of the primary disease, symptomatic and targeted pulmonary artery pressure reduction show a better prognosis.

Clinical characteristics of 11 cases of anti-melanoma differentiation associated gene 5 autoantibody-positive juvenile dermatomyositis / 中华实用儿科临床杂志

Objective:To clarity the clinical features of juvenile dermatomyositis (JDM) with positive anti-melanoma differentiation associated gene 5 (MDA5) antibody.Methods:Retrospective study.Clinical data of 11 anti-MDA5 autoantibody-positive JDM patients in the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2016 to January 2019 were retrospectively recruited for analyzing their clinical characteristics, pulmonary imaging and pulmonary function, thus summarizing treatment experiences.Results:A total of 11 children with anti-MDA5 autoantibody-positive JDM were recruited, involving 2 males and 9 females, with the average onset age of 1-13 (5.8±4.2) years.Clinical manifestations included rash in 11 cases (100.0%), arthritis in 5 cases (45.5%), and myasthenia in 4 cases (36.4%). Muscle enzyme elevated in 10 cases (90.9%) and serum ferritin (SF) elevated in 9 patients (81.8%). Ten cases (90.9%) showed interstitial lung disease (ILD), manifesting as ground glass opacity at subpleural area on CT scans, restrictive ventilation and decreased diffusion function on lung function test, while respiratory symptoms were absent.All patients were treated with glucocorticoid combined with immunosuppressor.Case 2 developed into rapid progressive pulmonary interstitial disease (RPILD), and died of respiratory failure 2 months later.The remaining was followed up for 1-2 years, and the ILD was relieved.Conclusions:All recruited children with anti-MDA5 autoantibody-positive JDM presented typical rash, and mild muscle weakness with a greater tendency to arthritis.Chinese pediatric patients are prone to complicate with ILD with no respiratory symptoms, but ground glass opacity at subpleural area on CT, and restrictive ventilation and decreased diffusion function on lung function test can be detected.Elevated SF is associated with the development of ILD.Glucocorticoid combined with immunosuppressive therapy is effective to JDM with ILD, but ineffective for RPILD.The mortality of anti-MDA5 autoantibody-positive JDM is high without an effective treatment.

Genome-wide analysis identify novel germline genetic variations in ADCY1 influencing platinum-based chemotherapy response in non-small cell lung cancer

To explore the pharmacogenomic markers that affect the platinum-based chemotherapy response in non-small-cell lung carcinoma (NSCLC), we performed a two-cohort of genome-wide association studies (GWAS), including 34 for WES-based and 433 for microarray-based analyses, as well as two independent validation cohorts. After integrating the results of two studies, the genetic variations related to the platinum-based chemotherapy response were further determined by fine-mapping in 838 samples, and their potential functional impact were investigated by eQTL analysis and in vitro cell experiments. We found that a total of 68 variations were significant at P < 1 × 10-3 in cohort 1 discovery stage, of which 3 SNPs were verified in 262 independent samples. A total of 541 SNPs were significant at P < 1 × 10-4 in cohort 2 discovery stage, of which 8 SNPs were verified in 347 independent samples. Comparing the validated SNPs in two GWAS, ADCY1 gene was verified in both independent studies. The results of fine-mapping showed that the G allele carriers of ADCY1 rs2280496 and C allele carriers of rs189178649 were more likely to be resistant to platinum-based chemotherapy. In conclusion, our study found that rs2280496 and rs189178649 in ADCY1 gene were associated the sensitivity of platinum-based chemotherapy in NSCLC patients.

Dosimetric comparison of three techniques in radiotherapy for breast cancer after modified mastectomy / 中华放射肿瘤学杂志

Objective:To analyze and compare the dosimetric differences based on volumetric-modulated arc therapy (VMAT), fixed field intensity-modulated radiotherapy (F_IMRT), and electron irradiation combined with VMAT (E&VMAT) in radiotherapy for breast cancer after modified mastectomy, aiming to provide reference for clinical selection of treatment plan.Methods:Ten patients with the left breast cancer who received radiotherapy after modified mastectomy were randomly selected. The target areas included chest wall and supraclavicular region, and the prescribed dose was 43.5 Gy in 15 fractions (2.9 Gy/F). Based on the Pinnacle 3 planning system, the VMAT, F_IMRT and E&VMAT plans (electron beam for chest wall, VMAT for supraclavicular area) were designed for each patient. The conformity and homogeneity of the target areas, the dose of organs at risk and treatment time were compared. Results:The VMAT plan could improve the dose distribution of the target areas. The conformity index and homogeneity index of the target dose were significantly better than those of the F_IMRT and E&VMAT plans (all P<0.05). The average dose, V 30Gy, V 20Gy, V 10Gy of the left lung in the VMAT plan were significantly better than those in the F_IMRT and E&VMAT plans (all P<0.05). The V 5Gy of the left lung in the VMAT plan was significantly better than that in the F_IMRT plan ( P<0.05). There was no statistical difference in the V 5Gy of the left lung between the VMAT and E&VMAT plans ( P>0.05). The heart, right breast and right lung of the VMAT plan could meet the clinical dose limit requirements. The treatment time of the VMAT, F_IMRT and E&VMAT plans was (326±27) s, (1 082±169) s, and (562±48) s, respectively. Conclusions:Compared with the F_IMRT and E&VMAT plans, the VMAT plan has better quality and shorter treatment time. VMAT plan has higher value in clinical application compared with the F_IMRT and E&VMAT plans.

Analysis of 7 cases of macrophage activation syndrome secondary to systemic juvenile idiopathic arthritis in infants and toddlers / 中华风湿病学杂志

Objective:To investigate the clinical features, diagnosis and treatment of systemic juvenile idiopathic arthritis (SJIA) complicated with macrophage activation syndrome (MAS).Methods:From January 1st, 2018 to January 1st, 2020, 7 cases of SJIA-MAS were diagnosed. Their clinical and laboratory data were collected and summarized.Results:In these 7 cases, 2 were males and 5 were females, the ratio of male to female was 2∶5. The age range was 11 months to 2 years old. The course of disease was 14 to 32 days. The clinical manifestations included fever and rash in 7 without arthritis; hepatomegaly, splenomegaly and lymphadenopathy in 7; hematological involvement in 7; nervous system involvement in 2; digestive system involvement in 7; respiratory system involvement in 7; cardiovascular involvement in 3. White blood cell was decreased in 1 case, platelet was decreased in 1 case and hemoglobin was decreased in 7 cases. Ferritin, triglyceride, alanine transaminas and aspartate aminotransferase were increased in 7 cases, fibrinogen was significantly decreased in 7 cases, and direct bilirubin was increased in 4 cases. IL-2R was significantly increased. Hemophagocytosis was observed in bone marrow of 4 cases. Cerebrospinal fluid protein was 2 005 mg/L in 1 case. All the 7 cases were tested for exon genes, and no pathogenic mutation was found. All of the 7 cases showed lung lesions in chest CT scan. Multiple demyelinating lesions were found in 1 case by head magnetic resonance imaging. One case was treated with high-dose intravenous methylprednisolone combined with IL-6 receptor antagonist(tocilizumab). The other 6 cases were treated with high-dose intravenous methylprednisolone combined with cyclosporine A (CsA). Two cases were treated with Janus kinases inhibitor(tofacitinib). After treatment, 7 cases got relieved, no death, no recurrence oocurred during the follow-up.Conclusion:Acute onset, multiple organ involvement and no joint inflammation are prominent in MAS of infants and toddlers. High fever, proressive reduction of blood cells and increase of SF are significant in SJIA-MAS. High dose glucocorticoid combined with CsA can benefit in most cases, and some severe cases need to be treated with biological agents.

Prohibitin regulates mTOR pathway via interaction with FKBP8 / 医学前沿

The ability of tumor cells to sustain continuous proliferation is one of the major characteristics of cancer. The activation of oncogenes and the mutation or inactivation of tumor suppressor genes ensure the rapid proliferation of tumor cells. The PI3K-Akt-mTOR axis is one of the most frequently modified signaling pathways whose activation sustains cancer growth. Unsurprisingly, it is also one of the most commonly attempted targets for cancer therapy. FK506 binding protein 8 (FKBP8) is an intrinsic inhibitor of mTOR kinase that also exerts an anti-apoptotic function. We aimed to explain these contradictory aspects of FKBP8 in cancer by identifying a "switch" type regulator. We identified through immunoprecipitation-mass spectrometry-based proteomic analysis that the mitochondrial protein prohibitin 1 (PHB1) specifically interacts with FKBP8. Furthermore, the downregulation of PHB1 inhibited the proliferation of ovarian cancer cells and the mTOR signaling pathway, whereas the FKBP8 level in the mitochondria was substantially reduced. Moreover, concomitant with these changes, the interaction between FKBP8 and mTOR substantially increased in the absence of PHB1. Collectively, our finding highlights PHB1 as a potential regulator of FKBP8 because of its subcellular localization and mTOR regulating role.

Circadian effects of ionizing radiation on reproductive function and clock genes expression in male mouse

BACKGROUND@#Exposure to the ionizing radiation (IR) encountered outside the magnetic field of the Earth poses a persistent threat to the reproductive functions of astronauts. The potential effects of space IR on the circadian rhythms of male reproductive functions have not been well characterized so far.@*METHODS@#Here, we investigated the circadian effects of IR exposure (3 Gy X-rays) on reproductive functional markers in mouse testicular tissue and epididymis at regular intervals over a 24-h day. For each animal, epididymis was tested for sperm motility, and the testis tissue was used for daily sperm production (DSP), testosterone levels, and activities of testicular enzymes (glucose-6-phosphate dehydrogenase (G6PDH), sorbitol dehydrogenase (SDH), lactic dehydrogenase (LDH), and acid phosphatase (ACP)), and the clock genes mRNA expression such as Clock, Bmal1, Ror-α, Ror-β, or Ror-γ.@*RESULTS@#Mice exposed to IR exhibited a disruption in circadian rhythms of reproductive markers, as indicated by decreased sperm motility, increased daily sperm production (DSP), and reduced activities of testis enzymes such as G6PDH, SDH, LDH, and ACP. Moreover, IR exposure also decreased mRNA expression of five clock genes (Clock, Bmal1, Ror-α, Ror-β, or Ror-γ) in testis, with alteration in the rhythm parameters.@*CONCLUSION@#These findings suggested potential health effects of IR exposure on reproductive functions of male astronauts, in terms of both the daily overall level as well as the circadian rhythmicity.

A method of determining the prescription isodose line for stereotactic radiotherapy VMAT plan / 中华放射医学与防护杂志

Objective:To establish a method of determining the prescription isodose line for steretactic radiotherapy (SRT) volumetric modulated arc radiotherapy (VMAT) plan.Methods:Eight patients with brain metastases treated with SRT were enrolled. The volume of planning target volume (PTV) ranged from 3.5 to 11.7 cm 3 (median 6.1 cm 3). Reference VMAT plans were designed for each patient with identical prescription dose. Then, the original PTV was contracted by a few millimeters to form a new target for optimization to get plans with different IDLs. The minimum margin which was needed to be contracted to achieve optimal IDL range for each PTV was also studied. Results:To achieve the optimal IDL range, 4 or 5 mm PTV contraction was needed for all patients, and the average IDL was (66.05±0.02)%. Compared with reference plans, the average gradient index (GI) of optimal IDL plans decreased by 20% from 4.05±0.39 to 3.37±0.24 ( Z=-2.521, P<0.05). The V40, V30, V5 and mean dose in normal brain tissue decreased by 11.5% ( Z=-1.973, P<0.05), 7.2% ( Z=-2.105, P<0.05), 12.8% ( Z=-2.521, P<0.05) and 8.1%, respectively ( Z=-2.382, P<0.05), and there was no statistically significant difference with V20, V10 and conformity index ( P>0.05). Conclusions:The optimization of IDL for SRT-VMAT plan can be achieved with the method of contracting PTV to form new target for planning. 4 or 5 mm is needed to be contracted to achieve the optimal IDL range, and to get lower GI and protect the normal brain tissue.

Analysis of clinical features of 14 infants with Takayasu arteritis / 中华实用儿科临床杂志

Objective To improve the understanding and diagnosis and treatment level of infant with Takayasu arteritis (TA) by analyzing the clinical features of 14 pediatric patients and reviewing related articles.Methods The clinical and follow-up data of infants with TA who were admitted to the Children's Hospital Affiliated to Capital Institute of Pediatrics between July 2016 and May 2019 were retrospectively analyzed.By reviewing related articles,the clinical features of this disease were summarized.Results The age of 14 patients (including 6 males and 8 females) were between 1 month and 23 days and 28 months.The most common clinical manifestations were fever in 10 cases (71.4％),hypertension in 9 cases (64.3％),weak or no pulse in 5 cases (35.7％).According to the clinical type of lesion vessels,11 cases (78.5％) were generalized type,3 cases (21.4％) were brachiocephalic artery type,and there was no thoracic abdominal aorta or single pulmonary artery type in this group.Among 14 infants with TA,12 cases had common carotid artery,carotid artery,subclavian artery,coronary artery and its branches (anterior descending branch,circumflex branch) involved (85.7％);11 cases had renal artery involved (78.6％);9 cases had radial artery involved (64.2％);8 cases had abdominal aorta involved (57.1％);6 cases had descending aorta involved (42.9％);6 cases had thoracic aorta involved (42.9％);6 cases had superior mesenteric artery involved (42.9％);5 cases had femoral artery involved (35.7％);5 cases had pulmonary artery involved (35.7％);and 4 cases had brachial artery involved (28.6％).In those 14 patients,11 cases were misdiagnosed,and 3 cases had unclear diagnosis,with misdiagnosis duration of 18 days to 2 months.In misdiagnosed cases,8 cases were misdiagnosed as atypical Kawasaki disease.Among those 14 cases,the ranges of most lesions were gradually decreased,and the slightly involved vessels even completely returned to normal state after treatment in 7 cases.The vascular imaging showed no significant exacerbation or imnprovement in 4 cases.Nine cases developed hypertension,the blood pressure of whom could be controlled within normal range with hypotensive drugs which could not be interrupted.Physical examination found weak or no pulse in 5 cases who were not improved.Among 14 patients,7 cases showed normal development,while the height and body mass of another 7 cases were the 25th percentile below those of normal children of the same age.All 14 patients were followed up for 2-22 months and received regular treatment without recurrence.Conclusions TA patients aged less than 3 years tend to have more blood vessels involved,be in serious condition and have higher rate of misdiagnosis.The disease can be controlled quickly after treatment,but vascular diseases may be developed easily.Some patients have a poor prognosis.

Analysis of clinical features of 14 infants with Takayasu arteritis / 中华实用儿科临床杂志

Objective@#To improve the understanding and diagnosis and treatment level of infant with Takayasu arteritis (TA) by analyzing the clinical features of 14 pediatric patients and reviewing related articles.@*Methods@#The clinical and follow-up data of infants with TA who were admitted to the Children′s Hospital Affiliated to Capital Institute of Pediatrics between July 2016 and May 2019 were retrospectively analyzed.By reviewing related articles, the clinical features of this disease were summarized.@*Results@#The age of 14 patients (including 6 males and 8 females) were between 1 month and 23 days and 28 months.The most common clinical manifestations were fever in 10 cases (71.4%), hypertension in 9 cases (64.3%), weak or no pulse in 5 cases (35.7%). According to the clinical type of lesion vessels, 11 cases (78.5%) were generalized type, 3 cases (21.4%) were brachiocephalic artery type, and there was no thoracic abdominal aorta or single pulmonary artery type in this group.Among 14 infants with TA, 12 cases had common carotid artery, carotid artery, subclavian artery, coronary artery and its branches (anterior descending branch, circumflex branch) involved (85.7%); 11 cases had renal artery involved (78.6%); 9 cases had radial artery involved (64.2%); 8 cases had abdominal aorta involved (57.1%); 6 cases had descending aorta involved (42.9%); 6 cases had thoracic aorta involved (42.9%); 6 cases had superior mesenteric artery involved (42.9%); 5 cases had femoral artery involved (35.7%); 5 cases had pulmonary artery involved (35.7%); and 4 cases had brachial artery involved (28.6%). In those 14 patients, 11 cases were misdiagnosed, and 3 cases had unclear diagnosis, with misdiagnosis duration of 18 days to 2 months.In misdiagnosed cases, 8 cases were misdiagnosed as atypical Kawasaki disease.Among those 14 cases, the ranges of most lesions were gradually decreased, and the slightly involved vessels even completely returned to normal state after treatment in 7 cases.The vascular imaging showed no significant exacerbation or improvement in 4 cases.Nine cases developed hypertension, the blood pressure of whom could be controlled within normal range with hypotensive drugs which could not be interrupted.Physical examination found weak or no pulse in 5 cases who were not improved.Among 14 patients, 7 cases showed normal development, while the height and body mass of another 7 cases were the 25th percentile below those of normal children of the same age.All 14 patients were followed up for 2-22 months and received regular treatment without recurrence.@*Conclusions@#TA patients aged less than 3 years tend to have more blood vessels involved, be in serious condition and have higher rate of misdiagnosis.The disease can be controlled quickly after treatment, but vascular diseases may be developed easily.Some patients have a poor prognosis.

The microdamage of " morphologically normal white matter" in patients with nasopharyngeal carcinoma after radiotherapy and its correlation with the irradiation dose / 中华放射肿瘤学杂志

Objective@#To explore the correlation between microdamage in white matter and radiotherapy dose at early stage after radiotherapy (RT) in patients with nasopharyngeal carcinoma (NPC).@*Methods@#Thirty-three patients who were initially diagnosed with NPC were recruited and received diffusion tensor imaging (DTI) scan and neuro-cognitive scale test within 1 week before RT and the first day after RT. DTI-related characteristic parameters including fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (λ‖), and radial diffusivity (λ⊥) were calculated based on whole-brain voxel analysis method. Paired t-test was conducted to evaluate statistical significance between pre-RT and post-RT groups. In the subgroup analysis, all the subjects were divided into 3 groups according to the average dose of temporal lobe, and each group was set with an equal dose interval range. The DTI-related parameters of whole brain pre-RT and post-RT in each group were statistically compared. All the statistical results were corrected by FDR with a threshold of P<0.05 and clusters>100.@*Results@#FA, MD, λ‖ and λ⊥in the post-RT group significantly differed compared with those in the pre-RT group (P<0.05). The values of FA, MD, λ‖ and λ⊥were 0.455±0.016, (9.893±0.403)×10-4, (13.441±0.412)×10-4 and (8.231±0.429)×10-4, respectively. Subgroup analysis showed that the extent and degree of λ‖ and λ⊥ changes were exacerbated with the increase of the average dose of temporal lobe after RT. Particularly in high-dose group, the average dose range was 25-35 Gy and the extent of regions with significant changes was significantly larger than those in the medium-dose (15-25 Gy) and low-dose groups (5-15 Gy)(P<0.05).@*Conclusions@#DTI can be utilized to detect" normal" brain tissue microdamage in NPC patients at early stage after RT. The average radiation dose of temporal lobe may be one of the reasons for the severity of cerebral microdamage. In the future, DTI technique may be useful for guiding exposure dose of organs at risk during RT planning and to evaluate the cohort with a high risk of cerebral microdamage at early stage after RT, thereby protecting normal cerebral tissues to the maximum extent.

Realization and application of automatic independent check software for radiotherapy treatment plans / 中华放射肿瘤学杂志

Objective@#To design and implement an automatic independent check software (AutoReview) for the radiotherapy treatment plan and improve the efficiency of independent check.@*Methods@#The patient′s electronic treatment plan chart in the record and verify (R&V) system database accessed by the SQL language was read and parsed. Based on the treatment plan information obtained from the chart, AutoReview extracted all parameters related to the treatment plan from the treatment planning system and the R&V system, and saved the structured data into a local database. For different check items, corresponding check logic was designed and the program was written to realize the automatic independent check. In total, 664 clinical treatment plans for one month were selected as a sample to evaluate the clinical application effect.@*Results@#Compared with manual check, AutoReview improved the check efficiency by nearly 60 times and the problem detection rate was elevated by 19.2%. In the two-year clinical application, the software operation was stable, and 14509 treatment plans were automatically checked and identified 588 cases with problems, accounting for 4.05%.@*Conclusion@#AutoReview can effectively improve the efficiency and accuracy of independent check in radiotherapy treatment plan.

Three cases report of juvenile dermatomyositis with positive anti-melanoma differentiation associated gene 5 (MDA5) antibody and severe interstitial lung disease and literature review / 中华儿科杂志

Objective@#To report the clinical features of anti-MDA5 antibody positive juvenile dermatomyositis (JDM) complicated with severe interstitial lung disease (ILD).@*Methods@#The clinical data of three patients, who was admitted to the Department of Rheumatology and Immunology, Children's Hospital of the Capital Institute of Pediatrics from September 2016 to July 2017, with anti-melanoma differentiation associated gene 5 (MDA5) antibody positive JDM complicated with ILD were retrospectively extracted and analyzed. Meanwhile, PubMed database, CNKI, Wanfang database and China Biology Medicine disc (from their establishment to February 2019) with the key words "juvenile dermatomyositis" "interstitial lung disease" , and "anti-MAD5 antibody" both in English and Chinese were searched.@*Results@#There were 2 females and 1 male (P1-P3), aged from 10 years 3 months to13 years 4 months, the time from onset to diagnosis were 2 months, 4 months and 10 months. All presented with rash. One of them had decreased muscle strength, and two had decreased activity tolerance. Creatine kinase was 588, 915 and 74 U/L, and serum ferritin were 1 792, >2 000 and 195.4 μg/L. All three patients had positive anti-MDA5 antibodies. At the time of diagnosis, all of them had ILD, pneumothorax and mediastinal emphysema, but had no respiratory symptoms. All three patients received oral methylprednisolone and cyclophosphamide pulse therapy, while human immunoglobulin was given only to P1 and P2. P1 developed rapid progressive pulmonary interstitial disease (RPILD) and died of respiratory failure after 2 months. While P2 and P3 were followed up for 1 to 2 years, who had complete remission, as anti-MDA5 antibody turned to negative and ILD improved significantly. Ten related reports in literature were retrieved, without reported Chinese cases, and most cases initiated with rash and very likely complicated with arthritis. Some of them were more likely to have ILD rather than muscle weakness. It also showed that Japanese JDM children had higher rate of positive anti-MDA5 antibody than patients from the U.S. and U.K., and are more susceptible to ILD and RPILD. The mortality rate of patients with RPILD is extremely high.@*Conclusions@#The cases of JDM with positive anti-MDA5 antibody mainly presented with rash and mild muscle weakness, and could be complicated with ILD, pneumothorax and mediastinal emphysema without respiratory symptoms at early stage. Anti-MDA5 antibody titer is related to disease activity and can turn to negative after treatment.

Minimally invasive treatment of pilon fracture with single main plate combined with multiple planar screws for support-ing fixation / 中华骨科杂志

Objective@#To explore the clinical effect of minimally invasive treatment of C type pilon fracture with single main plate combined with multiple planar screws for supporting fixation.@*Methods@#From January 2013 to March 2017, data of 22 patients treated by minimally invasive treatment with single main plate combined with multiple planar screws for supporting fixa-tion were retrospectively analyzed. There were 17 males and 5 females, aged from 23 to 69 years (average, 51.2 years). All cases were closed fractures involving fibula including weight hitting (5 cases), traffic accident (7 cases) and fall injury (10 cases). There were 3 cases of 43-C1 type, 11 cases of 43-C2 type and 8 cases of 43-C3 type according to AO/OTA classification. According to the Rüedi-Allgöwer classification, there were 5 cases of type Ⅱ and 17 cases of type Ⅲ. Complications were recorded postopera-tively and the articular surface reduction was evaluated using the Burwell-Charnley score. At the last follow-up, Tornetta’s pilon fracture clinical treatment outcome criteria was used to evaluate ankle joint function.@*Results@#All the patients were followed up for 13 to 25 months (average, 17.3 months). There were 20 cases of anatomical reduction, 1 case of fair reduction and 1 case of poor reduction according to the Burwell-Charnley score. All the 22 patients were healed with healing time of 3 to 6 months (aver-age, 4.8 months). The efficacy was evaluated according to the evaluation criteria of the clinical treatment results of pilon fracture by Tornetta: excellent in 8 cases, good in 10 cases, fair in 3 cases, and poor in 1 case. The excellent and good rate was 81.8% (18/22) . All patients had no complications such as wound edge blistering, skin necrosis and infection. Among them, 1 case of internal fixa-tion rejection occurred, and the incision was well healed after removal of the internal fixation after 3 months.@*Conclusion@#Mini-mally invasive treatment of C type pilon fracture with single main plate combined with multiple planar screws for supporting fixa-tion not only provides a better anatomical reduction of the articular surface, but also effectively reduces or even avoids incision complications. The long-term clinical results are satisfactory.

Minimally invasive treatment of pilon fracture with single main plate combined with multiple planar screws for supporting fixation / 中华骨科杂志

Objective To explore the clinical effect of minimally invasive treatment of C type pilon fracture with single main plate combined with multiple planar screws for supporting fixation.Methods From January 2013 to March 2017,data of 22 patients treated by minimally invasive treatment with single main plate combined with multiple planar screws for supporting fixation were retrospectively analyzed.There were 17 males and 5 females,aged from 23 to 69 years (average,51.2 years).All cases were closed fractures involving fibula including weight hitting (5 cases),traffic accident (7 cases) and fall injury (10 cases).There were 3 cases of 43-C1 type,11 cases of 43-C2 type and 8 cases of 43-C3 type according to AO/OTA classification.According to the Rtiedi-Allg(o)wer classification,there were 5 cases of type Ⅱ and 17 cases of type Ⅲ.Complications were recorded postoperatively and the articular surface reduction was evaluated using the Burwell-Charnley score.At the last follow-up,Tornetta's pilon fracture clinical treatment outcome criteria was used to evaluate ankle joint function.Results All the patients were followed up for 13 to 25 months (average,17.3 months).There were 20 cases of anatomical reduction,1 case of fair reduction and 1 case of poor reduction according to the Burwell-Charnley score.All the 22 patients were healed with healing time of 3 to 6 months (average,4.8 months).The efficacy was evaluated according to the evaluation criteria of the clinical treatment results of pilon fracture by Tornetta:excellent in 8 cases,good in 10 cases,fair in 3 cases,and poor in 1 case.The excellent and good rate was 81.8％(18/22).All patients had no complications such as wound edge blistering,skin necrosis and infection.Among them,1 case of internal fixation rejection occurred,and the incision was well healed after removal of the internal fixation after 3 months.Conclusion Minimally invasive treatment of C type pilon fracture with single main plate combined with multiple planar screws for supporting fixation not only provides a better anatomical reduction of the articular surface,but also effectively reduces or even avoids incision complications.The long-term clinical results are satisfactory.