Humans ; Amyotrophic Lateral Sclerosis/drug therapy* ; Benzofurans/therapeutic use* ; Double-Blind Method ; Treatment Outcome

Objective To explore the feasibility of quartz glass for radiotherapy dosimetry through the experimental study of the thermoluminescence characteristics of synthetic quartz glass. Methods The thermoluminescence glow curves of quartz glass under different annealing conditions were analyzed, the thermoluminescence characteristics of quartz glass were studied, and the measurement parameters were optimized. Using the Co-60 reference radiation field in the National Secondary Standard Dosimetry Laboratory, the quartz glass samples under different annealing conditions were irradiated following the dose levels of radiotherapy, i.e., 0.5, 1.0, 2.0, 4.0, 6.0, 8.0, and 10.0 Gy, respectively. According to the relationship between the absorbed dose of quartz glass and the relative thermoluminescence signal intensity, the linearity and dispersion of the dose response of quartz glass were obtained, and the feasibility of quartz glass for radiotherapy dosimetry was analyzed. Results The linear correlation coefficient of dose response of quartz glass under annealing condition of 430℃ for 10 min was 0.9984, and the dose response dispersion was 0.97% at the absorbed dose of 2 Gy. The linear correlation coefficient of dose response of quartz glass under annealing condition of 600℃ for 1 h was 0.9911, and the dose response dispersion was 1.4% at the absorbed dose of 2 Gy. Conclusion Preliminary results suggest that quartz glass with annealing condition of 430℃ for 10 min has the potential to be used for radiotherapy dosimetry.

Objective:To investigate the relationship between sarcopenia and the maximum diaphragm excursion(Dmax)observed on ultrasound in the elderly.Methods:Elderly volunteers(age≥60 years)were recruited from family members of patients at Guangdong Provincial People's Hospital.Their Dmax during forced inhalation was measured via ultrasound.The parameters for the diagnosis of sarcopenia included the appendicular skeletal muscle mass index(ASMI), handgrip strength and usual gait speed.We compared the differences in physical characteristics, pulmonary ventilation, physical performance and Dmax between patients with and without sarcopenia, and evaluated the relationship between sarcopenia and DEmax in the elderly via linear regression.Results:A total of 145 elderly volunteers[age(69.47±5.15)years]were included, and 28(19.31%)were diagnosed with sarcopenia.Body weight, ASMI, maximum inspiratory pressure(Pinmax), maximal power output(Wmax)and Dmax of patients with sarcopenia were significantly lower than those of patients without sarcopenia(all P<0.05).Dmax in the elderly was correlated with sex, height, ASMI, handgrip strength, usual gait speed, Pinmax and Wmax( r=0.181, 0.130, 0.322, 0.373, 0.401, 0.134, and 0.388, P=0.012, 0.037, 0.009, 0.002, 0.022, 0.009, and 0.002, respectively).After adjusting for sex, age, height and forced vital capacity(FVC), there was still a negative correlation between sarcopenia and Dmax in the elderly( β=-0.310, P=0.021). Conclusions:Dmax is related to Pinmax and physical performance in the elderly, and sarcopenia increases the risk of decline in the maximum diaphragm excursion in the elderly as observed on ultrasound.

Objective:The investigation and research on the application status of Hepatic Venous Pressure Gradient (HVPG) is very important to understand the real situation and future development of this technology in China.Methods:This study comprehensively investigated the basic situation of HVPG technology in China, including hospital distribution, hospital level, annual number of cases, catheters used, average cost, indications and existing problems.Results:According to the survey, there were 70 hospitals in China carrying out HVPG technology in 2021, distributed in 28 provinces (autonomous regions and municipalities directly under the central Government). A total of 4 398 cases of HVPG were performed in all the surveyed hospitals in 2021, of which 2 291 cases (52.1%) were tested by HVPG alone. The average cost of HVPG detection was (5 617.2±2 079.4) yuan. 96.3% of the teams completed HVPG detection with balloon method, and most of the teams used thrombectomy balloon catheter (80.3%).Conclusion:Through this investigation, the status of domestic clinical application of HVPG has been clarified, and it has been confirmed that many domestic medical institutions have mastered this technology, but it still needs to continue to promote and popularize HVPG technology in the future.

Objective:To summarize the clinical characteristics of type I interferonopathies and provide clues for early identification and diagnosis.Methods:Clinical data of 20 patients admitted to Department of Pediatrics, Peking Union Medical College Hospital and 5 patients admitted to Department of Rheumatology and Immunology, Shenzhen Children′s Hospital from January 2016 to September 2021 were retrospectively analyzed. The data included gene results, clinical manifestations and auxiliary examination results.Results:Of the 25 cases, 12 were males and 13 were females. Age of onset ranged from 1 day to 11 years. And 84% of them had the onset before the age of 3 years. The cases consisted of 14 cases of Aicardi-Goutières syndrome (AGS), 6 cases of adenosine deaminase 2 deficiency (DADA2), 3 cases of stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), and 2 cases of Spondyloenchondrodysplasia with immune dysregulation (SPENCDI). Eighteen patients (72%) experienced neurologic disorder, among whom 16 (64%) showed intracranial calcification, 11 (44%) had dystonia, 10 (40%) had leukodystrophy, 6 (24%) had epilepsy, 5 (20%) had brain atrophy and 5 (20%) had early-onset cerebrovascular events. Skin involvement occurred in 15 cases (60%), among whom 8 cases (32%) had chilblain-like rash, 4 cases (16%) had livedo reticularis, 3 cases (12%) had erythema, 2 cases (8%) had erythema nodosum and 2 cases (8%) had Raynaud′s phenomenon. In addition, 12 cases (48%) had positive autoimmune antibodies, 10 cases (40%) manifested as developmental retardation, 8 cases (32%) experienced lung interstitial lesions, and 7 cases (28%) demonstrated thyroid dysfunction. And 1 died (4%) at 11 years of age.Conclusions:Type Ⅰinterferonopathies can involve multiple organs, and share the characteristics of systemic inflammatory and autoimmune diseases. The early-onset neurological symptoms (early-onset cerebrovascular events, intracranial calcification, leukodystrophy and cerebral atrophy), rashes (chilblain-like rash, livedo reticularis and erythema), positive autoimmune antibodies, developmental delay, interstitial lung disease and thyroid dysfunction may indicate type Ⅰ interferonopathies.

Objectives:To observe the anatomical location and mechanism of axis ring fractures (ARF) using 3-D CT scans, and propose a new classification for such fractures.Methods:By reviewing prospectively maintained database collecting ARF from 7 medical centers in China, 202 patients were included in this study. According to anatomical location, ARFs were classified into axis arthrosis fracture (AAF) and axis bony damage (ABD). The axis ring was divided into anterior, middle, and posterior rings, based on the border of the pars interarticularis (or pedicle) of axis. According to the features of ARF and previous study, a new classification was proposed based on the anatomical features of different fracture patterns, which was divided into three types and six subtypes (A1, A2, B1, B2, C1 and C2). The incidence of AAF and ABD and their distribution in different location of axis ring and the new classification, were observed.Results:In 202 patients with ARF, 501 anatomical structures were involved. 288 AAFs were found in 178 patients (288/501, 57%), while 213 ABDs were found in 149 patients (213/501, 43%). In anterior ring, 304 structures (304/501, 61%) were involved in injury, with 225 AAF and 79 ABD. In middle ring, 99 structures (99/501, 20%) were involved in injury, and all of them were ABD. In posterior ring, 98 structures (98/501, 19%) were involved in injury, with 63 AAF and 35 ABD. The anterior ring injuries (61%) were more common than middle (20%) or posterior ring (19%). In anterior ring, AAF (84%) were morecommon than ABD (16%); In middle ring, all the injuries were ABD; In posterior ring, AAFs (64%) were more common than ABD (36%). Type A fractures were featured with pedicle fractures and were identified in 30 patients (30/202, 15%). Type A1 fractures were bilateral pedicle fracture lines symmetrically or asymmetrically and identified in 12 (6%) patients; Type A2 fractures were pedicle fracture lineson one side and inferior articular facet injuries or lamina fractures on the otherside and identified in 18 (9%) patients. Type B fractures were featured with superior articular facet injuries or posterior wall of C2 body fractures on one side and identified in 136 patients (67%). Type B1 fractures were superior articular facet injuries or posterior wall of C2 body fractures on one side and pedicle fracture on the other side and identified in 57 (28%) patients; Type B2 fractures were superior articular facet injuries or posterior wall of C2 body fractures on one side and inferior articular facet injuries or lamina fractures on the otherside and identified in 79 (39%) patients. Type C fractures were featured with bilateral superior articular facet injuries or posterior wall of C2 body fractures and identified in 36 patients (18%). Type C1 fractures were bilateral superior articular facet injuries or posterior wall of C2 body fractures symmetrically and identified in 22 (11%) patients; Type C2 fractures were bilateral superior articular facet injuries or posterior wall of C2 body fractures asymmetrically and identified in 14 (7%) patients.Conclusion:ARF could occur in different anatomical locations, and most of these fractures were caused by hyperextension and axial load on superior articular facet on one or two sides. The new CT classification of ARF with three types and six subtypes might provide all fracture patterns, which could be useful for the choice of proper diagnosis and treatment for such fractures.

Objective:To investigate the correlation between the muscle mass loss and severe postoperative pulmonary complications(PPC)in elderly patients with non-small cell lung cancer(NSCLC).Methods:Elderly patients with NSCLC undergoing lobectomy at the Lung Cancer Institute and the Department of Thoracic Surgery of Guangdong Provincial People's Hospital were recruited from Feb.2019 to Dec.2019.Data of the body composition, lung function, respiratory muscle strength test, cardiopulmonary exercise test were collected before operation.All patients were grouped into two groups: with versus without severe PPC at 30 d after operation.The differences of the above parameters were compared between the two groups.A multiple logistic regression analysis was used to analyze the risk factors for severe PPC.Results:In this study, 120 elderly NSCLC patients undergoing lobectomy were recruited, All evaluations were completed in 113 patients(aged 68.13±7.01 years)in whom, 21(18.58%, 21/113)patients had serious PPC.Compared with patients without PPC, patients with severe PPC had a lower appendicular skeletal muscle mass index(ASMI)(5.67±0.90 kg/m 2vs.7.71±1.40 kg/m 2, t=3.900, P=0.001), a lower forced expiratory volume in 1 second(FEV 1)(1.85±0.40 L vs.2.12±0.57 L, t=2.412, P=0.027), a lower maximal mid-expiratory flow(MMF)(1.40±0.69 L/s vs.2.11±1.09 L/s, t=2.502, P=0.021), a lower maximum inspiratory pressure(Pimax)(55.13±32.52 cmH 2O vs.64.71±20.60, t=0.778, P=0.047, 1 cmH 2O=0.098 kPa), a lower maximal oxygen consumption(Vo 2max)(1.14±0.41 L/min vs.1.40±0.34 L/min, t=0.779, P=0.046), a lower peak O 2 consumption(Vo 2max@kg)(20.00±1.91 L·min -1·kg -1vs.22.33±2.37 L·min -1·kg -1, t=0.813, P=0.041). Multiple logistic regression analysis showed that in addition to FEV 1( OR=2.824, 95% CI: 1.127-5.158, P=0.001)and Vo 2max@kg( OR=3.149, 95%CI: 1.829-6.592, P<0.001), ASMI was also an independent risk factor for serious PPC( OR=1.919, 95% CI: 1.604-3.466, P=0.006), in which the best cut-off value was 6.295 kg/m 2, the sensitivity and specificity were 0.816 and 0.818 respectively, and the area under the receiver operating characteristic(ROC)curve(AUC)was 0.887(95% CI: 0.793-0.981, P<0.0001). Conclusions:Muscle mass loss can increase the risk for the occurrence of severe PPC within 30 days after lobectomy in elderly patients with NSCLC.

Objective To evaluate the safety and effectiveness of modified total arch replacement by retrospectively analyzing the clinical outcome of surgical patients with Stanford type A aortic dissection (AAD). Methods From June 2015 to December 2016, 39 consecutive patients with AAD were recruited to this study. This modified technique was preformed under general anesthesia and a 30℃ hypothermia circulatory arrest (HCA) with continual bilateral antegrade cerebral perfusion. Different surgical approaches were applied according to the aortic root condition: Bentall procedure (4 patients), David procedure (2 patients), aortic valve plasty and ascending aortic replacement (25 patients) and Cabrol procedure (8 patients). Concomitant procedures included mitral valve plasty (1 patient) and tricuspid valve plasty (1 patient). Results The average cardiopulmonary bypass (CPB), aortic occlusion time (ACC), HCA and operation time was 218.5±42.2 min, 134.2±32.4 min, 4.9±2.3 min and 415.5±80.5 min respectively. Four patients required dialysis and 2 patients developed temporary neurological deficit. No permanent neurological deficit, postoperative paraplegia or in-hospital death occurred. Computed tomography examination was performed on all patients before discharge and 3 months after discharge. The follow-up result showed that 37 patients developed complete thrombosis in the false lumen and 2 patients developed partial thrombosis. Conclusion Modified total arch replacement is a safe and effective approach for AAD. It can greatly avoid postoperative complications and provide satisfactory short-term outcomes.

Objective     To retrospectively reviewed our experience of the surgical and perioperative treatment of patients suffering from critical Marfan syndrome with severe left ventricular dysfunction and to evaluate its therapeutic effect and prognosis. Methods     Between January 2012 and October 2016, 15 patients diagnosed with Marfan syndrome combined with severe left ventricular dysfunction (left ventricular ejection fraction≤40% or left ventricular end diastolic diameter≥75 mm) underwent operations for aortic root aneurysm in Zhujiang Hospital and Guangdong General Hospital. Among them, 11 were males and 4 were females with a mean age of 32.9±8.7 years ranging from 19 to 55 years. Five patients with aortic dissection underwent Bentall procedure and total arch reconstruction with stent graft implantation. Two patients underwent Bentall procedure and hemi-arch replacement, seven patients underwent Bentall procedure and one patient underwent Cabrol procedure. Concomitant procedures included mitral valve repair in 12 patients, mitral valve replacement in 3 patients and tricuspid valve repair in 12 patients. Results     There were 11 patients (73.3%) receiving intra-aortic balloon pumping implantation. One (6.7%) in-hospital death occurred. The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 58.3±6.0 mm (P<0.05) and the left ventricular ejection fraction improved from 37.3%±5.2% to 46.3%±4.4% 3 months postoperatively (P<0.05). The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 53.7±3.6 mm (P<0.05) and the left ventricular ejection fraction improved from 37.3%±5.2% to 57.7%±4.2% after one year (P<0.05). No death and reoperation occurred in the follow-up. Conclusion     Although the patients with Marfan syndrome and severe left ventricular dysfunction usually have a high surgical mortality, the key to satisfactory outcomes of severe Marfan syndrome is adequate preoperative preparation, complete correction of all vascular lesions during the operation, application of circulatory auxiliary device and perioperative strict and long-term ICU monitoring.

Objective To explore the effect of pravastatin on carotid atherosclerosis in the patients with type 2 diabetes mellitus (T2DM) complicating hypertension.Methods One hundred and six patients.with T2DM complicating hypertension treated in this hospital from April 2015 to April 2016 were selected and divided into the conventional treatment group (n=52) and pravastatin group (n=54).The patients of conventional treatment group were given the conventional therapy of diet control,reducing blood glucose,controlling hypertension,symptomatic and support treatment,while on the basis of conventional therapy the patients of pravastatin group were added with pravastatin.The carotid intima-medial thickness (IMT),detection rate of carotid atherosclerotic plaque and carotid plaque score were measured by ultrasound before and after the treatment in all cases.Meanwhile the levels of serum lipid and hyper sensitive C reactive protein (hs-CRP) were determined.Results The above indicators in the conventional treatment group had no statistically significant difference between before and after treatment (P＞0.05),but the IMT value,detection rate of carotid atherosclerotic plaque and carotid plaque score after treatment in the pravastatin group were decreased compared with before treatment,the levels of serum total cholesterol (TC),triacylglycerol (TG),low density lipoprotein cholesterol (LDL-C) and hs-CRP were also significantly reduced compared with before treatment,the differences were statistically significant (P＜0.05),moreover the above indicators after treatment in the pravastatin group were remarkably lower than those in the conventional treatment group (P＜0.05).There was no statistically significant difference in the incidence rate of adverse reactions between the two groups (P＞0.05).Conclusion Pravastatin can effectively and safely improve the carotid atherosclerosis degree in the patients with T2DM complicating hypertension.