Purpose: This Phase II trial was objected to evaluate the efficacy and safety of adding fulvestrant to neoadjuvant chemotherapy in patients with estrogen receptor (ER)+/human epidermal growth factor receptor 2 (HER2)– locally advanced breast cancer (LABC). Additionally, the study aimed to investigate the association of 16α-18F-fluoro-17β-fluoroestradiol (18F-FES) positron emission tomography (PET)–computed tomography (CT) and metabolites with efficacy. Materials and Methods: Fulvestrant and EC-T regimen were given to ER+/HER2– LABC patients before surgery. At baseline, patients received 18F-FES PET-CT scan, and plasma samples were taken for liquid chromatography–mass spectrometry analysis. The primary endpoint was objective response rate (ORR). Secondary endpoints included total pathologic complete response (tpCR) and safety. Results: Among the 36 patients enrolled, the ORR was 86.1%, the tpCR rate was 8.3%. The incidence of grade ≥ 3 treatment-emergent adverse events was 22%. The decrease in ER value in sensitive patients was larger than that in non-sensitive patients, as was Ki-67 (p < 0.05). The maximum standardized uptake value, mean standardized uptake values, total lesion ER expression of 18F-FES PET-CT in sensitive patients were significantly higher than those in non-sensitive patients (p < 0.05). Moreover, these parameters were significantly correlated with Miller and Payne grade and the change in ER expression before and after treatment (p < 0.05). Thirteen differential expressed metabolites were identified, which were markedly enriched in 19 metabolic pathways. Conclusion This regimen demonstrated acceptable toxicity and encouraging antitumor efficacy. 18F-FES PET-CT might serve as a tool to predict the effectiveness of this therapy. Altered metabolites or metabolic pathways might be associated with treatment response.

Purpose: This Phase II trial was objected to evaluate the efficacy and safety of adding fulvestrant to neoadjuvant chemotherapy in patients with estrogen receptor (ER)+/human epidermal growth factor receptor 2 (HER2)– locally advanced breast cancer (LABC). Additionally, the study aimed to investigate the association of 16α-18F-fluoro-17β-fluoroestradiol (18F-FES) positron emission tomography (PET)–computed tomography (CT) and metabolites with efficacy. Materials and Methods: Fulvestrant and EC-T regimen were given to ER+/HER2– LABC patients before surgery. At baseline, patients received 18F-FES PET-CT scan, and plasma samples were taken for liquid chromatography–mass spectrometry analysis. The primary endpoint was objective response rate (ORR). Secondary endpoints included total pathologic complete response (tpCR) and safety. Results: Among the 36 patients enrolled, the ORR was 86.1%, the tpCR rate was 8.3%. The incidence of grade ≥ 3 treatment-emergent adverse events was 22%. The decrease in ER value in sensitive patients was larger than that in non-sensitive patients, as was Ki-67 (p < 0.05). The maximum standardized uptake value, mean standardized uptake values, total lesion ER expression of 18F-FES PET-CT in sensitive patients were significantly higher than those in non-sensitive patients (p < 0.05). Moreover, these parameters were significantly correlated with Miller and Payne grade and the change in ER expression before and after treatment (p < 0.05). Thirteen differential expressed metabolites were identified, which were markedly enriched in 19 metabolic pathways. Conclusion This regimen demonstrated acceptable toxicity and encouraging antitumor efficacy. 18F-FES PET-CT might serve as a tool to predict the effectiveness of this therapy. Altered metabolites or metabolic pathways might be associated with treatment response.

Purpose: This Phase II trial was objected to evaluate the efficacy and safety of adding fulvestrant to neoadjuvant chemotherapy in patients with estrogen receptor (ER)+/human epidermal growth factor receptor 2 (HER2)– locally advanced breast cancer (LABC). Additionally, the study aimed to investigate the association of 16α-18F-fluoro-17β-fluoroestradiol (18F-FES) positron emission tomography (PET)–computed tomography (CT) and metabolites with efficacy. Materials and Methods: Fulvestrant and EC-T regimen were given to ER+/HER2– LABC patients before surgery. At baseline, patients received 18F-FES PET-CT scan, and plasma samples were taken for liquid chromatography–mass spectrometry analysis. The primary endpoint was objective response rate (ORR). Secondary endpoints included total pathologic complete response (tpCR) and safety. Results: Among the 36 patients enrolled, the ORR was 86.1%, the tpCR rate was 8.3%. The incidence of grade ≥ 3 treatment-emergent adverse events was 22%. The decrease in ER value in sensitive patients was larger than that in non-sensitive patients, as was Ki-67 (p < 0.05). The maximum standardized uptake value, mean standardized uptake values, total lesion ER expression of 18F-FES PET-CT in sensitive patients were significantly higher than those in non-sensitive patients (p < 0.05). Moreover, these parameters were significantly correlated with Miller and Payne grade and the change in ER expression before and after treatment (p < 0.05). Thirteen differential expressed metabolites were identified, which were markedly enriched in 19 metabolic pathways. Conclusion This regimen demonstrated acceptable toxicity and encouraging antitumor efficacy. 18F-FES PET-CT might serve as a tool to predict the effectiveness of this therapy. Altered metabolites or metabolic pathways might be associated with treatment response.

OBJECTIVE: To precise classify sacral meningeal cysts, effective guide minimally invasive neurosurgery and postoperative personalized rehabilitation by multiple dimensions radiographic reconstruction MRI. METHODS: From March to December 2021, based on the original 3D-fast imaging employing steadystate acquisition (FIESTA) scanning sequence, 92 patients with sacral meningeal cysts were pre-operatively evaluated by multiple dimensional reconstruction MRI. The shape of nerve root and the leakage of cyst were reconstructed according to the direction of nerve root or leakage track showed on original MRI scans. Sacral canal cysts were accurately classified as including nerve root and without nerve root, so as to accurately design the incision of skin and formulate corresponding open range of the posterior wall of the sacral canal. Under the microscope intraoperation, the shape of the nerve roots inside cysts or leakage track of the cysts without nerve roots were verified and explored. After the reinforcement and shaping operation, several reexaminations of multiple dimensional reconstruction MRI were performed to understand the deformation of the nerve root and hydrops in the operation cavity, so as to formulate a persona-lized rehabilitation plan for the patients. RESULTS: Among the 92 patients with sacral mengingeal cyst, 58 (63.0%) cysts with nerve root cyst, 29 (31.5%) cysts without nerve root cyst, and 5 (5.4%) cysts with mixed sacral canal cyst. In 58 patients with nerve root cysts, the accuracy of preoperative clinical classification on MRI image reached 96.6% (56/58) through confirmation by operating microscope. Only 2 cases of large single cyst with nerve root on the head of cyst were mistaken for without nerve root type. In 29 patients with sacral cyst without nerve root, the accuracy of preoperative image reached 100% through confirmation by operating microscope. The accuracy of judging the internal nerve root and leakage of 12 cases with recurrent sacral cyst was also 100%. Two cases of delayed postoperative hydrops were found one month after operation. After rehabilitation treatment by moxibustion and bathing, the hydrops disappeared 4-6 months after operation. CONCLUSION Multiple dimensional reconstruction MRI can precisely make clinical classification of sacral meningeal cysts before operation, guide minimally invasive neurosurgery effectively, and improve the rehabilitation effect.
Humans ; Magnetic Resonance Imaging/methods* ; Male ; Female ; Sacrum/surgery* ; Adult ; Middle Aged ; Imaging, Three-Dimensional/methods* ; Cysts/rehabilitation* ; Aged ; Adolescent ; Young Adult ; Spinal Nerve Roots/diagnostic imaging* ; Minimally Invasive Surgical Procedures ; Neurosurgical Procedures/methods*

Dystrophinopathies caused by variants of DMD gene are a group of muscular diseases including Duchenne muscular dystrophy, Becker muscular dystrophy, and DMD-associated dilated cardiomyopathy. With the advancement of genetic testing techniques and wider implementation of genetic screening, especially the expanded carrier screening, more and more individuals carrying DMD gene variants have been identified, whereas the genetic counseling capacity is relatively insufficient. Currently there is still a lack of professional norms for genetic counseling on dystrophinopathies. In this consensus, the main points to be covered in the pre- and post-test consultation have been discussed, with an aim to provide genetic counseling guidance for the disease diagnosis, treatment, and family reproduction.

V-ATPases are a class of multi-subunit protein complexes that utilize energy derived from ATP hydrolysis for mediating H + transport across cell membranes, which plays an important role in a range of life activities by acidifying the intracellular and extracellular environment. Variants of V-ATPase genes may lead to complete or partial loss of V-ATPase activity, which in turn may impair the ability of type A intercalated cells in renal tubules to pump H + into the tubular lumen, ultimately resulting in the onset of autosomal recessive distal renal tubular acidosis (dRTA). With the rapid development of molecular techniques, ATP6V0A4 and ATP6V1B1 have now been identified as the pathogenic genes for dRTA. Moreover, animal and cell experiments have substantiated the implication of V-ATPase subunit genes including ATP6V1C2 and ATP6V1G3 in the development of dRTA, though clinical evidence is still limited. This article has reviewed recent progress on the genetic and molecular mechanisms of V-ATPase subunit gene variants which can lead to dRTA, which may shed light on the diagnosis and treatment of this disease.

Background::Although overnight fasting is recommended prior to endoscopic retrograde cholangiopancreatography (ERCP), the benefits and safety of high-carbohydrate fluid diet (CFD) intake 2 h before ERCP remain unclear. This study aimed to analyze whether high-CFD intake 2 h before ERCP can be safe and accelerate patients’ recovery.Methods::This prospective, multicenter, randomized controlled trial involved 15 tertiary ERCP centers. A total of 1330 patients were randomized into CFD group ( n = 665) and fasting group ( n = 665). The CFD group received 400 mL of maltodextrin orally 2 h before ERCP, while the control group abstained from food/water overnight (>6 h) before ERCP. All ERCP procedures were performed using deep sedation with intravenous propofol. The investigators were blinded but not the patients. The primary outcomes included postoperative fatigue and abdominal pain score, and the secondary outcomes included complications and changes in metabolic indicators. The outcomes were analyzed according to a modified intention-to-treat principle. Results::The post-ERCP fatigue scores were significantly lower at 4 h (4.1 ± 2.6 vs. 4.8 ± 2.8, t = 4.23, P <0.001) and 20 h (2.4 ± 2.1 vs. 3.4 ± 2.4, t= 7.94, P <0.001) in the CFD group, with least-squares mean differences of 0.48 (95% confidence interval [CI]: 0.26–0.71, P <0.001) and 0.76 (95% CI: 0.57–0.95, P <0.001), respectively. The 4-h pain scores (2.1 ± 1.7 vs. 2.2 ± 1.7, t = 2.60, P = 0.009, with a least-squares mean difference of 0.21 [95% CI: 0.05–0.37]) and positive urine ketone levels (7.7% [39/509] vs. 15.4% [82/533], χ2 = 15.13, P <0.001) were lower in the CFD group. The CFD group had significantly less cholangitis (2.1% [13/634] vs. 4.0% [26/658], χ2 = 3.99, P = 0.046) but not pancreatitis (5.5% [35/634] vs. 6.5% [43/658], χ2 = 0.59, P = 0.444). Subgroup analysis revealed that CFD reduced the incidence of complications in patients with native papilla (odds ratio [OR]: 0.61, 95% CI: 0.39–0.95, P = 0.028) in the multivariable models. Conclusion::Ingesting 400 mL of CFD 2 h before ERCP is safe, with a reduction in post-ERCP fatigue, abdominal pain, and cholangitis during recovery.Trail Registration::ClinicalTrials.gov, No. NCT03075280.

At present, China has not yet established a comprehensive standard and evaluation system specifically for occupational health engineering ventilation inspection. In order to meet the requirements for the design of occupational-disease-prevention facilities, the evaluation of occupational disease hazard control effectiveness, and the protective facilities, the Guangdong Provincial Occupational Health Technical Quality Control Center developed the Guangdong Province Occupational Health Engineering Ventilation Inspection Work Specification (Trial) by referring domestic and international standards and norms related to occupational health engineering, industrial ventilation design manuals and professional literature, using brainstorming and experience-based methods. The work specification covers the key detection indicators of ventilation systems, and comprehensively covers the core links of occupational health engineering ventilation testing. The relevant indicators mainly include: detection of air velocity control; wind speed and air volume of exhaust hoods and exhaust vents of ventilation facilities; detection of wind pressure, wind speed and air volume inside the ventilation duct; fresh air volume detection; and calculation and evaluation of ventilation frequency. The specification is highly practical, indicting scientific and advanced principles, and ensures the fairness and reliability of the testing process and results. Its implementation will effectively promote the standardization and professionalism of occupational health engineering ventilation testing, providing robust technical support for safeguarding workers' health.

Objective:A dose verification system of two-dimensional semiconductor matrix(SRS MapCHECK)was used to verify the dose of the clinical treatment plan of patients who underwent CyberKnife(CK),which realized rapid verification for individualization of radiotherapy plans of patients through analyzed the γ-passing rates of them.Methods:A total of 253 patients with tumor who received CK clinical treatment in the First Medical Center of Chinese PLA General Hospital from March 2021 to May 2023 were selected.Among of them,121 cases received CK treatment on head,and 30 cases received that on lung,and 102 cases received CK treatment on abdomen and other metastatic tumor.In the MultiPlan treatment plan system,the plan of patient was mapped to the integrated model composed of StereoPHAN model and SRS MapCHECK matrix dose verification system by the means of the plan image center overlap.The dose verification was conducted on the plan of each patient on the basis of ensuring the consistency of the number of beam,direction of beam and the monitor unit.The different γ analysis standards(1%/1 mm,2%/1 mm,3%/1 mm,1%/2 mm,2%/2 mm,3%/2 mm,1%/3 mm,2%/3 mm and 3%/3 mm)were adopted to conduct global analysis of absolute dose for each verification plan,and the threshold(TH)of low dose was set as 10%.Results:The γ passing rates of phantom verification plans of 253 patients were respectively(88.64±5.91)%,(95.43±3.40)%,(97.90±2.06)%,(96.51±2.35)%,(98.15±1.68)%,(99.06±1.12)%,(98.30±1.39)%,(99.09±0.97)%and(99.52±0.63)%under different analysis standards.The γ passing rates of other standards of patients with tumor on different parts were larger than 95%except the analysis result of 1%1 mm standard.The overall analysis result of the deviation of central point dose was(-1.30±2.17)%,among of which the tumor of head,abdominal tumors and other metastatic tumor were about approximately-2%,while that of lung tumors were approximately-3%.The deviation of abdominal and other metastatic tumor was the minimum.The correlation analysis showed that the target volume and the size of the minimum collimator were respectively correlated to the dose deviation of the center.Conclusion:SRS MapCHECK dose verification system can conveniently and quickly realize the individualized verification for the plan of patients who receive CK treatment.

Objective To explore the clinical efficacy of prone position ventilation(PPV)in improving hypoxemia in patients with severe brain damage.Methods A retrospective research method was conducted,140 patients with severe brain damage who were admitted to the department of critical care medicine of the First Affiliated Hospital,Zhejiang University School of Medicine from August 2020 to August 2021 were selected as subject objected.According to the inclusion and exclusion criteria,20 patients with oxygenation index≤200 mmHg(1 mmHg≈0.133 kPa)who were treated with PPV were statistically analyzed.The patients'blood gas analysis related indicators[including arterial partial pressure of oxygen(PaO2),fractional of inspired oxygen(FiO2),oxygenation index,arterial oxygen saturation(SaO2),arterial partial pressure of carbon dioxide(PaCO2),pH value],ventilator-related parameters[including peak inspiratory pressure(PIP),positive end-expiratory pressure(PEEP),tidal volume(VT),lung dynamic compliance(Cdyn),etc.],and mean arterial pressure(MAP),heart rate(HR)were compared before PPV,12 hours after PPV,and 12 hours after reverting to supine position.At the same time,the related complications of patients during PPV were recorded.Results There were 15 males and 5 females,the mean age of the patients was(46.10±17.22)years old,the average PPV time was(22.20±5.94)hours.Compared with before PPV,patients showed significant increases in PaO2,oxygenation index,SaO2,VT,and Cdyn at 12 hours after PPV and 12 hours after recovery from supine position[PaO2(mmHg):98.35±21.85,98.45±17.90 vs.72.15±10.14,oxygenation index(mmHg):198.82±40.51,202.27±46.39 vs.133.20±33.95,SaO2:0.97±0.02,0.97±0.01 vs.0.94±0.03,VT(mL):558.42±111.23,580.29±119.44 vs.484.82±123.77,Cdyn(mL/cmH2O):26.11±5.42,27.90±5.80 vs.24.15±6.13,all P<0.05];Compared with 12 hours after PPV,the Cdyn of the patient still showed a significant increase after 12 hours of recovery from supine position(P<0.05).There were no statistical differences in the FiO2,PaCO2,pH value,PIP,PEEP,HR,and MAP of patients at various time points before and after PPV(all P>0.05).Five patients developed redness and swelling at the skin compression site mainly on the face after PPV,which gradually improved after returning to a supine position.During this period,there was no occurrence of catheter detachment,malignant arrhythmia,or significant hemodynamic instability.Conclusion PPV has a certain clinical effect on improving hypoxemia in patients with severe brain damage.