[Comparison of intraoperative neurophysiological monitoring between patients with arthrogryposis multiplex congenita and adolescent idiopathic scoliosis].

Objective: To compare the intraoperative neurophysiological monitoring (IONM) results between patients with arthrogryposis multiplex congenita (AMC) and adolescent idiopathic scoliosis (AIS) and to analyze the influence of congenital spinal deformity on IONM in AMC patients, thus to evaluate the efficiency of IONM in AMC patients. Methods: A cross-sectional study. The clinical data of 19 AMC patients underwent correction surgery from July 2013 to January 2022 in Nanjing Drum Tower Hospital were retrospectively reviewed. There were 13 males and 6 females with a mean age of (15.2±5.6) years, and the average Cobb angle of main curve was 60.8°±27.7°. And 57 female AIS patients of similar age and curve type with the AMC patients during the same period were selected as the control group, with an average age of (14.6±4.4) years and a mean Cobb angle of 55.2°±14.2°. The latency and amplitude of samatosensory evoked potentials (SSEPs) and transcranial electric motor evoked potentials (TCeMEPs) were compared between the two groups. The difference in IONM data between AMC patients with and without congenital spinal deformity was also evaluated. Results: The success rates of SSEPs and TCeMEPs were 100% and 14/19 for AMC patients, 100% and 100% for AIS patients. The SSEPs-P40 latency, SSEPs-N50 latency, SSEPs-amplitude, TCeMEPs-latency, TCeMEPs-amplitude showed no significant difference between AMC patients and AIS patients (P>0.05 for all). The side-difference of TCeMEPs-amplitude showed an increasing trend in AMC patients when compared with that in AIS patients, but there was no statistical difference between the two groups [(147.0±185.6) µV vs (68.1±311.4) µV, P=0.198]. The SSEPs-amplitude value was (1.4±1.1) µV on concave side in AMC patients with congenital spinal deformity, and it was (2.6±1.2) µV on concave side in AMC patients without congenital spinal deformity (P=0.041). The SSEPs-amplitude value was (1.4±0.8) µV on convex side in AMC patients with congenital spinal deformity, and it was (2.6±1.3) µV on convex side in AMC patients without congenital spinal deformity (P=0.028). Conclusions: The values of SSEPs-P40 latency, SSEPs-N50 latency, SSEPs-amplitude, TCeMEPs-latency and TCeMEPs-amplitude are similar in AMC and AIS patients. The SSEPs-amplitude of AMC patients with congenital spinal deformity is lower than that of AMC patients without congenital spinal deformity.

[Long-term mortality risk of valvular heart disease adults over 35 years old in Chinese communities].

Objective: To investigate the risk and influencing factors of long-term mortality of valvular heart disease (VHD) adults aged 35 years and over in Chinese communities. Methods: A cohort study was carried out. The data of the subjects who underwent echocardiography were collected from the Chinese Hypertension Survey between 2012 and 2015 and survival outcomes were followed up between 2018 and 2019. Kaplan-Meier survival curves were plotted and compared using log-rank test. Cox proportional hazards models were used to analyze the influence of VHD on mortality. Results: During an average follow-up time of (4.6±0.9) years, a total of 23 237 participants (10 881 males and 12 356 females) were pooled into the final analysis from 5 eastern, 5 central, and 4 western provinces, cities and autonomous regions in China, with a mean age of (56.9±13.2) years. Among the included participants, 1 004 had VHD (467 males and 537 females), with a mean age was of (68.1±12.6) years. In the Kaplan-Meier analysis, participants with VHD had a significantly increased risk of all-cause mortality (log-rank χ2=351.82, P<0.001) and cardiovascular mortality (log-rank χ2=284.14, P<0.001) compared with those without VHD. Multivariate Cox regression analysis showed that compared with those without VHD, the participants with rheumatic VHD had a 45% increased risk of all-cause mortality (HR=1.45, 95%CI: 1.12-1.89) and degenerative VHD increased the risk of cardiovascular mortality by 69% (HR=1.69, 95%CI: 1.19-2.38). The risk factors of cardiovascular mortality for VHD were age 55 years and over (55-<75 years: HR=4.93, 95%CI: 1.17-20.85;≥75 years: HR=11.92, 95%CI: 2.85-49.80) and diabetes mellitus (HR=1.71, 95%CI: 1.00-2.93). Conclusions: VHD is a risk factor of all-cause mortality and cardiovascular mortality among adults aged 35 years and over. Age 55 years and over and diabetes mellitus are adverse prognostic factors for patients with VHD.

[Clonality relatedness and molecular characteristics of Richter transformation].

Objective: To investigate the clinical, genetic, and clonality related aspects of individuals with Richter transformation (RT) . Methods: From January 2019 to December 2021, 18 RT patients with diagnoses at the First Affiliated Hospital of Nanjing Medical University (Pukou CLL center) were retrospectively examined. The immunoglobin heavy variable (IGHV) gene usage and IGHV-D-J rearrangement pattern of diagnosed CLL/SLL and transformed diffuse large B-cell lymphoma (DLBCL) were compared to determine the clonality relatedness. To investigate the risk factors of RT, Clinical and laboratory data from patients with newly diagnosed CLL/SLL and transformed DLBCL were gathered. Results: The median age of RT was 56.5 (41-75) years old. 17 patients transformed to DLBCL and 1 transformed to Hodgkin lymphoma (HL) . Of 17 individuals who had DLBCL transformation, 15 had CLL/SLL-related clonality and 2 had unrelated clonality. Next-generation sequencing (NGS) analysis of 11 paired initially diagnosed treatment-naive CLL/SLL and RT DLBCL found that EGR2、TP53 and NOTCH1 were among the most frequently mutated genes both in treatment-naive CLL/SLL and in RT DLBCL. In several cases, specific mutations were gained or lost throughout RT, indicating clonal evolution. Among 10 patients before exposure to BTK inhibitors before RT, four patients acquired BTK mutation. The aforementioned mutations should be considered high-risk variables for transformation; in addition, TP53 and EGR2 mutations could be linked to a poor prognosis following RT in patients receiving a cocktail of new medicines. Conclusion: Most RT DLBCL patients in our center are clonality related (15/17, 88.2% ) and we recommend all qualified centers to evaluate clonality relatedness of RT DLBCL patients. There was some variability in the mutational landscape between DLBCL that had undergone a transformation and initially diagnosed, treatment-naive CLL/SLL. The underlying molecular mechanism of RT needs more research.

Fast Gate-Based Readout of Silicon Quantum Dots Using Josephson Parametric Amplification.

Spins in silicon quantum devices are promising candidates for large-scale quantum computing. Gate-based sensing of spin qubits offers a compact and scalable readout with high fidelity, however, further improvements in sensitivity are required to meet the fidelity thresholds and measurement timescales needed for the implementation of fast feedback in error correction protocols. Here, we combine radio-frequency gate-based sensing at 622 MHz with a Josephson parametric amplifier, that operates in the 500-800 MHz band, to reduce the integration time required to read the state of a silicon double quantum dot formed in a nanowire transistor. Based on our achieved signal-to-noise ratio, we estimate that singlet-triplet single-shot readout with an average fidelity of 99.7% could be performed in 1 µs, well below the requirements for fault-tolerant readout and 30 times faster than without the Josephson parametric amplifier. Additionally, the Josephson parametric amplifier allows operation at a lower radio-frequency power while maintaining identical signal-to-noise ratio. We determine a noise temperature of 200 mK with a contribution from the Josephson parametric amplifier (25%), cryogenic amplifier (25%) and the resonator (50%), showing routes to further increase the readout speed.

Prominent contribution of Th1, Th17, and Tregs to the host response during M. neoaurum infection.

Nontuberculous mycobacteria are ubiquitous in outside environment and animals. As for nontuberculous mycobacteria infection, there is only limited information in humans regarding infection and the subsequent immune response, especially for Mycobacterium neoaurum. Here, haematoxylin-eosin and Ziehl-Neelsen staining were used to observe pathological changes and detect acid-fast bacilli in organ samples in mouse model. Flow cytometry and quantitative real-time polymerase chain reaction were performed to analyze the contribution of Th1, Th17 and Tregs to the host immune response. M. neoaurum caused chronic infection in mice, resulting in infiltrates with large aggregates of inflammatory cells, especially macrophages, in lung tissues. Our results indicated that 72% of CD4+ T cells appeared in the early days of infection, which was followed by a decrease to 47% by day 32, and then a rise to 76% by day 56. Moreover, we found higher frequency of IFN-g-producing CD4+ T cells and elevated mRNA expression of the transcription factor T-bet in the lungs; however, we observed lower mRNA expression of the transcription factor RORgt and lower frequency of IL-17-producing CD4+ T cells. A transient relative decrease in the number of Treg cells was observed in the lungs; however, the number of Tregs did not change significantly between the first and last day following infection. Thus, M. neoaurum causes chronic infection in C57BL/6 mice, with Th1, Th17, and Tregs playing a prominent role in the host response. The present study may lay the basis for further studies on the mechanisms underlying infection with nontuberculous mycobacteria.

Rapid increase of carbonate in cortical bones of hens during laying period.

The incorporation of carbonate has been recognized as an evident change in bone mineral (bioapatite) during aging. Laying hens (Gushi layer) at 4 stages of age (8 hens each stage) were studied by Raman spectroscopy and X-ray radiography to investigate the mineralogical changes and bone density, respectively. Cortical bones of the humerus and femur show a rapid increase of carbonate (∼1.9 wt.%) from sexual maturity to the peak of hens' laying period, while the densities of the cortical bones are relatively stable. Before sexual maturity, the density of the cortical bones increases considerably during aging. However, after the peak of the laying period, only femoral density continues elevating. Carbonate contents in the cortical bones reach the maximum at the peak of the laying period. Two pathways (halted growth of density and Ca-release due to the CO3 incorporation) could both contribute to the intense Ca requirement for egg laying. Crystallization, however, has no significant changes during aging and the laying period. This study could shed light on the mechanism of mineral losses due to CO3 incorporation, and also shows the advantages of Raman spectroscopy in tracking mineral loss in poultry bone.

[A novel open chest method of right ventricular hemodynamics measurement in pulmonary hypertensive mice].

Objective: Right heart catheterization is the gold-standard for measurement of right ventricular hemodynamics. However, it is difficult to perform in mice. We thus aimed to establish a new open chest method to record and analyze the right ventricular hemodynamic parameters in intubated mice connected to a respirator under anesthesia. Methods: Eight-week old male C57BL/6 mice were divided into the control group, and the hypoxia group using completely random method. Mice in the hypoxia group were exposed in low pressure oxygen chamber, which simulated the environment of plateau (5 500 m altitude, 10% oxygen) for 3 weeks. Mice in the control group were exposed in normal pressure and oxygen environment. The mice were intratracheally intubated and connected to a respirator, chest was opened, right ventricular hemodynamics were measured with a needle (0.7 mm×19 mm) punctured into right ventricle (5 mm in death), which was connected to the measuring equipment. Results: The open chest procedure could be finished within 5-10 minutes. The successful rate was 100%. There was no significant difference in heart rate between control group and hypoxia group under anesthetia ((306.4±11.5) bpm vs.(320.4±16.0) bpm, P>0.05). Compared with the control group, both the right ventricular systolic pressure (RVSP) and the mean right ventricular pressure(mRVP)were increased in hypoxia group ((17.1±1.0)mmHg(1 mmHg=0.133 kPa) vs. (22.6±1.0) mmHg, P<0.01; (9.6±0.8) mmHg vs.(12.4±0.3) mmHg, P<0.01). Moreover, the absolute value of the maximal rate of increase in right ventricular pressure (dp/dtmax) and the maximal rate of decrease in right ventricular pressure (dp/dtmin) were higher in the hypoxia group ((421.3±30.6)mmHg/s vs.(639.7±47.7)mmHg/s, P<0.01; (-324.3±24.0)mmHg/s vs. (-496.5±40.6) mmHg/s, P<0.01), indicating the pathological status after hypoxia treatment. Conclusions: Right ventricular hemodynamics measurement with this open chest technique is easy to handle and can accurately reflect the right ventricular pressure in mice under physiological and pathological conditions.

[A comparative study on sterols of ethanol extract and water extract from Hericium erinaceus].

OBJECTIVE: To study the pharmaceutic chemical basis of the different medicinal effects, and to compare and analyze the sterols in Hericium erinaceus mycelia and its ethanol extract, water extract derived from solid fermented mycelia. METHOD: The components of Hericium erinaceus mycelia and two kinds of extract were compared with some biochemical methods such as GC, RP-HPLC, etc. Sterol composition in Hericium erinaceus mycelia was determined by using GC-mass spectrometry. RESULTS: The content of crude polysaccharide, water-soluble protein and fatty acid of ethanol extract showed no obvious advantage over its water extract. However, there was significant difference between two kinds of extract in the content of sterols. And ergostane derivatives, beta-sitosterol and C28 sterol with four bonds were detected as the major sterols in Hericium erinaceus mycelia, among which ergosterol was the principal sterol. CONCLUSION: Sterols in Hericium erinaceus exist mainly in ethanol extract. And three sterols are found for the first time from Hericium erinaceus, which are ergostane derivatives, beta-sitosterol and C28 sterol with four bonds.

[Wind-damage effects on quality of heartwood of Lignum Santali Albi].

OBJECTIVE: To evaluate the wind-damage effects on quality of heartwood of Lignum Santali Albi. METHOD: GC-MS, TLC and pharmacodynamic test. RESULTS: The content of volatile oil from heartwood of Wind-damaged Lignum Santali Albi is 1.42%; the content of various components in the oil and the chromatography of different extracts are similar to those of reference drug and 25 years old trees. CONCLUSION: Wind-damage should accelerate the formation of heartwood of Lignum Santali Albi without influence on its quality.

Impact of interferon alpha-induced cytogenetic improvement on survival in chronic myelogenous leukaemia.

The objective of this study was to investigate the prognostic impact of the reduction of Philadelphia chromosome (Ph) positive metaphases by treatment of chronic myelogenous leukaemia (CML) with interferon (IFN) alpha. Therefore, we evaluated the outcome of patients with previously untreated chronic phase Ph-positive CML, enrolled from 1984 to 1990 into two consecutive IFN trials at our institution. Of a total of 71 patients, 62 (87%) were evaluable for cytogenetic response. No cytogenetic improvement was seen in 16 patients (23%), 28 patients (38%) had a decrease in Ph-positive bone marrow metaphases to levels ranging from 35% to 95%, and nine patients (13%) to levels between 5% and 34%. In nine patients (13%), Ph-positive metaphases were no longer detectable. After a median follow-up period of 33 months, the projected 5-year survival is 55% for the 62 patients evaluable for cytogenetic response. In this patient population there was no significant difference in the survival probability according to patients' risk status as defined by the Sokal score. Categorization according to the extent of Ph reduction, however, allowed three groups with significantly different prognoses to be identified. Patients achieving a Ph reduction to less than 35% were found to constitute a low risk group with a median survival not yet known and a projected 5-year survival of 90%. The 5-year survival rate was 55% for patients with a Ph reduction to levels between 35% and 95%, and less than 10% for those without any cytogenetic improvement. Thus, this study demonstrates that cytogenetic improvement on IFN treatment is an important prognostic factor for survival.

Translocation (8;21) in acute nonlymphocytic leukemia delineated by chromosomal in situ suppression hybridization.

In situ suppression hybridization with recombinant bacteriophage DNA libraries for chromosomes 8 and 21 was performed in two cases of acute nonlymphocytic leukemia, type FAB M2. In both cases, cytogenetic analysis by conventional G-banding revealed t(8;21)(q22;q22). In situ suppression hybridization was able to prove the reciprocal nature of the translocation in both cases by identifying the terminal end of chromosome 21 translocated to the derivative chromosome 8q-.

Seven variants including four new Philadelphia translocations.

This paper reports on seven atypical Philadelphia chromosome translocations in chronic myelocytic leukemia. Three of them, a t(16;22), t(17;22), and t(9;14;22) have already been observed before, while the t(X;9;11;22), t(X;22), t(3;22) and t(3;4;9;22) are newly reported.