Gut microbiota profiles in the perioperative period and long-term postoperative period in children with ventricular septal defect treated with cardiopulmonary bypass / 中华实用儿科临床杂志

Objective:To explore the characteristics of gut microbiota in the preoperative, short-term postoperative and long-term postoperative period at (15.61±4.51) months in children with ventricular septal defect (VSD) of congenital heart disease (CHD) treated with cardiopulmonary bypass (CPB).Methods:A prospective study was conducted.In Guangzhou Women and Children′s Medical Center, 13 patients with VSD who were scheduled for CPB and additional 10 age- and gender-matched healthy infants as pre-CPB control group from January 2021 to January 2022 were enrolled.Fecal samples were collected at pre- and early post-CPB.Meanwhile, 18 gender- and CHD diagnosis and operation-matched patients at (15.61±4.51) months after CPB and 8 healthy age- and gender-matched children as long-term control group after CPB were also enrolled, and fecal samples were collected.16S rRNA sequencing of fecal samples from all subjects were performed and comparing the differences in gut microbiota between two groups via comparing alpha and beta diversity, parameter test or nonparametric test, and LEfSe analysis.Results:Compared with those of pre-CPB control group, there was a significant difference in the composition of gut microbiota in the preoperative period of VSD children, with significantly increased abundances of Enterobacteriaceae and Shigella, and decreased abundance of Bifidobacterium (all P<0.05). The diversity of gut microbiota was comparable in VSD children before CPB and in the short period time after CPB (all P>0.05), except for the abundances of Clostridium and Streptococcus (all P<0.05), and there was no significant difference in the relative abundances of other highly abundant gut bacteria between the two periods (all P>0.05). Compared with that in VSD children in the short period time after CPB, the abundances of short-chain fatty acids-producing microbes were significantly higher at (15.61±4.51) months postoperatively (all P<0.05), and the gut bacteria profile was similar to that of the long-term control group after CPB (all P>0.05). Conclusions:Gut microbiota imbalance exists in VSD children before CPB.The gut microbiota profile is not influenced by CPB, which returns normal at (15.61±4.51) months postoperatively.

Significance of paroxysmal nocturnal hemoglobinuria clone in immunosuppressive therapy for children with severe aplastic anemia / 中国当代儿科杂志

OBJECTIVES@#To study the association between paroxysmal nocturnal hemoglobinuria (PNH) clone and immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA).@*METHODS@#A retrospective analysis was performed on the medical data of 151 children with SAA who were admitted and received IST from January 2012 to May 2020. According to the status of PNH clone, these children were divided into a negative PNH clone group (n=135) and a positive PNH clone group (n=16). Propensity score matching was used to balance the confounding factors, and the impact of PNH clone on the therapeutic effect of IST was analyzed.@*RESULTS@#The children with positive PNH clone accounted for 10.6% (16/151), and the median granulocyte clone size was 1.8%. The children with positive PNH clone had an older age and a higher reticulocyte count at diagnosis (P<0.05). After propensity score matching, there were no significant differences in baseline features between the negative PNH clone and positive PNH clone groups (P>0.05). The positive PNH clone group had a significantly lower overall response rate than the negative PNH clone group at 6, 12, and 24 months after IST (P<0.05). The evolution of PNH clone was heterogeneous after IST, and the children with PNH clone showed an increase in the 3-year cumulative incidence rate of aplastic anemia-PNH syndrome (P<0.05).@*CONCLUSIONS@#SAA children with positive PNH clone at diagnosis tend to have poor response to IST and are more likely to develop aplastic anemia-PNH syndrome.

Quantification of the iduronate-2-sulfatase activity in prenatal diagnosis of mucopolysaccharidosis type Ⅱ / 中华实用儿科临床杂志

Objective:To evaluate the activity of iduronate-2-sulfatase (IDS) in fetal villi and peripheral blood plasma of pregnant women at high risk of mucopolysaccharidosis type Ⅱ (MPS Ⅱ), and to discuss the application of gene analysis in prenatal diagnosis of MPS Ⅱ.Methods:The enzymatic testing and gene analysis results of 23 pregnant women at high risk of MPS Ⅱ, who underwent prenatal diagnosis in Guangzhou Women and Children′s Medical Center from February 2013 to December 2020, were analyzed retrospectively.The IDS activity in fetal villi (30 cases) and plasma (28 cases) was detected by artificial substrate fluorescence.The IDS activity in fetal villi (28 cases) and plasma (34 cases) of normal pregnant women was taken as control.Meanwhile, the fetal villi of both pregnant women at high risk of MPS Ⅱ and normal pregnant women were also analyzed by gene testing and for fetal sex identification.Data were compared between groups by the independent samples t test. Results:The normal reference values of the IDS activity in fetal villi and plasma of normal pregnant women were(71.2±23.4) nmol/(mg·4 h) and (611.1±114.5) nmol/(mL·4 h), respectively.Among the 30 cases of high-risk fetal villi, the IDS activity in fetal villi of 8 affected male fetuses was (1.7±0.3) nmol/(mg·4 h), which was significantly lower than that of 11 unaffected male fetuses (83.2±6.3) nmol/(mg·4 h) and that of 9 non-carrier female fetuses (80.0±7.5) nmol/(mg·4 h) ( t=10.8, 8.8; all P<0.01). Meanwhile, the IDS activity was measured in the maternal peripheral plasma of 28 pregnant women at high risk of MPS Ⅱ.Among them, the IDS activity in 8 affected male fetuses was(225.4±20.5) nmol/(mL·4 h), which was significantly lower than that in non-affected male fetuses[(451.0±15.1) nmol/(mL·4 h)] and that in non-carrier female fetuses[(467.7±45.3)nmol/(mL·4 h)]. Eight known pathogenic mutations were found in 30 cases at high risk of MPS Ⅱ of fetal villi, and the mutation types were c. 1048A>C, c.212G>A, c.514C>T, c.257C>T, c.425C>T, and c. 998C>T.Of the 8 cases, 6 affected male fetuses had significantly reduced IDS activities, and the other 2 female carriers had normal IDS enzyme activities. Conclusions:The IDS activity in fetal villi and peripheral plasma of pregnant woman is consistent with the gene analysis results.The IDS activity has an important reference value for the prenatal diagnosis of MPS Ⅱ in the first trimester.When no genetic mutations are found in the probands or the pathogenicity of the new mutation remains unclear, the IDS activity in fetal villi can be detected separately for the prenatal diagnosis of MPS Ⅱ.

Effects of non-muscle myosin Ⅱ silenced bone marrow-derived mesenchymal stem cells transplantation on lung extracellular matrix in rats after endotoxin/lipopolysaccharide-induced acute lung injury / 中华烧伤杂志

Objective: To investigate the effects of non-muscle myosin Ⅱ (NMⅡ) gene silenced bone marrow-derived mesenchymal stem cells (BMMSCs) on pulmonary extracellular matrix (ECM) and fibrosis in rats with acute lung injury (ALI) induced by endotoxin/lipopolysaccharide (LPS). Methods: The experimental research methods were adopted. Cells from femur and tibial bone marrow cavity of four one-week-old male Sprague-Dawley rats were identified as BMMSCs by flow cytometry, and the third passage of BMMSCs were used in the following experiments. The cells were divided into NMⅡ silenced group transfected with pHBLV-U6-ZsGreen-Puro plasmid containing small interference RNA sequence of NMⅡ gene, vector group transfected with empty plasmid, and blank control group without any treatment, and the protein expression of NMⅡ at 72 h after intervention was detected by Western blotting (n=3). The morphology of cells was observed by an inverted phase contrast microscope and cells labeled with chloromethylbenzoine (CM-DiⅠ) in vitro were observed by an inverted fluorescence microscope. Twenty 4-week-old male Sprague-Dawley rats were divided into blank control group, ALI alone group, ALI+BMMSC group, and ALI+NMⅡ silenced BMMSC group according to the random number table, with 5 rats in each group. Rats in blank control group were not treated, and rats in the other 3 groups were given LPS to induce ALI. Immediately after modeling, rats in ALI alone group were injected with 1 mL normal saline via tail vein, rats in ALI+BMMSC group and ALI+NMⅡ silenced BMMSC group were injected with 1×107/mL BMMSCs and NMⅡ gene silenced BMMSCs of 1 mL labelled with CM-DiⅠ via tail vein, and rats in blank control group were injected with 1 mL normal saline via tail vein at the same time point, respectively. At 24 h after intervention, the lung tissue was collected to observe intrapulmonary homing of the BMMSCs by an inverted fluorescence microscope. Lung tissue was collected at 24 h, in 1 week, and in 2 weeks after intervention to observe pulmonary inflammation by hematoxylin eosin staining and to observe pulmonary fibrosis by Masson staining, and the pulmonary fibrosis in 2 weeks after intervention was scored by modified Ashcroft score (n=5). The content of α-smooth muscle actin (α-SMA), matrix metalloproteinase 2 (MMP-2), and MMP-9 was detected by immunohistochemistry in 2 weeks after intervention (n=3), the activity of superoxide dismutase (SOD), malondialdehyde, myeloperoxidase (MPO) was detected by enzyme-linked immunosorbent assay at 24 h after intervention (n=3), and the protein expressions of CD11b and epidermal growth factor like module containing mucin like hormone receptor 1 (EMR1) in 1 week after intervention were detected by immunofluorescence staining (n=3). Data were statistically analyzed with one-way analysis of variance, Bonferroni method, and Kruskal-Wallis H test. Results: At 72 h after intervention, the NMⅡprotein expression of cells in NMⅡ silenced group was significantly lower than those in blank control group and vector group (with P values <0.01). BMMSCs were in long spindle shape and grew in cluster shaped like vortexes, which were labelled with CM-DiⅠ successfully in vitro. At 24 h after intervention, cell homing in lung of rats in ALI+NMⅡ silenced BMMSC group was more pronounced than that in ALI+BMMSC group, while no CM-DiⅠ-labelled BMMSCs were observed in lung of rats in blank control group and ALI alone group. There was no obvious inflammatory cell infiltration in lung tissue of rats in blank control group at all time points, while inflammatory cell infiltration in lung tissue of rats in ALI+BMMSC group and ALI+NMⅡ silenced BMMSC group was significantly less than that in ALI alone group at 24 h after intervention, and alveolar wall turned to be thinner and a small amount of congestion in local lung tissue appeared in rats of the two groups in 1 week and 2 weeks after intervention. In 1 week and 2 weeks after intervention, collagen fiber deposition in lung tissue of rats in ALI alone group, ALI+BMMSC group, and ALI+NMⅡ silenced BMMSC group was significantly aggravated compared with that in blank control group, while collagen fiber deposition in lung tissue of rats in ALI+BMMSC group and ALI+NMⅡ silenced BMMSC group was significantly improved compared with that in ALI alone group. In 2 weeks after intervention, modified Ashcroft scores for pulmonary fibrosis of rats in ALI alone group, ALI+BMMSC group, and ALI+NMⅡ silenced BMMSC group were 2.36±0.22, 1.62±0.16, 1.06±0.26, respectively, significantly higher than 0.30±0.21 in blank control group (P<0.01). Modified Ashcroft scores for pulmonary fibrosis of rats in ALI+BMMSC group and ALI+NMⅡ silenced BMMSC group were significantly lower than that in ALI alone group (P<0.01), and modified Ashcroft score for pulmonary fibrosis of rats in ALI+NMⅡ silenced BMMSC group was significantly lower than that in ALI+BMMSC group (P<0.01). In 2 weeks after intervention, the content of α-SMA in lung tissue of rats in ALI+BMMSC group and ALI+NMⅡ silenced BMMSC group were significantly decreased compared with that in ALI alone group (P<0.05 or P<0.01). The content of MMP-2 in lung tissue of rats in the 4 groups was similar (P>0.05). The content of MMP-9 in lung tissue of rats in ALI alone group was significantly increased compared with that in blank control group (P<0.01), and the content of MMP-9 in lung tissue of rats in ALI+BMMSC group and ALI+NMⅡ silenced BMMSC group was significantly decreased compared with that in ALI alone group (P<0.01). At 24 h after intervention, the activity of malondialdehyde, SOD, and MPO in lung tissue of rats in ALI alone group, ALI+BMMSC group, and ALI+NMⅡ silenced BMMSC group were significantly increased compared with that in blank control group (P<0.01), the activity of malondialdehyde in lung tissue of rats in ALI+NMⅡ silenced BMMSC group and the activity of SOD in lung tissue of rats in ALI+BMMSC group and ALI+NMⅡ silenced BMMSC group were significantly increased compared with that in ALI alone group (P<0.05 or P<0.01), and the activity of SOD in lung tissue of rats in ALI+NMⅡ silenced BMMSC group was significantly decreased compared with that in ALI+BMMSC group (P<0.01). The activity of MPO in lung tissue of rats in ALI+BMMSC group and ALI+NMⅡ silenced BMMSC group was significantly decreased compared with that in ALI alone group (P<0.01), and the activity of MPO in lung tissue of rats in ALI+NMⅡ silenced BMMSC group was significantly decreased compared with that in ALI+BMMSC group (P<0.01). In 1 week after intervention, the protein expression of CD11b in lung tissue of rats in ALI+NMⅡ silenced BMMSC group was significantly increased compared with those in the other three groups (P<0.05 or P<0.01), while the protein expressions of EMR1 in lung tissue of rats in the four groups were similar (P>0.05). Conclusions: Transplantation of NMⅡ gene silenced BMMSCs can significantly improve the activity of ECM components in the lung tissue in LPS-induced ALI rats, remodel its integrity, and enhance its antioxidant capacity, and alleviate lung injury and pulmonary fibrosis.

Xiaoyaosan Promotes Myelin Regeneration and Alleviates Depressive Symptoms in Vascular Dementia Mice with Depression via Promoting Microglia Polarization / 中国实验方剂学杂志

Objective:To investigate the effect of Xiaoyaosan on depressive behavioral phenotype in mice with vascular dementia （VaD） mice and its possible mechanism. Method:Sixty three-month-old male C57/BL6 mice were divided into the normal control group， model group， positive control group， as well as low-， medium-， and high-dose Xiaoyaosan groups. Mice in all groups except for the normal control group underwent bilateral carotid artery stenosis. Two weeks later， they were subjected to chronic restraint stress， 6 h per day， for inducing VaD complicated with depression. Mice in the low-， medium-， and high-dose Xiaoyaosan groups were treatment with intragastric administration of Xiaoyaosan decoction （5， 10， 20 g·kg^-1）， the ones in the positive control group with fluoxetine （10 mg·kg^-1）， and those in the normal control group and model group with an equal volume of normal saline for four weeks， during which the restraint stress was maintained. The depressive behavioral phenotype of mice was observed in sugar water preference test and tail suspension test. The fluorescence expression of myelin basic protein （MBP） in ventral hippocampus （vHIP） was detected by fluorescence immunoassay. The ultrastructure of myelin sheath in vHIP was observed by transmission electron microscopy. The protein expression levels of MBP， myelin oligodendrocyte glycoprotein （MOG）， myelin-associated glycoprotein （MAG）， triggering receptor expressed on myeloid cells-2 （TREM2）， inducible nitric oxide synthase （iNOS）， arginase 1 （Arg1）， interleukin-I<italic>β</italic> （IL-1<italic>β</italic>）， tumor necrosis factor-<italic>α</italic> （TNF-<italic>α</italic>）， interleukin-4 （IL-4）， and interleukin-10 （IL-10） were assayed by Western blot. Result:As revealed by behavioral test， compared with the normal control group， the model group exhibited prolonged immobility time and decreased percentage of sugar water preference （<italic>P</italic><0.01）. Compared with the model group， Xiaoyaosan significantly shortened the immobility time of mice （<italic>P</italic><0.05） and increased the percentage of sugar water preference （<italic>P</italic><0.01）. Western blot results showed that the protein expression levels of MBP， MOG， and MAG in vHIP of the model group were remarkably decreased as compared with those of the normal control group （<italic>P</italic><0.01）. The protein expression levels of MBP， MOG， and MAG in vHIP of the low-dose Xiaoyaosan group were increased in contrast to those in the model group （<italic>P</italic><0.05， <italic>P</italic><0.01）， while the protein expression of iNOS was decreased （<italic>P</italic><0.01）. The protein expression levels of MBP， MOG， MAG， TREM2， Arg1， IL-4， and IL-10 in the medium- and high-dose Xiaoyaosan groups were up-regulated （<italic>P</italic><0.05， <italic>P</italic><0.01）， whereas those of iNOS， IL-1<italic>β</italic>， and TNF-<italic>α</italic> were down-regulated （<italic>P</italic><0.01）. The immunofluorescence findings demonstrated that the mean fluorescence intensity of MBP in the model group declined in comparison with that in the normal control group （<italic>P</italic><0.01）， while the mean fluorescence intensities of MBP in the low-， medium-， and high-dose Xiaoyaosan groups were enhanced to different degrees （<italic>P</italic><0.01）. It was observed under the transmission electron microscope that the myelin structure of the model group was loosened and the dense layer was separated and irregularly arranged. Xiaoyaosan improved the structural integrity of myelin sheath and the looseness of lamellar structure. Conclusion:Xiaoyaosan ameliorates the depressive behavioral phenotype of VaD mice， which may be related to the up-regulation of TREM2， the induction of M2 polarization of microglia cells， the enhancement of their anti-inflammatory and phagocytic abilities， and the promotion of damaged myelin sheath regeneration.

Clinical effect of multicenter multidisciplinary treatment in children with renal malignant tumors / 中国当代儿科杂志

OBJECTIVE@#To study the long-term clinical effect of multicenter multidisciplinary treatment (MDT) in children with renal malignant tumors.@*METHODS@#A retrospective analysis was performed on the medical data of 55 children with renal malignant tumors who were diagnosed and treated with MDT in 3 hospitals in Hunan Province from January 2015 to January 2020, with GD-WT-2010 and CCCG-WT-2016 for treatment regimens. A Kaplan-Meier survival analysis was used to analyze the survival of the children.@*RESULTS@#Of the 55 children, 10 had stage I tumor, 14 had stage Ⅱ tumor, 22 had stage Ⅲ tumor, 7 had stage IV tumor, and 2 had stage V tumor. As for pathological type, 47 had FH type and 8 had UFH type. All children underwent complete tumor resection. Of the 55 children, 14 (25%) received preoperative chemotherapy. All children, except 1 child with renal cell carcinoma, received postoperative chemotherapy. Among the 31 children with indication for radiotherapy, 21 (68%) received postoperative radiotherapy. One child died of postoperative metastasis. The incidence rate of FH-type myelosuppression was 94.4%, and the incidence rate of UFH-type myelosuppression was 100%. The median follow-up time was 21 months and the median survival time was 26 months for all children, with an overall survival rate of 98% and an event-free survival rate of 95%.@*CONCLUSIONS@#Multicenter MDT has the advantages of high success rate of operation and good therapeutic effect of chemotherapy in the treatment of children with renal malignant tumors, with myelosuppression as the most common side effects, and radiotherapy is safe and effective with few adverse events. Therefore, MDT has good feasibility, safety, and economy.

Assessment of the Severity of Coronavirus Disease:Quantitative Computed Tomography Parameters versusSemiquantitative Visual Score

Objective@#To compare the accuracies of quantitative computed tomography (CT) parameters and semiquantitative visualscore in evaluating clinical classification of severity of coronavirus disease (COVID-19). @*Materials and Methods@#We retrospectively enrolled 187 patients with COVID-19 treated at Tongji Hospital of Tongji MedicalCollege from February 15, 2020, to February 29, 2020. Demographic data, imaging characteristics, and clinical data werecollected, and based on the clinical classification of severity, patients were divided into groups 1 (mild) and 2 (severe/critical). A semiquantitative visual score was used to estimate the lesion extent. A three-dimensional slicer was used toprecisely quantify the volume and CT value of the lung and lesions. Correlation coefficients of the quantitative CT parameters,semiquantitative visual score, and clinical classification were calculated using Spearman’s correlation. A receiver operatingcharacteristic curve was used to compare the accuracies of quantitative and semi-quantitative methods. @*Results@#There were 59 patients in group 1 and 128 patients in group 2. The mean age and sex distribution of the two groupswere not significantly different. The lesions were primarily located in the subpleural area. Compared to group 1, group 2 hadlarger values for all volume-dependent parameters (p < 0.001). The percentage of lesions had the strongest correlation withdisease severity with a correlation coefficient of 0.495. In comparison, the correlation coefficient of semiquantitative scorewas 0.349. To classify the severity of COVID-19, area under the curve of the percentage of lesions was the highest (0.807;95% confidence interval, 0.744–0.861: p < 0.001) and that of the quantitative CT parameters was significantly higher thanthat of the semiquantitative visual score (p = 0.001). @*Conclusion@#The classification accuracy of quantitative CT parameters was significantly superior to that of semiquantitativevisual score in terms of evaluating the severity of COVID-19.

Literature analysis on the impact of implementing the diagnosis-related groups prospective payment system on the quality of medical services / 中华医院管理杂志

Objective:To analyze the effect of diagnosis-related groups prospective payment system(DRG-PPS) on medical service quality.Methods:Literature on DRG-PPS impact on medical service quality was searched in Chinese and English databases, and inclusion and exclusion criteria were formulated. Meta analysis was performed on the average length of stay, mortality and readmission rate, and the results of other indicators were described and integrated.Results:A total of 33 articles were included for analysis. Meta analysis showed that the implementation of DRG-PPS could shorten the average length of stay, WMD=-0.67(95% CI: -0.91 to-0.43); the implementation of DRG-PPS had no significant impact on the readmission rate( OR=0.97, 95% CI: 0.87-1.08); the implementation of DRG-PPS did not have a significant impact on the death rate of patients( OR=0.98, 95% CI: 0.93-1.03). The results of narrative integration showed that DRG-PPS had no significant effect on the incidence of complications, patient satisfaction, quality of life, service and operation process indicators. Conclusions:DRG-PPS can shorten the average length of stay, but has no significant effect on mortality and readmission rate.

New progress in genetic study of nemaline myopathy / 中华神经科杂志

Nemaline myopathy is a common type of congenital myopathy,and various gene mutations of thin filaments and related components are the causes of the disease.In recent years,a total of 13 gene mutations have been found to be associated with the disease.We review the six new pathogenic genes discovered in the past five years,and summarize gene function,encoded protein,mutation type and clinical features of nemaline myopathy.The pathogenesis of nemaline myopathy is needed to be further explored.

Unusual facies and recurrent high triglycerides for more than one year in a girl / 中国当代儿科杂志

A girl, aged 1 year and 9 months, was found to have hypertriglyceridemia in the neonatal period, with unusual facies and signs of dark skin all over the body, disappearance of subcutaneous adipose, acanthosis nigricans of the neck, excessive and thick hair, empty cheeks, muscle hypertrophy of the extremities, hepatomegaly, and neutrophil deficiency. Whole exome sequencing of monogenic disorder revealed a homozygote mutation in the BSCL2 gene, c.974 (exon 7)_c.975 (exon 7) insG. Her parents were heterozygotes for this locus. The girl was diagnosed with congenital generalized lipodystrophy (CGL), but the association between CGL and neutrophil deficiency remained unclear. Triglyceride was maintained at a normal level after the treatment with a low-fat and high-carbohydrate diet, and there were no obvious changes in signs. CGL is a rare autosomal recessive systemic disease manifested as disappearance of systemic subcutaneous adipose, muscle hypertrophy of the extremities, and metabolic disorders in the neonatal period, such as high triglycerides, hyperinsulinemia, and hyperglycemia. About 95% of CGL cases are caused by mutations in the AGPAT2 or BSCL2 gene.

Diffusion kurtosis imaging can be predictive in high-aggressive prostate cancer / 实用放射学杂志

Objective To investigate whether diffusion kurtosis imaging(DKI)can be predictive in high-aggressive prostate cancer (PCa).Methods 51 patients with pathologically confirmed PCa underwent preoperative DK-MR imaging(b of 0,700,1 400 and 2 100 s/mm2).Data was post-processed by mono-exponential and non-Gauss DK model,respectively,for quantitation of apparent diffusion coefficient(ADC),apparent non-Gaussian diffusion coefficient(Dapp)and apparent non-Gauss kurtosis coefficient(Kapp). The ability of Dapp,Kapp and ADC for predicting high-aggressive(Gleason score>4+3)PCa was analyzed by ROC regression.Results There were 29 low-aggressive and 33 high-aggressive PCa on pathologic findings.High-aggressive PCa had significantly lower ADC[(0.764 ± 0.114)×10-3mm2/s vs(0.825 ± 0.116)×10-3mm2/s,P=0.004],lower Dapp[(1.212 ± 0.194)×10-3mm2/s vs (1.472 ± 0.297)×10-3mm2/s,P< 0.001],while higher Kapp(1.114 ± 0.177 vs 0.835 ± 0.192,P<0.001)than that for low-aggressive PCa.Dapp and Kapp had significantly higher sensitivity(Dapp:75.3%;Kapp:74.1%),specificity(Dapp:85.4%;Kapp:86.7%)and area under curve(AUC)(Dapp:0.889;Kapp:0.894)than that for ADC(64.1%;76.4%;0.738;P<0.01)in differentiating low-aggressive from high-aggressive PCa.Conclusion DKI can be a reliable way for predicting high-aggressive PCa.

The diagnostic value of spectral CT in atypical tuberculosis ball and lung cancer / 实用放射学杂志

Objective To study the diagnostic value of spectral CT in atypical tuberculosis ball and lung cancer.Methods 45 cases of pulmonary nodule or mass received unenhanced and two phase enhanced scan in gemstone spectral imaging mode,and all pulmonary nodule or mass were confirmed by pathology.Selecting unenhanced,arterial phase and venous phases of spectrum image,atypical tuberculosis ball group and lung cancer group were analyzed.Normalized iodine concentration (NIC), the spectral curve slope (λHU),CT value of unenhanced and two phase enhanced spectrum image were measured and calculated.Two independent samples t-test was used for the statistic analysis of each spectrum parameters between the two groups.The receiver operating characteristic (ROC) curve was obtained for comparing the sensitivity and specificity of each spectral parameter.Results Among the 45 patients,11 were atypical tuberculosis ball, and 34 were lung cancer.Comparison NIC,two phase enhanced λHU and the net enhanced CT values of 70 keV monoenergetic image of the two groups, lung cancer group was higher than atypical tuberculosis ball group,and the difference was statistically significant(P0.05).When the NIC diagnostic threshold was 0.105, the maximum Youden index (0.733), the area under the ROC curve was 0.89, and sensitivity and specificity was 82.4% and 90.9%, respectively.Conclusion The quantitative parameters of spectral CT is helpful in the diagnosis of atypical tuberculosis ball and lung cancer,especially the NIC with high value.

Increased Expression of the NOD-like Receptor Family, Pyrin Domain Containing 3 Inflammasome in Dermatomyositis and Polymyositis is a Potential Contributor to Their Pathogenesis / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Dermatomyositis (DM) and polymyositis (PM) are common inflammatory myopathies whose immunopathogenic mechanisms remain poorly understood. The NOD-like receptor family, pyrin domain containing 3 (NLRP3) inflammasome is a type of cytoplasmic multiprotein inflammasome and is responsible for the activation of inflammatory reactivations. Responding to a wide range of exogenous and endogenous microbial or sterile stimuli, NLRP3 inflammasomes can cleave pro-caspase-1 into active caspase-1, which processes the pro-inflammatory cytokines pro-interleukin (IL)-1β and pro-IL-18 into active and secreted IL-1β and IL-18. The NLRP3 inflammasome is implicated in infectious and sterile inflammatory diseases. However, it remains unclear whether it is involved in the pathogenesis of DM/PM, which we aim to address in our research.</p><p><b>METHODS</b>In this study, 22 DM/PM patients and 24 controls were recruited. The protein and RNA expression of IL-1β, IL-18, NLRP3, and caspase-1 in serum and muscle samples were tested and compared between the two groups.</p><p><b>RESULTS</b>The serum IL-1β and IL-18 levels were significantly higher in DM/PM patients than those in the controls by enzyme linked immunosorbent assay (ELISA, DM vs. control, 25.02 ± 8.29 ng/ml vs. 16.49 ± 3.30 ng/ml,P < 0.001; PM vs. control, 26.49 ± 7.79 ng/ml vs. 16.49 ± 3.30 ng/ml,P < 0.001). Moreover, the real-time quantitative reverse transcription-polymerase chain reaction (qRT-PCR) showed that DM/PM patients exhibited higher RNA expression of IL-1β, IL-18, and NLRP3 in the muscle (for IL-1β, DM vs. control, P= 0.0012, PM vs. control, P= 0.0021; for IL-18, DM vs. control, P= 0.0045, PM vs. control, P= 0.0031; for NLRP3, DM vs. control, P= 0.0017, PM vs. control, P= 0.0006). Moreover, the protein expression of NLRP3 and caspase-1 in muscle samples of DM/PM patients were also significantly elevated compared to that in the muscles of the controls.</p><p><b>CONCLUSIONS</b>Our findings demonstrate that the NLRP3 inflammasome is implicated in the pathogenesis of DM/PM. High NLRP3 expression led to elevated levels of IL-1β and IL-18 and could be one of the factors promoting disease progress.</p>

Oculopharyngeal Weakness, Hypophrenia, Deafness, and Impaired Vision: A Novel Autosomal Dominant Myopathy with Rimmed Vacuoles / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Myopathies with rimmed vacuoles are a heterogeneous group of muscle disorders with progressive muscle weakness and varied clinical manifestations but similar features in muscle biopsies. Here, we describe a novel autosomal dominant myopathy with rimmed vacuoles in a large family with 11 patients of three generations affected.</p><p><b>METHODS</b>A clinical study including family history, obstetric, pediatric, and development history was recorded. Clinical examinations including physical examination, electromyography (EMG), serum creatine kinase (CK), bone X-rays, and brain magnetic resonance imaging (MRI) were performed in this family. Open muscle biopsies were performed on the proband and his mother. To find the causative gene, the whole-exome sequencing was carried out.</p><p><b>RESULTS</b>Disease onset was from adolescence to adulthood, but the affected patients of the third generation presented an earlier onset and more severe clinical manifestations than the older generations. Clinical features were characterized as dysarthria, dysphagia, external ophthalmoplegia, limb weakness, hypophrenia, deafness, and impaired vision. However, not every patient manifested all symptoms. Serum CK was mildly elevated and EMG indicated a myopathic pattern. Brain MRI showed cerebellum and brain stem mildly atrophy. Rimmed vacuoles and inclusion bodies were observed in muscle biopsy. The whole-exome sequencing was performed, but the causative gene has not been found.</p><p><b>CONCLUSIONS</b>We reported a novel autosomal dominant myopathy with rimmed vacuoles characterized by dysarthria, dysphagia, external ophthalmoplegia, limb weakness, hypophrenia, deafness, and impaired vision, but the causative gene has not been found and needs further study.</p>

Human experiments of metabolism, blood alkalization and oxygen effect on control and regulation of breathing. I: room air exercise test / 中国应用生理学杂志

<p><b>OBJECTIVE</b>Under the guidance of the holistic integrative physiology medicine, we reanalyzed the data during symptom-limited maximum cardiopulmonary exercise testing (CPET) in order to investigate control and regulatory mechanism of breathing.</p><p><b>METHODS</b>This study investigated 5 normal volunteers who accepted artery catheter, performed CPET room air. Continuous measured pulmonary ventilation parameters and per minute arterial blood gas (ABG) analysis sample parameters during exercise. All CPET and ABG data changes were standard analyzed and calculated.</p><p><b>RESULTS</b>With gradually increasing power, minute oxygen uptake(every breath oxygen uptake x respiratory rate = O2 paulse x heart rate) and minute ventilation (tidal volume x respiratory rate) showed nearly linear progressive increase during the CPET(compared with the rest stage, P < 0.05 - 0.001); Minute ventilation increased even more significant after the anaerobic threshold (AT) and respiratory compensation point. PaO2 was increased at recovery 2 minutes (P < 0.05); PaCO2 was decreased after anaerobic threshold 2 minutes (P < 0.05); [H+]a was increased from AT (P < 0.05), and rapidly raised at last 2 minutes, remained high at recovery. Lactate was increased rapidly from AT (compared with resting, P < 0.05); bicarbonate decreased rapidly from AT (compared with resting, P < 0.05) and it's changed direction was contrary to lactic acid.</p><p><b>CONCLUSION</b>In order to overcome the resistance of the power during exercise, metabolic rate othe body increased, respiratory change depend upon the change metabolism, and the accumulation of acidic products exacerbated respiratory reactions at high intensity exercise.</p>

Human experiments of metabolism, blood alkalization and oxygen effect on control and regulation of breathing. II: room air exercise test after blood alkalization / 中国应用生理学杂志

<p><b>OBJECTIVE</b>Basis on the dynamic changes of the ventilation and arterial blood gas parameters to symptom-limited maximum cardiopulmonary exercise testing (CPET), we further investigate the effect of alkalized blood by drinking 5% NaHCO3 on ventilation during exercise.</p><p><b>METHODS</b>After drinking 5% NaHCO3 75 ml (3.75 g) every 5 min, total dosage of 0.3 g/Kg, 5 volunteers repeated CPET. All CPET and ABG data changes were analyzed and calculated. At the same time, CPET and ABG parameters after alkalized blood were compared with those before alkalized blood (control) used paired t test.</p><p><b>RESULTS</b>After alkalized blood, CPET response patterns of parameters of ventilation, gas exchange and arterial blood gas were very similar (P > 0.05). All minute ventilation, tidal volume, respiratory rate, oxygen uptake and carbon dioxide elimination were gradually increased from resting stage (P < 0.05-0.001), according to the increase of power loading. During CPET after alkalized blood, ABG parameters were compared with those of control: hemoglobin concentrations were lower, CaCO2 and pHa were increased at all stages (P < 0.05). The PaCO2 increased trend was clear, however only significantly at warm-up from 42 to 45 mmHg (P < 0.05). Compared with those of control, only the minute ventilation was decreased from 13 to 11 L/min at resting (P < 0.05).</p><p><b>CONCLUSION</b>Even with higher mean CaCO2, PaCO2 and pHa, lower Hba and [H+]a, the CPET response patterns of ventilatory parameters after alkalized blood were similar.</p>

Human experiments of metabolism, blood alkalization and oxygen effect on control and regulation of breathing. III: pure oxygen exercise test after blood alkalization / 中国应用生理学杂志

<p><b>OBJECTIVE</b>After performed symptom-limited maximum cardiopulmonary exercise testing (CPET) before and after acute alkalized blood, we repeated CPET with pure oxygen.</p><p><b>METHODS</b>Five volunteers, 3hr after alkalizing blood room air CPET, re-performed CPET inhaling from Douglas bag connected with pure oxygen tank. We compared with those of room air CPETs before and after alkalized blood.</p><p><b>RESULTS</b>After alkalized blood oxygen CPET had a similar response pattern as those of CPETs before and after blood alkalization. During the CPET, all breath frequency, minute ventilation and tidal volume at each stage were similar to those of CPETs before and after alkalized blood (P > 0.05),except there was a lower peak tidal volume than those of both CPETs and a slightly higher resting minute ventilation only than CPET after alkalized blood (P > 0.05). After alkalized blood, oxygen CPET, all PaO2 and SaO2 and most Hb were lower than those of both CPETs (P < 0.05). The pHa and [HCO3-]a were higher than those of CPET before alkalized blood (P < 0.05); but were not CPET after alkalized blood (P > 0.05). PaCO2 was similar to that of CPET before alkalized blood (P > 0.05), but was lower than that of CPET after alkalized blood at resting and warm-up (P < 0.05); then was similar to both CPETs at anaerobic threshold (P > 0.05); but was higher at peak exercise higher than those of both CPETs (P < 0.01). Oxygen increased 2,3 volunteers' workload and time at AT and peak exercises.</p><p><b>CONCLUSION</b>Respiratory response pattern to oxygen CPET after alkalized blood is similar to those of both CPETs before and after alkalized blood. The CPET response is dominantly depended upon metabolic rate, but not levels of pHa, PaCO2 and PaO2.</p>

Kniest dysplasia due to mutation of COL2A1 gene / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To detect potential mutation of COL2A1 gene in two children suspected for Kniest dysplasia.</p><p><b>METHODS</b>The 54 exons and splicing regions of the COL2A1 gene were amplified with PCR and the product was subjected to direct sequencing.</p><p><b>RESULTS</b>A missense mutation (c.905C>T, p.Ala302Val) was found in the coding region of the COL2A1 gene, which has been previously reported in abroad. The patients appeared to have short trunk dwarfism, enlarged joints and midface hypoplasia.</p><p><b>CONCLUSION</b>The probands are the first cases of Kniest dysplasia described in China, and so was the p.Ala302Val mutation.</p>

Neuropeptide trefoil factor 3 increases reward effects of cocaine in rats / 中华行为医学与脑科学杂志

Objective To investigate the role of neuropeptide trefoil factor 3 (TFF3) in cocaine reward responses and the underlying mechanism.Methods Fifty SD rats were divided into 6 groups including Control group,Cocaine group,Cocaine + TFF3 (0.01 mg/kg) group,cocaine + TFF3 (0.1 mg/kg) group,cocaine + TFF3 (0.5 mg/kg) group and TFF3 (0.1 mg/kg) group,ip,n=7 ～ 9),and were treated with TFF3 / saline (ip),30 min later,rats were injected with cocaine (10 mg/kg ip) followed by 1 h hypedocomotion test.Immediately after behavioral test,rats (n=4～6) were decapitated and tissues of nucleus accumbens (NAc) were isolated and prepared for neurotransmitters analysis by HPLC; Another twenty one rats were divided into control and TFF3-treatment group,and the rats were trained with a modified 2-day cocaine CPP conditioning procedure.During cocaine CPP conditioning process,saline or TFF3 (0.1 mg/kg ip) was injected 30 min prior to cocaine injection (5 mg/kg ip).Results Systemic administration of TFF3 (0.1 mg/kg,ip) significantly increased the cocaine-induced locomotor activity (Total distance in 1 hour were (180±41) cm,(359±53) cm,(590±75) cm,(153±27) cm for Control,Vehicle + Cocaine,TFF3+Cocaine and TFF3+Vehicle groups respectively) and augmented cocaine rewarding effects in CPP(Post-training CPP score were (98± 18) s,(187±24) s for Vehicle + Cocaine,TFF3+Cocaine groups respectively).TFF3 (0.1 mg/kg ip) administration increased the dopamine concentration in the NAc induced by cocaine injection ((0.65±0.1) ng/ml,(1.24±0.14) ng/ml,(1.75±0.23) ng/ml,(0.74±0.21) ng/ml for Control,Cocaine,TFF3 + Cocaine and TFF3 + Vehicle groups respectively).Conclusion TFF3 is involved in regulation of behavioral response to cocaine,which is associated with the increasing of dopamine in the NAc.

The role of ADC difference value in identification between benign and malignant breast lesions / 实用放射学杂志

Objective To evaluate the difference of the surrounding normal tissue and lesion apparent diffusion coefficient (appar-ent diffusion coefficient,ADC)value in identify malignant and benign breast lesions.Methods There were 65 suspicious malignant breast lesions received by DWI scan and dynamic contrast-enhanced MRI (DEC-MRI)in 64 patients.The ADC value of breast lesions and peripherial glands were respectively measured,and then the difference was calculated.Receiver operating characteristic curve (ROC)analysis was used to evaluate the difference’s performance in identification between benign and malignant breast lesions.Re-sults There were 35 malignant lesions and 30 benign lesions.For the malignant lesions,the optimal threshold values for ADC of breast lesions were 1.28×10-3 mm2/s.The area under the ROC was 92.2%,the sensitivity and specificity were 76.7% and 94.3%.While the optimal threshold values for ADC were 0.63×10 -3 mm2/s in the benign lesions.The area under the ROC was 94.9%,the sensitivity and specificity were 88.6% and 96.7%.Conclusion ADC difference can reduce the influences of individual physiological factors,different machine,scanning parameters,which play an important role in identification between benign and malignant breast lesions.