Atractylodis rhizoma is a perennial herb of the Asteraceae family,which mainly divided into A.chinensis(DC)Koidz and Atractyiodes lancea(Thunb)DC,with the effects of strengthening the spleen,drying dampness,brightening the eyes,etc.Atractylodis rhizoma is mainly used in the clinical treatment of spleen deficiency and dampness,night blindness,eye fatigue and other symptoms.According to the clinical effect,and modern pharmacological researches have confirmed,the Chinese herbal medicine Atractylodis rhizoma contains a variety of active ingredients,such as volatile oils,alkynes,glycosides,etc.In recent years,pharmacological studies on Atractylodis rhizoma have found that atractylodin in polyalkynes has good activity in anti-inflammation,treatment of bacterial resistance,and inhibition of cholangiocarcinoma cell migration,and its high biological activity may be related to the conjugated enyne structure.Therefore,this article summarizes the studies on chemical components and pharmacological effects of polyalkynes in Atractylodis rhizoma that have been published in recent years,and comprehensively expounds the research progress of polyalkynes in Atractylodis rhizoma,so as to provide reference for scientific researchers and promote the in-depth development and utilization of the medicinal value of Atractylodis rhizoma.

OBJECTIVES: To investigate genotype-phenotype characteristics and long-term prognosis of neonatal carbamoyl phosphate synthetase 1 (CPS1) deficiency among children through newborn screening in Zhejiang province. METHODS: The clinical and follow-up data of children with CPS1 deficiency detected through neonatal screening and confirmed by tandem mass spectrometry and genetic testing in Zhejiang Province Newborn Disease Screening Center from September 2013 to August 2023 were retrospectively analyzed. RESULTS: A total of 4 056 755 newborns were screened and 6 cases of CPS1 deficiency were diagnosed through phenotypic and genetic testing. Ten different variations of CPS1 genewere identified in genetic testing, including 2 known pathogenic variations (c.2359C>T and c.1549+1G>T) and 8 unreported variations (c.3405-1G>T, c.2372C>T, c.1436C>T, c.2228T>C, c.2441G>A, c.3031G>A, c.3075T>C and c.390-403del). All patients had decreased citrulline levels (2.72-6.21 μmol/L), and varying degrees of elevated blood ammonia. The patients received restricted natural protein intake (special formula), arginine and supportive therapy after diagnosis, and were followed-up for a period ranging from 9 months to 10 years. Three patients experienced hyperammonemia, and one patient each had attention deficit hyperactivity disorder, transient facial twitching and increased muscle tone. One patient died, while the other five surviving patients had normal scores of the Ages & Stages Questionnaires (ASQ) and Griffiths Development Scales up to the present time; 4 cases had combined height or weight lag and one case was normal in height and weight. CONCLUSIONS Low citrulline levels and hyperammonemia are common in CPS1 deficiency patients in Zhejiang. Most gene variants identified were specific to individual families, and no hotspot mutations were found. Early diagnosis through newborn screening and following standardized treatment can significantly improve the prognosis of the patients.
Child ; Humans ; Infant, Newborn ; Carbamoyl-Phosphate Synthase I Deficiency Disease/therapy* ; Neonatal Screening ; Follow-Up Studies ; Hyperammonemia ; Citrulline/genetics* ; Retrospective Studies ; Mutation

Objective:To investigate the characteristics of early myocardial mechanics changes in diabetic cardiomyopathy (DCM).Method:Sixty healthy 4-week-old male C57BL/6J mice were randomly divided into the T2DM group ( n=30) and the control group ( n=30). The T2DM group was fed with high-fat diet for 4 weeks, and accepted injection of a single high-dose of streptozotocin (STZ) intraperitoneally. Finally, the model was established successfully in 23 mice. The control group was fed with a normal diet and treated with citrate buffer liquid at an equal dose as T2DM group. Then, nine mice were randomly selected from each of the two groups every 4 weeks until the end of the 24th week. Six of the nine mice were randomly selected to perform 7.0 T MR scanning after measuring blood glucose and body weight. Cine images were acquired through cardiovascular MR feature tracking (CMR-FT). The obtained parameters included the left ventricle global peak circumferential strain (LV-GPCS), left ventricle global peak radial strain(GPRS) and the ejection fraction (EF), etc. The rest three mice were sacrificed for observation of the changes of interstitial fibers and micro-vessels in myocardial tissue with Sirius red staining. One-way analysis of variance (ANOVA) and t test were used for comparison. Results:There were significant differences in blood glucose levels between the two groups during the observation period ( P<0.05). In the 4 th-24 th week, the value of GPCS in T2DM group showed a downward trend, and the difference was statistically significant ( F = 8.23, P<0.001). Compared with the control group, the value of GPCS in T2DM group was statistically significant at the 20 th and 24 th week (the 20 th week: -11.4%±2.1% in the T2DM group vs. -14.3%±1.9% in the control group, t=2.54, P=0.029;the 24 th week: -12.3%±1.7% in the T2DM group vs. -14.6%±1.8% in the control group, t=2.35 , P=0.040), while the EF value was different at the 24 th week (51%±5% in the T2DM group vs. 62%±6% in the control group, t=3.38, P=0.007). There was no significant difference in the GPRS of the T2DM mice group over time or compared with the controls ( P>0.05). Moreover, the pathological results showed that the myocardial interstitial fibers in the T2DM group had remarkably increased since the 12 th week. Conclusions:The alterations in myocardial interstitial fibers and myocardial contractility appeared early in T2DM mice. Especially, the left ventricle global peak circumferential strain value is superior to the EF value in reflecting the early changes in DCM.

Background: Enteritis is one of the most frequently reported symptoms in piglets infected with porcine circovirus type 2 (PCV2), but the immunopathogenesis has not been reported. Objectives: This study examined the effect of a PCV2 infection on the intestinal mucosal immune function through morphological observations and immune-related molecular detection. Methods: Morphological changes within the ileum of piglets during a PCV2 infection were observed. The expression of the related-molecules was analyzed using a gene chip. The immunocyte subsets were analyzed by flow cytometry. The secretory immunoglobulin A (SIgA) content was analyzed by enzyme-linked immunosorbent assay. Results: The PCV2 infection caused ileal villus damage, intestinal epithelial cells exfoliation, and an increase in lymphocytes in the lamina propria at 21 days post-infection.Differentially expressed genes occurred in the defense response, inflammatory response, and the complement and coagulation cascade reactions. Most of them were downregulated significantly at the induction site and upregulated at the effector site. The genes associated with SIgA production were downregulated significantly at the induction site. In contrast, the expression of the Toll-like receptor-related genes was upregulated significantly at the effector site. The frequencies of dendritic cells, B cells, and CD8 + T cells were upregulated at the 2 sites. The SIgA content decreased significantly in the ileal mucosa. Conclusions PCV2 infections can cause damage to the ileum that is associated with changes in immune-related gene expression, immune-related cell subsets, and SIgA production.These findings elucidated the molecular changes in the ileum after a PCV2 infection from the perspective of intestinal mucosal immunity, which provides insights into a further study for PCV2-induced enteritis.

Objective To investigate the value of phase-contrast magnetic resonance imaging (PC-MRI)in evaluating pulmonary artery blood flow and right ventricular(RV)function in elderly patients with chronic obstructive pulmonary disease (COPD).Methods Twenty-four elderly patients with COPD admitted to Beijing Anzhen Hospital between February 2016 and June 2017 were enrolled in this retrospective study.Based on pulmonary artery pressure evaluated by right heart catheterization,COPD patients were divided into a pulmonary hypertension group (PH group,n =12)and a non-PH group(n=12).Pulmonary artery blood flow velocity and volume,pulmonary arterial compliance and right heart function parameters including ejection fraction,end diastolic volume,endsystolic volume and cardiac output,and 6-min walking distance evaluated by PC-MRI were compared between the two groups.Results There were significant differences in peak flow velocity[(88.1 ±16.0)cm/s vs.(59.8± 13.8) cm/s,P =0.005],flow volume [(80.7± 22.0) ml/s vs.(53.2± 26.7)ml/s,P=0.012],main pulmonary artery compliance[(42.3± 14.6)vs.(22.7± 10.8),P =0.001],right ventricular ejection fraction [(48.4 ± 13.4) ％ vs.(37.6 ± 11.1) ％,P =0.000],end-diastolic volume[(64.6±22.5)ml vs.(72.5±22.8)ml,P =0.030]and end-systolic volume[(50.6±33.1)ml vs.(41.7±33.1)ml,P =0.040]between the non-PH and PH groups,while there was no significant difference in cardiac output between the non-PH and PH groups[(34.2±10.8)ml vs.(34.4±8.3)ml,P =0.080].Pearson's correlation analysis showed that 6-minute walking distance had good correlations with right ventricular ejection fraction(r =0.49),forced expiratory volume in 1 s(FEV1)(r=0.60)and main pulmonary artery diameter(r=0.61).Conclusions PC-MRI is a noninvasive imaging method for quantitative analysis of pulmonary artery dynamics and right heart function for the elderly with COPD,and it can provide information for evaluating therapeutic effects and prognosis.

Objective To observe the value of optimized temporal parallel acquisition technique (TPAT) sequence in evaluating cardiac structure and function in arrhythmia patients.Methods Totally 33 arrhythmia patients (arrhythmia group) and 48 normal rhythm subjects (normal group) underwent cardiac MRI with conventional cine (balanced steadystate free-precession [bSSFP]) sequence and optimized TPAT sequence.Myocardial thickness,cardiac function,myocardial strain parameters of left ventricle and image quality of 2 sequences were compared in the two groups,respectively.Results In arrhythmia group,there was statistical difference of myocardial thickness in 12 myocardial segments between the 2 sequences (all P ＜ 0.05),as well as peak and average values of myocardial radial and circumferential strain (all P＜0.05).In normal group,there was no statistical difference of myocardial thickness and stain parameters between the 2 sequences (all P＞0.05).Additionally,no statistical difference of cardiac function was found between the 2 sequences in two groups (all P＞0.05).In arrhythmia group,the image quality of optimized TPAT sequence was better than that of bSSFP sequence (P＜0.05).Conclusion For arrhythmia patients,optimized TPAT cine sequence could improve image quality of cardiac MRI.

Objective To analyze the impact of dual energy monochromatic reconstructions (50-160 keV) on coronary calcified plaque stenosis quantification in a cardiac phantom with the real stenosis as standard of reference.Methods Signal-to-noise(SNR)and contrast-to-noise ratios(CNR)were calculated. In conventional 120 kV coronary CT angiography (CCTA) images as well as 12 monochromatic series(50-160 keV),luminal narrowing of calcified plaque was measured and compared to the real stenosis. Bland-Altman plots were performed to analyze the correlation of reference standard with conventional 120 kV and 12 monochromatic series (50-160 keV). The sensitivity, specificity, and accuracy of CCTA for detection of≥50% stenosis were calculated and receiver operating characteristic(ROC)curve was analyzed with the real stenosis as reference standard.Results The SNR and CNR were decreased with the increase of keV from 70 keV.The SNR and CNR of monochromatic imaging were lower compared with conventional 120 kV images(SNR:76.4±16.1;CNR:274.7±54.1)(P<0.05).The Bland-Altman plots presented a smaller measurement bias towards 90-160 keV than conventional 120 kV, and smallest measurement bias was revealed in 100-130 keV imaging(100-130 keV:bias 17.2% vs 120 kV:21.4%).Using the stenosis≥50% as cut-off value, the specificity were higher in the monochromatic series (70-160 keV) than conventional 120 kV CCTA.The specificity(75.0%)were the highest in the 120 keV and 130 keV images.The area under the ROC curve(AUC)of 90-130 keV(0.991-0.995)was higher than that in conventional 120 kV imaging (0.990). Conclusions The use of monochromatic imaging improves the overall accuracy of stenosis evaluation in coronary calcified plaques. Reconstructions at 100-130 keV for calcified lesions yielded the optimal results.

Objective To compare the diagnostic accuracy of clinically significant prostate cancer by general radiologist and uroradiology specialist based on the Prostate Imaging Reporting and Data System (PI-RADS).Methods A total of 45 men from Beijing United Family Hospital and Clinics undergoing prostate mpMRI examination and subsequent MRI-targeted biopsy were included in the study.The age of patients was (60.0 ± 8.0) years,the median PSA level was 7.2 ng/ml (1.2-95.8 ng/ml) and the median prostate volume was 45.0 ml (18.3-127.0 ml).The general radiologists from Beijing United Family Hospital and Clinics made the diagnosis according to PI-RADS 2.0.One uroradiology specialist from Beijing Anzhen hospital reviewed all the mpMRIs retrospectively and marked new PI-RADS score based on PI-RADS 2.0.The PI-RADS ≥ 3 lesion was recognized as suspicious of clinically significant prostate cancer.The distribution of PI-RADS score from different doctors and the diagnostic accuracy of clinically significant prostate cancer was compared.Results All the 45 patients underwent MRI-targeted cognitive biopsy and 14 cases of prostate cancer were detected,including 9 cases of clinically significant prostate cancer.There was no significant difference in the distribution of PI-RADS by general radiologist and uroradiology specialist (P =0.064).82.8％ (37/45) and 37.8％ (17/45) patients were diagnosed with PI-RADS ≥ 3 by general radiologist and uroradiology specialist respectively.The interobserver agreement was only 17.8％ (8/45).The positive predictive value of PI-RADS≥3 was 35.1％ (13/37) and 76.5％ (13/17) for prostate cancer by general radiologist and uroradiology specialist respectively,and for clinically significant prostate cancer,the positive predictive value of PI-RADS ≥ 3 was 21.6％ (8/37) and 52.9％ (9/17) respectively.Conclusions Uroradiology specialist achieved significantly superior in predictive value of PI-RADS for clinically significant prostate cancer compared with general radiologist.In the experienced centers,MRI-targeted biopsy could be performed only on high PI-RADS score lesions,thus to reduce unnecessary biopsies and to avoid over diagnosis and over treatment of prostate cancer.

PurposeTo determine the feasibility and accuracy of dual source CT (DSCT) in assessing myocardial delayed-enhancement and left ventricular wall thickness of hypertrophic cardiomyopathy (HCM) in comparison with cardiac magnetic resonance (CMR).Materials and MethodsEighty patients with HCM confirmed by clinical diagnosis were enrolled in the study. DSCT images and CMR images were acquired at the arterial and lag phases. According to 17-segment model provided by American Heart Association, the left ventricular wall thickness and location of delayed-enhancement were verified, and the correlation of these two methods were analyzed in terms of the diagnosis of myocardial delayed enhancement (MDE).Results1360 myocardial segments for 80 patients were assessed. The left ventricular wall thickness determined by DSCT was significantly correlated with MR results (r=0.88,P<0.01). DSCT and MDE showed substantial agreement on per-patient (n=74,Kappa=0.751,P<0.05) and per-segment (n=1238, Kappa=0.746,P<0.01) levels. For dense myocardial delayed enhancement, CT findings were significantly correlated with those of CMR (r=0.89, P<0.01), but CT scan slightly underestimated the lesion scope of fibrosis. Bland-Altman analysis showed that CT and MRI were different in measuring the lesion volume of myocardial delayed enhancement (mean standard deviation was 2.71%).ConclusionThe cardiac CT examination provides comprehensive information in coronary artery and myocardial assessment, and MDE-DSCT is also effective in the diagnosis of myocardial fibrosis in HCM since it can be used in assessing myocardial fibrosis.