The seed kernel of Momordica cochinchinensis, i.e., Momordicae Semen, is used for medicinal purposes, but to date, no research has been reported on its chemical constituents. In this study, the chemical constituents of Momordicae Semen were investigated for the first time using silica gel column chromatography, semi-preparative HPLC, HR-MS, and NMR. As a result, eight compounds were isolated and identified as: p-hydroxybenzoic acid-7-O-trehaloside(mubeside A, 1), 2,6-dimethoxyphenol-O-β-D-apiosyl-(1→2)-β-D-glucoside(mubeside B, 2), 1-O-p-methoxybenzoyl-1,4-benzenediol-4-O-β-D-apiosyl-(1→2)-β-D-glucoside(mubeside C, 3), 1-O-p-hydroxybenzoyl-1,4-benzenediol-4-O-β-D-apiosyl-(1→2)-β-D-glucoside(mubeside D, 4), gypsogenin-3-O-β-D-galactosyl-(1→2)-β-D-glucuronoside(5), quillaic acid-3-O-β-D-galactosyl-(1→2)-β-D-glucuronoside(6), violanthin(7), and kaempferitrin(8). Compounds 1-4 are new compounds, while compounds 5-8 were isolated from Momordicae Semen for the first time.
Glycosides/isolation & purification* ; Drugs, Chinese Herbal/isolation & purification* ; Molecular Structure ; Magnetic Resonance Spectroscopy ; Chromatography, High Pressure Liquid

Objective To investigate the disease spectrum and pathogenic genes of inherited metabolic disorder(IMD)among neonates in Gansu Province of China.Methods A retrospective analysis was conducted on the tandem mass spectrometry data of 286 682 neonates who received IMD screening in Gansu Provincial Maternal and Child Health Hospital from January 2018 to December 2021.A genetic analysis was conducted on the neonates with positive results in tandem mass spectrometry during primary screening and reexamination.Results A total of 23 types of IMD caused by 28 pathogenic genes were found in the 286 682 neonates,and the overall prevalence rate of IMD was 0.63‰(1/1 593),among which phenylketonuria showed the highest prevalence rate of 0.32‰(1/3 083),followed by methylmalonic acidemia(0.11‰,1/8 959)and tetrahydrobiopterin deficiency(0.06‰,1/15 927).In this study,166 variants were identified in the 28 pathogenic genes,with 13 novel variants found in 9 genes.According to American College of Medical Genetics and Genomics guidelines,5 novel variants were classified as pathogenic variants,7 were classified as likely pathogenic variants,and 1 was classified as the variant of uncertain significance.Conclusions This study enriches the database of pathogenic gene variants for IMD and provides basic data for establishing an accurate screening and diagnosis system for IMD in this region.

OBJECTIVES: To systematically evaluate the value of the platelet-to-lymphocyte ratio (PLR) in predicting coronary artery lesions (CAL) in Chinese children with Kawasaki Disease (KD). METHODS: A comprehensive search was conducted in databases including PubMed, Embase, Cochrane Library, Web of Science, China National Knowledge Infrastructure, Wanfang Data, China Biomedical Literature Database, and China Science and Technology Journal Database from inception to December 2022. The quality of the included literature was assessed using the Newcastle-Ottawa Scale, and a Meta analysis was performed using Stata 15.1. RESULTS: A total of ten published reports, involving 3 664 Chinese children with KD, were included in this Meta analysis, of whom 1 328 developed CAL. The Meta analysis revealed a sensitivity of 0.78 (95%CI: 0.71-0.83), specificity of 0.71 (95%CI: 0.61-0.80), overall diagnostic odds ratio of 8.69 (95%CI: 5.02-15.06), and an area under the curve of the summary receiver operating characteristic of 0.82 (95%CI: 0.78-0.85) for PLR in predicting CAL in the children with KD. The sensitivity, specificity, and area under the curve of summary receiver operating characteristic were lower for PLR alone compared to PLR in combination with other indicators. Sensitivity analysis demonstrated the stability of the Meta analysis results with no significant changes upon excluding individual studies. However, a significant publication bias was observed (P<0.001). CONCLUSIONS PLR demonstrates certain predictive value for CAL in Chinese children with KD.
Child ; Humans ; Mucocutaneous Lymph Node Syndrome/pathology* ; Coronary Vessels/pathology* ; Lymphocytes ; Biomarkers ; China ; Coronary Artery Disease/pathology*

The pharmaceutical manufacturing model is gradually changing from intermittent manufacturing to continuous manufacturing and intelligent manufacturing. This paper briefly reviewed the supervision and research progress in continuous pharmaceutical manufacturing in China and abroad and described the definition and advantages of continuous pharmaceutical manufacturing. The continuous manufacturing of traditional Chinese medicine(TCM) at the current stage was summarized in the following three terms: the enhancement of the continuity of intermittent manufacturing operations, the integration of continuous equipment to improve physical continuity between units, and the application of advanced process control strategies to improve process continuity. To achieve continuous manufacturing of TCM, the corresponding key technologies, such as material property characterization, process modeling and simulation, process analysis technology, and system integration, were analyzed from the process and equipment, respectively. It was proposed that the continuous manufacturing equipment system should have the characteristics of high speed, high response, and high reliability, "three high(H~3)" for short. Considering the characteristics and current situation of TCM manufacturing, based on the two dimensions of product quality control and production efficiency, a maturity assessment model for continuous manufacturing of TCM, consisting of operation continuity, equipment continuity, process continuity, and quality control continuity, was proposed to provide references for the application of continuous manufacturing technology for TCM. The implementation of continuous manufacturing or the application of key continuous manufacturing technologies in TCM can help to systematically integrate advanced pharmaceutical technology elements and promote the uniformity of TCM quality and the improvement of production efficiency.
Medicine, Chinese Traditional ; Reproducibility of Results ; China ; Quality Control ; Pharmaceutical Preparations

Objective: To examine the association of body mass index (BMI) and waist circumference (WC) with frailty among oldest-old adults in China. Methods: A total of 7 987 people aged 80 years and older (oldest-old) who participated in the Chinese Longitudinal Healthy Longevity Survey (CLHLS) in 2017-2018 were included. Information on demographic characteristics, behavior pattern, diet, activities of daily living, cognitive function, health status, disease condition were collected by questionnaire and physical examination. Generalized linear mixed model and restricted cubic splines (RCS) were used to analyze the association of BMI and WC with frailty. Results: The mean age of all participants was 91.7 years, and their mean BMI and WC were (21.3±3.5) kg/m² and (82.9±10.5) cm, respectively. The proportion of male was 42.3% (3 377/7 987), and the proportion of people with frailty was 33.7% (2 664/7 987). After controlling confounding factors, compared with T₂ (19.1-22.1 kg/m²) of BMI, the OR (95%CI) of the female T₁ (<19.1 kg/m²) and T₃ (≥22.2 kg/m²) group was 1.39 (1.17-1.65) and 1.27 (1.07-1.52), respectively. Compared with T₂ (77-85 cm) of WC, the OR (95%CI) of female T₁ (<77 cm) and T₃ (≥86 cm) group was 1.20 (1.01-1.42) and 1.10 (0.93-1.31), respectively. The results of multiple linear regression model with restrictive cubic spline showed that there was a non-linear association of BMI and WC with frailty in female. Conclusion: There is a U-shaped association of BMI and WC with frailty in female participants.
Adult ; Male ; Female ; Humans ; Aged, 80 and over ; Waist Circumference ; Body Mass Index ; Frailty/epidemiology* ; Activities of Daily Living ; China/epidemiology* ; Risk Factors

Objective: To investigate the clinicopathological features, clinical manifestations and different diagnosis of patients with complicated lymphatic anomaly. Methods: The clinical and pathologic data of four patients with complicated lymphatic anomaly diagnosed and treated in Peking Union Medical College Hospital from January 2000 to December 2021 were collected and analyzed. Results: One Gorham-Stout disease case and three generalized lymphatic anomaly cases were included in this cohort. Patients' ages ranged from 7 to 32 years. There were three males and one female. The positions of biopsy included three bone biopsy and one bronchus biopsy. Microscopically, all cases showed diffuse enlarged lymphatic channels. At the same time, osteogenesis was obvious in Gorham-Stout disease case. Radiologically, cortical loss was seen in Gorham-Stout disease, and lytic bone confined to the medullary cavity presented in generalized lymphatic anomaly. The three generalized lymphatic anomaly cases also had coagulopathy, and two had effusion. Conclusions: The histologic feature of complicated lymphatic anomaly was diffuse lymphatic malformation, and the diagnosis depends on clinical and pathologic information. The treatment and prognosis of these diseases are different, and therefore it is necessary to understand their clinical and pathologic features and make the correct diagnosis.
Male ; Humans ; Female ; Child ; Adolescent ; Young Adult ; Adult ; Osteolysis, Essential/pathology* ; Lymphatic Abnormalities/surgery* ; Bone and Bones/pathology* ; Diagnosis, Differential ; Prognosis

Objective: To analyze the clinical characteristics of children with Burkitt lymphoma (BL) and to summarize the mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 (CNCL-B-NHL-2017) regimen. Methods: Clinical features of 436 BL patients who were ≤18 years old and treated with the CNCL-B-NHL-2017 regimen from May 2017 to April 2021 were analyzed retrospectively. Clinical characteristics of patients at disease onset were analyzed and the therapeutic effects of patients with different clinical stages and risk groups were compared. Survival analysis was performed by Kaplan-Meier method, and Cox regression was used to identify the prognostic factors. Results: Among 436 patients, there were 368 (84.4%) males and 68 (15.6%) females, the age of disease onset was 6.0 (4.0, 9.0) years old. According to the St. Jude staging system, there were 4 patients (0.9%) with stage Ⅰ, 30 patients (6.9%) with stage Ⅱ, 217 patients (49.8%) with stage Ⅲ, and 185 patients (42.4%) with stage Ⅳ. All patients were stratified into following risk groups: group A (n=1, 0.2%), group B1 (n=46, 10.6%), group B2 (n=19, 4.4%), group C1 (n=285, 65.4%), group C2 (n=85, 19.5%). Sixty-three patients (14.4%) were treated with chemotherapy only and 373 patients (85.6%) were treated with chemotherapy combined with rituximab. Twenty-one patients (4.8%) suffered from progressive disease, 3 patients (0.7%) relapsed, and 13 patients (3.0%) died of treatment-related complications. The follow-up time of all patients was 24.0 (13.0, 35.0) months, the 2-year event free survival (EFS) rate of all patients was (90.9±1.4) %. The 2-year EFS rates of group A, B1, B2, C1 and C2 were 100.0%, 100.0%, (94.7±5.1) %, (90.7±1.7) % and (85.9±4.0) %, respectively. The 2-year EFS rates was higher in group A, B1, and B2 than those in group C1 (χ²=4.16, P=0.041) and group C2 (χ²=7.21, P=0.007). The 2-year EFS rates of the patients treated with chemotherapy alone and those treated with chemotherapy combined with rituximab were (79.3±5.1)% and (92.9±1.4)% (χ²=14.23, P<0.001) respectively. Multivariate analysis showed that stage Ⅳ (including leukemia stage), serum lactate dehydrogenase (LDH)>4-fold normal value, and with residual tumor in the mid-term evaluation were risk factors for poor prognosis (HR=1.38,1.23,8.52,95%CI 1.05-1.82,1.05-1.43,3.96-18.30). Conclusions: The CNCL-B-NHL-2017 regimen show significant effect in the treatment of pediatric BL. The combination of rituximab improve the efficacy further.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Burkitt Lymphoma/drug therapy* ; Child ; Disease-Free Survival ; Female ; Humans ; Lactate Dehydrogenases ; Lymphoma, B-Cell/drug therapy* ; Male ; Prognosis ; Retrospective Studies ; Rituximab/therapeutic use* ; Treatment Outcome

Objective:To observe and analyze the macular choroidal thickness and choroidal blood perfusion (CBP) in eyes with idiopathic macular hole (IMH) and their correlation.Methods:A cross-sectional observational clinical study. From March 2019 to October 2021, 60 IMH patients with 60 eyes (IMH group) and 60 healthy volunteers with 60 eyes (control group) who consecutively visited Department of Ophthalmology of The First Affiliated Hospital of Zhengzhou University were included in the study. Among the 60 eyes in the IMH group, 8, 8, 15, and 29 eyes were at stage Ⅰ, Ⅱ, Ⅲ, and Ⅳ, respectively. There was no significant difference in age, spherical equivalent power and axial length between the two groups ( t=1.327, 0.157, 0.542; P>0.05). The average macular choriodal thickness (AMCT) and CBP in different regions of the macular region of the examined eye were measured using a swept-frequency light source optical coherence tomography scanner. According to the zoning method for the treatment of diabetic retinopathy, the choroid within 6 mm of the fovea was divided into 3 concentric circles with the fovea as the center. They are the central area with a diameter of 1 mm, the inner ring area of 1-3 mm, and the outer ring area of 3-6 mm; the inner ring area and the outer ring area were divided into 4 areas by 2 radiations respectively, including the upper part of the inner superior (IS), the lower part of the inner inferior (Ⅱ ), and the nasal side of the inner nasal (IN), inner temporal (IT), outer superior (OS), outer inferior (OI), outer nasal (ON), outer temporal (OT), a total of 9 regions. The distribution characteristics of AMCT and CBP in different regions were observed. The correlation between AMCT and CBP was analyzed by Pearson correlation; the correlation between AMCT, CBP and IMH stage was analyzed by Spearman correlation. Results:Compared with the eyes of the control group, the AMCT of the affected eyes in the IMH group was significantly thinner in all areas of the macula, and the difference was statistically significant ( t=2.378, 4.641, 2.888, 3.390, 3.575, 4.870, 4.077, 4.946, 4.578; P<0.05). Compared with the control group, the CBP in the OS and OT regions of the affected eyes in the IMH group was significantly lower, the difference was statistically significant ( t=3.424, 4.516; P<0.05). The results of Pearson correlation analysis showed that there was a significant positive correlation between AMCT and CBP in the OT region ( r=0.314, P<0.001). Spearman correlation analysis showed that there was a significant positive correlation between AMCT and IMH staging in each region ( r=0.375, 0.374, 0.289, 0.379, 0.441, 0.392, 0.303, 0.341, 0.292; P<0.05). There was no significant correlation between CBP and IMH staging in IN, OI and OT regions ( r=-0.138, -0.016, -0.221; P>0.05); CBP and IMH staging in other regions were significantly negatively correlated ( r=-0.560, -0.390,-0.819, -0.692, -0.329, -0.587; P<0.05). Conclusions:The choroidal thickness in the macular region of the eyes with IMH is significantly thinner than that of the normal subjects; there is choroidal hypoperfusion in local areas. There is a significant positive correlation between local regional AMCT and CBP; IMH stage is higher, the trend of AMCT in each region is thickening, and the CBP in most regions decrease.

The present study aimed to determine whether the number of patients with symptomatic benign prostatic hyperplasia (BPH) who preferred surgery decreased during the past 11 years at our center (West China Hospital, Chengdu, China), and whether this change affected the timing of surgery and the physical condition of surgical patients. This retrospective study included 57 557 patients with BPH treated from January 2008 to December 2018. Of these, 5427 patients were treated surgically. Surgical patients were divided into two groups based on the time of treatment (groups 8-13 and groups 13-18). The collected data comprised the percentage of all patients with BPH who underwent surgery, baseline characteristics of surgical patients, rehabilitation time, adverse events, and hospitalization costs. The surgery rates in groups 8-13 and groups 13-18 were 10.5% and 8.5% (P < 0.001), respectively. The two groups did not clinically differ regarding patient age and prostate volume. The rates of acute urinary retention and renal failure decreased from 15.0% to 10.6% (P < 0.001) and from 5.2% to 3.1% (P < 0.001), respectively. In groups 8-13 and groups 13-18, the mean catheterization times were 4.0 ± 1.7 days and 3.3 ± 1.6 days (P < 0.001), respectively, and the mean postoperative hospitalization times were 5.1 ± 2.4 days and 4.2 ± 1.8 days (P < 0.001), respectively. The incidences of unplanned second surgery and death reduced during the study period. The surgery rate decreased over time, which suggests that medication was chosen over surgery. However, the percentage of late complications of BPH also decreased over time, which indicates that the timing of surgery was not delayed.