Effects of sarcopenia on the occurrence of overt hepatic encephalopathy in patients with liver cirrhosis / 中华消化杂志

Objective:To investigate the risk factors of overt hepatic encephalopathy (OHE) in patients with liver cirrhosis, and to clarify the effect of sarcopenia on OHE.Methods:Based on the liver cirrhosis cohort established by our research group, from January 1, 2013 to December 31, 2017, 480 patients diagnosed with liver cirrhosis and underwent upper abdominal computed tomography were selected from 3 centers, including the Second Affiliated Hospital of Naval Medical University (Shanghai Changzheng Hospital), Shanghai East Hospital Affiliated to Tongji University, and Shandong Provincial Hospital. The L3 skeletal muscle index (L3-SMI) <44.77 cm 2/m 2 for males and L3-SMI <32.50 cm 2/m 2 for females were used as the diagnostic criterion for sarcopenia. The clinical data of all the patients were collected, including baseline medical history, age, serum total bilirubin, serum levels of aspartate aminotransferase (AST), γ-glutamyl transpeptidase (GGT), albumin, sodium, prothrombin time (PT), international normalized ratio (INR), hemoglobin, platelet count, etc, as well as Child-Pugh classification of liver function, and model for end-stage liver disease (MELD) score. Independent sample t test, rank sum test, and chi-square test were used for statistical analysis. Binary logistic regression analysis was used to analyze the independent risk factors for OHE in patients with liver cirrhosis, and Kaplan-Meier method was used to analyze the effect of sarcopenia on the incidence of OHE in patients with liver cirrhosis. Results:After 2 years of follow-up, the incidence of OHE was 16.2% (78/480). The age, serum total bilirubin level, AST, GGT, PT, INR, Child-Pugh score, and MELD score of OHE patients were all higher than those of non-OHE patients ((59.67±10.30) years old vs. (53.41±12.06) years old, 35.25 μmol/L(20.10 μmol/L, 60.53 μmol/L) vs. 22.70 μmol/L(15.10 μmol/L, 35.20 μmol/L), 40.00 U/L(27.25 U/L, 61.00 U/L) vs. 33.00 U/L(24.75 U/L, 47.00 U/L), 52.50 U/L(26.25 U/L, 86.75 U/L) vs. 34.50 U/L(22.00 U/L, 73.00 U/L), (17.71±3.52) s vs. (15.50±2.98) s, 1.50±0.34 vs. 1.31±0.29, 8.95±2.19 vs.7.20±1.94, 13.56±4.42 vs.11.42±3.92), while serum albumin, serum sodium and platelet count of OHE patients were all lower than those of non-OHE patients ((29.72±5.55) g/L vs. (33.19±5.89) g/L, 139.00 mmol/L(136.00 mmol/L, 142.00 mmol/L)vs.140.00 mmol/L (138.00 mmol/L, 142.00 mmol/L), 60.00×10 9/L(43.75×10 9/L, 90.25×10 9/L) vs. 80.00×10 9/L(56.00×10 9/L, 131.00×10 9/L)), and the differences were statistically significant ( t=-4.77; Z=-4.10, -3.13, -2.24; t=-5.19, -4.71, -6.57, -3.98, 4.99; and Z=2.44 and 3.48; all P<0.05). The proportions of ascites, hepatic encephalopathy, hepatorenal syndrome, spontaneous bacterial peritonitis at baseline, and the incidence of sarcopenia in OHE patients were all higher than those in non-OHE patients (82.1%, 64/78 vs. 63.7%, 256/402; 41.0%, 32/78 vs. 3.5%, 14/402; 5.1%, 4/78 vs. 1.0%, 4/402; 14.1%, 11/78 vs. 2.5%, 10/402; 37.2%, 29/78 vs. 19.7%, 79/402), and the L3-SMI of OHE patients was lower than that of non-OHE patients ((43.14±8.97) cm 2/m 2 vs. (46.29±8.49) cm 2/m 2), and the differences were statistically significant ( χ2=9.11, 101.97, 4.52, 18.38, 10.53; t=2.86; all P<0.05). The results of binary logistic regression analysis indicated that platelet count ( OR=0.995, 95% confidence interval (95% CI) 0.991 to 1.000, P=0.038), L3-SMI ( OR=0.959, 95% CI 0.922 to 0.997, P=0.035) and hepatic encephalopathy ( OR=14.724, 95% CI 6.741 to 32.161, P<0.001) were independent influencing factors for OHE in patients with liver cirrhosis. The incidence of OHE in patients with sarcopenia was higher than that of patients without sarcopenia (26.9%, 29/108 vs. 13.2%, 49/372), and the difference was statistically significant ( χ2=10.53, P=0.001). The results of Kaplan-Meier analysis demonstrated that patients with sarcopenia were more likely to develop OHE ( P<0.001). Conclusion:Sarcopenia is closely correlated to OHE and is an independent predictor of OHE in patients with liver cirrhosis.

Erratum: Author correction to "TMEM16A inhibits angiotensin II-induced basilar artery smooth muscle cell migration in a WNK1-dependent manner" Acta Pharm Sin B 11(12) (2021) 3994-4007

[This corrects the article DOI: 10.1016/j.apsb.2021.04.013.].

Establishment of a nomogram for survival rate after liver resection for primary small hepatocellular carcinoma based on SEER data and external validation / 临床肝胆病杂志

Objective To establish a nomogram for overall survival rate after liver resection for primary small hepatocellular carcinoma based on SEER data and external validation of Chinese data. Methods The data of 1809 patients, registered in National Cancer Institute SEER database in 2004-2015, who underwent hepatectomy for primary small hepatocellular carcinoma were extracted as modeling group, and 158 patients with small hepatocellular carcinoma who underwent hepatectomy in Affiliated Hospital of North Sichuan Medical College from 2010 to 2017 were collected as validation group. The univariate Cox risk regression analysis, lasso regression analysis, and multivariate Cox hazard regression analysis were used to investigate the influencing factors for OS after hepatectomy in patients with small hepatocellular carcinoma. A nomogram was established based on the independent influencing factors for OS, and index of concordance (C-index), calibration curves, and receiver operating characteristic (ROC) curve were used to analyze the predictive ability of the nomogram. The Kaplan-Meier survival analysis and the log-rank test were used to investigate the difference in survival between the high- and low-risk groups. Results The multivariate Cox hazard regression analysis showed that sex (hazard ratio [ HR ]=1.22, 95% confidence interval [ CI ]: 1.05-1.41, P =0.010), Seer stage ( HR =1.51, 95% CI : 1.23-1.85, P < 0.001; HR =10.31, 95% CI : 2.53-42.04, P =0.001), tumor diameter ( HR =1.22, 95% CI : 1.06-1.39, P =0.004), vascular invasion or metastasis ( HR =1.43, 95% CI : 1.24-1.65, P < 0.001), and alpha-fetoprotein ( HR =1.33, 95% CI : 1.16-1.54, P < 0.001) were independent risk factors for OS after hepatectomy for small hepatocellular carcinoma. The modeling group had a C-index of 0.621, and its area under the ROC curve at 1, 2, and 3 years was 0.666(95% CI 0.628-0.704), 0.678(95% CI 0.647-0.708), and 0.663(95% CI : 0.635-0.690), respectively; the validation group had a C-index of 0.718, and its area under the ROC curve at 1, 2, and 3 years was 0.695(95% CI : 0.593-0.797), 0.781(95% CI : 0.706-0.856), and 0.759(95% CI 0.669-0.848), respectively. Risk stratification was performed based on the nomogram, and the Kaplan-Meier survival analysis showed that for both the modeling group and the validation group, the low-risk group had a significantly better prognosis than the high-risk group ( P < 0.01). Conclusion The model established for survival rate after liver resection for primary small hepatocellular carcinoma can predict the 1-, 2-, and 3-year OS rates and can thus be used in clinical practice in China.

Difficult and complicated oral ulceration: an expert consensus guideline for diagnosis / 国际口腔科学杂志·英文版

The complexity of oral ulcerations poses considerable diagnostic and therapeutic challenges to oral specialists. The expert consensus was conducted to summarize the diagnostic work-up for difficult and complicated oral ulcers, based on factors such as detailed clinical medical history inquiry, histopathological examination, and ulceration-related systemic diseases screening. Not only it can provide a standardized procedure of oral ulceration, but also it can improve the diagnostic efficiency, in order to avoid misdiagnosis and missed diagnosis.

Current Status and Research Trends of Panax Between 1900-2019: A Bibliometric Analysis / 中国结合医学杂志

OBJECTIVE@#To investigate the current status and further development of Panax genus and 6 important individual species including P. notoginseng, P. quinquefolium, P. vietnamensis, P. japonicus, P. stipuleanatus and P. zingiberensis.@*METHODS@#The bibliometric analysis was based on the Web of Science core database platform from Thomson Reuters. Totally, 7,574 records of scientific research of Panax species published from 1900-2019 were analyzed. The statistical and visualization analysis was performed by CiteSpace and HistCite software.@*RESULTS@#The academic research of Panax species increase promptly. Plant science is the main research field while research and experimental medicine and agricultural engineering will be the further development tendency. Particularly, the discrimination research of P. notoginseng will be the research tendency among Panax species, especially diversity research. In addition, P. vietnamensis deserves more attention in the genus Panax.@*CONCLUSION@#This research provides a reference for further research of the genus and individual species.

Effect of DC vaccine sensitized with CpG ODN combined with tumor antigen on murine melanoma / 药学学报

The potential application of dendritic cells (DC) sensitized with cytosine-phosphoric acid-guanine (CpG) oligodeoxynucleotide (ODN) and tumor antigen as a vaccine against murine melanoma was investigated with freshly isolated mouse bone marrow-derived dendritic cells. For the DC vaccine preparation, DC were sensitized with the B16 tumor antigen and CpG ODN was used to promote further maturation of the DC. The immunogenic activity of the vaccine was evaluated in vitro by determining the proliferation of T lymphocytes and the killing effect of cytotoxic T lymphocytes (CTL) on B16 tumor cells. The DC vaccine was injected intraperitoneally and tumor inhibition in mice bearing B16 xenografts was examined. All mice were cared for under an approved SIMM Institutional Animal Care and Use Committee (IACUC) protocol. In vitro, this DC vaccine promoted the proliferation of T lymphocytes and showed a potent killing effect on the target B16 cells. In vivo experiments showed that after treatment or pre-immunization both the tumor volume and weight were significantly decreased. The DC vaccine with CpG ODN and tumor antigen exhibited an inhibitory effect against melanoma, providing a potential method for melanoma cancer treatment.

Investigation and analysis of children's choice of environment in oral clinics / 华西口腔医学杂志

OBJECTIVES@#To investigate and analyze children,s choice of environment in oral clinics so as to provide evidence for improving the oral clinic environment.@*METHODS@#A total of 110 children in the Pediatric Dentistry of Qingdao Stomatological Hospital Affiliated to Qingdao University were selected as the research subjects from September to December 2019 to conduct a questionnaire survey, and the results were statistically analyzed by using a Chi-square test.@*RESULTS@#A total of 107 effective questionnaires were collected. The children surveyed liked the room with pink and blue walls (61.68%), cartoon murals (57.94%), and arranged toys (61.68%). Most of them did not dislike the special smell in the room (62.62%) and liked the smell of orange essential oil (52.34%). During treatment, the children liked watching cartoons the best (61.68%), expected doctors to be dressed in white (51.40%), and expected doctors to be treated by female doctors (68.22%). Most children wanted to be accompanied by family members during treatment (62.62%). The Chi-square test showed statistically significant differences in seven questions between preschool and school-age children: the environment of the clinic room, the color of the walls, the clinic decoration, the entertainment during treatment, color of the doctors' dress and gender, and company of the family members during treatment (@*CONCLUSIONS@#Children have their own preferences for the environment in the oral clinic. For preschool children, we need to create a soothing and warm atmosphere with warm colors liked by children. For school-age children, a calm and relaxed clinic atmosphere with cold colors should be created.

TMEM16A inhibits angiotensin II-induced basilar artery smooth muscle cell migration in a WNK1-dependent manner

Vascular smooth muscle cell (VSMC) migration plays a critical role in the pathogenesis of many cardiovascular diseases. We recently showed that TMEM16A is involved in hypertension-induced cerebrovascular remodeling. However, it is unclear whether this effect is related to the regulation of VSMC migration. Here, we investigated whether and how TMEM16A contributes to migration in basilar artery smooth muscle cells (BASMCs). We observed that AngII increased the migration of cultured BASMCs, which was markedly inhibited by overexpression of TMEM16A. TMEM16A overexpression inhibited AngII-induced RhoA/ROCK2 activation, and myosin light chain phosphatase (MLCP) and myosin light chain (MLC20) phosphorylation. But AngII-induced myosin light chain kinase (MLCK) activation was not affected by TMEM16A. Furthermore, a suppressed activation of integrin

Pathogenic role of NDUFA13 inactivation in spontaneous hepatitis in mice and the mechanism / 南方医科大学学报

OBJECTIVE@#To investigate the role of NDUFA13 inactivation in the pathogenesis of spontaneous hepatitis in mice and explore the possible mechanisms.@*METHODS@#Hepatocyte-specific NDUFA13 knockout (NDUFA13@*RESULTS@#Liver-specific NDUFA13 heterozygous knockout mice were successfully constructed as verified by PCR results. HE staining revealed severe liver damage in both 4- week-old and 2-year-old NDUFA13@*CONCLUSIONS@#Hepatocytes-specific NDUFA13 ablation can trigger spontaneous hepatitis in mice possibly mediated by the activation of ROS/NF-κB/NLRP3 signaling.

Research progress on Melkersson-Rosenthal syndrome / 浙江大学学报·医学版

Melkersson-Rosenthal syndrome (MRS) is a rare neuro-muco-cutaneous syndrome, which is characterized by recurrent orofacial swelling, recurrent facial paralysis and fissured tongue. It has a high prevalence in young adults. Up to now, the etiology of MRS is still not clear, it may related to infection, immune deficiency and hereditary factors. The pharmacological therapy and surgery are the main treatment. Corticosteroids seems to be the drug of choice for MRS patient, but the specific dosage and therapeutic effect have not yet been determined. Surgeries of lips provide excellent results in persistentlip edema MRS cases. This article reviews the research progress on MRS, focusing on its epidemiology, etiology, histopathological characteristics, clinical manifestations, classification, diagnostic criteria, differential diagnosis and treatment, to provide information for its early diagnosis and appropriate treatment.

Diagnostic strategies for oral manifestations of infectious diseases / 浙江大学学报·医学版

Infectious diseases are caused by infection of organism with pathogenic agents, which may lead to epidemics. Many infectious diseases such as syphilis, diphtheria, measles, and acquired immunodeficiency syndrome have specific and non-specific manifestations in the oral cavity. However, the same disease may have different oral manifestations in different patients clinically. We can apply the strategy of "three unusuals and one change" in clinical reasoning for diagnosis of infectious diseases with oral manifestations. The so-called "three unusuals" refer to unusual age, unusual location and unusual effect, and "one change" means the change from rare to common. In this article, we will give a comprehensive introduction about the oral manifestations of common communicable diseases and their diagnostic strategies.

Application of medical magnifying loupes in diagnosis of oral mucosal diseases / 浙江大学学报·医学版

: To evaluate the application of medical magnifying loupes in diagnosis of oral mucosal diseases. Twenty-four patients with plaque-type oral lichen planus or homogeneous oral leukoplakia were inspected by naked eyes or assistance with magnifying loupes. Histopathological results were used as the gold standard to evaluate the sensitivity, specificity and accuracy of the two methods in clinical diagnosis. Questionnaires were used to evaluate the subjective effect of magnifying loupes on the diagnosis efficiency of oral mucosal diseases and to explore the most suitable parameters for application. The sensitivity, specificity and accuracy of medical magnifying loupes for the identification of plaque-type oral lichen planus and homogeneous oral leukoplakia were 94.74%, 100.00% and 95.83%, respectively, which were significantly higher than those of naked eye inspection (89.47%, 80.00% and 87.50%). The effective rate of magnifying loupes assisted diagnosis was 91.76% according to physicians' subjective evaluation. The most suitable parameters were 3.5 times magnification and working distance. The medical magnifying loupes can effectively improve the efficiency of the inspection and diagnosis of oral mucosal diseases, and have the characteristics of convenience and real-time. The recommended clinical parameters are 3.5 times magnification and working distance.

Establishment and predictive value of an early warning system for recurrence after radical resection of BCLC stage 0/A hepatocellular carcinoma / 临床肝胆病杂志

Objective To establish an Early Warning System for Recurrence Scoring after Radical Resection of BCLC stage 0/A Primary Liver Cancer (PLC-EWSPRS), and to investigate its predictive value. Methods A retrospective analysis was performed for the clinical data of 232 patients with BCLC stage 0/A liver cancer who underwent radical resection in Affiliated Hospital of Chuanbei Medical College from January 2009 to January 2015, and according to the presence or absence of recurrence within 5 years after surgery based on telephone or outpatient follow-up data, the patients were divided into recurrence group with 103 patients and non-recurrence group with 129 patients. The t -test was used for comparison of normally distributed continuous data between groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between groups; the chi-square test or Fisher's exact test was used for comparison of categorical data between groups. The indices with statistical significance were included in the binary logistic regression analysis to investigate the risk factors for recurrence of BCLC stage 0/A liver cancer after surgery. Two points were assigned for independent risk factors and one point was assigned for risk factors to establish the PLC-EWSPRS system. The receiver operating characteristic (ROC) curve and the area under the ROC curve (AUC) were used to evaluate the diagnostic efficiency of this system. Results Compared with the non-recurrence group, the recurrence group had significantly higher levels of aspartate aminotransferase and alanine aminotransferase (ALT) and a significantly lower level of albumin (Alb) before surgery ( Z =3.864 and 4.587, t =-5.628, all P < 0.001), as well as a significantly higher proportion of patients with positive HBsAg, capsular invasion, microvascular invasion (MVI), tumor diameter ≥5 cm, liver cirrhosis (moderate-to-severe), non-R0 resection, or death within 5 years ( χ 2 =35.539, 22.325, 13.398, 7.130, 4.312, 4.034, and 18.527, all P < 0.05). The regression analysis showed that preoperative Alb < 40 g/L (odds ratio [ OR ]=5.796, P < 0.001), preoperative ALT ≥40 U/L ( OR =3.029, P =0.002), MVI ( OR =3.981, P =0.003), positive HBsAg ( OR =7.829, P < 0.001), capsular invasion ( OR =5.357, P < 0.001), and non-R0 resection ( OR =3.048, P =0.018) were independent risk factors for recurrence of BCLC stage 0/A liver cancer within 5 years after surgery. According to the assignment criteria of the PLC-EWSPRS system, the recurrence group had the lowest score of 2 points and the highest score of 14 points, while the non-recurrence had the lowest score of 0 point and the highest score of 11 points, and the recurrence group had a significantly higher score than the non-recurrence group ( P < 0.05). The ROC curve analysis showed that the PLC-EWSPRS system had an AUC of 0.918 (95% confidence interval [ CI ]: 0.883-0.953, P < 0.001) in predicting recurrence within 5 years after surgery in patients with BCLC stage 0/A liver cancer undergoing radical resection, and subgroup analysis showed that the system had an AUC of 0.796 (95% CI : 0.695-0.896, P =0.002), 0.859 (95% CI : 0.791-0.927, P < 0.001), and 0.944 (95% CI : 0.839-1.000, P =0.044), respectively, in predicting recurrence within 5 years after surgery in patients with a low score of 0-5 points, a moderate score of 6-10 points, and a high score of 11-14 points. Conclusion The PLC-EWSPRS system has a good value in predicting the recurrence of BCLC stage 0/A liver cancer within 5 years after surgery and thus has important guiding significance for postoperative reexamination and treatment strategy for patients with BCLC stage 0/A liver cancer undergoing radical resection.

Recent progress in targeting degradation of FAK based on PROTAC / 药学学报

Local focal adhesion kinase (FAK) is a non-receptor intracellular tyrosine kinase that plays an important role in tumor initiation, development, metastasis and invasion, and is considered to be an important target for the development of antineoplastic drugs. It has both kinase-dependent and non-kinase-dependent scaffolding functions. However, traditional small molecular inhibitors can only inhibit its kinase-dependent activity, so it is difficult to target the kinase-independent scaffolding function. Therefore, there is an urgent need for novel strategies to enhance FAK targeting to lay the foundation for determining the druggability and discovery of FAK inhibitors. Proteolysis targeting chimera (PROTAC) is a new drug development strategy that can recruit E3 ligase to specifically ubiquitinylate target proteins for degradation through the proteasome system. The unique mechanism of action of the PROTAC system could be used to target and degrade the FAK protein, thus eliminating the scaffolding function of FAK. In this review, FAK protein, the signaling pathway, and small molecule inhibitors are briefly described, and the latest research progress in targeting the degradation of FAK using PROTAC technology is summarized.

High expression of ACE2 receptor of 2019-nCoV on the epithelial cells of oral mucosa / 国际口腔科学杂志·英文版

It has been reported that ACE2 is the main host cell receptor of 2019-nCoV and plays a crucial role in the entry of virus into the cell to cause the final infection. To investigate the potential route of 2019-nCov infection on the mucosa of oral cavity, bulk RNA-seq profiles from two public databases including The Cancer Genome Atlas (TCGA) and Functional Annotation of The Mammalian Genome Cap Analysis of Gene Expression (FANTOM5 CAGE) dataset were collected. RNA-seq profiling data of 13 organ types with para-carcinoma normal tissues from TCGA and 14 organ types with normal tissues from FANTOM5 CAGE were analyzed in order to explore and validate the expression of ACE2 on the mucosa of oral cavity. Further, single-cell transcriptomes from an independent data generated in-house were used to identify and confirm the ACE2-expressing cell composition and proportion in oral cavity. The results demonstrated that the ACE2 expressed on the mucosa of oral cavity. Interestingly, this receptor was highly enriched in epithelial cells of tongue. Preliminarily, those findings have explained the basic mechanism that the oral cavity is a potentially high risk for 2019-nCoV infectious susceptibility and provided a piece of evidence for the future prevention strategy in dental clinical practice as well as daily life.

Construction of an oral squamous cell carcinoma cell line for stable PA28γ overexpression / 华西口腔医学杂志

OBJECTIVE@#To construct a PA28γ overexpression cell line and determine its effects after infecting an oral squa-mous cell carcinoma (OSCC) cell line.@*METHODS@#The PA28γ gene was cloned into the pLOV.CMV.cherry.2A.EF1a.PuroR lentiviral vector by polymerase chain reaction (PCR), and PCR and DNA sequencing alignment analysis were used for identification. Then, 293T cells were used to package viral diseases. Infected OSCC cells were used to construct a cell line with stable PA28γ overexpression. Finally, the level of PA28γ expression in the OSCC cell line was detected through Western blot.@*RESULTS@#The successful construction of PA28γ recombinant lentiviral vectors was confirmed by DNA sequencing. The results of immunofluorescence showed that the PA28γ overexpression lentivirus successfully infected the OSCC cells and showed cherry red fluorescence. The results of Western blot demonstrated that the constructed cells with stable PA28γ overexpression significantly increased the expression of PA28γ.@*CONCLUSIONS@#The PA28γ overexpression lentiviral vector can significantly increase its protein expression in OSCC cells. We provide a stable OSCC cell line for further study on the effect of PA28γ in OSCC.

Analysis of Key Varieties and Rational Allocation of Traditional Chinese Medicine Resources Under COVID-19 Epidemic Situation / 中国实验方剂学杂志

Traditional Chinese medicine (TCM) plays an important role in the fight against coronavirus diseasef-2019 (COVID-19) epidemic. A total of 241 Chinese patent medicines and 242 prescriptions were recommended by the state and 26 provinces (autonomous regions and municipalities directly under the central government) for the prevention and treatment of COVID-19. A total of 53 varieties of commonly used medicinal materials were selected by analysis, of which 20 were common key varieties in medicinal materials of Chinese patent medicines and prescriptions, including Glycyrrhizae Radix et Rhizoma, Forsythiae Fructus, Pogostemonis Herba, etc. At the same time, some cold-quilt medicines and ethnic medicines also played an important role in the epidemic. By evaluating the supply and regeneration ability of the key varieties of TCM resources under the current COVID-19 epidemic situation, means of the source, regeneration cycle, medicinal parts, new production and market information of 53 kinds of TCM at present, it is suggested that the artificial cultivation of Glycyrrhizae Radix et Rhizoma and Paeoniae Radix Rubra can be strengthened, the development of Lonicerae Japonicae Flos, Isatidis Radix and other domestic medicinal materials can be controlled. In response to the change in market demand, the production variety structure and planting area of Chinese medicinal materials were timely adjusted to improve the quality standard and safety index of Chinese medicinal materials, and in order to provide ideas for the rational allocation of TCM resources and the development of Chinese medicinal materials industry under the epidemic situation.

Research progress of dyskeratosis congenita / 中华口腔医学杂志

Dyskeratosis congenita (DC) is a rare disease and a genetic heterogeneity of bone marrow failure, characterized by muco-cutaneous triad of mucosal leukoplakia, abnormal skin pigmentation, nails dystrophy and often involving multiple organs or systems. The inheritance patterns of DC include X-linked recessive, autosomal dominant and recessive patterns. However, the inheritance patterns in 30%-40% of DC patients remained unknown. Dyskeratosis congenita is difficult to diagnose because of its genetic and clinical heterogeneity. This article will review and discuss the state-of-the-art progresses in genetics, clinical manifestation, diagnosis, differential diagnosis, treatment and prognosis of DC.

Analyze the national directory of rare diseases, define the diagnostic role of the stomatologists / 中华口腔医学杂志

A rare disease, also referred to as an orphan disease, is defined as the disease with a low prevalence or that affects a small percentage of the population. It is a well model of human disease, which can facilitate the in-depth study and understanding of related diseases. Therefore, five Chinese governmental authorities, including the National Health Commission of the People′s Republic of China, jointly issued the "First National Directory of Rare Diseases" (the First List) on May 11, 2018. The First List covers 121 rare indications. In the analysis of the directory, we found that among the 121 diseases, there are 51 (42.2%) with oral characterization. Oral manifestations mainly include craniofacial abnormalities, dentition (dental) abnormalities, oral soft tissue lesions, jaw bone lesions, salivary gland related diseases, etc., even some of them are the first, earliest and inevitable clinical manifestations of some patients with rare diseases. In order to strengthen the understanding of stomatological counterparts on the importance of the national directory of rare diseases and deeply understand the important and irreplaceable role of stomatologists in the diagnosis and treatment of rare diseases, the present review article is specifically written to introduce the oral characterization of the rare diseases listed in the catalogue, aiming at improving the diagnosis and treatment capabilities of these diseases by peers and benefiting the public.

Advances in research on related mechanisms of immune-targeted therapy on osteosarcoma / 中国免疫学杂志

Osteosarcoma, originated from mesenchymal tissue, is one of the highest incidence of primary malignant bone tumors which were characteristic double peak distribution both in adolescents and elderly. Immune-targeted therapy could block the tumor cell signaling pathway and promote cancer cell death by apoptosis. Immune-targeted therapy is an effective treatment of anti-osteosarcoma after surgery and chemotherapy in recent years. The paper reviews the advances in latest research on the related mechanisms of the immune-targeted therapy on osteosarcoma, and hope to provide a theoretical basis for clinical treatment.