The construction of a training system for visiting physicians in the department of rare diseases in China is an important measure to improve the overall diagnosis and treatment capacity for rare diseases and address the critical challenge of insufficient knowledge and skills among clinicians in practice. This article systematically describes the visiting physician training system established by the Department of Rare Diseases at Peking Union Medical College Hospital. It summarizes the training objectives and positioning, design logic, and learning modules of the system, aiming to provide a reference for the construction of the specialized talent team for rare diseases in China.

Gorham-Stout disease(GSD) is a rare osteolytic disorder characterized by spontaneous and progressive osteolysis, along with abnormal angiogenesis and lymphangiogenesis, with no new bone formation. We present a case of a 15-year-old female admitted due to " recurrent right leg pain for 5 years, 11 months after undergoing right femoral fracture surgery". Through comprehensive integration of the patient's clinical phenotype, laboratory tests, imaging findings, pathological examinations, and molecular biological test results, GSD was considered highly likely. A multidisciplinary treatment approach was conducted, including a combination of zoledronic acid and sirolimus to inhibit osteolysis, along with rehabilitation training and orthopedic intervention, providing a personalized and comprehensive treatment strategy.

OBJECTIVE: To provide the experience and demonstration for the transformation of acupuncture-moxibustion techniques standards from Chinese national standards to international standards. METHODS: Questionnaire research, literature research, semi-structured interviews and expert consultation were used. RESULTS: The safety of acupuncture-moxibustion techniques was evaluated through literature research, and based on the results of the questionnaire survey, expert interviews, and expert consultation, 11 main bodies and structure of the former Chinese national standard, Technical Benchmark of Acupuncture and Moxibustion: General Rules for Drafting, were adjusted and optimized in accordance with the requirements of international standard (including the language, normative references, purpose, scope, applicable environment, target population, work team, terms and definitions, general principles and basic requirements, structural elements and text structure, and compilation process); and the first international standard, World Federation of Acupuncture-Moxibustion Societis (WFAS) Technical Benchmark of Acupuncture and Moxibustion: General Rules for Drafting was formulated to specify the general rules for drafting. CONCLUSION The 3 key questions, "international compatibility", "technical operability" and "safety" should be solved technically on the basis of explicit international requirements. It is the core technical issue during transforming the national standards of technical benchmark of acupuncture and moxibustion into international standards.
Moxibustion/methods* ; Acupuncture Therapy/methods* ; Humans ; Translational Research, Biomedical/standards* ; Surveys and Questionnaires ; China ; Benchmarking/standards*

Objective To characterize the epidemiological,radiographic and clinical features of transmigrated mandibular canines by cone-beam CT(CBCT),in order to provide assistance for treatment planning.Methods Patients treated at our institution diagnosed with mandibular canines transmigration were retrospectively screened using CBCT images from June 2012 to December 2018.Clinical and radiographic parameters including location,classification,neighbouring structures,and associated anomalies were evaluated.Re-sults Eighty-two patients with 85 transmigrated mandibular canines were enrolled,establishing a prevalence of 0.11％(82/75 021).Among these cases,79 patients had unilateral transmigration of mandibular canines;the other 3 had bilateral transmigrations.Thirty-nine cases were male and 43 were female,with age ranging from 10 to 58.According to the Mupparapu classification,type Ⅰ(42,49.41％)was the most prevalent followed by type Ⅱ(19,22.35％)and type Ⅲ(15,17.65％).Fifty-three(62.35％)crowns were located labially,and often associated with odontoma,cyst and supernumerary tooth.Seventy transmigrated canines were surgically re-moved while 2 cases were tried to orthodontically aligned,failed and finally extracted.Ten cases received routine monitoring.Conclu-sion The transmigration of mandibular canines is rarely occurring abnormality with certain clinical imaging features which can assist in clinical diagnosis and treatment planning.

Objectives:To investigate the prognostic difference of different revascularization strategies in AMI patients with multi-vessel disease and diabetes.Methods:AMI patients with multi-vessel disease and diabetes admitted to the Department of Cardiology of the First Hospital of Lanzhou University from January 2022 to June 2024 were retrospectively included.Patients were divided into non complete revascularization(NCR)group(n=166),staged complete revascularization(SCR)group(n=152)and immediate complete revascularization(ICR)group(n=120).Baseline clinical characteristics,coronary angiography data and postoperative medication were compared among the groups.Primary endpoint was the major adverse cardiovascular and cerebrovascular events(MACCE)during follow-up.MACCE includes all-cause mortality,cardiogenic mortality,non-fatal myocardial infarction,unplanned revascularization and stroke.The log-rank test was used to analyze the significance of the differences in the cumulative incidence of MACCE among the three groups.Cox regression was used to explore the influencing factors of poor prognosis in patients.Results:There were statistically significant differences among NCR group,SCR group and ICR group in terms of the history of previous percutaneous coronary intervention,the use of intraoperative coronary intravascular ultrasound(IVUS)and the dosage of contrast agent(all P<0.05).During a median follow-up of 21(11,25)months,MACCE events occurred in 59 cases(35.5%)in the NCR group,26 cases(17.1%)in the SCR group,and 30 cases(25.0%)in the ICR group.The Kaplan-Meier curve showed that the differences in the cumulative incidence of MACCE among the three groups were statistically significant(log-rank P<0.001).Using the Bonferroni correction(adjusted α′=0.05/3≈0.0167),pairwise comparisons revealed statistically significant differences between the NCR and SCR groups(log-rank P<0.001)and between the NCR and ICR groups(log-rank P=0.011).However,no statistically significant difference was observed between the SCR and ICR groups(log-rank P=0.228).Cox multivariate regression analysis demonstrated that history of hypertension was an independent risk factor for MACCE in AMI patients with multivessel coronary artery disease and diabetes(HR=1.71,95%CI:1.10-2.64,P<0.05).The difference in the incidence of MACCE between the SCR group and the NCR group was statistically significant(HR=0.45,95%CI:0.28-0.73,P=0.001).Conclusions:Staged complete revascularization serves as the preferred revascularization strategy for AMI patients with multivessel coronary disease and diabetes mellitus.Additionally,for patients with concomitant hypertension,blood pressure management should be intensified to reduce the risk of MACCE.

OBJECTIVE To analyze the clinical characteristics and the correlation between laboratory indicators and prognosis of severe Mycoplasma pneumoniae pneumonia(SMPP)in children.METHODS A total of 85 children with SMPP admitted to Anhui Provincial Children's Hospital from Nov.2021 to May 2024 were selected as the study subjects.Based on clinical typing at admission,they were divided into a high-risk group(n=59)and a low-risk group(n=26).The clinical manifestations,laboratory indicators and outcomes at 28 days of treatment were compared between the two groups.RESULTS The duration of fever and cough before admission in the high-risk group was(7.17±1.09)days and(6.79±1.25)days,respectively,which was longer than that in the low-risk group(P＜0.05).There were no statistically significant differences in pulmonary auscultation(wheezing rales,moist rales)and extrapulmonary complications between the two groups.The levels of C-reactive protein(CRP),serum amyloid A(SAA),platelets(PLT),fibrinogen(FIB),D-dimer(DD)and N-terminal pro-brain natriuretic peptide(NT-proBNP)in the high-risk group were(11.62±1.45)mg/L,(226.88±36.83)mg/L,(3 18.57±39.82)×109/L,(4.28±0.74)g/L,(0.81±0.12)μg/ml and(2 295.48±413.75)pg/ml,respectively,all of which were higher than those in the low-risk group(P＜0.05).Within 28 days after treatment of children in both groups,one patient in the high-risk group died.CONCLUSIONS Compared with children with SMPP in the low-risk group,those in the high-risk group have a higher risk of prognostic mor-tality,suggesting a correlation between the children's blood CRP,SAA,PLT,FIB,DD and NT-proBNP levels and the prognosis of children with SMPP.

Patients with periodontal disease often require combined periodontal-orthodontic interventions to restore periodontal health, function, and aesthetics, ensuring both patient satisfaction and long-term stability. Managing these patients involving orthodontic tooth movement can be particularly challenging due to compromised periodontal soft and hard tissues, especially in severe cases. Therefore, close collaboration between orthodontists and periodontists for comprehensive diagnosis and sequential treatment, along with diligent patient compliance throughout the entire process, is crucial for achieving favorable treatment outcomes. Moreover, long-term orthodontic retention and periodontal follow-up are essential to sustain treatment success. This expert consensus, informed by the latest clinical research and practical experience, addresses clinical considerations for orthodontic treatment of periodontal patients, delineating indications, objectives, procedures, and principles with the aim of providing clear and practical guidance for clinical practitioners.
Humans ; Consensus ; Orthodontics, Corrective/standards* ; Periodontal Diseases/complications* ; Tooth Movement Techniques/methods* ; Practice Guidelines as Topic

Cemental tear is a rare and indetectable condition unless obvious clinical signs present with the involvement of surrounding periodontal and periapical tissues. Due to its clinical manifestations similar to common dental issues, such as vertical root fracture, primary endodontic diseases, and periodontal diseases, as well as the low awareness of cemental tear for clinicians, misdiagnosis often occurs. The critical principle for cemental tear treatment is to remove torn fragments, and overlooking fragments leads to futile therapy, which could deteriorate the conditions of the affected teeth. Therefore, accurate diagnosis and subsequent appropriate interventions are vital for managing cemental tear. Novel diagnostic tools, including cone-beam computed tomography (CBCT), microscopes, and enamel matrix derivatives, have improved early detection and management, enhancing tooth retention. The implementation of standardized diagnostic criteria and treatment protocols, combined with improved clinical awareness among dental professionals, serves to mitigate risks of diagnostic errors and suboptimal therapeutic interventions. This expert consensus reviewed the epidemiology, pathogenesis, potential predisposing factors, clinical manifestations, diagnosis, differential diagnosis, treatment, and prognosis of cemental tear, aiming to provide a clinical guideline and facilitate clinicians to have a better understanding of cemental tear.
Humans ; Dental Cementum/injuries* ; Consensus ; Diagnosis, Differential ; Cone-Beam Computed Tomography ; Tooth Fractures/therapy*

Objectives:To investigate the factors affecting the formation of coronary collateral circulation(CCC)in patients with chronic total occlusion(CTO)lesion of coronary artery.Methods:A total of 305 consecutive patients who were hospitalized in the department of cardiology of the First Hospital of Lanzhou University from December 2022 to December 2023 and CTO lesions were confirmed by coronary angiography in at least one major coronary artery were included.The clinical data were collected,and the patients were divided into poor CCC group(Rentrop grade 0-1,n=109)and good CCC group(Rentrop grade 2-3,n=196)according to Rentrop criteria.Univariate logistic regression and multivariate logistic regression analysis were used to investigate the risk factors of poor CCC formation in CTO lesion patients.Results:There were 109 patients with poor CCC formation and 196 patients with good CCC formation in this cohort.The levels of white blood cell count,neutrophil count,platelet count,platelet to lymphocyte ratio,neutrophil to lymphocyte ratio,systemic immunoinflammatory index(SII),lipoprotein a and fibringen(Fib)were significantly higher,lymphocyte count and mean platelet volume were significantly lower in patients with poor CCC formation than in patients with good CCC formation(all P<0.05).Multivariate logistic regression analysis showed that higher SII(OR=1.004,95%CI:1.003-1.006,P<0.001),Fib(OR=1.546,95%CI:1.038-2.301,P=0.032)levels were independent predictors of poor CCC formation in CTO lesion patients.Conclusions:Higher levels of SII and Fib are independently correlated with poor CCC formation,which may be used as clinical predictor of poor CCC formation in CTO lesion patients.

The presence of magnetic fields in a magnetic resonance accelerator (MR-linac) can affect the reference dosimetry, and thus the existing Code of Practices (CoPs) are inadequate for MR-linac. In this article, the characteristics of adsorbed dose to water and ionization chamber response in the presence of magnetic fields were introduced and a formalism for reference dosimetry in MR-linac was developed based on the existing CoPs, aiming to provide reference for dosimetric quality control and research work of MR-linac in China.