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.

Clonal cell abnormalities represent a shared initiating event for systemic inflammation and organ injury across a spectrum of diseases, with diverse clonal origing including somatic mutations, germline mutations, and aberrant monoclonal immunoglobulin secretion. This review focuses on the clinical features, mechanism studies and therapeutic advances of the diseases, including VEXAS (vacuoles, E1 enzyme, X-linked, autoin-flammatory, somatic) syndrome, GATA2 deficiency, STAT3 gain-of-function (STAT3-GOF)syndrome, secondary hemophagocytic lymphohistiocytosis, and Schnitzler syndrome. It aims to deepen clinical understanding of these diseases and provide references for precision treatment.

Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic immune-mediated disorder characterized by infiltration of IgG4-positive plasma cells and multiorgan fibrosis. Among them, cases with multiple pancreatic cystic lesions as the prominent manifestation are relatively rare, and they are easily misdiagnosed as pancreatic tumors or pancreatitis in clinical practice. Herein, we report one patient with IgG4-RD admitted to Peking Union Medical College Hospital. The patient initially presented with multiple pancreatic cystic lesions and aneurysmal dilatation of the abdominal aorta, accompanied by markedly elevated serum IgG4 levels. Combined with pathological findings from submandibular gland biopsy, the diagnosis of IgG4-RD was ultimately established. After treatment with a regimen of glucocorticoids combined with cyclophosphamide, the patient′s condition improved. Meanwhile, we performed a literature review of cases with IgG4-RD-related pancreatic cystic lesions, aiming to improve clinicians′ understanding of this rare disease phenotype.

The Expert Consensus on Clinical Application of Qinbaohong Zhike Oral Liquid in Treatment of Acute Bronchitis and Acute Attack of Chronic Bronchitis （GS/CACM 337-2023） was released by the China Association of Chinese Medicine on December 13^th， 2023. This expert consensus was developed by experts in methodology， pharmacy， and Chinese medicine in strict accordance with the development requirements of the China Association of Chinese Medicine （CACM） and based on the latest medical evidence and the clinical medication experience of well-known experts in the fields of respiratory medicine （pulmonary diseases） and pediatrics. This expert consensus defines the application of Qinbaohong Zhike oral liquid in the treatment of cough and excessive sputum caused by phlegm-heat obstructing lung， acute bronchitis， and acute attack of chronic bronchitis from the aspects of applicable populations， efficacy evaluation， usage， dosage， drug combination， and safety. It is expected to guide the rational drug use in medical and health institutions， give full play to the unique value of Qinbaohong Zhike oral liquid， and vigorously promote the inheritance and innovation of Chinese patent medicines.

Central venous pressure(CVP)is a commonly used hemodynamic index in clinical practice,and the accuracy of its measurement results is influenced by the measurement technical specifications.Based on research evidence and clinical practice,this article interprets the main content of the group standard of CVP measurement technology,and proposes suggestions for its use.It aims to guide clinical nursing staff to correctly understand the principles and methods of CVP measurement,and promote the standardization of nursing practice and the accuracy of measurement results.

Objective:To analyze the factors influencing postoperative complications and survival in patients undergoing hepatectomy for primary liver cancer (PLC).Methods:Two hundred and eighty-eight patients with PLC who underwent hepatectomy at Tianjin Medical University Cancer Hospital from April 2018 to December 2020 were prospectively enrolled, including 235 males and 53 females, aged (58.5±9.5) years. Nutritional evaluations included the nutritional risk screening 2002 (NRS-2002), patient-generated subjective global assessment (PG-SGA), and physical metrics such as body mass index, handgrip strength, arm circumference, mid-arm muscle circumference (MAMC), and triceps skinfold thickness (TSF). Based on the occurrence of postoperative complications during hospitalization including abdominal infection, bleeding, bile leakage, intra-abdominal fluid accumulation, pulmonary infection, intestinal fistula, and wound dehiscence, patients were divided into two groups: the complication group ( n=205) and the non-complication group ( n=83). Multivariate logistic regression analysis was performed to identify the factors related to postoperative complications, while Cox proportional hazards regression was used to evaluate the impact of various variables on postoperative survival. Survival analysis was conducted using the Kaplan-Meier method, and differences in survival rates were compared using the log-rank test. Results:Compared with the non-complication group, patients in the complication group had a significantly longer postoperative hospital stay [13(8, 16) d vs. 7(5, 9) d], higher proportions of low MAMC [34.1% (24/83) vs. 20.0% (41/205)], lower grip strength [28.9% (24/83) vs. 17.6% (36/205)], higher incidence of intraoperative blood loss ≥200 ml [44.6% (37/83) vs. 22.0% (45/205)], and greater proportion of resection involving ≥3 liver segments [45.8% (38/83) vs. 26.3% (54/205)] (all P<0.05). Multivariate logistic regression analysis identified the low MAMC ( OR=2.01, 95% CI: 1.09-3.71, P=0.025), intraoperative blood loss ≥200 ml ( OR=2.75, 95% CI: 1.55-4.90, P=0.001), and resection involving ≥3 liver segments ( OR=2.02, 95% CI: 1.15-3.55, P=0.015) as independent risk factors for postoperative complications in patients undergoing hepatectomy for PLC. Multivariate Cox regression analysis revealed that low TSF ( HR=1.94, 95% CI: 1.01-3.72, P=0.047), low handgrip strength ( HR=2.23, 95% CI: 1.29-3.88, P=0.004), and resection involving ≥3 liver segments ( HR=1.96, 95% CI: 1.15-3.36, P=0.014) were risk factors associated with poor survival after surgery. Conclusions:Low MAMC, intraoperative blood loss ≥200 ml, and resection involving ≥3 liver segments are risk factors for postoperative complications in patients undergoing hepatectomy for PLC, while low TSF, reduced handgrip strength, and resection involving ≥3 liver segments are predictors of poor postoperative survival.

Dysfunction of the diaphragm is a common problem in mechanically ventilated patients and is closely related to various adverse outcomes.This review summarizes the evaluation methods of diaphragm function,the association between diaphragm dysfunction and adverse clinical outcomes in critically ill mechanically ventilated patients,and the protective effect of diaphragm function on adverse outcomes related to mechanical ventilation.The aim is to provide references for medical staff to optimize evaluation techniques,develop personalized nursing plans,and improve patients' quality of life and health outcomes.

Objective To compare the efficacy,acceptability and safety of a low-volume magnisium sodicum potassium sulfate oral sulfate solution(OSS)with polyethylene glycol(PEG)electrolytes powder in bowel preparation for colonoscopy.Methods A prospective,single-blinded and single-center cohort study was conducted.The ambulatory and hospitalized 1 037 patients who underwent colonoscopy from April 2023 to January 2024 were enrolled.Participants were divided into OSS group(639 cases)and PEG group(398 cases),according to the bowel cleansing drugs taken orally.After propensity score matching(PSM),each group included 385 cases.The success rate of bowel preparation,scores of Boston bowel preparation scale(BBPS),medication taste,patients'satisfaction and the occurrence of adverse events were compared.Results The success rate of bowel preparation in the OSS group was 96.4％(371/385),higher than the 91.7％(353/385)in the PEG group,and the difference was statistically significant(P＜0.05).The total and segmented BBPS scores of the OSS group were higher than those of the PEG group,the differences were statistically significant(P＜0.05).The medication taste and patients satisfaction of the OSS group were significantly better than those of the PEG group,the differences were statistically significant(P＜0.05).There was no statistically significant difference in incidence of adverse reactions between the two groups(P=0.800).Conclusion Compared to PEG,OSS has a better intestinal cleaning effect,medication taste,and patients satisfaction.In addition,OSS has security that is not inferior to PEG.

Objective To analyze the geographical distribution characteristics of TCM syndromes of coronary heart disease in the elderly based on the literature;To summarize the characteristics of TCM syndromes of coronary heart disease patients in different geographical areas;To provide evidence-based data for the standardization of TCM syndromes and syndrome differentiation treatment of coronary heart disease in the elderly.Methods Literature on TCM syndrome of coronary heart disease in the elderly was retrieved from CNKI,Wanfang Data,VIP,CBM and PubMed,from the establishment of each database to December 31,2024.The common TCM syndrome types,syndrome frequency and regional distribution of coronary heart disease in the elderly were statistically analyzed.Results Forty articles were included,with a total number of 6 447 cases and a male-to-female ratio of 1.1∶1.The top 5 highest percentage of co-morbidities of coronary artery disease in the elderly were 1 308 cases of hypertension(24.11%),1 022 cases of type 2 diabetes mellitus(18.84%),787 cases of respiratory disease(14.51%),517 cases of heart failure(9.53%),and 348 cases of hyperlipidemia(6.41%).The top 5 TCM syndromes in elderly patients with coronary heart disease included 779 cases of qi deficiency and blood stasis(12.08%),692 cases of qi-yin deficiency(10.73%),367 cases of phlegm-blood stasis(5.69%),363 cases of heart blood stagnant blockade(5.63%),and 343 cases of qi stagnation and blood stasis(5.32%).The distribution of TCM syndromes in different regions was characterized by qi deficiency,blood stasis and phlegm stasis in the northeast;qi deficiency,blood stasis and qi yin deficiency in the north;qi deficiency,blood stasis and phlegm internal obstruction in the east;qi and yin deficiency and heart blood stasis in the central;qi deficiency,blood stasis and phlegm stasis in the south;phlegm and yin paralysis obstruction,qi and yin deficiency in the southwest;stasis blockage of the cardiac veins,qi yin deficiency in the northwest.The overall trend was that the number of syndromes in the north was higher than that in the south;the number of syndromes in the east,centre and west was decreasing.The distribution of yin deficiency syndrome was highest in the southwest(23.33%)and lowest in the east(10.93%);yang deficiency syndrome was highest in the southwest(13.30%)and lowest in the central(3.32%);qi deficiency and blood stasis syndrome was highest in the northeast(32.89%)and lowest in the northwest(7.24%);the distribution of qi-yin deficiency was highest in the southwest(23.33%)and lowest in the east(7.22%);the distribution of phlegm and stasis syndrome was highest in the northeast(25.67%)and lowest in the east(1.69%).The overall generalization was that deficiency,stasis and phlegm were the main syndrome factors.Conclusion The main symptoms of coronary heart disease in the elderly manifest as a mixture of deficiency and excess,qi deficiency and blood stasis syndrome is the most syndrome type of coronary heart disease in the elderly and is mainly distributed in the northeast.The distribution of TCM syndromes has regional characteristics.

Objective:To conduct a multi-center cross-sectional study of elderly patients with cognitive impairment based on real-world data; To analyze the clinical characteristics and core medication law of the disease population.Methods:The medical records of elderly patients diagnosed with cognitive impairment from January 1, 2016 to December 31, 2024 were retrieved from Chinese Evidence-based TCM Database. Excel 2010 software was used to collect general information, TCM syndrome elements and syndrome types, use frequency of Chinese materia medica, property, taste, meridian tropism, efficacy classification and other information, using PivotTable to establish Chinese materia medica matrix. IBM SPSS Modeler 18.0 software was used to display the frequency of co-occurrence among high-frequency Chinese materia medica, and the association rules were analyzed based on the Apriori algorithm. R language 4.4.1 was used for clustering analysis on Chinese materia medica and the cluster pedigree and correlation heat map were drawn.Results:A total of 1 194 elderly patients with cognitive impairment were enrolled, with a mean age of (79.51±7.65) years, and the highest frequency of comorbidities was hypertension (891 cases, 74.62%); AD was the most frequent diagnosis of cognitive impairment (367 times, 30.74%). The proportion of mild cognitive impairment in the low value group of Systemic Immune Inflammation Index (SII) was relatively high, and the high value group of SII was mainly vascular dementia and AD. Wind-phlegm blocking syndrome (106 times, 13.73%) topped the list; phlegm was the most frequent element of pathogenicity (353 times, 45.73%), and meridians and collaterals (194 times, 25.13%). Totally 328 prescriptions were included, involving 308 kinds of Chinese materia medica. The total recorded frequency of Chinese materia medica was 5 665, with tonifying herbs constituting the most frequently used category, accounting for 1 633 times; the medicinal property of drugs for treating elderly patients with cognitive disorders was mostly warm (103 times, 33.44%), the taste was mostly bitter (141 times, 45.78%), and attributed to the liver meridian (145 times, 47.08%); among the two and three association rules, the drug pairs with the highest confidence were Gastrodiae Rhizoma→Uncariae Ramulus cum Uncis (97.22%) and Hyperici Perforati Herba→Acanthopanacis Senticosi Radix et Rhizoma seu Caulis+Alpiniae Oxyphyllae Fructus (97.06%); five medicinal combinations were obtained by high frequency drug clustering analysis.Conclusions:Elderly patients with cognitive impairment are mainly diagnosed with AD. There may be a potential correlation between SII level and the progression of cognitive impairment. The drugs are used to tonify the kidney and replenish essence, detoxify and unblock collaterals, replenish qi and activate blood circulation, and awaken the brain and open the orifices. Shengmai Decoction, Renshen Yangrong Decoction and other TCM classic prescriptions can be used in clinic.