[Clinical features of non-cirrhotic portal hypertension in patients with common variable immunodeficiency].

We wished to summarize the clinical features of common variable immunodeficiency (CVID) complicated by non-cirrhotic portal hypertension (NCPH) and to deepen our understanding of it. The case data of CVID complicated with NCPH admitted to Peking Union Medical College Hospital from January 1983 to May 2021 were analyzed retrospectively to summarize their clinical characteristics. Six patients with CVID combined with NCPH (three of each sex; 16-45 years) were assessed. Four patients had portal hypertension. All patients had anemia, splenomegaly, a normal serum level of albumin and transaminases, and possibly increased levels of alkaline phosphatase and gamma-glutamyl transpeptidase. Two patients were diagnosed with esophagogastric fundic varices by gastroscopy. Two patients underwent splenectomy (which improved hematologic abnormalities partially). Four patients had autoimmune disease. Two cases were diagnosed with nodular regenerative hyperplasia (NRH) upon liver biopsy. Six patients were administered intravenous immunoglobulin-G (0.4-0.6 g/kg bodyweight) once every 3-4 weeks as basic therapy. Often, CVID complicated with NCPH has: (1) The manifestations of portal hypertension as the primary symptom. (2) Autoimmune-related manifestations. Imaging can provide important diagnostic clues. The etiology may be related to hepatic NRH and splenomegaly due to recurrent infections.

[Epidemiological characteristics of pneumoconiosis deaths in a certain area from 1961 to 2020].

Objective: To investigate the epidemiological characteristics of pneumoconiosis deaths in Putuo District of Shanghai, and to provide evidence for prevention and management of pneumoconiosis in the future. Methods: In June 2021, the data of 263 patients with pneumoconiosis who died from January 1961 to December 2020 in Putuo District of Shanghai were retrospectively analyzed. Trend χ(2) test, analysis of variance and Spearman rank correlation methods were used to analyze the basic information, age of exposure to dust, promotion period, course of disease and direct cause of death of patients with pneumoconiosis. Results: Among the 263 cases of pneumoconiosis patients who died in Putuo District of Shanghai from 1961 to 2020, 260 cases (98.86%) were male. The main types of pneumoconiosis were foundry worker pneumoconiosis (53.23%, 140/263) and silicosis (43.73%, 115/263). The main types of pneumoconiosis were sand cleaner (36.50%, 96/263). The age of onset was (53.42±10.13) years old, the age of death was (76.14±9.12) years old, and the age of exposure to dust was (20.91±8.99) years. The duration of dust exposure was negatively correlated with the duration of disease (r(s)=-0.24, P<0.001). With the increase of pneumoconiosis stage, the mortality of silicosis and foundry worker pneumoconiosis also showed an increasing trend (χ(2)(trend)=4.22, 3.87, P=0.040, 0.049). 31.94% (84/263) of pneumoconiosis patients died directly from pneumoconiosis, ranking first among the direct causes of death. Conclusion: The death cases of pneumoconiosis in Putuo District of Shanghai are mainly foundry worker pneumoconiosis and silicosis, and pneumoconiosis is the main cause of death.

[Application of augmented reality and mixed reality navigation technology in laparoscopic limited right hepatectomy].

Objective: To investigate the application effect of augmented reality and mixed reality navigation technology in three-dimensional(3D) laparoscopic narrow right hepatectomy(LRH). Methods: A retrospective analysis was performed on the clinical data of 5 patients with hepatic malignancy admitted to the First Department of Hepatobiliary Surgery,Zhujiang Hospital,Southern Medical University from September 2020 to June 2021,all of whom were males,aged from 42 to 74 years.Preoperative evaluation was performed using the self-developed 3D abdominal medical image visualization system; if all the 5 patients were to receive right hemihepatectomy,the remnant liver volume would be insufficient,so LRH were planned.During the operation,the independently developed 3D laparoscopic augmented reality and mixed reality surgical navigation system was used to perform real-time multi-modal image fusion and interaction between the preoperative 3D model and 3D laparoscopic scene.Meanwhile,intraoperative ultrasound assisted indocyanine green fluorescence was used to determine the surgical path.In this way,the LRH under the guidance of augmented reality and mixed reality navigation was completed.The predicted liver resection volume was evaluated before surgery,actual resected liver volume,surgical indicators and postoperative complications were analyzed. Results: All the 5 patients completed LRH under the guidance of augmented reality and mixed reality navigation technology,with no conversion to laparotomy.The median operative time was 300 minutes(range:270 to 360 minutes),no intraoperative blood transfusion was performed,and the median postoperative hospital stay was 8 days(range:7 to 9 days).There were no perioperative deaths,or postoperative complications such as liver failure,bleeding,or biliary fistula. Conclusion: For patients who need to undergo LRH,the use of augmented and mixed reality navigation technology can safely and effectively guide the implementation of surgery,retain more functional liver volume,improve surgical safety,and reduce postoperative complications.

[Contemporary dietary patterns and oral disease].

The achievements and breakthroughs in scientific field have provided scientific evidence for the relationship amongst diet, nutrition and chronic diseases, including oral diseases. Thus many international organizations and the governments of most countries were pushed to pay their attention to the prevention of chronic diseases by dietary patterns. From 1989 to 2018, the Food and Agriculture Organization of the United Nations and World Health Organization organized expert consultations for many times to issue a series of global strategies and action plans on diet and health. The governments of China, Japan and US had produced the Dietary Guidelines for Residents. The results showed that the morbidity and mortality of many chronic diseases had been decreased by following the dietary guidance. However, the scientific achievements did not seem to produce tremendous enhancement in public health for various reasons. In the present article, the authors analyses the relationship between contemporary dietary patterns and oral health, and make suggestions on preventing and controlling oral diseases via dietary patterns for policy makers and dental professionals.

[Development and improvement of Chinese oral public health system].

The pandemic of COVID-19 in early 2020 is a big challenge to the mechanism of major epidemic prevention and control, the national public health management system, as well as medical and health resources in China. Going through the COVID-19 crisis, we should fully understand the problems and vulnerabilities of the current public health system in China, and it is imperative to deepen and strengthen the public health system which should be throughout whole life cycle and whole process. Oral cavity is the first portal of human body, while illness finds its way in by the mouth. Oral health is highly related to general health and quality of life. The outbreak has drawn the public's attention to the public health system. The establishment and improvement of the oral public health system with the concept of "public health with Chinese characteristics" should also attract people's attentions and thoughts. Furthermore it is urgent to recognize the importance of oral public health.

[Recommendations of diagnosis and treatment of gout in China].

Gout is a crystal associated arthritis caused by monosodium urate (MSU) accumulating in joint, and it belongs to metabolic rheumatic disease. In China, gout is common but it is insufficient for education of standardized diagnosis and treatment for gout. Based on the evidence and guidelines from China and other countries, Chinese gout Collaborative Research Group developed standardization of diagnosis and treatment of gout in China. The purpose is to standardize the methods for diagnosis of gout, treatment opportunity and strategies in order to reduce misdiagnosis, missed diagnosis and irreversible damage.

[Prevention and control of oral diseases via "Common Risk Factor Approach"].

People are generally more aware of infectious diseases, cardiovascular diseases, trauma and other fatal diseases rather than oral diseases. In fact, the oral disease as a whole is ranked the third among the chronic diseases of human beings. There have been accumulating evidences that oral health plays a significant role in general health and quality of life. There is a wide recognition that oral health shares common risky/pathogenic factors with other Non-Communicable Diseases (NCD). Oral microorganisms may cause dental caries and periodontal diseases, and they may also cause atherosclerosis and heart diseases, thus these usually end up to chronic diseases. On the other hand, unhealthy diet, physical inactivity, tobacco and alcohol consumption, psychological stress, poor sanitation, accidents and a sedentary lifestyle contribute to both oral diseases and a number of general chronic diseases. Oral health cannot be dealt with in isolation from other health issues. That is why the WHO recommended the "Common Risk Factor Approach (CRFA)" for oral health promotion. In this article, the authors give essential interpretation of CRFA and make suggestions on preventing and controlling oral diseases via CRFA.

[The interpretation of 2018 updated European League Against Rheumatism evidence based recommendations for the diagnosis of gout].

With the improvement of people's living conditions and the prolonged lifespan, gout and hyperuricemia have increasingly become common chronic diseases in daily clinical practice for other physicians in addition to rheumatologists, even including general practitioners and primary care doctors. The European League Against Rheumatism (EULAR) firstly issued recommendations on gout diagnosis and management in 2006, which has been updated several times. Using a Delphi consensus approach, 2018 updated European League Against Rheumatism evidence-based recommendations for the diagnosis of gout finally formulated eight main recommendations, which was published online in June 2019. Not only does this edition emphasize the role of specialists, joint puncture or tophus aspirates and ultrasound in diagnosis, but also calls on all clinicians to pay more attention to risk factors of gout and associated comorbidities.

[The evaluation of training program "resident team leader in the Department of General Internal Medicine" at Peking Union Medical College Hospital].

Objective: To evaluate senior resident training program "resident team leader in the Department of General Internal Medicine" at Peking Union Medical College Hospital. Methods: We surveyed the residents or the fellows who had been selected as resident team leaders and received the training from October 2014 to September 2018 on their comments and suggestions. Results: Twenty-two rotated senior residents who were selected as team leaders in the Department of General Internal Medicine completed the survey. Almost all (21/22, 95.5%) of the respondents reported that they learnt more in general as team leaders by Visual Analog Scale (VAS). The mean VAS scores of clinical skills were 7.23±1.27, 7.86±1.32 in teaching abilities, 8.14±0.89 in leadership evaluation. Scales as chief resident assistants were 8.44±1.26. Sixteen respondents (72.7%) considered that pre-job training by attending doctors was necessary. Another 8 (36.4%) respondents addressed their demands on training of teaching skills. Conclusions: The senior resident training program "resident team leader in the Department of General Internal Medicine" improves the competency of rotated senior residents. It is a valuable pilot study on senior resident training and worthy of further application in other departments and hospitals.

[A questionnaire survey for gout management in physicians in Beijing].

Objective: To provide helpful continued medical education (CME) for physicians and improve gout treatment, we conducted a questionnaire survey to investigate physicians' knowledge in nine districts of Beijing. Methods: A questionnaire survey including ten gout-related questions was conducted among 298 physicians in Beijing. Demographic data and previous gout CME experience were collected. Chi-square test or Student's t test, univariate analysis and logistic regression analysis were used to evaluate the relevant factors of physicians' knowledge level. Results: A total of 250 valid copies were collected including 127 from community service centers (CSC), 123 from tertiary hospitals. The correct answer rate of gout etiology, pathogenesis and attack symptoms were over 70% in both groups. 45.5% (56/123) CSC doctors and 57.4% (66/115) tertiary doctors answered right drugs to control acute gout attack (P=0.067). Only 42.3% (52/123) in CSC and 53.4% (63/118) in hospitals chose allopurinol as a urate-lowering drug (ULT), while 46.3% (57/123) and 32.2% (38/118) doctors considered colchicine as a ULT drug (P=0.084) respectively. Near half doctors considered that gout patients should take long-term ULT [40.5% (51/126) vs. 57.6%(68/118)respectively, P=0.007]. Univariate analysis showed that CME training could improve gout-related knowledge in CRC doctors. Conclusion: Most CSC doctors generally understand basic knowledge of gout, while confusion of treatment is still significant. CME especially including standard gout treatment should be performed by doctors in tertiary hospitals.

[Clinical features of adult patients with chronic active Epstein-Barr virus infection].

Objective: To investigate the clinical features of adult-onset chronic active Epstein-Barr virus infection (CAEBV). Methods: A total of 21 adult patients with CAEBV who were admitted to the department of General Internal Medicine at Peking Union Medical College Hospital from January 2006 to January 2016 were retrospectively analyzed. Demographic data, disease duration, clinical manifestations, laboratory findings, treatments and prognosis were reviewed. Results: Eighteen females and 3 males were enrolled with a mean age of 39 years. The most common clinical manifestations included fever in 20 patients, splenomegaly in 20 patients, lymphadenopathy in 18 patients, and hepatomegaly in 10 patients, followed by laryngopharyngeal disorders in 6 patients, pleural effusion and peritoneal effusion each in 5 patients, rash in 4 patients, interstitial lung disease in 3 patients, gastrointestinal hemorrhage in 2 patients, and peripheral neuropathy and pulmonary hypertension each in 1 patient. Six patients were complicated with hemophagocytic lymphohis-tioncytosis(HLH) that developed 5-17 (mean: 9) months following CAEBV onset, all of whom experienced hyperpyrexia, pancytopenia, lymphadenopathy, splenomegaly, and liver dysfunction, 3 with hepatomegaly. Nineteen of the 21 patients had received steroid therapy including 10 combined with immunosuppressive agents, 11 with antiviral therapy, and 8 with intravenous immunoglobulin. Thirteen patients died, including 10 of multiple organ failure, (including 6 of HLH) 2 of severe pulmonary infection, and 1 of lymphoma. Six patients remained on follow-up, yet 2 were missing. Conclusions: CAEBV is expected with severe condition and poor prognosis, which is likely to be complicated with HLH. Clinical physicians should pay attention to adult patients with fever, hepatosplenomegaly and lymphadenopathy, which suggests possible CAEBV.

[An analysis of residents' self-evaluation and faculty-evaluation in internal medicine standardized residency training program using Milestones evaluation system].

Objective: To assess the value of internal medicine residency training program at Peking Union Medical College Hospital (PUMCH), and the feasibility of applying revised Milestones evaluation system. Methods: Postgraduate-year-one to four (PGY-1 to PGY-4) residents in PUMCH finished the revised Milestones evaluation scales in September 2017. Residents' self-evaluation and faculty-evaluation scores were calculated. Statistical analysis was conducted on the data. Results: A total of 207 residents were enrolled in this cross-sectional study. Both self and faculty scores showed an increasing trend in senior residents. PGY-1 residents were assessed during their first month of residency with scores of 4 points or higher, suggesting that residents have a high starting level. More strikingly, the mean score in PGY-4 was 7 points or higher, proving the career development of residency training program. There was no statistically significant difference between total self- and faculty-evaluation scores. Evaluation scores of learning ability and communication ability were lower in faculty group (t=-2.627, -4.279, all P<0.05). The scores in graduate students were lower than those in standardized training residents. Conclusions: The goal of national standardized residency training is to improve the quality of healthcare and residents' career development. The evaluation results would guide curriculum design and emphasize the importance and necessity of multi-level teaching. Self-evaluation contributes to the understanding of training objectives and personal cognition.

[Glycogen storage disease type â a: a rare cause of gout in adolescent and young adult patients].

Objective: To analyze the clinical features of secondary gout in glycogen storage disease type Ⅰa (GSD Ⅰa), so as to improve the awareness of this disease. Methods: The clinical features, laboratory findings, treatments and prognosis of 5 GSD Ⅰa patients with secondary gout who had been admitted to the Peking Union Medical College Hospital during 2006 to 2016 were collected and analyzed. GSD Ⅰa was confirmed by liver biopsy and genotyping. Results: Among the 5 patients (median age: 27 years), 3 were males and 2 were females. The mean age of gout onset was 17 ranging from 10 to 22 years old. The common manifestations of GSD included hepatomegaly since childhood, hypoglycemia, growth retardation, anemia, hyperlactacidemia and hyperlipidemia. All the 5 patients were complicated with gouty tophi and kidney stone. Gouty tophi and kidney stone were identified 3.8 years and 10.2 years after the first occurrence of articular symptoms, respectively. Renal damage occurred in 3 cases. All the patients underwent several therapeutic modalities including lifestyle intervention, allopurinol, and raw corn starch treatment. Conclusions: Determination of the presence of primary disease should be performed actively for young-onset gout with early occurrence of gouty tophi. GSD should be suspected if there exist clinical manifestations like hepatomegaly, recurrent hypoglycemia, growth retardation. Early management of hyperuricemia and gout in GSD patients is important to prevent complications and improve prognosis.