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Orbital disorders include conditions originating from the orbital bones, surrounding tissues, and post-orbital septum. They also include systemic ailments affecting the orbit. Different clinical symptoms make up the complex range of orbital disorders. Because these disorders mostly impact the orbital area instead of the intraocular compartment, there is little diagnostic usefulness for typical ophthalmic visual tests. As such, the primary instruments for diagnosing and evaluating orbital illnesses have become ophthalmic imaging modalities, including ocular ultrasonography(B-scan), computed tomography(CT), and magnetic resonance imaging(MRI). One way to improve the precision and promptness of diagnosing orbital diseases is to standardize the functioning of widely used imaging equipment and define the radiological features of orbital abnormalities. Such programs are crucial for the care of patients with orbital disorders since they considerably reduce the number of misdiagnoses and missed diagnoses in these individuals. The underlying concepts, operational techniques, and normal and pathological imaging findings associated with common diagnostic tools for orbital illnesses are all thoroughly reviewed in this guideline. The objective is to improve primary healthcare settings' diagnostic competence in the field of orbital pathology and to standardize procedures for diagnosing orbital disorders.
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Objective:To investigate the effects of sclerostin (SOST) and WNT/CTNNB1 signaling pathway on the cell cycle, migration and invasion of human uveal melanoma (UM) cells and its related mechanism.Methods:UM tissues from 20 cases of epithelioid UM and 16 cases of spindle cell type UM were collected.The contents of SOST, Wnt-1 and Catenin beta-1 proteins in the collected tissues were detected by immunohistochemical staining.Three human UM tissue derived cell lines OCM-1 (primary spindle cell type), Mum-2B (metastatic epithelioid) and Mum-2C (metastatic spindle cell type) were selected and divided into three groups, blank control group not transfected, empty vector group transfected with SOST negative control vector and SOST siRNA group transfected with SOST siRNA.After 24-hour transfection, the mRNA and protein expression levels of SOST, CTNNB1, WNT protein family 1 (WNT1), CCND1, matrix metalloproteinase (MMP)2 and MMP9 were detected by real-time fluorescence quantitative PCR and Western blot, respectively.The invasion and migration ability of the transfected cells were measured by transwell method, and the cell cycle distribution was detected by flow cytometry.Another 9 female BALB/c nude mice were selected and randomized into OCM-1 group, OCM-1 empty vector group and SOST shRNA group, inoculated with OCM-1 without lentivirus infection, OCM-1 with blank lentivirus infection and OCM-1 with SOST shRNA lentivirus infection, respectively.Six weeks after inoculation, the in situ formation of tumor was observed.The interaction between SOST and low density lipoprotein receptor related protein(LRP)-5/6 in OCM-1 cells was explored by co-immunoprecipitation assay.The study protocol was approved by the Ethics Committee of Tianjin Medical University Eye Hospital (2018KY[L]-20).Results:Immunohistochemical staining results showed that the SOST expression level was higher and the expression levels of Wnt-1 and Catenin beta-1 were lower in spindle cell type UM tissues than in epithelioid UM tissues, and the differences were all statistically significant (all at P<0.01). The real-time fluorescence quantitative PCR results showed that the relative expression of SOST mRNA was significantly lower and the relative expressions of CCND1, WNT1 and MMP9 mRNA were significantly higher in SOST siRNA groups than in corresponding empty vector groups in the three cell lines (all at P<0.05). In OCM-1 and Mum-2C cell lines, the relative expressions of CTNNB1 mRNA were significantly higher in SOST siRNA groups than in empty vector groups (all at P<0.01). Western blot results showed that the relative expression of SOST protein was significantly lower and the relative expressions of Wnt-1, Catenin beta-1, cyclin-D1, MMP2 and MMP9 proteins were significantly higher in SOST siRNA groups than in empty vector groups (all at P<0.01). Transwell assay showed that the cell invasion and migration ability of SOST siRNA group was significantly higher than that of blank control group and empty vector group in the three cell lines (all at P<0.01). Flow cytometry showed that the proportion of G1-phase cells and the G1/S-phase ratio were significantly lower in SOST siRNA group than in blank control groups and empty vector groups (all at P<0.01). The eyeball volume of OCM-1 group, OCM-1 empty vector group and SOST shRNA group was (42.7±4.6), (49.0±22.9) and (135.2±32.7)mm 3, respectively, showing a significant overall difference ( F=19.963, P<0.01). The eyeball volume of SOST shRNA group was larger than that of OCM-1 group and OCM-1 empty vector group, and the differences were statistically significant (both at P<0.05). Co-immunoprecipitation results showed that SOST could interact with LRP-5 and LRP-6 by binding to them, respectively. Conclusions:Silencing SOST can promote the invasion and migration of UM cells, and increase the proportion of UM cells in the division phase.Silencing SOST can promote tumor growth in eyes of nude mice.SOST may play this function by interacting with the membrane receptor LRP-5/LRP-6 and then regulating the WNT/CTNNB1 signal pathway.
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Objective:This study aimed to explore the safety and feasibility of gasless transoral vestibular robotic resection of thyroglossal duct cysts. Methods:The clinical data of patients who underwent gasless transoral vestibular robotic resection of thyroglossal duct cysts at the Department of otolaryngology, Sun Yat-sen Memorial Hospital, Sun yat-sen university from September 2020 to May 2022 were analyzed. The operative time, blood loss, postoperative complications, postoperative pain score, postoperative aesthetic score, and recurrence were prospectively evaluated. Results:All patients completed the operation successfully and no case conversed to an open operation. The operation time was 104.00（95.00, 131.25） minutes, and the surgical blood loss was 15.00（10.00, 16.25） mL. The drainage volume was（59.71±9.20） mL. Postoperative pathology was consistent with thyroglossal duct cysts. There was no local reswelling, subcutaneous hematoma, emphysema, skin flap necrosis, infection and other complications. The postoperative hospital stay was 3.00（2.00, 3.00） days. Six patients had mild sensory abnormalities of the lower lip 3 months after surgery, and all patients were satisfied with the cosmetic outcomes. No recurrence was found during the 5-26 months follow-up. Conclusion:gasless transoral vestibular robotic resection of thyroglossal duct cysts is safe and feasible, with hidden postoperative scars and good cosmetic outcomes. It can provide a new choice for patients with thyroglossal duct cysts.
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Humanos , Procedimentos Cirúrgicos Robóticos , Cisto Tireoglosso/patologia , Complicações Pós-Operatórias , Cicatriz/patologia , Dor Pós-OperatóriaRESUMO
Objective:To summarize and analyze the clinical, laboratory and imaging characteristics of patients with Caroli syndrome (CS), so as to deepen the understanding of the disease and explore the possible methods for improving early diagnosis.Methods:From January 2008 to June 2021, the clinical data of 18 CS patients who were hospitalized in Peking Union Medical College Hospital and diagnosed by pathology or by clinical and imaging features were collected. The general data, clinical manifestations, laboratory examination (white blood cell count, hemoglobin, etc.), imaging features (ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI)), and diagnosis and treatment of the patients were retrospectively analyzed. Descriptive method was used for statistical analysis.Results:The median age of 18 CS patients was 18 years old (ranged from 1 to 39 years old); there were 10 male and 8 female patients with a male-to-female ratio of 5∶4. The median time from symptom onset to diagnosis was 24 months (ranged from 1 month to 28 years). At the time of diagnosis, 15 patients had already had portal hypertension-related complications, and 8 patients had biliary infections. The common symptoms included abdominal distension (6 cases), fever with or without abdominal pain (5 cases), and loss of appetite (3 cases). The common abnormal laboratory findings included peripheral white blood cell count, hemoglobin level and platelet count below the normal reference value range, alanine aminotransferase and bilirubin level above the normal reference value range, and 4 patients had positive autoantibodies. Four patients were clearly diagnosed according to the pathology of liver biopsy, 14 patients were clearly diagnosed by imaging. Among which the diagnostic rate of abdominal ultrasonography for CS was 4/18, CT was 11/15, and MRI or magnetic resonance cholangiopancreatography was 12/16. The typical features of abdominal ultrasonography were liver cysts with splenomegaly, typical manifestation of CT was intrahepatic bile duct dilatation with " central dot sign", and MRI typically manifested as multiple cystic dilatations of intrahepatic bile ducts. Among the 18 patients with CS, 1 case underwent right hepatectomy, 3 cases were waiting for liver transplantation, and the other 14 patients chose symptomatic treatment due to economic reasons.Conclusions:The clinical manifestations and laboratory findings of CS patients lack specificity, and the diagnosis of most patients is delayed. The lack of understanding of the disease by clinical and imaging doctors may be one of the reasons affecting the early diagnosis of CS patients. The findings of splenomegaly and liver cysts by abdominal ultrasound may provide clues for the diagnosis of CS for clinical and imaging doctors.
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Objective:To construct a geriatric comprehensive health evaluation system, and establish the norm of corresponding score.Methods:The methods of Delphi survey was implemented to develop the geriatric comprehensive health evaluation system.The weight coefficients of the items were calculated and assigned according to the expert-graded importance score and Saaty analytic hierarchy process.Reliability and validity of the system were examined.Goodness of fit was evaluated by confirmatory factor analysis.The demarcation norm of geriatric comprehensive health score was established based on an empirical study of 2, 118 non-hospitalized elderly population.Results:During the process of Delphi expert consultation and item analysis, nine items were deleted and two items were added.In the end, the 29-item comprehensive geriatric health evaluation system was developed, which included three dimensions of physiology, psychology, as well as social support and environment.The Cronbach's α coefficient of the evaluation system was 0.709.The exploratory factor analysis-extracted ten common factors meet ten second-level items.Confirmatory factor analysis indicated that the goodness of fit of the system was good.Geriatric health status was divided into five grades of very poor, poor, general, good and excellent by 0.5 to 1 standard deviation.The demarcation norm of geriatric comprehensive health score for different age and gender was established accordingly.Conclusions:The reliability and validity of the geriatric comprehensive health evaluation system are satisfactory.The construction of comprehensive health evaluation system and the establishment of the norm can facilitate the quantitative assessment of geriatric health status, and provide a reference for the screening and grading of individual health status in the elderly.
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Objective:To analyze the expression and clinical significance of serum retinol binding protein 4 (RBP4), superoxide dismutase (SOD) and hypersensitive C-reactive protein (hs-CRP) in patients with acute ST-segment elevated myocardial infarction (STEMI).Methods:Seventy-eight patients with STEMI who underwent percutaneous coronary intervention (PCI) admitted to Nanxishan Hospital of Guangxi Zhuang Autonomous Region from January 2017 to March 2019 were enrolled. On the basis of the routine treatment, atorvastatin calcium or rosuvastatin calcium treatment was given. According to the results of coronary angiography, the patients were divided into three groups: single-vessel, double-vessel and three-vessel lesions, and the severity of coronary artery disease score (SYNTAX score) was performed. The serum RBP4, SOD and hs-CRP were measured and compared among different groups before and after treatment. The correlation between the three serum parameters of STEMI patients and the SYNTAX score, risk prediction (STEMI high-risk score) and prognosis were recorded and compared among different groups.Results:There was no significant difference in serum RBP4, SOD and hs-CRP among different groups before treatment. In multivessel (double-vessel and three-vessel) vascular disease group, SYNTAX score was significantly correlated with RBP4 and hs-CRP (double-vessel lesions: r values were 0.616 and 0.489 respectively, three-vessel lesions: r values were 0.423 and 0.357 respectively, all P < 0.05), but had no correlation with SOD ( r values were 0.108 and 0.055 respectively, both P > 0.05), and high-risk score was closely correlated with RBP4 and hs-CRP levels ( r values were 0.581 and 0.623 respectively, both P < 0.01). There was no significant difference in the prognosis of patients treated with different statins. There were 8 and 7 high-risk patients in the atorvastatin calcium group and rosuvastatin calcium group, respectively, and 2 and 3 dead patients respectively (both P > 0.05). Conclusion:Combined detection of serum RBP4, SOD and hs-CRP levels can be used as better indicators to predict the risk of STEMI, which is worthy of clinical application.
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Objective:Due to the circumstances where people being required to home quarantine and eye hospitals being unable to receive patient normally, The application of internet plus technology is explored to build Remote Dedicated Doctor Platform of Ophthalmology (RDDPO) for online consultation, accessing to medication, medical services and advises orderly, and to help to further develop and apply the platform.Methods:In 2018, RDDPO was jointly developed.It has been modified and improved to the formulation of national medical policy and medical insurance policy and the outbreak of COVID-19 at the end of 2019, and it has been carried out comprehensive application verification during the prevention and control of COVID-19 epidemic.With a B/S structure, platform docks the official accounts of Wechat platform.On the server side, three J2EE frameworks of the integrated development, including Struts2, Spring and Hibernate are adopted; on the mobile client side, a combination of development tools including React-Native and Redux is used.Meanwhile, a micro-services Architecture (msA) of RESTful WebAPI is chosen at the business logic layer.The terminal data information is used to summarize the results of the platform's functional services for ophthalmic patients, such as remote diagnosis and treatment, online prescription, medicine delivery and health education, etc., and to make statistics on all kinds of data during the epidemic prevention and control period, so as to verify the operations management capabilities of the platform's functions.Results:During the period of COVID-19 epidemic prevention and control, the number of monthly visits of RDDPO increased from 1063 person times from September 1, 2019 to the end of September to 30 002 person times from February 1, 2020 to February 28, 2020.The proportion of diagnosis and treatment person times of this platform in the total number of diagnosis and treatment person times increased from 19.1% (203/1 063) to 92.3% (277/3 002), which can effectively avoid the risk of personal contact infection and meet the patients' medical needs.95.8% of the patients were very satisfied.95.8% of the patients were very satisfied.Conclusions:The RDDPO establishes a channel for doctor-patient communication during the epidemic, which can be considered as an important way to effectively address the needs of patients for medical treatment.
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Objective@#To survey the nutritional status of maintenance hemodialysis (MHD) patients and explore the role of oral nutritional supplement (ONS) in improving nutritional status among MHD patients.@*Methods@#From January 2016 to December 2017, one hundred and sixty-two MHD patients were selected randomly from hemodialysis centre of No. 2 peoples’ Hospital and assessed malnutrition incidence by objective score of nutrition on dialysis (OSND). Patients with malnutrition were divided into control group and ONS group randomly, patients in control group were conducted with traditional nutritional education, while patients in ONS group were conducted with ONS as a substitution of diet on the basis of control group. Finally, nutrition-related indexes were observed after 6 months.@*Results@#The malnutrition incidence of MHD patients was 66.7% (108/162) screening by OSND, the malnutrition incidence of patients over 60 years old was 82.8% (72/87), significantly higher than below 60 years old, 49.3%(37/75)(P<0.05). After 6-month intervention, the differences of nutrition-related physical and laboratory indexes between control group and ONS group was observed [BW:(53.2±6.5)kg vs. (50.7±6.0)kg, t=2.08, P=0.04; TSF:(10.3±3.1)mm vs. (9.0±3.0)mm, t=2.21, P=0.03; ALB:(39.6±5.6)g/L vs. (35.6±5.5)g/L, t=3.74, P<0.01; HB:(104.5±10.8)g/L vs. (99.7±12.6)g/L, t=2.13, P=0.04; TF:(2.0±0.5)g/L vs. (1.5±0.4)g/L, t=5.74, P<0.01], while opposite before intervention (P>0.05). There was no significant difference of OSND, GNRI and MIS scores between control group and ONS group before intervention [OSND:(24.1±1.9) vs. (25.3±1.7), t=3.46, P<0.01; GRNI:(90.4±5.4) vs. (92.4±4.5), t=2.09, P=0.04; MIS:(9.2±3.2) vs. (10.9±3.3), t=2.72, P=0.01], while opposite after intervention (P<0.05). Subjective feeling score of patients in ONS group was significantly higher than control group after intervention [(10.7±3.5)vs(8.9±4.0), t=2.49, P=0.01]. The extra cost in control group and ONS group was (688.8±254.6) RMB and (749.2±44.9) RMB respectively with no significance (t=1.72, P=0.09).@*Conclusion@#The malnutrition incidence is high among MHD patients, traditional nutrition education takes poor patients compliance, while ONS could improve patients′ compliance and nutritional status because of scientific formula and good taste, which provide another diet selection for MHD patients.
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A questionnaire survey was conducted among 14 senior residents or fellows who received the attending ward-round training in Department of General Internal Medicine of Peking Union Medical College Hospital during the period from January 2015 to September 2018. The attitudes and suggestions towards the training program, and to what extent they benefited from the program were assessed. Based on Visual Analog Scale (VAS), the mean score on general training improvement was (8.29±1.25). The self-reported benefits in decision making, teaching skills and leadership of residents participating in the attending ward-round training program were significantly higher than those receiving regular rotating and training (t=5.53, 7.78, 13.00; P<0.01). Eight respondents suggested that the training frequency could be increased from once a week to at least twice a week and 10 respondents wished that the supervisors could give feedback on their decision-making performance as quickly as possible after the attending round. The training program "attending ward-round by senior resident" facilitates to improve the competency of rotating senior residents, which may be worth extending to more hospitals.
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A questionnaire survey was conducted among 14senior residents or fellows who received the attending ward-round training in Department of General Internal Medicine of Peking Union Medical College Hospital during the period from January 2015 to September 2018.The attitudes and suggestions towards the training program,and to what extent they benefited from the program were assessed.Based on Visual Analog Scale (VAS),the mean score on general training improvement was (8.29± 1.25).The self-reported benefits in decision making,teaching skills and leadership of residents participating in the attending ward-round training program were significantly higher than those receiving regular rotating and training(t=5.53,7.78,13.00;P＜0.01).Eight respondents suggested that the training frequency could be increased from once a week to at least twice a week and 10 respondents wished that the supervisors could give feedback on their decision-making performance as quickly as possible after the attending round.The training program"attending ward-round by senior resident"facilitates to improve the competency of rotating senior residents,which may be worth extending to more hospitals.
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Objective@#To explore the related factors and working way of adolescent injury,and to provide a basis for the effective prevention and intervention of adolescent injury.@*Methods@#The framework of the questionnaire in this study was developed based on the Social Ecology Model. A cross-sectional survey was conducted on a valid sample of 4 309 students from 8 junior high schools and 8 senior high schools in Beijing and Zhongshan city respectively, using stratified random cluster sampling method. Students from grade 7 to grade 12 were invited to participate and investigated with injury prevalence.@*Results@#The incidence of adolescent injury by person and by person-time was 15.53% and 22.49% respectively, and there were differences by sex, age and regions(χ2=15.92，11.45，20.33，P<0.05). The occurrence of adolescent injury was affected by adolescent psychological behavior and social environment through different underlying pathways. The intrapersonal factors and perception of environmental safety showed direct effects on the adolescents’ injuries (effect size was 0.29 and 0.05 respectively, P<0.05).@*Conclusion@#Adolescent injury is associated with diverse factors and pathways in a synergistic and complex manner, suggesting that a three-dimensional and diverse strategy should be taken to intervene the injures.
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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.
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Objective To survey the nutritional status of maintenance hemodialysis (MHD) patients and explore the role of oral nutritional supplement (ONS) in improving nutritional status among MHD patients. Methods From January 2016 to December 2017, one hundred and sixty-two MHD patients were selected randomly from hemodialysis centre of No. 2 peoples’Hospital and assessed malnutrition incidence by objective score of nutrition on dialysis (OSND). Patients with malnutrition were divided into control group and ONS group randomly, patients in control group were conducted with traditional nutritional education, while patients in ONS group were conducted with ONS as a substitution of diet on the basis of control group. Finally, nutrition-related indexes were observed after 6 months. Results The malnutrition incidence of MHD patients was 66.7% (108/162) screening by OSND, the malnutrition incidence of patients over 60 years old was 82.8% (72/87), significantly higher than below 60 years old, 49.3%(37/75)(P<0.05). After 6-month intervention, the differences of nutrition-related physical and laboratory indexes between control group and ONS group was observed [BW:(53.2±6.5)kg vs. (50.7± 6.0)kg, t=2.08, P=0.04; TSF:(10.3±3.1)mm vs. (9.0±3.0)mm, t=2.21, P=0.03; ALB:(39.6±5.6)g/L vs. (35.6± 5.5)g/L, t=3.74, P<0.01; HB:(104.5±10.8)g/L vs. (99.7±12.6)g/L, t=2.13, P=0.04; TF:(2.0±0.5)g/L vs. (1.5± 0.4)g/L, t=5.74, P<0.01], while opposite before intervention (P>0.05). There was no significant difference of OSND, GNRI and MIS scores between control group and ONS group before intervention [OSND:(24.1± 1.9) vs. (25.3 ± 1.7), t=3.46, P<0.01; GRNI:(90.4 ± 5.4) vs. (92.4 ± 4.5), t=2.09, P=0.04; MIS:(9.2 ± 3.2) vs. (10.9±3.3), t=2.72, P=0.01], while opposite after intervention (P<0.05). Subjective feeling score of patients in ONS group was significantly higher than control group after intervention [(10.7±3.5)vs(8.9±4.0), t=2.49, P=0.01]. The extra cost in control group and ONS group was (688.8±254.6) RMB and (749.2±44.9) RMB respectively with no significance (t=1.72, P=0.09). Conclusion The malnutrition incidence is high among MHD patients, traditional nutrition education takes poor patients compliance, while ONS could improve patients′ compliance and nutritional status because of scientific formula and good taste, which provide another diet selection for MHD patients.
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BACKGROUND: Glucosamine and chondroitin sulfate are important compositions of cartilage tissues. Animal experiments have shown that glucosamine and chondroitin sulfate can treat osteoporosis synergistically, but the effect of their combinations with regular exercise on the bone and joint health of menopausal women is little reported. OBJECTIVE: To observe the effects of the combination of glucosamine, chondroitin sulfate and bone health exercises on skeletal and joint health in postmenopausal women by a random community trial. METHODS: From January to June 2016, 206 eligible postmenopausal women were selected from Bishan District of Chongqing and randomly divided into two groups by random digital method. The trial group received glucosamine and chondroitin sulfate (3 capsules/day, and bone health exercises (at least 3 times/week), while the control group did not receive special treatment. After 6 months of intervention, the bone mineral density, muscle strength of habitual knee, incidence of fracture, osteoarthritis and fall, and levels of symptom and function were compared between the two groups. The peak torque of extension and flexion of habitual knee at 0 and 60 (°) /s angular velocity was detected using Isomed 2000 Dynamometer. RESULTS AND CONCLUSION: (1) The bone mineral density and the peak torque of extension and flexion of habitual knee at 0 and 60 (°) /s angular velocity in the trial group were significantly higher than those in the control group at 6 months after treatment (P ＜ 0.05). (2) The incidence of fall in the trail group was lower than that in the control group at 6 months (17.00％ vs. 5.94％, P ＜ 0.05). The incidence of fracture and osteoarthritis had no significant difference between two groups (P＞ 0.05). (3) The self-ranking of joint pain, holding power of muscle, walking distance and joint stiff had significant differences between two groups (P ＜ 0.05). (4) These results indicate that glucosamine and chondroitin sulfate supplements combined with regular bone health exercises can improve bone mineral density and muscle strength, and prevent joint pain and stiff in postmenopausal women to some extent, thus preventing fall, fracture and osteoarthritis of postmenopausal women.
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Objective To describe the study design,the characteristics of participants as well as the pedigrees included in the baseline survey of Fujian Tulou Family Cohort Study.Methods Fujian Tulou Family Cohort Study was a prospective open cohort study with a biological sample bank.A baseline survey was conducted in Tulou areas of Nanjing county in Fujian province from 2015 to 2018,including questionnaire survey,physical and biochemical indicators examinations,and blood sample collection in adults aged ≥ 18 years.In addition,family relationship of the participants was also recorded.The pedigree information of the juveniles under 18 years old were also collected.Results The baseline survey included 2 727 individuals in two clans,of whom 2 373 (87.0％) were adults,and 2 126 participants completed questionnaires,physical examinations and biochemical tests.The average age of the 2 126 participants was (57.9 ± 13.3) years,with 39.4％ being males.The current smoking rates in male and female participants were 41.2％ and 2.1％,respectively.The corresponding rates of current alcohol consumption were 19.0％ and 2.6％.For common chronic diseases,the prevalence rates were 51.3％ for hypertension,9.7％ for diabetes and 26.7％ for hyperlipemia according to the self-reported disease diagnoses,health examination results and biochemical examination results in class 1Ⅱor Ⅲ hospitals.Based on the family relationship information and genealogical data,710 pedigrees were finally identified,consisting of 5 087 family members.The numbers of five,four,three,and two generations pedigrees were 3,88,238 and 381,respectively.The pairs of the first to the fifth degree relatives were 12 039,2 662,1 511,202 and 31,respectively.Conclusion The establishment of Fujian Tulou Family Cohort provides valuable resources for exploring the genetic risk factors,environmental risk factors and gene-environment interactions contributing to the risk of common chronic diseases.
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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.
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Objective To analyze the clinical features of secondary gout in glycogen storage disease type Ⅰ a (GSD Ⅰ a),so as to improve the awareness of this disease.Methods The clinical features,laboratory findings,treatments and prognosis of 5 GSD Ⅰ a patients with secondary gout who had been admitted to the Peking Union Medical College Hospital during 2006 to 2016 were collected and analyzed.GSD Ⅰ a 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.
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Objective To describe the study design,the characteristics of participants as well as the pedigrees included in the baseline survey of Fujian Tulou Family Cohort Study.Methods Fujian Tulou Family Cohort Study was a prospective open cohort study with a biological sample bank.A baseline survey was conducted in Tulou areas of Nanjing county in Fujian province from 2015 to 2018,including questionnaire survey,physical and biochemical indicators examinations,and blood sample collection in adults aged ≥ 18 years.In addition,family relationship of the participants was also recorded.The pedigree information of the juveniles under 18 years old were also collected.Results The baseline survey included 2 727 individuals in two clans,of whom 2 373 (87.0％) were adults,and 2 126 participants completed questionnaires,physical examinations and biochemical tests.The average age of the 2 126 participants was (57.9 ± 13.3) years,with 39.4％ being males.The current smoking rates in male and female participants were 41.2％ and 2.1％,respectively.The corresponding rates of current alcohol consumption were 19.0％ and 2.6％.For common chronic diseases,the prevalence rates were 51.3％ for hypertension,9.7％ for diabetes and 26.7％ for hyperlipemia according to the self-reported disease diagnoses,health examination results and biochemical examination results in class 1Ⅱor Ⅲ hospitals.Based on the family relationship information and genealogical data,710 pedigrees were finally identified,consisting of 5 087 family members.The numbers of five,four,three,and two generations pedigrees were 3,88,238 and 381,respectively.The pairs of the first to the fifth degree relatives were 12 039,2 662,1 511,202 and 31,respectively.Conclusion The establishment of Fujian Tulou Family Cohort provides valuable resources for exploring the genetic risk factors,environmental risk factors and gene-environment interactions contributing to the risk of common chronic diseases.
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Objective To investigate the predictive value of pregnancy-associated plasmaprotein-A (PAPP-A) and GRACE risk score for death and nonfatal myocardial infarction (combined endpoint) in AMI patients.Methods All AMI patients hospitalized in our department during July 2011 to July 2015 were included consecutively in this prospective study.Plasma PAPP-A were measured at admission.GRACE risk score was acquired with the application of GRACE risk score calculator.Patients were followed up for at least 1 year for any nonfatal myocardial infarction or MACE.Kaplan Meier survival study was analysed according to PAPP-A and GRACE score risk stratification respectively.A cutoff value of 3.0 ng/ml of PAPP-A was chosen from pilot work in this cohort.Results A total of 220 patients were enrolled in the study.The death and nonfatal myocardial infarction during follow-up were significantly higher in patients with PAPP-A≥3.0 ng/ml compared to patients with PAPP-A<3.0 ng/ml (15.7% vs.6.0%, log-rank χ2=5.684, P=0.017).The area under ROC curve of PAPP-A was 0.796(95%CI 0.696-0.896, P<0.01) and the ROC curve of PAPP-A GRACE risk stratification was 0.715 (95%CI 0.567-0.863,P<0.01).Subgroup analysis showed that death and nonfatal myocardial infarction during follow-up was significantly higher in patients with PAPP-A≥3.0 ng/ml compared to patients with PAPP-A<3.0 ng/ml in intermediate and low risk group by GRACE risk stratifcation (log-rank χ2=14.63,P<0.001).Conclusions PAPP-A could predict mortality and nonfatal myocardial infarction in patients with AMI.PAPP-A combined with GRACE risk score can better predict outcome than GRACE risk score alone in intermediate and low risk patients by GRACE risk stratifcation.
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Objective@#To assess the safety and curative effect of gasless endoscopic selective lateral neck dissection (GESLND) via an anterior chest approach for papillary thyroid carcinoma (PTC).@*Methods@#Eighteen patients with PTC(T1-2N1bM0, size<3.0 cm), having GESLND via an anterior chest approach, were included from November 2008 to December 2016.@*Results@#GESLND via an anterior chest approach was successfully performed in all 18 PTC patients (seven male and eleven female) with 83.3% of T1 and 16.7% of T2. The mean operative time of selective lateral neck dissection was 73 min (range 51-92 min). The mean of intraoperative bleeding was 61.1 ml (range 30-120 ml). No major complications occurred except one transient hypoparathyroidism. No residual thyroid glands were detected on ultrasonography and thyroglobulin was(0.73±0.16)ng/ml three months postoperatively. The median of follow-up was 54.5 months (range 6-104 months). No recurrence disease was observed in any patient on ultrasonography, computer tomography, thyroglobulin or selective iodine-131 scan during the follow-up period. The cosmetic result and functional preservation was excellent, when the assessments were performed three months postoperatively.@*Conclusion@#GESLND via an anterior chest approach is feasible and safe for selected PTCs, with superior appearance.