The diagnosis and treatment resources for rare diseases in China are highly imbalanced. The basic diagnosis and treatment capabilities are weak, the diagnosis period for patients is long, and the rates of missed diagnosis and misdiagnosis are relatively high. The establishment of a hierarchical diagnosis and treatment system is the inevitable approach to enhancing the diagnosis and treatment standards of rare diseases. Currently, the implementation of the domestic hierarchical diagnosis and treatment system for rare diseases still confronts numerous challenges, such as ambiguous referral standards and processes of primary medical institutions, and ineffective information interaction among institutions at all levels. Thus, it is essential to facilitate high-level information construction for the hierarchical diagnosis and treatment of rare diseases. This paper explores the process of constructing a multidisciplinary joint remote diagnosis and treatment platform and a health management platform through informatization, with the hope of establishing two closed loops of digital diagnosis and treatment services and health follow-up management for patients with rare diseases, as well as achieving timely diagnosis and lifelong health management for patients. It integrates and optimizes auxiliary diagnostic tools, promotes the rapid dissemination of rare disease diagnosis and treatment experiences to the grassroots, enhances the information construction level of the hierarchical diagnosis and treatment system, and endeavors to address the practical predicament of weak diagnosis and treatment capabilities of rare diseases in grassroots medical institutions. Additionally, this paper proposes an essential approach for multi-dimensional independent innovation to guide the popularization of efficient and high-quality rare disease diagnosis and treatment services. By encompassing innovating the rare disease diagnosis and treatment collaboration network and multidisciplinary diagnosis and treatment model, facilitating the application of the latest biomedical and informatics technologies to the grassroots, and constructing a national intelligent data platform for rare disease innovation, a new model for rare disease services with Chinese characteristics will be established. This will significantly enhance the medical treatment level of rare diseases in China and strive for more benefits for patients.

Objective Based on mosq-ovitrap monitoring method of Aedes albopictus， to determine the spatial and temporal distribution of Aedes albopictus in Songjiang District and provide scientific evidence for prevention and control of mosquito-borne diseases. Methods From May to October， 2018‒2020， density of Aedes albopictus was monitored by using the mosq-ovitrap method. Sub-districts and towns were used as the monitoring units and the difference in mosquito ovitrap index （MOI） was compared by seasons， years and habitats. Results The total number of positive mosquito ovitraps was 1049 in 2018‒2020， and the average MOI was 8.10. Four sub-districts/towns in the urban area and the northern area had the relatively high MOIs. Moreover， MOIs in residential and external environments were higher than other habitats. In addition， MOI of Aedes albopictus decreased over years from 2018 to 2020. Conclusion Aedes albopictus is widely distributed in Songjiang District. Although the number of mosquito decreased over years after the implementation of mosquito control measures， reproduction of mosquito remains excessive in some areas and seasons. It is necessary to perform targeted control measures based on comprehensive monitoring methods.

Objective:To explore the safety and feasibility of optimized pathological evaluation system of donor's kidney and modified surgery during adult dual kidney transplantation(DKT)and evaluate its effectiveness to provide more alternative protocols for kidney transplantation from extended criteria donors.Methods:DKT was performed in 10 recipients using the same protocol from June 2019 to May 2021.And retrospective reviewing was performed for clinical data, including characteristics of donors and recipients, optimized pathological evaluation system, modified surgery, treatment regimens, complications and follow-ups.Results:There were 8 male and 2 female donors with an age of(57.9±12.8)years and BMI(24.1±4.1)kg/m 2.The percentage of DCD was 70% and DBD 30%.The serum creatinine before procurement was 107.6(93.3-163.5)μmol/l.Zero-point puncture biopsy was performed for both kidneys and optimized pathological evaluation system was implemented(Banff criteria & Remuzzi score). The pathological results indicated that glomerular sclerosis for left and right kidneys were 2.0(1.5-2.0)and 1.5(1.0-2.0). And Remuzzi score for left and right kidneys were(4.4±1.2)and(3.6±1.5)points respectively.All recipients were male with an age of(43.1±9.0)years and BMI(22.2±1.9)kg/m 2.All PRAs were negative pre-operation.Modified surgery was performed in all recipients(two kidneys were implanted outside iliac vessels without patch and artery of superior kidney was anastomosed to internal iliac artery). Operative duration was(195±54.3)min and serum creatinine before discharge 125.0(102.0-199.0)μmol/L.Renal dynamic scintigraphy indicated that glomerular filtration rate was(30.0±8.2)ml/min for left kidney and(29.2±13.9)ml/min for right kidney.MRA results indicated that morphologies of renal arteries and veins were regular.The time between operation and discharge was(22.4±4.7)days.Compared with SKT, serum creatinine before discharge of DKT was lower and DGF incidence of DKT was higher without statistical significance.The time between operation and discharge was longer for DKT than that for SKT( P<0.05). The complications consisted of 20% donor derived infection(DDI)and 50% DGF.And there was no surgical complication associated with vessels and ureter.Renal function remained stable during 6-month follow-ups. Conclusions:Optimized pathological evaluation system of donor's kidney and modified surgery during adult dual kidney transplantation are both safe and feasible.The postoperative function of transplanted dual kidney is successfully restored.However, long-term follow-ups are required for evaluating its effectiveness.

Objective To evaluate the feasibility and validity of near infrared fluorescence imaging with indocyanine green in sentinel node biopsy for cN0 oral carcinoma.Methods Thirty cases of previously untreated oral carcinoma staged cT1-3 N0M0 were enrolled in this study.1 ml of indocyanine green (25 mg/ 5 ml) was injected both around the primary tumor in a 4 quadrant pattern and in the base of the tumor before skin incision.After elevation of the platysma flap and posterior retraction of the sternocleidomastoid muscle,fluorescence images were taken with a near infrared fluorescence detector until the hotspots were captured,then the hotspot lymph nodes were removed.Lymph nodes identified with fluorescent hotspots and verified in vivo were defined as sentinel nodes,and they were harvested and sent together with neck dissection specimen for pathologic study.Results Sentinel nodes were successfully harvested in all 30 cases.The number of sentinel nodes per case varied from 1 to 9,with an average number of 3.4.Routine pathology demonstrated that occult metastasis was exclusively found in the sentinel nodes in 5 cases (16.67％),and all the other lymph nodes were free from metastasis.No tracer associated adverse effects occurred in this series.Conclusion Near infrared fluorescence imaging with indocyanine green has a high detection rate in sentinel node biopsy for cN0 oral/oropharyngeal carcinoma and the sentinel nodes can evaluate the cervical metastatic status accurately.It is an easy,feasible and promising method,which is worthy of further investigation.

[ABSTRACT]OBJECTIVEThis study was designed to compare the quality of life between patients who
underwent a tongue reconstruction with radial forearm flap (RFF) and infrahyoid myocutaneous flap (IHMCF) after hemiglossectomy for their tongue cancers, and to figure out an optimal reconstructive method for the defects resulted from hemiglossectomy.METHODSA non-randomized case-control study was performed on 24 patients with tongue squamous cell carcinoma who underwent a standard hemiglossectomy combined with perfectly tongue reconstruction from June 2005 to June 2012. All of the cases were without tongue base invasion. Of the 24 cases, 19 had T2 disease, 5 had T3 disease, and they were divided into RFF group (n=10) and IHMCF group (n=14). The quality of life were evaluated one year after operation using EORTC-QLQ30 and FACT-H&N35 and compared between the two groups.RESULTSThe scores were comparable between the two group with regard to all domains of EORTC-QLQ30,with all P values>0.05.The scores of swallowing(P=0.005), speech (P=0.008), teeth (P=0.014), and cough (P=0.009) domains were significantly higher in IHMCF group than in RFF group, with P value of 0.005, 0.008, 0.014 and 0.009 respectively, while the other domains of FACT-H&N35 were comparable between the two groups, with allP values>0.05.CONCLUSIONOverall quality of life was similar in the two groups. Oral function domains were better in IHMCF group than in RFF group. When guarantee of flap survival is available, IHMCF could be used as a good alternative flap to RFF in tongue reconstruction after hemiglossectomy.

Objective: To study the effect of berberine (BR) on ventricular remodeling in experimental rats with myocardial infarction (MI) and its mechanisms.
Methods: The MI model of experimental rats was established by ligation of the left anterior descending coronary artery and the MI animals were randomly divided into 3 groups: MI+BR group, in which the rats received BR 20 mg/kg.d, Sham group and MI group, the rats in those 2 groups received the same volume of normal saline. All animals were treated for 8 weeks. The cardiac function and structure were assessed by echocardiography, cardiac interstitial collagen deposition was evaluated by Masson stain, the myocardial cell apoptosis was detected by Tunel method, and the activation of nuclear factor (NF-κB) was also examined.
Results: For echocardiography, MI group had enlarged left ventricular end diastolic diameter (7.28 ± 0.29) mm than Sham group (6.86 ± 0.36) mm,P<0.05, but it decreased in MI+BR group (6.89 ± 0.99) mm,P>0.05. MI group had increased left ventricular end systolic diameter (5.88 ± 0.33) mm than Sham group (4.61 ± 0.31) mm, but it decreased in MI+BR group (4.68 ± 1.17) mm, allP< 0.01. MI group showed increased left ventricular posterior wall compensatory hypertrophy (1.81 ± 0.85) mm than Sham group (1.67 ± 0.16 mm),P<0.05, while in MI+BR group, it was deereased to (1.65 ± 0.14) mm. MI group presented decreased LVEF (45.77 ± 3.17) % than Sham group (67.28 ± 4.15) %, but it increased in MI+BR group (64.64 ± 5.82) %, allP<0.01. For Masson stain, cardiac interstitial collagen deposition in MI group (11.39 ± 0.45) % was higher than Sham group (2.65 ± 0.45) %, but less in MI+BR group (7.00 ± 0.87) %, allP<0.01. For Tunel examination, the myocardial cell apoptosis index was increased in MI group (21.31 ± 2.34) than Sham group (0.99 ± 0.38), but decreased in MI+BR group (14.15 ± 1.62), allP<0.01. For NF-κB activation study, the nuclear protein p65 content was higher in MI group (0.14 ± 0.02) ng/ml than Sham group (0.06 ± 0.01) ng/ml, but lower in MI+BR group (0.10 ± 0.02) ng/ml, allP<0.01.
Conclusion: Application of BR may improve the ventricular remodeling and cardiac function in experimental MI rats, it might be because of BR partially inhibit NF-κB activation, reduce collagen deposition and help anti-apoptosis in myocardial cells.

Objective To evaluate if Ⅵ region lymph nodes metastasis status can be a reliable indicator in prediction of lateral lymph nodes metastasis in papillary thyroid carcinoma (PTC) with negative cervical lymph nodes (cN0).Methods Retrospectively reviewed the medical records from January 2003 to October 2011 of 73 patients with PTC who underwent prophylactic lateral neck dissection (Ⅱ-Ⅴ region or Ⅱ-Ⅳ region).The relationship between cervical lymph nodes metastasis and lateral lymph nodes metastasis was assessed.Results The rate of lateral lymph nodes metastasis was 16.4％(12/73).The rate of Ⅵ region lymph nodes metastasis was 42.5％(31/73).Multivariate analysis showed that Ⅵ region lymph nodes metastasis was the risk factor of lateral lymph nodes metastasis in cN0 patients with PTC(OR =7.3,P=0.020).Conclusion Ⅵ region lymph nodes metastasis status can be a reliable indicator of lateral lymph nodes metastasis in cN0 patients with PTC.

The effect of cognition need on information seeking behaviors of information users and the difference in behaviors of information users were investigated in 184 undergraduates from the same grade with different back-grounds of subjects using the cognition need scale and undergraduates information-seeking behaviors scale ,respec-tively.The data were analyzed by descriptive statistical analysis, correlation analysis, rank test, and multivariate linear regression analysis , respectively , showing that the cognition need is significantly correlated with the informa-tion seeking behaviors of undergraduates in terms of information evaluation, information searching, information management and information use .

Objective To compare the treatment outcome of underwent surgery plus radiotherapy and radiotherapy/chemoradiotherapy plus salvage surgery for stage Ⅲ/Ⅳ laryngeal carcinoma,to investigate an optimized treatment for the patients of stage Ⅲ/Ⅳ laryngeal carcinoma.Methods Clinical data from 103 patients with stage Ⅲ (39 cases) or stage Ⅳ (64 cases) laryngeal carcinoma were retrospectively analyzed.The patients were divided into surgery plus radiotherapy group (S±R,46 cases) and radiotherapy/chemoradiotherapy plus salvage surgery group (R±S,57 cases).Overall survival,relapse free survival,and laryngeal preservation rate were used to compare the treatment outcome between two groups.Multivariate regression models were used to analyze the independent factors for survival and laryngeal preservation rate.Results Survival rate was higher in S±R group than in R±S group [2 year overall survival/relapse free survival 74.7 ％ (34/46) / 72.4 ％ (33/46) vs 46.4 ％ (26/57) / 40.9 ％ (23/57),P ＜ 0.05].Laryngeal preservation rate was higher in R±S group than in S±R group [93.0 ％ (15/46) vs 32.6 ％ (53/57),P ＜ 0.05].Multivariate analysis demonstrated that treatment modality and T stage were independent factors for long-term survival,while treatment modality was the only an independent factor for laryngeal preservation rate.Conclusions Surgery plus radiotherapy result in better survival and lower laryngeal preservation rate than radiotherapy/chemoradiotherapy plus salvage surgery in treatment of stage Ⅲ/Ⅳ laryngeal carcinoma.Surgery plus radiotherapy should be the first choice for treatment of locally advanced laryngeal carcinoma.Improvement of the quality of life could be achieved by laryngeal preservation surgery and phonation reconstruction procedures.

ObjectiveTo research the endothelial dysfunction and early changes in arterial elasticity in patients with hyperlipidemia and effects of atorvastatin on these changes.Methods40 patients with hyperlipidemia but without any treatment in our hospital were selected as a study group,and 30 healthy people were selected as control group.Use the Flow mediated dilation,FMD detection which bases on the echo-trackingtechnology,eTRACYING to evaluate the right brachial atherosclerosis parameters and vascular diastolic parameters,including the pressure strain elastic modulus(Ep) ;stiffness index (β) ;compliance (AC) ;FMDs and FMDd.The study group take atorvastatin 20mg per day,then retested above parameters and TC,LDL-C after 12 weeks and analyzed all parameters.ResultsThe values of β and Ep in study group are significantly higher than the control group (all P ＜ 0.001 ),but AC;FMDs and FMDd are significantly lower than the control group( all P ＜ 0.001 ).The results of the study group after the treatment of atorvastatin are as follwing:TC,LDL-C,β and Ep are lower than before,AC,FMDs and FMDd are higher than before;and the differences are of significance in statistics ( all P ＜ 0.001 ).ConclusionHyperlipidemic patients had shown the vascular endothelial injury and vascular early hardening before the abnormal changes in intimal,but the atorvastatin intervention could reverse these changes.