BACKGROUND:Pierre Robin Sequence is a congenital malformation which is characterized by micrognathia, glossoptosis and respiratory tract obstruction with or without cleft palate. SOX9, KCNJ2, Ptprs and Ptprf are probably connected with Pierre Robin Sequence. OBJECTIVE:To review the recent progress in the researches on the related genes about Pierre Robin Sequence. METHODS:A computer-based online search of CNKI database and PubMed database was performed to retrieve the relevant articles published from January 1999 to September 2014 with the key words of“micrognathia, Pierre Robin Sequence, mutation, gene locus”in Chinese and English, respectively. Final y, 58 articles were included for review after deleting unrelated and repetitive ones. RESULTS AND CONCLUSION:SOX9, KCNJ2, Ptprs and Ptprf are probably connected with Pierre Robin Sequence. Recently, the research on the genes connected with Pierre Robin Sequence focuses on 17q23-24, and smal sample cases are commonly seen. But, further large sample test and case analysis, as wel as related animal models are needed to analyze the role of these genes in the pathogenesis of Pierre Robin sequence, as wel as consequently, we can analyze the etiology and pathogenesis of Pierre Robin sequence.

OBJECTIVE:To explore optimization method and effect of clinical pharmacists on anticoagulants therapy plan for cancer patient with venous thromboembolism(VTE). METHODS:Clinical pharmacists participated in the whole process of antico-agulant therapy for one case of breast cancer complicated with VTE. Clinical pharmacists suggested patient to initially take low mo-lecular weight heparin sodium 0.6 ml,sc,qd;and then take Warfarin sodium tablet 3 mg,po,qd;initial plan and oral dosage form plan superimposed and alternated,and pharmaceutical care and medication education were also provided for the patient. RESULTS:Physicians adopted clinical pharmacist's suggestions,and the patient received anticoagulant therapy for 27 days and paclitaxel che-motherapy once. Coagulation function INR was 2.71;the patient didn't felt discomfort and then discharged from hospital. CON-CLUSIONS:The participation of clinical pharmacists in the optimization of individualized anticoagulant therapy and pharmaceutical care is able to promote rational drug use,prevent severe ADR in the clinic,guarantee the safety of drug use and improve medica-tion compliance.

Objective To evaluate the application of abbreviated protocol (AP) of magnetic resonance imaging (MRI) in screening of breast cancer with dense breast tissue.Methods Total 478 patients with dense breast tissue,who had negative X-ray mammography (MG),underwent dynamic contrast-enhanced MRI (DCE-MRI).The AP MRI,which consisted of first postcontrast subtracted (FAST) and maximum-intensity projection (MIP),and full diagnostic protocol (FDP) MRI images were analyzed by Breast Imaging Reporting and Data System (BI-RADS).Among 478 patients the histopathological diagnosis was available in 39 cases (41 breast lesions).With pathological diagnosis as gold standard,the sensitivity,specificity,positive predictive value (PPV) and negative predictive value (NPV) of AP and FDP in breast cancer detection were evaluated.Results In 41 breast lesions there were 16 malignant lesions and 25 benign lesions.FDP detected all 16 malignant lesions,including 9 cases of ductal carcinoma in situ,6 cases of invasive ductal carcinoma and 1 case of mucinous carcinoma;AP detected 15 out of 16 cases of breast cancer.The sensitivity of FDP and AP in detection of breast malignant lesions was 16/16 and 15/16,respectively (x2 =0.725,P =0.224).The specificity of FDP and AP was 92％ (23/25) and 76％ (19/25),respectively (x2 =6.327,P =0.012).Conclusion For women with dense breast tissue AP of MRI can be used in early screening of breast cancer.

To summarize the nursing experience of a patient with bilothorax after autolo-gous liver transplantation.The key points of nursing include:early identification of reexpan-sion pulmonary edema,the start of the hospital rapid response team to give efficient rescue;early identification of bilothorax,and maintenance of effective drainage combined with accurate anti-infection treatment;individual and stage lung rehabilitation training to promote patients lung rehabilitation;step anticoagulant therapy is safely adminis-tered under dynamic assessment of bleeding risk.Through careful treatment and nursing,the patient was discharged 63 days after surgery.

Objective To study classification of vascular sign of tiny ground glass nodules-like lung adenocarcinoma in HRCT, and explore its value to differentiate benign from malignant of the ground glass nodules(GGN).Methods 87 patients with tiny ground glass nodules-like lung adenocarcinoma examined on HRCT were retrospectively evaluated.According to the new pathological classification standard of lung adenocarcinoma,they were divided into three groups:(1)pre-invasive group(n=25),including 14 cases of atypical adenomatous hyperplasia(AAH)and 11 cases of adenocarcinoma in situ(AIS);(2)minimally invasive adenocarcinoma (MIA)group(n=35);(3)invasive adenocarcinoma(IAC)group(n=27).The lesions were divided into three types according to the grinding of glass composition proportion:Type A,pure ground glass nodules(pGGN);Type B,mixed ground glass nodules (mGGN)which contained glass composition≥50%;Type C,mGGN which contained glass composition < 50%.The vascular sign of GGN were divided into four types:Type 1,without vessels passing through the GGN,or vessels passing by GGN;Type 2,intact vessels passing through GGN,but vascular morphology is normal;Type 3,single vessels passing through GGN,and distorted,stiff vessels seen within GGN;Type 4,two or more vessels passing through GGN,and branches between vessels formed in GGN,and the diameter of vessel was irregular,partial enlargement.The relationship among the size of the GGN,content of the grinding of glass proportion and the vascular sign of GGN were analyzed both in axial images and reconstruction images.Results There were significant differences in size among the three groups(P=0.032,P=0.000,P=0.000).There were significant differences between content of the grinding of glass proportion and classification of vascular sign of GGN(P=0.000).Type 1 and type 2 vascular sign were dominant in the infiltrating former group,a total of 24 cases(96%).The incidence of type 3 and type 4 vascular sign in MIA group and IAC group was 60% and 74% respectively,and there were significant differences with the infiltrating former group(P=0.000,0.000).Further analysis indicated that type 3 was more commonly seen in MIA with comparison to type 4 which was more likely seen in IAC,the difference was statistically significant(P=0.043).Conclusion To study HRCT vascular sign of tiny ground glass nodules-like lung adenocarcinoma can improve the ability of the GGN benign and malignant diagnosis,provide reliable basis for clinical diagnosis and treatment.

Objective:To observe the changes of peri-implant tissue around the individualized abutment that was grinded from zirconia provisional crown in one year.Methods:In this research, a prosthodontic-driven virtual implant planning and immediate provisionalization were conducted in computer assisted design software. And computer-aided design/computer-aided manufacturing (CAD/CAM) techniques were used to fabricate the zirconia provisional crown and surgical guide template before surgery. The implant was accurately placed with the surgical guide, and the zirconia provisional crown was immediately delivered after surgery. Three months later, the implant osseointegration was completed, and zirconia provisional crown was prepared intraorally to generate customized zirconia abutment for final prosthesis. The study included 30 patients with single anterior tooth loss, including 18 males and 12 females, aged from 26 to 50 years old, and the mean age was (36.2±6.1) years old. The patients were from the Center of Oral Implantology, The First Affiliated Hospital of Zhejiang University Medical College from January 2017 to February 2018. After cementation of the final prosthesis, the cases were followed up at 6 and 12 months time intervals. Implant survival rate, probing depth, bleeding on probing, marginal bone level loss and papilla index score (PIS) were recorded in every appointment.Results:The survival rate of 30 implants was 100%, and the probing depths were less than 5 mm. The bone resorption at 6 and 12 months follow-up after the final delivery was 0 (0, 0) mm and 0 (-0.2, 0) mm, respectively, and the difference was not statistically significant ( P>0.05). The PIS was 3.0 (2.0, 4.0), 3.0 (2.8, 4.0) and 3.0 (3.0, 4.0) on the final delivery, 6 and 12 months after final delivery, respectively. Conclusions:Marginal bone level and bone loss were stable with this new implant clinical protocol at the one-year follow-up.

Objective:To explore the prognostic influencing factors of adult lymphoma-associated hemophagocytic syndrome (LAHS) based on multicenter data.Methods:The clinical data of 86 LAHS patients diagnosed in 9 medical centers of Huaihai Lymphoma Working Group from January 2015 to August 2020 were retrospectively analyzed. The optimal cut-off value of continuous variables was obtained based on MaxStat algorithm. Cox proportional hazard regression model was used for univariate and multivariate analyses. Kaplan-Meier method was used for survival analysis, and log-rank test was performed.Results:Among the 86 adult LAHS patients, 50 (58.1%) were males and 36 (41.9%) were females, the median age of the patients was 57 years old (19-76 years old), and the median overall survival (OS) time was 1.67 months (95% CI 0.09- 3.24 months). The most common pathologic type was diffuse large B-cell lymphoma (58 cases, 67.44%). Based on MaxStat algorithm, the optimal cut-off values of age, albumin, serum creatinine, lactate dehydrogenase, fibrinogen and platelet count were 64 years old, 30.1 g/L, 67 μmol/L, 1 045 U/L, 4.58 g/L and 72×10 9/L, respectively. Multivariate analysis showed that patient's age, lactate dehydrogenase, albumin and fibrinogen levels were independent influencing factors for OS (all P < 0.05). Conclusions:LAHS is dangerous and progresses quickly. Patients with age ≥ 64 years old, lactate dehydrogenase ≥ 1 045 U/L, fibrinogen ≥ 4.58 g/L and albumin < 30.1 g/L have poor survival.

Objective:To explore the prognostic value of lymphocyte subsets in adult hemophagocytic syndrome (HPS).Methods:A total of 172 adult HPS patients diagnosed in 8 medical centers from January 2013 to August 2020 were selected for the study, of whom 87 were male (50.6%, 87/172), and 85 were female (49.4%, 85/172), with 68 survivors and 104 deaths. The clinical data were summarized, and variables such as lymphocyte subsets, immunoglobulin characteristics and fibrinogen were retrospectively analyzed, and the correlation between the mentioned variables and patient prognosis was analyzed. The optimal cut-off values of continuous variables were calculated by MaxStat, and the prognostic factors of HPS patients were screened based on the Cox proportional hazard regression model.Results:The median age of HPS patients was 56 (42, 66) years old, and the 5-year cumulative survival rate was 37.4% (37.4/100). The median age, platelet and albumin were 48 (27, 63) years, 84×10 9/L and 32.3 g/L in the survival group, and 59 years, 45.5×10 9/L, and 27.3 g/L in the death group, respectively. The differences between the two groups was statistically significant ( Z=?3.368, P=0.001; Z=?3.156, P=0.002; Z=?3.431, P=0.001). Patients with differentiated cluster 8+(CD8+)<11.1%, CD3+<64.9%, CD4+>51%, and CD4/CD8 ratio>2.18 had poor prognosis (χ 2=7.498, P=0.023; χ 2=4.169, P=0.041; χ 2=4.316, P=0.038; χ 2=9.372, P=0.002). Multivariable analysis showed that CD4/CD8 ratio, age, fibrinogen and hemoglobin were independent prognostic factors in HPS patients ( HR=2.435, P=0.027; HR=5.790, P<0.001; HR=0.432, P=0.018; HR=0.427, P=0.018). Conclusion:Peripheral blood lymphocyte subsets can be used to evaluate the prognosis of patients with HPS; CD4/CD8 ratio, age, fibrinogen, and hemoglobin are independent prognostic factors in HPS patients.

Objective:Factors influencing the prognosis of hemophagocytic lymphohistiocytosis (HLH) in adults were analyzed based on multicentric data.Methods:Clinical data of 124 adult patients with HLH diagnosed in eight medical centers in the Huaihai Lymphoma Working Group from March 2014 to July 2020 were collected. The optimal truncation value of continuous variables was obtained based on the Maxstat algorithm, X-Tile software, and restricted cubic spline. Cox proportional risk regression model was used to construct the adult HLH risk prediction model, and the visualization of the model was realized through the histogram. The bootstrap resampling method was used to verify the model, C-index and calibration curve was used to verify the histogram, and the prediction accuracy was checked. Kaplan-Meier analysis was used to calculate the survival rate and draw the survival curve. Furthermore, the differences between groups were tested by log-rank.Results:The median age of the 124 patients was 55 (18-84) years, including 61 (49.19%) males. The most common etiology was infection. Serum ferritin increased in 110 cases (88.71%) , hepatosplenomegaly in 57 cases (45.97%) . Of the 124 patients, 77 (62.10%) died, and the median survival time of the patients was 7.07 months. Univariate results showed that the prognosis of adult HLH was influenced by sex, age, fibrinogen, serum creatinine, alanine aminotransferase, and albumin ( P<0.05) . The results of multivariate analysis showed that gender, platelet, albumin, alanine aminotransferase, and treatment regimens were independent influencing factors for prognosis. Based on the above five risk factors, the prediction model of the histogram was established, and the C-index of the model was 0.739. Finally, the calibration chart showed good consistency between the observed and predicted values of HLH. Conclusion:The prognosis of the adult hemophagocytic syndrome is influenced by many factors. Gender, platelet, albumin, alanine aminotransferase, and treatment regimens are independent risk factors. Therefore, the established histogram provides a visual tool for clinicians to evaluate the prognosis of adult HLH.