Objective To determine the clinical characteristics of neonatal asymmetric crying facies and to review the latest progress in clinical research of this condition. Methods Clinical and laboratory data of a case of neonatal asymmetric crying facies syndrome admitted to Peking Union Medical College Hospital in March, 2013 was reported. Clinical charateristics, chromosome abnormalities, treatment and prognosis of neonatal asymmetric crying facies reported in China were analyzed, and pertinent literatures in China Knowledge Resource Integrated Database and CQVIP Database were reviewed. Results Eighteen Chinese reports on this syndrome were retrieved and 48 patients, 31 males and 17 females including our patient, were analyzed. Twelve patients had neonatal asymmetric crying facies and 36 patients had neonatal asymmetric crying facies syndrome. Anomalies in these children included malformations of the heart (26 cases, 72.2%), ear (11 cases, 30.6%), gastrointestinal tract (4 cases, 11.1%), cleft palate (2 cases, 5.6%) and fingers (2 cases, 5.6%). Standard chromosome analysis in three studies was normal. In one case, chromosome 22q11.2 microdeletion was not found using flucrescence in situ hyloridization analysis. There were few studies with long-term follow-up in China. Conclusions Neonatal asymmetric crying facies are complicated with a high rate of other malformations. Identification of associated malformations and close follow-up are required, and intervention should be carried out earlier so as to ensure a good outcome.

OBJECTIVE:To investigate general regularity and characteristics of adverse drug reactions/events(ADR/ADE)in-duced by Dezocine injection,and to provide reference for safe and rational drug use in clinic. METHODS:Retrieved from Jiangsu Province ADR Database during Jan. 2009-Aug. 2016,ADR/ADE induced by Dezocine injection was analyzed statistically in re-spects of patient's gender and age,usage and dosage,organs/systems involved in ADR,clinical manifestations and outcome. RE-SULTS:Among 1290 ADR/ADE induced by Dezocine injection,there were 40 cases of severe ADR/ADE(3.10％);medical insti-tutions were main reporting units (1276 cases,98.81％). In ADR/ADE cases,female was more male,with ratio of male to fe-male was 1:1.45. ADR/ADE mostly occurred in 45-65 age group(43.49％). Intravenous medication was main route of administra-tion (1137 cases,88.14％). Initial dose of 577 cases was excessive dose (50.75％). ADR/ADE mainly involved gastrointestinal system (54.99％),central and peripheral nervous system (24.72％) and systemic lesions (5.20％). Severe ADR/ADE manifested as anaphylactic shock,palpitation and dyspnea,etc. But anaphylactic shock was not mentioned in drug package inserts. 1283 ADR/ADE(99.46％)were cured or improved . CONCLUSIONS:It is necessary to strengthen drug safety reevaluation of Dezocine injec-tion after being on the market,improve drug package inserts and enhance patients education as so as to reduce and avoid the occur-rence of ADR/ADE.

Objective To study the clinicopathological characteristics and diagnosis of hepatic perivascular epithelioid cell tumour (PEComa).Methods Retrospective analysis was made on the clinicopathological and immunohistochemical phenotype of the 9 PEComa cases.Results The average age was 39 years,male to female ratio was 1∶ 8.Tumours were grossly well-circumscribed and solid.The maximum diameter were from 1.0 cm to 9.0 cm,with the average of 3.6 cm.The tomours were of nested architecture and composed of uniform epithelioid cells with clear cytoplasm and round nuclei.A subset of tumours was dominated by spindle cells.Thick-wall blood vessels and fat cells were common.Tumour cells were positive for HMB45,MelanA,and SMA (8/8,7/7,4/4).Some of them were positive for S-100 (4/ 8).All of the 9 cases were negitive for hepatocyte (0/9).Ki-67 index of the tumours were below 10％ (9/ 9).All 9 cases were followed up from 3 months to 6 years,all were alive and recurrence free.Conclusion Hepatic PEComa is often seen in adult women with some specific histological patterns and immunophenotypes.Definite diagnosis of PEComa can be made by combining clinicopathological characteristics with a panel of immunohistochemical marks.PEComas grow slowly,often responds to surgical resection and rarely recur.

Objective:To compare three nutritional screening tools in predicting dialysis-related pro tein-energy wasting(PEW) among maintenance hemodialysis(MHD) patients and to find a more rapid,accurate,and feasible screening tool.Methods:The nutrtional risk screening 62002 (NRS 2002),7-point subjective global assessment(SGA) and malnutrition inflammation scores (MIS) were compared among 205 MHD patients.Correlations between the serum biochemistry,anthropometry and different screening tools were analyzed,and comparisons were made with established guidelines by International Society of Renal Nutrition and Metabolism (ISRNM) for PEW.Results:A total of 31.2％ of HD patients had PEW by ISRNM criteria,whilst using NRS 2002,7-point SGA and MIS,58％,40.8％ and 46.8％ of MHD patients exhibited PEW respectively.The results of three screening tools were closely related to those by serum biochemistry and anthropometry (P ＜ 0.05),but the composite correlation coefficient between the screening tools for PEW and the objective nutritional index commonly used was higher in NRS 2002(r =0.787,P ＜ 0.001) than in 7-Point SGA or MIS.NRS 2002 had higher sensitivity and accuracy values (87.5％ and 59.1％,respectively) in MHD Patients.Conclusion:MIS,7-PointSGA and NRS 2002 are valid tools for nutrition screening of dialysis patients,and NRS 2002 may be the best one.

Objective To evaluate the use of bioelectrical impedance phase angle (PhA) for predicting protein-energy wasting (PEW) in hemodialysis patients.Methods This cross-sectional study involved 144 patients who were on maintenance hemodialysis (MHD) in the blood purification centers of two tertiary hospitals in Guangzhou,where the body impedance analysis (BIA) was performed after the hemodialysis procedure and the prevalence of protein-energy wasting (PEW) was investigated by the 7-point subjective global assessment (SGA) among.Nutritional indices of the patients with PEW were compared with the well-nourished patients to identify independent variables that can predict PEW and determine the cut-off value of PhA as a predictor.Results The patients with PEW had significantly lower PhA values than the Non-PEWpatients (t =8.68,P=0.00).PhA was positively related with the nutritional indices reflecting function of body proteins or muscles,such as serum albumin (rs=0.25,P=0.00),prealbumin (rs=0.31,P=0.00),free fat mass index (rs=0.25,P=0.00),and mid-arm muscle circumference (rs =O.44,P =O.00).However,the correlations of PhA with fat mass (rs =0.04,P=0.66) and body fat percentage (rs =-0.04,P=0.66) were no statistical significance.Multivariate logistic regression analysis revealed that PhA (OR =4.48,P =0.00) and body mass index (OR =1.59,P =0.00) were independent predictors of PEW,but PhA was more potent than BMI.Receiver operating characteristic curve analysis suggested that the optimum PhA cut-off point for predicting PEW was 4.6.Conclusion PhA can be a useful independent predictor for PEW in hemodialysis patients and its cut-off value is 4.6.

Objectives: To study the significance of SATB2 expression in colon adenocarcinoma and its differential diagnosis function for ovarian metastatic adenocarcinoma. Methods: Immunohistochemistry was used to detect the expression level of SATB2 in 130 cases of colon adenocarcinoma. The relationship between the positive rate of SATB2 expression in colon cancer and clinicopathological factors was studied. Forty-seven cases of pancreatic ductal adenocarcinoma, 22 cases of cholangiocarcinoma, 46 cases of gastric adenocarcinoma, and 53 cases of ovarian mucinous adenocarcinoma were studied respectively. Results: The positive expression rate of SATB2 in 130 cases of colon adenocarcinoma is 73.8%. The SATB2 expression bears no correlation with gender, age, tumor size, location, histology type, lymph node metastasis, staging, local recurrence, distant metastasis, survival, Kras mutation, and microsatellite stability. The expression rate of SATB2 is significantly higher in well differentiated and moderately differentiated colon adenocarcinoma than that in poorly differentiated adenocarcinoma (χ²=12.804, P=0.002); the expression rate in the cases without tumor deposit is significantly higher than in cases with tumor deposit (χ²=6.485, P=0.011). There was no positive expression in all cases of pancreatic adenocarcinoma, cholangiocarcinoma, gastric adenocarcinoma, nor in ovarian mucinous adenocarcinoma. Conclusion The expression of SATB2 is associated with the differentiation of colon adenocarcinoma and the formation of tumor deposit. SATB2 can be used as an effective tumor marker for identifying colorectal cancer ovarian metastases.

Objective: To investigate the expression of immunomarkers CK7, CK20, CK17, CDX2, MUC1 and MUC2 in primary adenocarcinoma of the ampulla of Vater, to explore the role of these markers in the histopathologic subclassification of ampullary carcinoma; and to provide biologic basis for precision treatment of patients with different types of ampullary carcinoma. Methods: Forty-two cases of primary ampullary carcinoma were collected at Peking University People′s Hospital, from 2012 to 2018 year. There were 22 males and 20 females. Aged range 42 to 88 years old, with mean aged (62±11) years. Among the patients, 6 was high differentiation, 19 median differentiation, and 17 low differentiation. Immunohistochemical studies on the expression of CK7, CK20, CK17, CDX2, MUC1 and MUC2 were performed in 42 cases of primary ampullary carcinoma. The relationship between different ampullary carcinoma subtypes and clinicopathologic survival data was analyzed using SPSS 16.0 statistical software. Results: Three histopathologic subtypes were observed. Among 42 cases, 8(19.0%)were classified as intestinal subtype, which showed a positive expression rate of 8/8 for both CK20 and CDX2, and 5/8 for MUC2. Both CK7 and CK17 were weakly expressed in one case (1/8). No expression was observed for MUC1 in this subtype. Twenty-two (52.4%,22/42) cases were classified as pancreaticobiliary subtype, which showed a positive expression rate of 100.0%(22/22) for both CK7 and MUC1, and 90.9% (20/22) for CK17. No expression was observed for CK20, CDX2 and MUC2.The remaining 12 (28.6%) cases were classified as mixed subtype, which showed variable expression patterns. The expression frequencies of these 6 immunomarkers in different subtypes of ampullary carcinoma did not correlate with various clinicopathologic factors such as patient gender and age, tumor size, histologic differentiation, pancreatic and bile duct invasion, or the depth of duodenal invasion. However, stage Ⅲ+Ⅳ diseases were more commonly seen in pancreaticobiliary type (63.6%,14/22) than intestinal type (2/8) and mixed type (3/9; χ²=6.508, P=0.039). Follow-up data showed a trend of better survival rate for patients with intestinal subtype than those with mixed and pancreaticobiliary subtypes. Conclusions Ampullary carcinoma can be subclassified into three different subtypes using a panel of six immunomarkers, especially for the identification of subtypes of poorly differentiated carcinoma. CK7, CK17 and MUC1 are major markers of pancreaticobiliary subtype, whereas CK20, CDX2 and MUC2 are useful markers for intestinal subtype. The mixed subtype variably expresses these markers. The prognosis of patients with intestinal subtype appears better than that of pancreaticobiliary and mixed subtypes. Accurate subtyping of ampullary carcinoma is clinically important to patient management and prognosis assessment.