ObjectiveTo explore the clinical features and diagnosis of one Kennedy's disease.MethodsOne patient was clinically diagnosed as Kennedy's disease on the basis of the clinical features including slowing progression of disease, symptoms, nervous system signs, electromyography and nerve conduction velocity results and family history. His CAG number from the repetitive CAG sequence in the first exon of androgen receptor gene was determined using PCR.ResultsThe progression of Kennedy's disease is usually much slower. The CPK and testosterone levels increased in patient. EMG revealed neurogenic injury. The numbers of CAG region of the first exon of androgen receptor gene were 51 in the patient.ConclusionDespite its relatively typical manifestations, the definite diagnosis of Kennedy's disease should be made by detecting the number of CAG from the repetitive CAG region in the first exon of androgen receptor gene.

Background and purpose:Due to the lack of cost-effective pre-treatment predictors for advanced cervical squamous cell carcinomas treated with concurrent chemoradiotherapy (CCRT), both baseline circulating CD4+CD25+CD127Low/- regulatory T cell (Treg) count and serum squamous cell carcinoma antigen (SCC-Ag) level were measured for this feasibility study. Methods: Peripheral blood samples were collected from 44 patients with stageⅡB-ⅣA cervical squamous carcinomas before CCRT. Flow cytometry immunophenotyping and enzyme-linked immunosorbent assay were used for circulating CD4+CD25+CD127Low/-Treg count and serum SCC-Ag level testing,respectively. Clinical and pathological characteristics were retrospectively reviewed to analyze the predictive value of the 2 indexes. Results:The baseline circulating CD4+CD25+CD127Low/-Treg count was lower in the patient group with positive treatment response than in the group with negative response [(8.78±2.80)%vs (10.95±2.56)%, P<0.05], and the serum SCC-Ag level showed no signiifcant difference between the 2 groups. No correlation was detected between these 2 markers (Spearman’rho=-0.093, P=0.540). Determined by plotting receiver operating characteristic curves, the best cut-off points were 9.76%for circulating CD4+CD25+CD127Low/-Treg count and 9.50 ng/mL for serum SCC-Ag level, respectively. Univariate analysis showed that pretherapeutic circulating CD4+CD25+CD127Low/-Treg count (OR=1.901, 95%CI:1.112-3.219, P=0.017), but not serum SCC-Ag level (OR=0.998, 95%CI:0.001-4.253, P=0.897), was predictive of clinical response to CCRT. Multivariate Logistic regression analysis revealed that pre-treatment CD4+CD25+CD127Low/-Treg count was an independent predictor for clinical response to CCRT (OR=3.115, 95%CI:1.253-7.742, P=0.014). Conclusion:Pretherapeutic circulating CD4+CD25+CD127Low/-Treg count is a feasible method to predict clinical response to CCRT in patients with advanced cervical squamous cell carcinomas.

Foreign specialist physician system has been implemented for more than 100 years. China piloted its own specialist physician system from 2004. The article compared foreign specialist physician system with China’s pilot system. Combined with TCM clinical characteristics and laws, the article also discussed to set up Chinese medical specialist system which is in accordance with both international conventions and TCM laws of development as well as docking the pilot system.

Objective To compare the scores from the simpliifed COMFORT scale with those from the bispectral index (BIS) and analyze the variation among different evaluators in children in an intensive care unit, and to investigate the reliability;validity of simpliifed COMFORT scale. Methods Twenty children between 3 and 16 years of ages on mechanical ventilation and sedation were simultaneously classiifed based on the BIS and the simpliifed COMFORT scale evaluated by 4 independent evalua-tors. The Kappa test was performed and the correlation between the two methods (Pearson correlation) was tested. Results In to-tal, 70 observations were performed on 20 patients. Based on the Kappa coefifcient, the agreement among evaluators ranged from 0.62 to 0.77 (P<0.001). There was a correlation between the BIS and the simpliifed COMFORT scale (r=0.53-0.61,P<0.01). Conclusions Due to the strong consistency between the independent evaluators and the correlation between the two methods, the reliability and validity of simpliifed COMFORT scale are preferable and simpliifed COMFORT scale is useful in classifying the level of sedation in children on mechanical ventilation.

Objective:To prepare a bone substitute using microsphere scaffold containing adiponectin(APN)and to investigate the release behavior of the scaffold in vitro.Methods:Chitosan microsphere was developed by an emulsion-ionic cross-linking method. Poly (L-lactic-co-glycolic)acid (PLGA)and β-tricalcium phosphate (β-TCP)were used to prepare microsphere scaffold containing APN.The morphology,particle size,drug loading,incorporation efficiency and release behavior of the microsphere were examined. Results:The APN containing microsphere showed good spherical geometry,suitable size and microporosity under scanning electron microscope.The average diameter of the milipore was 20 -200 μm;the drug loading and incorporation efficiency were 1 .3% and 70.3% respectively.The controled-release process continued for 91 days.The extract solution from the APN microsphere-scaffold promoted MC3T3 cell proliferation without cytotoxicity.Conclusion:The APN microsphere-scaffold has sustained release function and may promote osteoblast proliferation.

Objective To strengthen the recognition of atypical POEMS syndrome in order to improve diagnosis rate of rare cases of POEMS syndrome. Methods The diagnosis and treatment of a rare case of POEMS syndrome coexisting with Castleman disease but without M protein in serum, urine and bone marrow who was admitted to Xuanwu Hospital, Capital Medical University in November 2017 were retrospectively analyzed with review of the literature. Results The elder male was suspected with a diagnosis of POMES syndrome, but absence of monoclonal plasma cell disease that made it difficult to diagnose. Systemic PET-CT found an active metabolic lesion in left iliac bone. Although the lymph node biopsy had been performed for a diagnosis of Castleman disease, a bone biopsy was also done for a definite diagnosis. Pathological result indicated a plasmacytoma which confirmed a diagnosis of POEMS syndrome without M protein in serum, urine and bone marrow. Literature review suggested that the application of immunofixation electrophoresis was helpful to improve the diagnostic rate of POEMS syndrome. For patients with a suspected diagnosis of POEMS syndrome, bone biopsy, flow cytometry and systemic PET-CT may assist in the search for monoclonal plasma cell. Periphery neuropathy, bone lesion and treatment response were helpful in distinguishing Castleman disease coexisting with POEMS syndrome from Castleman disease without POEMS syndrome. Conclusions When a mandatory major criterion of POEMS syndrome is not sufficient, it should be actively sought, especially for patient with a suspected diagnosis of POEMS syndrome. For patients with multiple lesions, multi-site biopsies are necessary to assist in diagnosis.

Objective To evaluate the application value of polarized light dermoscopy in the diagnosis of vitiligo and differential diagnosis between vitiligo and other common hypopigmented diseases.Methods Dermoscopic images of lesions of 235 patients with confirmed vitiligo (130 with active vitiligo and 105 with stable vitiligo) were collected from the database in the Department of Dermatology,Xijing Hospital from January 2018 to July 2018,and retrospectively analyzed.Meanwhile,dermoscopic images of amelanotic nevus (151 cases),pityriasis alba (113 cases) and idiopathic guttate hypomelanosis (54 cases) were compared with the dermoscopic images of vitiligo.Chi-square test was used for comparison of rates among groups.Results The prevalence of perifollicular pigmentation,reticular pigmentation,starburst,Tapioca sago and micro-Koebner phenomenon was significantly higher in the patients with active vitiligo than in those with stable vitiligo (x2 =36.5,151.2,13.0,14.9,8.4 respectively,all P ＜ 0.05).The prevalence of perifollicular pigmentation,perilesional hyperpigmentation,perifollicular telangiectasia,leukotrichia was significantly higher in patients with stable vitiligo than in those with active vitiligo (x2 =91.2,166.0,54.3,41.1 respectively,all P ＜ 0.05).The prevalence of residual perifollicular pigmentation was significantly higher in patients with active (89.2％,116/130) or stable vitiligo (54.3％,57/105) than in those with amelanotic nevus (20.5％,31/151,P ＜ 0.05),pityriasis alba (12.4％,15/113,P ＜ 0.05)or idiopathic guttate hypomelanosis (9.3％,5/54,P ＜ 0.05).Conclusion Residual perifollicular pigmentation could serve as an important feature for distinguishing vitiligo from achromic nevus,pityriasis alba and idiopathic guttate hypomelanosis,and polarized light dermoscopy shows good value in the differential diagnosis between vitiligo and some hypopigmented diseases.

Objective:To establish the risk prediction models for postoperative delirium (POD) in elderly patients undergoing non-cardiac surgery and to evaluate the predictive efficacy.Methods:A total of 685 patients of both sexes, aged 65-90 yr, of American Society of Anesthesiologists (ASA) physical status Ⅰ-Ⅳ, who underwent non-cardiac elective surgery requiring tracheal intubation during general anesthesia in general surgery, orthopedics, urology, hepatobiliary and pancreatic surgery in our hospital from January 2020 to December 2020, were selected.Patients were assigned to the training set and validation set at a ratio of 7∶3 using a simple random sampling method.The clinical data of patients in the perioperative period were collected, and the patients were followed up within 1-7 days after operation (or before discharge), and the occurrence of POD was recorded.Univariate and multivariate logistic regression analysis was used to identify the independent risk factors for POD.The risk prediction model for POD was established based on the results of multivariate logistic regression analysis of the training set, a nomogram and receiver operating characteristic (ROC) curve were drawn, and the area under the curve (AUC) was calculated.The validation set was used to verify the prediction model and assess the efficacy of the risk prediction model for POD.Results:A total of 653 patients were enrolled in this study, 139 patients developed POD, and the incidence was 21.3%.The results of multivariate logistic regression analysis showed that advanced age, high ASA physical status classification, low preoperative Mini-Mental State Examination score, complication with diabetes mellitus, low years of education, high preoperative Pittsburgh Sleep Quality Index scale score, long anesthesia time and high numerical rating scale score after operation were independent risk factors for POD in elderly patients undergoing non-cardiac surgery.The risk prediction model for POD was established based on the independent risk factors mentioned above.The AUC of the training set was 0.981, the Youden index was 0.881, the sensitivity was 95.95%, and the specificity was 92.92%; the AUC of the validation set was 0.939, the Youden index was 0.795, the sensitivity was 94.44%, and the specificity was 85.09%.Conclusion:The risk prediction model for POD established based on age, ASA physical status classification, history of diabetes melittus, years of education, preoperative Mini-Mental State Examination score, preoperative Pittsburgh sleep quality index scale score, anesthesia time and postoperative numerical rating scale score has good predictive efficacy in elderly patients undergoing non-cardiac surgery.

Objective:To investigate dermoscopic manifestations and features of melanonychia.Methods:A retrospective analysis was carried out on dermoscopic images of 4 common types of melanonychia collected in Department of Dermatology, Xijing Hospital, the Fourth Military Medical University from January 2016 to July 2020.Results:A total of 266 cases of melanonychia were collected, including 64 (24.1%) of subungual melanoma, 52 (19.5%) of nail matrix nevi, 89 (33.5%) of subungual hemorrhage, and 61 (22.9%) of onychomycosis. Subungual melanoma and nail matrix nevi mostly occurred in the fingernails. To be specific, subungual melanoma most frequently occurred in the thumbnails (62.8%) , while nail matrix nevi mostly involved the 2 nd - 5 th fingernails (73.9%) . Subungual hemorrhage and onychomycosis mostly occurred in the toenails, and there were 51 (57.3%) cases of subungual hemorrhage of the toenails and 46 (75.4%) cases of onychomycosis of the toenails. Subungual melanoma mostly occurred in patients aged over 40 years (49 cases, 76.8%) , while the other 3 types of melanonychia mostly affected patients aged under 40 years. Dermoscopic manifestations of subungual melanoma mainly included regular longitudinal bands (35 cases, 54.7%) or irregular bands (25 cases, 39.0%) whose width was greater than 3 mm in 87.5% cases, Hutchinson sign (36 cases, 56.3%) , and ruptures (15 cases, 23.4%) which mainly were black-brown in color; dermoscopic manifestations of nail matrix nevi mainly were a single regular pigmented band (52 cases, 100%) whose width was less than 3 mm in 36 (69.2%) cases, and Hutchinson sign (26 cases, 50%) , while no ruptures were observed in nail matrix nevus lesions; subungual hemorrhage dermoscopically manifested as diffuse macules (74 cases, 83.1%) , and globular dark red or black hemorrhagic structures were observed in 85 (95.5%) cases; fungal melanonychia was dermoscopically characterized by irregular dark brown longitudinal bands (54 cases, 88.5%) . Conclusions:Subungual melanoma was dermoscopically characterized by regular longitudinal bands with a width of greater than 3 mm, nail matrix nevi by regular longitudinal bands, subungual hemorrhage by diffuse macules, and onychomycosis by irregular longitudinal bands. Dermatoscopy can be used to identify melanonychia lesions and provide a basis for auxiliary diagnosis of subungual melanoma.

Objective:To investigate the value of α-synuclein (α-syn) concentration in cerebrospinal fluid (CSF) in predicting postoperative delirium (POD).Methods:One thousand patients underwent elective surgery with combined epidural-spinal anesthesia in our hospital from January 2018 to September 2020 were selected.The epidural puncture was performed at L 3, 4 interspace, and 2 ml of CSF was collected after the needle reaching the subarachnoid space.The concentrations of α-syn, β-amyloid (Aβ)40, Aβ42, total tau protein (T-tau), and phosphorylated tau protein (P-tau) in CSF were determined by enzyme-linked immunosorbent assay.The concentrations of α-syn in CSF and occurrence of POD in patients of different ages were recorded.Patients were divided into POD group and non-POD group according to whether POD occurred, and frequency matching (1∶1) was performed based on five matching variables of age, ASA physical status, education level, duration of operation, and intraoperative blood loss. Results:Eight hundred and forty-one patients were finally included in the study, and the incidence of POD was 15.0%. There were 126 cases in POD group and 126 cases in non-POD group after matching. The concentrations of α-syn in CSF and incidence of POD were gradually increased with age ( P<0.05). Compared with non-POD group, the concentrations of α-syn, T-tau and P-tau in CSF were significantly increased, the concentrations of Aβ40 and Aβ42 were decreased, Aβ40/P-tau, Aβ42/P-tau, Aβ42/Aβ40 and P-tau/T-tau were decreased in POD group ( P<0.05). After confounding factors were corrected by logistic regression analysis, increased concentrations of α-syn, p-tau, and T-tau in CSF were risk factors for POD ( P<0.05). Increased concentrations of Aβ40 and Aβ42 in CSF and increased Aβ40/P-tau and Aβ42/P-tau were protective factors for POD ( P<0.05). Multiple linear regression analysis showed that the concentration of α-syn in CSF was negatively correlated with Aβ40 and Aβ42 concentrations and positively correlated with P-tau and T-tau concentrations ( P<0.05). The area under the receiver operating characteristic curve of concentrations of α-syn in CSF predicting POD was 0.895, Youden index was 0.664, sensitivity was 80.00%, and specificity was 86.36% ( P<0.001). Conclusion:The concentration of α-syn in CSF is related to the occurrence of POD, and it provides higher accuracy in predicting POD.