Objective To investigate the clinical and genetic characteristics of a progressive symmetric erythrokeratoderma (PSEK).Methods The clinical and genetic characteristics of a PSEK were compared and analyzed with clinical collection and review of 17 PSEK families reported in China since 1980.Results PSEK was consistent with autosomal dominant inheritance.PSEK had the feature with incomplete penetrance and variable expression.The clinical features included hyperkeratotic plaques with distinct border and strikingly symmetric distribution pattern on the extremities.Part of patients was extended to other areas of the body.The onset of the disease commonly started in infancy or childhood.The general health condition was not affected.PSEK might be associated with other clinical symptoms.The diseased potential increased in the family of consanguineous marriage.Conclusions PSEK has genetic heterogeneity.Its causative genes have not been determined.Further studies are needed.

Objective To investigate the time course of nuclear factor-κB(NF-κB) and the effects of pyrrolidine dithiocarbamate (PDTC) on the expressions of NF-κB,tumor necrosis factor-α (TNF-α) and interleukin1β(IL-1β) in hippocampus after seizures.Methods Epilepsy were induced by [PTZ] through Intraperitoneal injection.Western blotting was used to detect NF-κB p65 expression in nucleus at various experiment groups in different time points( 14d,21 d,28d,35d).Moreover,mRNA and protein expressions of TNF-α and IL-1β in different experiment groups in different time points by Real-time quantitative PCR and ELISA analysis.Results The expression of NF-κB p65 began to increase in the nuclear fraction in 14d,kept rising in 28d and returned to 14d level in 35d after epilepsy seizures,At 14d,21d,28d and 35d,the expressions of NF-κB in PDTC groups ( (0.54 ±0.07),(0.65 ± 0.08 ),(0.78 ± 0.10),(0.78 ± 0.10) ) was significantly lower than those in PTZ groups ((1.20 ±0.11),(1.42 ±0.14),(1.88 ±0.16),(1.25 ±0.10)) (P＜0.01).After epilepsy seizures,the expression of TNF-α 、IL-1β mRNA was increased in PTZ groups( ( 1.34 ±0.13,0.81 ± O.17 ),( 1.64 ±0.17,1.56±0.20),(2.03 ±0.16,1.65 ±0.18),(1.40 ±0.10,1.30 ±0.13) ) than those in NS groups(P＜0.01 ) ;and compared with PTZ groups PDTC significantly decrease the mRNA expressions of TNF-α and IL-1 β in PDTC groups( (0.96 ±0.1,0.57 ±0.07),(1.36 ±0.15,1.09 ±0.18),(1.47 ±0.14,1.25 ±0.16),(1.12 ±0.12,O.85 ± 0.12) ) (P ＜ 0.05 ).The expressions of TN F-α,IL-1β protein were similar in hippocampal by ELISA.Conclusion Seizures induces NF-κB nucleus translocation and promotes the expressions of TNF-ot and IL-1 β in hippocampus and pyrrolidine dithiocarbamate suppresses NF-κB associated inflammatory pathway in epileptic rat hippocampus.

Objective To explore the clinical significance of cerebrospinal fluid neuropeptide Y in epileptic patients with intelligence disturbance.Methods The Full Intelligence Quotient (FIQ) of 78 cases of epileptic patients were assessed by WAIS-RC.The subjects were divided into intelligence disturbance group and non-intelligence disturbance group.Their cerebrospinal fluid neuropeptide Y Was tested by using radio immunoassay.Results The content of neuropeptide Y in intelligence disturbance group Was obviously higher than that in non-intelligence disturbance group (P<0.01).Conclusion Intelligence disturbance in epileptic patients is related to the increased Neuropeptide Y.Neuropeptide Y may reflect the intelligence condition in epileptic patients.

Objective To discuss the management level positioning of scientific research secretary in university affiliated hospitals,also propose suggestions for the team building.Methods Discussed the characteristics and particularities of the university affiliated hospitals,analyzed management level of scientific research secretary.Based on the fact that doctors or professional technicians are commonly part-time scientific research secretaries in these hospitals,discussed the responsibilities and team building.Results The university affiliated hospitals should establish a scientific research secretary as a management level between administrative entities and researchers,to form a four-level management mode.To fulfill this,detailed suggestions on qualifications,occupational instructions,selection process,job task and position transition of scientific research secretary were discussed in this article.Conclusions The responsibility of scientific research secretaries should be positioned to assist the scientific research management departments,to extend better need-based services to all scientific researchers,with the aim of promoting scientific research management services to "implement to people" and implementing the "people-oriented" concept.

Objective To study the relationship between ischemic stroke and gene polymorphism of an-giotensin-converting enzyme (ACE), apolipoprotein E (APOE) and methylene tetrahydrofolate reductase (MTHFR) among the population of Zhuang nationality in western Gui. Methods We directly sequenced ACE, APOE and MTHFR genes in 149 cases of ischemic stroke and 109 cases of normal people in western Gui. χ2 test was used to measure the relationship between gene polymorphism and ischemic stroke. Hardy-Weinberg genetic equilibrium test was used to evaluate the reliability of these data. Results In the ischemic stroke group, 62 cases, 22 cases and 65 cases carried II genetype, DD genetype and ID genetype in ACE. χ2 test showed no relationship between ACE gene polymorphism and ischemic stroke. In analysis of the polymorpism of APOE in the ischemic stroke and control group, no relationship between APOE gene polymorphism and ischemic stroke was found by χ2 test. MTFHR gene polymorphism was significantly related with ischemic stroke by χ2 test (P = 0.019). Conclusion Polymorphism of gene MTFHR but neither ACE nor APOE is significantly associated with ischemic stroke.

Objective:To analyze the associated symptoms of progressive symmetrical erythema keratosis (PSEK) and the related literature was reviewed.Methods:Two Mongolian PSEK families in the dermatology department of the People′s Hospital of Inner Mongolia Autonomous Region from 2016 to 2017 were collected, and 40 complete PSEK families and 156 sporadic cases at home and abroad published since 1980 to 2020 were searched by using the database to analyze the concomitant symptoms of PSEK.Results:A total of 40 complete PSEK families were included, including 714 cases. The incidence of PSEK was 57.38% in foreign countries and 37.42% in China; The most common concomitant symptoms abroad were palmoplantar keratosis (PPK), followed by nail changes, neurological symptoms, dysplasia, combined with variable erythema keratosis (EKV), and the concomitant symptoms were more serious; The most common concomitant symptoms in China were nail changes, followed by PPK, damp hyperhidrosis, pruritus, pain and some skin diseases, and the concurrent symptoms were mild.Conclusions:PSEK has many associated symptoms and the molecular genetic mechanism is still unclear. It is necessary to conduct a more comprehensive and in-depth study and understanding of the disease through the development of sequencing technology and the expansion of clinical cases.