BACKGROUND: Generalized pustular psoriasis (GPP), a rare and recurrent autoinflammatory disease, imposes a substantial burden on patients and society. Awareness of GPP in China remains limited. METHODS: This cross-sectional survey, conducted between September 2021 and May 2023 across 14 hospitals in China, included GPP patients of all ages and disease phases. Data collected encompassed demographics, clinical characteristics, economic impact, disease severity, quality of life, and treatment-related complications. Risk factors for GPP recurrence were analyzed. RESULTS: Among 127 patients (female/male ratio = 1.35:1), the mean age of disease onset was 25 years (1st quartile [Q1]-3rd quartile [Q3]: 11-44 years); 29.2% had experienced GPP for more than 10 years. Recurrence occurred in 75.6% of patients, and nearly half reported no identifiable triggers. Younger age at disease onset ( P  = 0.021) and transitioning to plaque psoriasis ( P  = 0.022) were associated with higher recurrence rates. The median diagnostic delay was 8 months (Q1-Q3: 2-41 months), and 32.3% of patients reported misdiagnoses. Comorbidities were present in 53.5% of patients, whereas 51.1% experienced systemic complications during treatment. Depression and anxiety affected 84.5% and 95.6% of patients, respectively. During GPP flares, the median Dermatology Life Quality Index score was 19.0 (Q1-Q3: 13.0-23.5). This score showed significant differences between patients with and without systemic symptoms; it demonstrated correlations with both depression and anxiety scores. Treatment costs caused financial hardship in 55.9% of patients, underscoring the burden associated with GPP. CONCLUSIONS The substantial disease and economic burdens among Chinese GPP patients warrant increased attention. Patients with early onset disease and those transitioning to plaque psoriasis require targeted interventions to mitigate the high recurrence risk.
Humans ; Male ; Female ; Psoriasis/pathology* ; Adult ; Cross-Sectional Studies ; Adolescent ; Child ; Young Adult ; Quality of Life ; Middle Aged ; China/epidemiology* ; Recurrence ; Risk Factors ; Surveys and Questionnaires ; East Asian People

Objectives:To explore the association between the r'wave amplitude in lead V1 and impedance changes with left bundle branch pacing electrode implantation depth. Methods:A total of 78 patients with normal heart structure and underwent left bundle branch area pacing(LBBAP)in the Second Affiliated Hospital of Nanchang University from January 1,2019 to December 31,2021 were included in this retrospective analysis.Baseline data,intraoperative and imaging data,and 3,6,9 and 12 months of follow-up results were collected.Correlation and regression analysis were performed to define the feasibility using the r'wave in lead V1 during pacing and impedance changes to estimate the electrode depth. Results:r'waves at the end of the QRS complex in lead V1 during pacing were found in 70 cases(89.7%),and 8 cases(10.3%)showed rS,RS type QRS waves,or no r'wave at the end.Correlation analysis showed that r'wave amplitude was positively correlated with electrode depth(r=0.424,P<0.01),negatively correlated with impedance(r=-0.256,P=0.03).There was no significant statistical correlation between electrode implantation depth and impedance(r=-0.132,P=0.27).Regression analysis found that electrode depth was an important factor affecting r'wave amplitude(regression coefficient=0.056,P=0.000).Combined with the established regression model and impedance,it was found that the amplitude of the r'wave in lead V1 is at the range of 0.24-0.69 mV,and the impedance ranges from 648.30 to 828.90 Ω,and the electrode implantation depth is 6-11 mm,which is most suitable.The risk of perforation is low,and the left bundle branch can be successfully captured with a high probability.The pacing parameters are satisfactory,and the pacing QRS wave duration is narrow.During the intraoperative,postoperative 48 hours,and 12-month follow-up period,the patient did not experience complications such as electrode perforation,thromboembolism,cardiac tamponade,infection,or wire dislocation. Conclusions:Left bundle branch region pacing is a safe and feasible pacing method.During LBBAP,the amplitude of the r'wave in lead V1 at the range of 0.24-0.69 mV,and the impedance ranges from 648.30 to 828.90 Ω can be used to guide the pacing in the left bundle branch region and reduce the risk of electrode perforation.

Objective:To analyze the relationship between ataxia telangiectasia mutated (ATM) single nucleotide polymorphism (SNP) at rs1801516 and rs1800054 and sporadic breast cancer (SBC) in Inner Mongolia.Methods:A total of 102 patients with SBC (72 Han and 30 Mongolian) who were admitted to the Affiliated Hospital of Inner Mongolia Medical University from January 2018 to September 2019 were prospectively collected as case group and 102 healthy women (72 Han and 30 Mongolian) during the same period as control group. 2 ml of venous blood was collected to extract DNA. According to the Single Nucleotide Polymorphism Database (dbSNP), the highly polymorphic sites rs1801516 and rs1800054 of ATM gene were selected. The polymerase chain reaction (PCR) and direct sequencing were used to detect the polymorphism of the two sites, and the correlation between the single nucleotide polymorphism of the two sites and the susceptibility of SBC in Inner Mongolia was analyzed. The potential association between clinicopathological factors and ATM gene polymorphism in patients with SBC in Inner Mongolia were explored.Results:GG, GA and AA genotypes were detected in rs1801516 locus of ATM gene. Only CC genotype was detected in the rs1800054 locus of ATM gene. There was no significant difference in the distribution of genotype frequency and allele frequency between Mongolian breast cancer group and Han breast cancer group, Mongolian control group and Han control group, Mongolian breast cancer group and Mongolian control group, Han breast cancer group and Han control group (all P>0.05). Logistic regression analysis showed that allele G was the susceptibility gene of SBC in Inner Mongolia ( OR: 1.775, 95% CI: 1.04-3.03, P=0.04). ATM rs1801516 polymorphism may be associated with increased risk of breast cancer in patients with mass diameter ≤2 cm and/or without lymph node metastasis (all P<0.05). Conclusions:The polymorphism of ATM gene rs1801516 and rs1800054 may not be significantly correlated with the risk of SBC in Inner Mongolia. The rs1801516 locus may be associated with increased risk of breast cancer in patients with mass diameter ≤2 cm and/or without lymph node metastasis. Gene G may be one of the susceptible genes of SBC in Inner Mongolia.

Objective:To explore the occurrence and influencing factors of perimenopausal syndrome among Han, Hui and Tibetan women in Gansu Province.Methods:A stratified cluster sampling method was used to select 7290 women aged 40-55 years as the research subjects for cross-sectional survey. The questionnaire was used to investigate demographic characteristics, marriage and childbearing menstrual history, and the modified Kupperman score was used to investigate the symptoms related to perimenopausal syndrome. The data were statistically analyzed using SPSS20.0 software.Results:The natural menopausal age of women in Gansu Province was (47.43±3.99) years old, Hui women had the earliest age of menopause (47.08±4.29). There were statistically significant differences in occupation, education level and residence among the three ethnic groups ( P<0.001, P=0.001, P=0.005). The incidence of perimenopausal syndrome was 58.85% (246/418) for Hui, 44.16% (136/308) for Tibetans, and 41.06% (2695/6564) for Hans. The incidence of perimenopausal syndrome and the grade of related symptoms were significantly different among the three ethnic groups (all P<0.001). Han women had the highest proportion of mild symptoms, while Hui women and Tibetan women had the highest proportion of moderate symptoms. The differences of the incidence of mild and severe symptoms among the three ethnic groups were statistically significant (all P<0.001). Logistic regression analysis showed that engaging in heavy physical labor is a common risk factor for the incidence of perimenopausal syndrome in the three ethnic groups. Conclusion:The prevention and treatment of perimenopausal syndrome can be carried out from the following aspects: promoting economic development, improving living conditions, changing life and work patterns, increasing investment in education, medical and health care, and strengthening knowledge popularization.

Objective:To explore the occurrence and influencing factors of perimenopausal syndrome among Han, Hui and Tibetan women in Gansu Province.Methods:A stratified cluster sampling method was used to select 7290 women aged 40-55 years as the research subjects for cross-sectional survey. The questionnaire was used to investigate demographic characteristics, marriage and childbearing menstrual history, and the modified Kupperman score was used to investigate the symptoms related to perimenopausal syndrome. The data were statistically analyzed using SPSS20.0 software.Results:The natural menopausal age of women in Gansu Province was (47.43±3.99) years old, Hui women had the earliest age of menopause (47.08±4.29). There were statistically significant differences in occupation, education level and residence among the three ethnic groups ( P<0.001, P=0.001, P=0.005). The incidence of perimenopausal syndrome was 58.85% (246/418) for Hui, 44.16% (136/308) for Tibetans, and 41.06% (2695/6564) for Hans. The incidence of perimenopausal syndrome and the grade of related symptoms were significantly different among the three ethnic groups (all P<0.001). Han women had the highest proportion of mild symptoms, while Hui women and Tibetan women had the highest proportion of moderate symptoms. The differences of the incidence of mild and severe symptoms among the three ethnic groups were statistically significant (all P<0.001). Logistic regression analysis showed that engaging in heavy physical labor is a common risk factor for the incidence of perimenopausal syndrome in the three ethnic groups. Conclusion:The prevention and treatment of perimenopausal syndrome can be carried out from the following aspects: promoting economic development, improving living conditions, changing life and work patterns, increasing investment in education, medical and health care, and strengthening knowledge popularization.

Objective:To construct an intelligent performance measurement system of gastrointestinal endoscopy and to analyze its value for endoscopic quality improvement.Methods:The intelligent gastrointestinal endoscopy performance measurement system was developed by using the deep convolutional neural network (DCNN) and deep reinforcement learning, based on the Digital Imaging and Communications in Medicine. Images were acquired of patients undergoing gastrointestinal endoscopy at Digestive Endoscopy Center of Renmin Hospital of Wuhan University from December 2016 to October 2018. The system applied cecum recognition model (DCNN1), images in vitro and in vivo recognition model (DCNN2), and identification model at 26 gastric sites (DCNN3) to monitor indices such as cecal intubation rate, colonoscopic withdrawal time, gastroscopic inspection time, and gastroscopic coverage. Images of 83 gastroscopies and 205 colonoscopies acquired at Digestive Endoscopy Center of Renmin Hospital of Wuhan University from March to November 2019 were randomly selected to examine the effectiveness of the system. Results:The intelligent gastrointestinal endoscopy performance measurement system consisted of quality analysis of both gastroscopy and colonoscopy, including all indices, and could be generated automatically at any time. The accuracy for cecal intubation rate, colonoscopic withdrawal time, gastroscopic inspection time, and gastroscopic coverage were 92.5% (172/186), 91.7% (188/205), 100.0% (83/83), 89.3% (1 928/2 158), respectively.Conclusion:The intelligent performance measurement system for gastrointestinal endoscopy can be recommended for the quality control of gastrointestinal endoscopy, from which endoscopists can get feedback and improve the quality of gastrointestinal endoscopy.

Objective: To evaluate the efficacy and safety of nifekalan (NIF) on cardioversion in atrial fibrillation (AF) patients post radiofrequency ablation, and investigate the relevant factors related to the cardioversion efficacy of NIF. Methods: We screened patients with sustained AF rhythm after radiofrequency ablation between November 2016 and July 2018. Participants were treated with intravenous NIF 0.4 mg/kg within 5-10 minutes after ablation. We observed the adverse reaction, and monitored the rhythm, heart rate, QT interval and QTc interval before the medication and at 5, 10, 20, 120 min after the medication. According to the drug outcome of NIF, patients were divided into conversion group and non-conversion group, related factors affecting conversion efficacy were evaluated using logistic regression analysis. Results: (1)A total of 116 patients were enrolled in the study (63 males and 53 females, mean age was (64±18) years). Among them, 72 patients were converted to sinus rhythm, and the overall successful rate was 62.1%. There were 84 patients with persistent AF, of which 50 cases (59.2%) were restored to sinus rhythm. There were 32 patients with paroxysmal AF, 22 cases (68.8%) of them were restored to sinus rhythm. The conversion time was 1.5 to 12 (6.8±3.4)min. (2) In 116 patients, the QT interval and QTc interval were significantly longer after medication than before the drug administration (P<0.01), and peaked at about 10th min, and restored to the level before drug administration at about 120th min. (3) There were 8 cases of bradycardia (6.9%), 3 cases of frequent and short ventricular tachycardia (2.6%). (4) The duration of atrial fibrillation was shorter and left atrial diameter was smaller in the cardioversion group than in the non-cardioversion group (both P<0.05). There were no significant differences in gender, disease history, atrial fibrillation type and structural heart disease between the two groups (P>0.05). (5) Multifactorial logistic regression analysis showed that the duration of atrial fibrillation (OR=0.980, 95%CI 0.966-0.994, P=0.004) and the left atrial diameter (OR=0.888, 95%CI 0.814-0.967, P=0.007) were the factors that influence the cardioversion efficacy of NIF on atrial fibrillation post ablation. Conclusions The total effective rate of NIF was 62.1% in patients witrh sustained AF post radiofrequency ablation, was 68.8% in patients with paroxysmal AF. Besides, NIF has the advantage of short conversion time and few adverse reactions. Left atrium diameter and AF duration were relevant factors that influence the efficacy of NIF of cardioversion in patients with sustained AF after radiofrequency ablation.

Objective To evaluate the efficacy and safety of nifekalan (NIF) on cardioversion in atrial fibrillation (AF) patients post radiofrequency ablation, and investigate the relevant factors related to the cardioversion efficacy of NIF. Methods We screened patients with sustained AF rhythm after radiofrequency ablation between November 2016 and July 2018. Participants were treated with intravenous NIF 0.4 mg/kg within 5-10 minutes after ablation. We observed the adverse reaction, and monitored the rhythm, heart rate, QT interval and QTc interval before the medication and at 5, 10, 20, 120 min after the medication. According to the drug outcome of NIF, patients were divided into conversion group and non?conversion group, related factors affecting conversion efficacy were evaluated using logistic regression analysis. Results (1)A total of 116 patients were enrolled in the study (63 males and 53 females, mean age was (64±18) years). Among them, 72 patients were converted to sinus rhythm, and the overall successful rate was 62.1%. There were 84 patients with persistent AF, of which 50 cases (59.2%) were restored to sinus rhythm. There were 32 patients with paroxysmal AF, 22 cases (68.8%) of them were restored to sinus rhythm. The conversion time was 1.5 to 12 (6.8±3.4)min. (2) In 116 patients, the QT interval and QTc interval were significantly longer after medication than before the drug administration (P<0.01), and peaked at about 10th min, and restored to the level before drug administration at about 120th min. (3) There were 8 cases of bradycardia (6.9%), 3 cases of frequent and short ventricular tachycardia (2.6%). (4) The duration of atrial fibrillation was shorter and left atrial diameter was smaller in the cardioversion group than in the non?cardioversion group (both P<0.05). There were no significant differences in gender, disease history, atrial fibrillation type and structural heart disease between the two groups (P>0.05). (5) Multifactorial logistic regression analysis showed that the duration of atrial fibrillation ( OR=0.980, 95%CI 0.966-0.994, P=0.004) and the left atrial diameter ( OR=0.888, 95%CI 0.814-0.967, P=0.007) were the factors that influence the cardioversion efficacy of NIF on atrial fibrillation post ablation. Conclusions The total effective rate of NIF was 62.1% in patients witrh sustained AF post radiofrequency ablation, was 68.8% in patients with paroxysmal AF. Besides, NIF has the advantage of short conversion time and few adverse reactions. Left atrium diameter and AF duration were relevant factors that influence the efficacy of NIF of cardioversion in patients with sustained AF after radiofrequency ablation.

OBJECTIVE:To explore the present situation of scientific research of clinical pharmacists in domestic third-grade class A hospitals,and discuss its feasible research ideas. METHODS:Questionnaire was conducted for the scientific research situa-tion of clinical pharmacists who obtained the certification in domestic 63 third-grade class A hospitals,the questionnaire data was collected,and data was statistically analyzed by using IBM SPSS Statistics 22.0 software. RESULTS:Totally 315 questionnaires were sent out,282 were effectively received,with effective recovery of 89.52%. In terms of attitude to scientific research,propor-tion of the surveyed clinical pharmacists who thought it was quite necessary and necessary was more than 70%;except for academ-ic degree(P<0.05),the different gender,age and job title did not cause the differences in scientific research attitude(P>0.05). In terms of the present situation of scientific research,achievements of them were relatively simple,thinking the biggest difficulty was insufficient of experiment condition;there was significant difference in the numbers of authorized patents in recent 5 years by different gender (P<0.05),the numbers of applications for approved funds,authorized patents and paper published in recent 5 years by different academic degrees(P<0.05),as well as the numbers of applications for approved off-campus funds(title also re-lated to the number of hospital funds)and the numbers of paper published in domestic magazine in recent 5 years by different ages and job titles (P<0.05). In terms of direction of scientific research,most of surveyed clinical pharmacists thought they should strengthen communicate with physicians and look for the breakthrough point,they also thought the optimal one was clinical re-search;the direction selection was basically similar no matter the gender,academic degree,age and job title(P>0.05). In terms of ability enhancement,the surveyed pharmacists thought liter-ature review,evidence-based literature/experimental interpreta-tion and foreign language reading were the abilities most need to be enhanced;except for gender,academic degree,age andjob title showed similarity in enhancing the literature review,evidence-based literature/experimental interpretation ability (P>0.05),while there were significant differences in enhancing foreign language reading ability by different gender,academic degree, age and job title (P<0.05). CONCLUSIONS:Clinical pharmacists show certain differences in the attitude to scientific research, present situation and direction of scientific research,and ability enhancement because of different gender,academic degree,age and job title,but there are also common. In order to improve the competence of scientific research and promote the discipline devel-opment of clinical pharmacy,pharmacy administrator in hospital should improve the academic degree and job title on clinical phar-macists and provide necessary advanced scientific platform and pertinent training at the same time. Additionally,except for the ratio-nal drug use,evidence-based pharmacy and pharmacoeconomics,clinical pharmacists can find new breakthrough point from phar-maceutical care,drug treatment decisions,translational medicine and medicine large data.

Objective: To explore the clinical and molecular genetic features of a Chinese patient with catecholaminergic polymorphic ventricular tachycardia (CPVT). Methods: Clinical data including resting electrocardiography, echocardiography and treadmill exercise testing of a patient with CPVT admitted to our department in March 2013 were analyzed, and the peripheral venous blood samples of the patient and his family members and 400 ethnicity-matched healthy controls were obtained. All exons and exon-intron boundaries of the six CPVT-related genes including RYR2, CASQ2, TRDN, CALM1, KCNJ2 and ANKB were sequenced to detect the variants related to CPVT. The relationship between the genotypes and phenotypes was analyzed to direct the target therapy. Results: Recurrent syncope induced either by exercise or extreme frightened fear was observed in this patient. There was no positive family history of syncope or sudden death. The resting electrocardiography and echocardiography of the patient were normal, while the exercise testing revealed bidirectional and polymorphic ventricular tachycardia. A cardiac ryanodine receptor gene mutation (R2401H) was identified in this patient, while this mutation was absent in his parents and sister and 400 controls. No variant was detected in the remaining five candidate genes. Treatment with high dose of metoprolol succinate (118.75 mg/d) was effective and patient was free of syncopal attack during the 2 years follow-up. Conclusion This is the first report on RyR2-R2401H mutation in Chinese patient with CPVT, and high dose of metoptolol is the effective therapy option for CPVT related to RyR2 mutation.