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

IKBKG is the essential modulator for nuclear factor-κB(NF-κB) signaling pathway, and mutations within this gene can lead to anhidrotic ectodermal dysplasia and immunodeficiency (EDA-ID). Here we report a male patient, who presented with mild frontal bossing, sparse hair, skin pigmentation, conical teeth, and recurrent infections involving bacteria, fungi, and viruses after one month of age, together with hypogammaglobulinemia. These symptoms were consistent with the phenotype of EDA-ID. Genetic analysis revealed a hemizygous mutation c.1249T > G (p.Cys417Gly) in exon 10 of the IKBKG gene in the patient. The mother of the patient was identified as heterozygous carriers of the same mutation. Immunological assessment revealed a significant reduction in memory B cells. The patient showed no skewed TCR diversity. PHA-induced T-cell proliferation was impaired. IFN-γ secretion by Th cells was significantly reduced after PHA stimulation. IκBα degradation rate retained the same in the patient. We summarized the clinical features of the patient and conducted immunological analysis, in order to increase pediatricians′awareness of this rare disease. For boys with early-onset recurrent infections together with ectodermal dysplasia, IKBKG mutation should be considered. In addition, genetic analysis is needed to exclude pseudogene interference and functional evaluations are required.

With the process of China’s aging population intensifying， palliative care， as an important guarantee for improving the quality of life of terminally ill patients， is receiving more and more social attention， and the demand is constantly increasing. Palliative care needs versatile professionals， and general education can enhance people’s awareness and understanding of it， enabling more people to understand， accept， and participate in palliative care. With the advancement of knowledge and technology in palliative care， the traditional cramming education models are no longer able to meet the actual needs. Therefore， there is an urgent need to innovate palliative care education strategies. By analyzing the current problems in the general education of palliative care in China， this paper proposed thoughts and suggestions for general and innovative education of palliative care in several aspects， such as establishing general and innovative education systems and evaluation systems of palliative care， diversifying educational contents and methods， strengthening medical staffs training， promoting diversified student groups， and strengthening the popularization of palliative care knowledge among the public.

Lactic acid bacteria(LAB)were isolated and cultivated from the intestinal contents of rabbits by MRS-CaCO3 solid medium.Identification was achieved through morphological observa-tion,Gram staining,physiological and biochemical characterisation,16S rDNA sequence analysis,and ERIC-PCR analysis.Strains displaying typical Lactobacilli characteristics were exanimated for their biological characteristics,resistance properties,adherence capacity in vitro,colonization abili-ty in vivo,and safety profile.In this study,a total of four strains of Lactobacillus reuteri were iso-lated from rabbits,all of which exhibited typical biological characteristics of LAB.These strains demonstrated inhibitory effects on common pathogenic bacteria in the gastrointestinal tract,with the primary inhibitory substance being bacteriocin.Furthermore,they showed sensitivity to chlor-amphenicol,rifampicin,and erythromycin,and displayed a degree of tolerance to gastrointestinal conditions and high temperature.These stains were capable of successful colonization in rabbits with a higher degree of safety.This study lays a foundation for the development of LAB prepara-tions for the prevention and treatment of rabbit intestinal diseases.

Objective:To evaluate the effect of short-chain fatty acids on microglial synapse engulfment in aged rats with postoperative cognitive dysfunction (POCD).Methods:Forty-eight healthy male Sprague-Dawley rats, aged 18 months, weighing 520-650 g, were divided into 4 groups ( n=12 each) using a random number table method: control group (group C), short-chain fatty acids group (group S), POCD group (group P), and POCD+ short-chain fatty acids group (group PS). Rats received short-chain fatty acids (sodium propionate 25.9 mmol/L, sodium butyrate 40 mmol/L and sodium acetate 67.5 mmol/L) in the free drinking water for 28 days in S and PS groups. On day 29, anesthesia was induced with 4%-5% sevoflurane and maintained with 3% sevoflurane, and the tibial fracture internal fixation was performed to prepare a rat model of POCD in P group and PS group. Morris water maze test was performed at day 7 after surgery. The escape latency, times of crossing the original platform, mean swimming speed and time spent in the original platform quadrant were recorded. The rats were sacrificed at the end of Morris water maze test, and the brains were collected to analyze the number and density of dendritic spines in the hippocampal CA1 region (by Golgi staining) and to determine the expression of postsynaptic density 95 (PSD95) and complement 1q (C1q) in the hippocampal CA1 region (by immunofluorescence). Results:Compared with group C, the times of crossing the original platform were significantly decreased, the time spent in the original platform quadrant was shortened, the escape latency was prolonged, the number and density of dendritic spines and the number of intersection points between dendrites and concentric circles were decreased, the expression of PSD95 was down-regulated, and the expression of C1q was up-regulated in P and PS groups ( P<0.05). Compared with group P, the times of crossing the original platform were significantly increased, the time spent in the original platform quadrant was prolonged, the escape latency was shortened, the number and density of dendritic spines and the number of intersection points between dendrites and concentric circles were increased, the expression of PSD-95 was up-regulated, and the expression of C1q was down-regulated in group PS ( P<0.05). Conclusions:The mechanism by which short-chain fatty acids attenuates POCD is related to decreased microglial engulfment of synapses in aged rats.

Strengthening the management of key positions in medical and health institutions, implementing rotation management of personnels in key positions, is of great significance for regulating the operation of power and preventing corruption risks. A tertiary grade A hospital in Qingdao has implemented a rotation system for personnels in key positions among functional department from an internal control perspective since 2022. On the basis of conducting job risk assessment, the hospital has developed a key position rotation system, determining the scope, rotation cycle, and rotation method for key position personnels, and formulated key position rotation plan. The Party Committee Office and the Human Resources Department were responsible for organizing and implementing it, and evaluating the implementation effect. From 2022 to 2023, 61 key positions were rotated between departments and within departments, accounting for 37.7% of the total number of key positions. Compared with 2021, the number of petitions and reports received by the Discipline Inspection and Supervision Office for key positions in 2022 has decreased by 12.5%, which can provide reference for other medical and health institutions to carry out anti-corruption risk management.

Objective To observe the clinical efficacy of Wenyang Huayin Prescription(cooked Aconiti Lateralis Radix Praeparata,Cinnamomi Ramulus,Codonopsis Radix,Atractylodis Macrocephalae Rhizoma,Poria,Descurainiae Semen Lepidii Semen,etc.)combined with conventional western medicine in the treatment of elderly patients with chronic heart failure(CHF).Methods A total of 120 elderly CHF patients with fluid retention due to yang deficiency syndrome who were treated in People's Hospital of Chenghai District from December 2021 to December 2022 were selected as the research objects.They were randomly divided into control group and observation group,with 60 cases in each group.The control group was given conventional western medicine treatment,and the observation group was treated with Wenyang Huayin Prescription on the basis of the treatment of the control group.The course of treatment was four weeks.The indexes of the two groups were observed before and after treatment:(1)TCM syndrome scores;(2)serum levels of N-terminal pro-B-type natriuretic peptide(NT-proBNP),high-sensitivity C-reactive protein(hs-CRP),tumor necrosis factor-α(TNF-α)and interleukin-6(IL-6);(3)left ventricular ejection fraction(LVEF),left ventricular end-diastolic diameter(LVEDD);(4)6-minute walking distance.The clinical efficacy and safety of the two groups were compared.Results After treatment,(1)the scores of TCM syndromes in the two groups were significantly lower than those before treatment(P＜0.05),and the scores in the observation group were significantly lower than those in the control group(P＜0.05).(2)The levels of serum hs-CRP,TNF-α,IL-6 and NT-proBNP in the two groups were significantly lower than those before treatment(P＜0.05),and the levels in the observation group were significantly lower than those in the control group(P＜0.05).(3)The levels of LVEF in the two groups were significantly higher than those before treatment(P＜0.05),and the levels of LVEDD were significantly lower than those before treatment(P＜0.05).The level of LVEF in the observation group was significantly higher than that in the control group(P＜0.05),and the level of LVEDD was significantly lower than that in the control group(P＜0.05).(4)The 6-minute walking distance in the two groups was significantly increased when compared with that before treatment(P＜0.05),and the increase in the observation group was significantly greater than that in the control group(P＜0.05).(5)The total effective rate of the observation group(96.67%)was significantly higher than that of the control group(86.67%)(P＜0.05);the total incidence of adverse reactions in the observation group(1.67%)was significantly lower than that in the control group(11.67%)(P＜0.05).Conclusion Wenyang Huayin Prescription combined with conventional western medicine treatment can effectively reduce the TCM syndrome score of elderly CHF patients with fluid retention due to yang deficiency syndrome,improve clinical symptoms and cardiac function,and reduce the body's inflammatory response.The clinical efficacy is superior to that of western medicine alone,and the safety is relatively high.

Objective:To describe the current status of the resilience of the primary caregivers ′ families of patients with first-ever stroke and to explore the factors that influence it, in order to assist medical professionals in timely identification of related factors, formulate targeted nursing rehabilitation programs, and provide better support and care to patients and their families. Methods:Using convenience sampling, the primary caregivers ′ families of patients with first-ever stroke admitted to The First Affiliated Hospital of Naval Medical University of the People ′s Liberation Army of China and Shanghai Tenth People ′s Hospital from May 2021 to January 2022 were selected as subjects for a cross-sectional survey of family resilience. The Family Resilience Assessment Scale was used to evaluate the primary caregivers of first-ever stroke patients, and multiple linear regression was employed to clarify the influencing factors of the family resilience of primary caregivers. Results:A total of 307 questionnaires were distributed, and 288 valid questionnaires were collected. There were 84 males and 204 females, aged ≤30 years old with 23 cases, aged 31-40 years old with 63 cases, aged 41-50 years old with 63 cases, aged 51-60 years old with 32 cases, aged ≥ 61 years old with 107 cases. The total score of family resilience of the primary caregivers was (210.80±17.32) points. Univariate analysis showed that there were statistical differences in the caregiver ′s occupational status, relationship with the patient, age, family residence, marital status, type of family, daily caregiving time, and the patient ′s gender and age with the caregiver ′s family resilience score ( t=2.06, -3.38, F values were 4.96-21.32, all P<0.05). The level of family resilience of primary caregivers was positively correlated with social support, positive coping methods, and family support ( r=0.375, 0.627, 0.642, all P<0.05), and negatively correlated with caregiving burden and negative coping methods ( r=-0.444, -0.563, both P<0.05). The multiple linear regression equation showed that social support, family support, positive coping, and negative coping were the main influencing factors of the family resilience of primary caregivers of patients with first-ever stroke ( t values were -6.68-6.27, all P<0.05), explaining 57.5% of the variance in family resilience. Conclusions:The family resilience of primary caregivers of patients with first-ever stroke is at a high level. Primary caregivers who receive more family support and social support, and who adopt positive coping methods and reduce negative coping methods, have higher levels of family resilience.

Objective To compare the difference and consistency between OPD-Scan Ⅲ and IOL-Master 700 in the measurement of corneal curvature(flat keratometry,K1 and steep keratometry,K2)and horizontal corneal diameter(white-to-white(WTW)distance).Methods Totally 268 patients with 328 eyes(164 right eyes and 164 left eyes)who underwent cataract surgery at the Department of Ophthalmology of the Aerospace Centre Hospital from October 2021 to September 2022 were selected for this study.The K1,K2,and WTW values of the sampled right or left eyes were measured and analyzed using OPD-Scan Ⅲ and IOL-Master 700,respectively.Parameter comparisons were performed through paired t-tests;correlations between parameters were detected through the Pearson correlation analysis;and the Bland-Altman method and intragroup correlation coefficient(ICC)analysis were employed to determine the consistency of parameter measurements between the two instruments.Results The K1 and K2 values measured by OPD-Scan Ⅲ were greater than those measured by IOL-Master 700,while the WTW values were lower than those measured by IOL-Master 700,but the differences were statistically significant(all P＜0.001).K1,K2,and WTW values measured by OPD-Scan Ⅲ were positive-ly correlated with the corresponding values measured by IOL-Master 700,and the differences were statistically significant(all P＜0.001).The proportions of K1,K2,and WTW values outside the 95％limits of agreement for both instruments were within 5％,but the absolute value of the difference in values within the 95％limits of agreement was close to or more than(1.0)D,indicating a sizeable clinical deviation.ICC analysis confirmed a good consistency between K1 and K2 values of the left and right eyes measured by the two instruments(ICC＞0.90).The difference in WTW values measured by the two instruments was significantly correlated with K2 values(both P＜0.05).There were 5 samples(83.33％)outside the positive deviation range of WTW values,of which K2 measured by IOL-Master 700 was above 47.03.Conclusion The OPD-Scan Ⅲ and IOL-Master 700 have been found to have measurement biases when assessing K1,K2 and WTW.In clini-cal practice,the two instruments cannot be interchanged as an alternative to each other.The WTW values measured by IOL-Master 700 are greater than those obtained by OPD-Scan Ⅲ;when K2≥47.03,the WTW values may not be reliably ref-errable.