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

Intravascular large B-cell lymphoma (IVLBCL) is a rare large B-cell lymphoma subtype. We report a patient who presented with "recurrent fever and pancytopenia." A 64-year-old female patient had previously been diagnosed with Waldenstrom’s macroglobulinemia and had received zanubrutinib treatment. In February 2023, the patient revisited due to "recurrent fever and pancytopenia." A positron emission tomography/computed tomography scan demonstrated significant enlargement of the bilateral adrenal glands. After an adrenal biopsy, she was diagnosed with diffuse large B-cell lymphoma, not otherwise specified. The patient received chemotherapy with the R-CHOP regimen (rituximab + cyclophosphamide + doxorubicin + vincristine + prednisone). After three treatment courses, a cranial magnetic resonance imaging examination indicated central nervous system infiltration of the lymphoma. After reviewing the pathology of the adrenal biopsy, the final diagnosis was revised as IVLBCL. Despite aggressive treatment, the disease continued to progress, and the patient died two months later. According to a multidisciplinary level, this article discusses the case from the perspective of a multidisciplinary team collaboration, involving imaging, pathology, dermatology, and lymphoma, to provide reference opinions for the clinical diagnosis and treatment of IVLBCL.

Paraplegia caused by spinal cord injury is a serious neurological complication, for which surgery is currently the main treatment method. Due to different surgical approaches, patients are usually expected to maintain a passive prone position for a long time or switch between the supine and prone positions. Affected by multiple factors such as neurogenic sensory disorders, pathological changes in muscle tone and operative duration, the risk of intraoperative acquired pressure injury (IAPI) is significantly increased. Current clinical prevention strategies for IAPI in these patients predominantly focus on localized pressure relief during positioning, lacking systematic, standardized comprehensive prevention protocols or evidence-based guidelines. To address it, Department of Nursing, Orthopedics Branch, China International Exchange and Promotive Association for Medical and Health Care, Spinal Trauma Professional Committee, Orthopedics Branch, Chinese Medical Doctor Association, Nursing Group of Spine and Spinal Cord Professional Committee of Chinese Association of Rehabilitation Medicine organized experts in relevant fields to formulate Guideline for the prevention of intraoperative acquired pressure injury in paraplegic patients with spinal cord injury ( version 2025), based on evidence-based medical evidence and latest research results and clinical practice at home and abroad. Eleven recommendations were put forward from the aspects of preoperative risk assessment, intraoperative prevention strategies, postoperative handover and monitoring, and supportive mechanisms for IAPI prevention, aiming to standardize the prevention measures and management strategies of IAPI in paraplegic patients with spinal cord injury and accelerate the recovery of patients and improve the therapeutic effect.

Paraplegia caused by spinal cord injury is a serious neurological complication, for which surgery is currently the main treatment method. Due to different surgical approaches, patients are usually expected to maintain a passive prone position for a long time or switch between the supine and prone positions. Affected by multiple factors such as neurogenic sensory disorders, pathological changes in muscle tone and operative duration, the risk of intraoperative acquired pressure injury (IAPI) is significantly increased. Current clinical prevention strategies for IAPI in these patients predominantly focus on localized pressure relief during positioning, lacking systematic, standardized comprehensive prevention protocols or evidence-based guidelines. To address it, Department of Nursing, Orthopedics Branch, China International Exchange and Promotive Association for Medical and Health Care, Spinal Trauma Professional Committee, Orthopedics Branch, Chinese Medical Doctor Association, Nursing Group of Spine and Spinal Cord Professional Committee of Chinese Association of Rehabilitation Medicine organized experts in relevant fields to formulate Guideline for the prevention of intraoperative acquired pressure injury in paraplegic patients with spinal cord injury ( version 2025), based on evidence-based medical evidence and latest research results and clinical practice at home and abroad. Eleven recommendations were put forward from the aspects of preoperative risk assessment, intraoperative prevention strategies, postoperative handover and monitoring, and supportive mechanisms for IAPI prevention, aiming to standardize the prevention measures and management strategies of IAPI in paraplegic patients with spinal cord injury and accelerate the recovery of patients and improve the therapeutic effect.

Intravascular large B-cell lymphoma (IVLBCL) is a rare large B-cell lymphoma subtype. We report a patient who presented with "recurrent fever and pancytopenia." A 64-year-old female patient had previously been diagnosed with Waldenstrom’s macroglobulinemia and had received zanubrutinib treatment. In February 2023, the patient revisited due to "recurrent fever and pancytopenia." A positron emission tomography/computed tomography scan demonstrated significant enlargement of the bilateral adrenal glands. After an adrenal biopsy, she was diagnosed with diffuse large B-cell lymphoma, not otherwise specified. The patient received chemotherapy with the R-CHOP regimen (rituximab + cyclophosphamide + doxorubicin + vincristine + prednisone). After three treatment courses, a cranial magnetic resonance imaging examination indicated central nervous system infiltration of the lymphoma. After reviewing the pathology of the adrenal biopsy, the final diagnosis was revised as IVLBCL. Despite aggressive treatment, the disease continued to progress, and the patient died two months later. According to a multidisciplinary level, this article discusses the case from the perspective of a multidisciplinary team collaboration, involving imaging, pathology, dermatology, and lymphoma, to provide reference opinions for the clinical diagnosis and treatment of IVLBCL.

Objective:To investigate the role of DNA damage and repair inhibition in the effect of ginkgo biloba on liver injury in patients with coal-burning-borne arsenism.Methods:In March 2017, the investigation was conducted in Jiaole village arsenic poisoning area in Yuzhang Town, Xingren County, Guizhou Province. According to the "Diagnosis of Endemic Arsenicosis" (WS/T 211-2015) and the "Diagnostic Criteria of Occupational Toxic Hepatopathy" (GBZ 59-2010), 52 patients with arsenism were selected as the ginkgo biloba intervention group, and 49 cases of arsenism patients as intervention control group. Ginkgo biloba tablets were given orally for 3 months (1 tablet/time, 3 times/d) according to the commonly used clinical methods, and no other drugs were given to all subjects during the intervention period. The intervention control group was given placebo in the same way as that of ginkgo biloba intervention group. A total of 41 residents who did not burn high arsenic coal 12 km away with no abnormal liver function were selected as normal control group. Physical examinations were performed before the intervention and at the end of the intervention at 3 months. After receiving signed informed consent, morning urine and peripheral venous blood samples were collected to detect urinary arsenic content by inductively coupled plasma mass spectrometry (ICP-MS); liver function biochemical indexes [albumin (ALB), albumin/globulin (A/G), cholinesterase (CHE), total bile acid (TBA)] were determined by automatic biochemical analyzer, DNA damage by single-cell gel electrophoresis assay, and the expression of miR-145 (repair inhibition index) by qRT-PCR.Results:There were 116 subjects, 41 in normal control group, 39 in ginkgo biloba intervention group and 36 in intervention control group. In ginkgo biloba and intervention and intervention control groups, there was no significant difference in age, gender, smoking habits and drinking compared with normal control group ( P > 0.05). Urinary arsenic content, TBA level, DNA damage degree [comet tail DNA percentage (TailDNA%) and olive tail moment (OTM)] and plasma miR-145 expression level [(38.75 ± 19.09) μg/g Cr, (11.13 ± 1.55) μmol/L, 8.50 ± 0.88, 7.43 ± 0.68, 5.78 ± 0.75, respectively] in ginkgo biloba intervention group patients before intervention were higher than those in normal control group [(11.62 ± 5.33) μg/g Cr, (5.36 ± 0.87) μmol/L, 5.24 ± 0.33, 4.71 ± 0.29, 2.05 ± 0.27, respectively], the differences were statistically significant ( P < 0.05); the levels of ALB, A/G and CHE were significantly lower than those in normal control group ( P < 0.05). After the intervention of ginkgo biloba, urinary arsenic content, TBA level, DNA damage degree (TailDNA% and OTM) and plasma miR-145 expression level in patients were significantly lower than those before the intervention ( P < 0.05); the levels of ALB, A/G and CHE were significantly higher than those before the intervention ( P < 0.05). There was no significant difference in the above indexes before and after intervention in the intervention control group ( P > 0.05). The results of correlation analysis between DNA damage degree, miR-145 and liver function indexes after the intervention of ginkgo biloba showed that, DNA damage degree (TailDNA% and OTM) was negatively correlated with the levels of ALB, A/G and CHE ( r = - 0.34, - 0.33, - 0.48, - 0.31, - 0.31, - 0.42, P < 0.05), and positively correlated with the level of TBA ( r = 0.49, 0.48, P < 0.05); miR-145 was negatively correlated with the levels of ALB, A/G and CHE ( r = - 0.26, - 0.23, - 0.38, P < 0.05), which was positively correlated with the level of TBA ( r = 0.32, P < 0.05); and DNA damage degree was positively correlated with the expression of miR-145 ( r = 0.65, 0.52, P < 0.05). Conclusion:Ginkgo biloba tablets can alleviate the liver damage caused by arsenic through coal burning, and the mechanism of this process is related to its inhibition of miR-145 expression and reduction of DNA damage.

Objective:To investigate the role of liver X-activated receptor (LXRα)/sterol-regulatory element binding protein (SREBP-1c) in arsenic induced lipid metabolism disorders in rats, and to provide a basis for study the mechanism of arsenic induced lipid metabolism disorders.Methods:Twenty-four healthy clean grade Wistar rats, were randomly divided into 4 groups according to body weight (80 - 100 g) by the random number table method, with 6 rats in each group, half male and half female. Rats in control group were given deionized water by gavage. The low, medium and high arsenic dose groups were given 2.5, 5.0 and 10.0 mg·kg -1·d -1 sodium arsenite solution by gavage, respectively. They were exposed to arsenic for 6 days a week for 4 months. At the end of the experiment, blood and liver samples of rats in each group were collected. The hepatic arsenic content was determined by inductively coupled plasma mass spectrometry (ICP-MS); the serum levels of triglyceride (TG), total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C) and high-density lipoprotein cholesterol (HDL-C) were measured by automatic biochemical analyzer. The mRNA expression levels of LXRα and SREBP-1c in liver tissues were determined by real-time PCR; the protein expression levels of LXRα, SREBP-1c, acetyl CoA carboxylase (ACC) and phospho-ACC (pACC) in liver tissues were determined by Western blotting. Results:The hepatic arsenic contents of rats in low, medium and high arsenic dose groups were (61.04 ± 4.98), (62.66 ± 6.71) and (87.86 ± 13.89) μg/g, respectively, which were higher than that in control group [(2.43 ± 0.63) μg/g, P < 0.05], and the hepatic arsenic content of rats in high arsenic dose group was higher than those in low and medium arsenic dose groups ( P < 0.05). The serum TG levels of rats in low, medium and high arsenic dose groups were (0.90 ± 0.17), (1.28 ± 0.24) and (1.82 ± 0.18) mmol/L, respectively, which were higher than that in control group [(0.50 ± 0.12) mmol/L, P < 0.05]; the serum LDL-C levels of rats in low, medium and high arsenic dose groups were (0.54 ± 0.04), (0.63 ± 0.07) and (0.69 ± 0.08) mmol/L, respectively, which were higher than that in control group [(0.27 ± 0.05) mmol/L, P < 0.05]; the serum TC levels of rats in medium and high arsenic dose groups were (1.88 ± 0.23) and (2.10 ± 0.10) mmol/L, respectively, which were higher than that in control group [(1.51 ± 0.14) mmol/L, P < 0.05]; the serum HDL-C levels of rats in medium and high arsenic dose groups were (0.84 ± 0.11) and (0.71 ± 0.14) mmol/L, respectively, which were lower than that in control group [(1.15 ± 0.08) mmol/L, P < 0.05]; and the serum levels of TG and LDL-C in medium and high arsenic dose groups were higher than those in low arsenic dose group ( P < 0.05), and the serum level of TG in high arsenic dose group was higher than that in medium arsenic dose group ( P < 0.05). The mRNA expression level of hepatic LXRα of rats in high arsenic dose group was higher than those in control group and low arsenic dose group ( P < 0.05); there was no significant difference in mRNA expression levels of hepatic SREBP-1c of rats between low, medium and high arsenic dose groups and control group ( P > 0.05). The protein expression levels of hepatic LXRα of rats in medium and high arsenic dose groups were higher than that in control group ( P < 0.05), and high arsenic dose group was higher than low arsenic dose group ( P < 0.05); the protein expression levels of hepatic SREBP-1c and ACC of rats in high arsenic dose group were higher than that in control group ( P < 0.05). There was a positive correlation between hepatic arsenic content in arsenic-exposed rats and the serum levels of TG, TC, LDL-C, the mRNA expression level of hepatic LXRα, the protein expression levels of hepatic LXRα, SREBP-1c and ACC ( r = 0.84, 0.62, 0.89, 0.55, 0.54, 0.64, 0.70, P < 0.05), and the serum level of HDL-C was negatively correlated with the hepatic arsenic content in arsenic-exposed rats ( r = - 0.75, P < 0.001). Conclusion:Sodium arsenite can increase the serum levels of TG, TC and LDL-C, decrease the serum level of HDL-C and increase the protein expression levels of LXRα and SREBP-1c in liver tissues, suggesting that arsenic induced lipid metabolism disorders in rats may be related to the upstream regulation mechanism of LXRα/SREBP-1c.

Objective:To understand the relationship between the disease condition of patients with coal-burning-borne endemic arsenic poisoning (abbreviated as coal-burning-borne arsenic poisoning) and serum lipid metabolism indicators.Methods:Using a case-control study method, in the coal-burning-borne arsenic poisoning village of Yuzhang Town, Qianxinan Prefecture, Guizhou Province, 204 patients with arsenic poisoning diagnosed according to the standard of "Diagnosis of Endemic Arsenicosis" (WS/T 211-2015) were included in case group, including 87 males and 117 females, aged(53.37 ± 8.06) years old; and they were divided into mild arsenic poisoning group (71 cases), moderate arsenic poisoning group (59 cases) and severe arsenic poisoning group (74 cases) according to the clinical grading. Another 63 residents were selected into control group in a non-arsenic-exposed village about 12 km away from the diseased village, including 23 males and 40 females, aged (53.78 ± 9.10) years old. A face-to-face questionnaire survey was conducted for each group of people, including basic information such as general demographic characteristics, smoking status, and drinking status; fasting peripheral blood was collected, and an automatic biochemical analyzer was used to detect serum total cholesterol (TC) and triglyceride (TG), low-density lipoprotein cholesterol (LDL-C), and high-density lipoprotein cholesterol (HDL-C) levels.Results:There were significant differences of serum TC [(4.94 ± 1.00), (5.00 ± 0.99), (5.27 ± 0.94), (5.57 ± 1.07) mmol/L], TG [(2.17 ± 0.90), (2.25 ± 1.31), (2.66 ± 1.43), (2.78 ± 1.40) mmol/L], LDL-C [(2.51 ± 0.79), (2.74 ± 0.64), (2.97 ± 0.66), (3.15 ± 0.80) mmol/L], and HDL-C levels [(1.57 ± 0.55), (1.42 ± 0.43), (1.36 ± 0.42), (1.30 ± 0.38) mmol/L] in control group, mild, moderate and severe arsenic poisoning groups ( F = 5.83, 3.64, 9.72, 4.41, P < 0.01 or < 0.05). Among them, the serum TC level in severe arsenic poisoning group, serum TG and LDL-C levels in moderate and severe arsenic poisoning groups were significantly higher than those in control group ( P < 0.05); the serum HDL-C level in moderate and severe arsenic poisoning groups were lower than that in control group ( P < 0.05); the serum TC, TG and LDL-C levels in severe arsenic poisoning group were significantly higher than those in mild arsenic poisoning group ( P < 0.05). After linear trend test, serum TC, TG and LDL-C levels all showed an upward trend with the degree of arsenic poisoning ( Ftrend = 15.77, 10.14, 29.15, P < 0.05), and serum HDL-C level showed a downward trend with the degree of arsenic poisoning ( Ftrend = 12.75, P < 0.05). There were significant differences in the abnormal rates of serum TC, TG and LDL-C levels among control group and mild, moderate and severe arsenic poisoning groups (χ 2 = 21.16, 16.60, 8.29, P < 0.01 or < 0.05). Among them, the serum TC and TG levels abnormal rates in moderate and severe arsenic poisoning groups and serum LDL-C level abnormal rate in severe arsenic poisoning group were higher than those in control group ( P < 0.05), the serum TC, TG and LDL-C levels abnormal rates in severe arsenic poisoning group were higher than those in mild arsenic poisoning group ( P < 0.05). There was no significant difference of the serum HDL-C level abnormal rate among four groups (χ 2 = 2.11 , P > 0.05). The results of trend chisquare analysis showed that the abnormal rates of serum TC, TG and LDL-C levels presented an increasing trend with the degree of arsenic poisoning (χ 2trend = 19.90, 15.25, 7.63, P<0.05). The results of logistic regression analysis showed that the risk of abnormal serum TC level in patients with severe arsenic poisoning was 2.90 times that in control group [odds ratio ( OR) = 2.90, 95% confidence interval ( CI): 1.43 - 5.91], and the risk of abnormal serum LDL-C level in patients with severe arsenic poisoning was 2.87 times that in control group ( OR = 2.87, 95% CI: 1.22 - 6.71). Conclusion:There is a correlation between the disease condition of patients with coal-burning-borne arsenic poisoning and their dyslipidemia.

Objective:Comparative analysis of diabetes was carried out in coal-burning arsenic poisoning areas and non-arsenic exposed villages of Yuzhang Town, so as to explore the relationship between arsenic exposure and diabetes.Methods:Data of basic information of 594 people who were diagnosed and included in the diabetes management in Central Health Center of Yuzhang Town in Qianxinan Prefecture Guizhou Province in 2018 were collected. According to the "Standards for the Determination and Classification of Endemic Arsenic Poisoning Areas" (WS 277-2007), 11 administrative villages in the town were divided into 5 arsenic poisoning villages and 6 non-arsenic exposure villages. The prevalence (%) was used for statistical description.Results:In 2018, the prevalence of diabetes in Yuzhang Town was 1.74% (594/34 218), 1.38% (243/17 665) for men and 2.12% (351/16 553) for women, the gender difference was statistically significant (χ 2=27.794, P < 0.05). The prevalence of standardized diabetes in arsenic poisoning villages was 3.38%; the prevalence of standardized diabetes in non-arsenic exposure villages was 3.13%. After sex stratification analysis, the non-arsenic exposed villages were used as reference. The OR and 95% CI of diabetic patients in arsenic poisoning villages were 0.65 (0.50-0.81) for males and 1.35 (1.09-1.67) for females. Conclusions:The association between arsenic exposure and diabetes is related to gender. The risk of diabetes mellitus in women is higher than that in men.