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

BACKGROUND:Allogeneic hematopoietic stem cell transplantation is an important treatment for malignant hematological diseases,and delayed postoperative platelet implantation is a common complication that seriously affects the quality of patient survival;however,there are no standard protocols to improve platelet implantation rates and prevent platelet implantation delays. OBJECTIVE:To compare the safety and efficacy of oral Herombopag Olamine versus subcutaneous recombinant human thrombopoietin for promoting platelet implantation in patients with malignant hematological diseases undergoing haploid hematopoietic stem cell transplantation. METHODS:Clinical data of 163 patients with malignant hematological diseases who underwent haploidentical hematopoietic stem cell transplantation from January 2016 to October 2022 were retrospectively analyzed.A total of 72 patients who started to subcutaneously inject recombinant human thrombopoietin at+2 days were categorized into the recombinant human thrombopoietin group;a total of 27 patients who started to orally take Herombopag Olamine at+2 days were categorized into the Herombopag Olamine group;and 64 patients who did not apply Herombopag Olamine or recombinant human thrombopoietin were categorized into the blank control group.The implantation status,incidence of acute graft-versus-host disease of degree II-IV within 100 days,1-year survival rate,1-year recurrence rate,and safety were analyzed in the three groups. RESULTS AND CONCLUSION:(1)The average follow-up time was 52(12-87)months.The implantation time of neutrophils in the blank control group,recombinant human thrombopoietin group,and Herombopag Olamine group was(12.95±3.88)days,(14.04±3.71)days,and(13.89±2.74)days,respectively,with no statistically significant difference(P=0.352);the implantation time of platelets was(15.16±6.27)days,(17.67±6.52)days,and(17.00±4.75)days,with no statistically significant difference(P=0.287).(2)The complete platelet implantation rate on day 60 was 64.06%,90.28%,and 92.59%,respectively,and the difference was statistically significant(P<0.001).The subgroup analysis showed that the difference between the blank control group and the recombinant human thrombopoietin group was statistically significant(P<0.001),and the difference between the blank control group and the Herombopag Olamine group was statistically significant(P=0.004).The difference was not statistically significant between the recombinant human thrombopoietin group and Herombopag Olamine group(P=0.535).(3)100-day II-IV degree acute graft-versus-host disease incidence in the blank control group,recombinant human thrombopoietin group,and Herombopag Olamine group were 25.00%,30.56%,and 25.93%,respectively,and the difference was not statistically significant(P=0.752).(4)The incidence of cytomegalovirus anemia,cytomegalovirus pneumonia,and hepatic function injury had no statistical difference among the three groups(P>0.05).(5)During the follow-up period,there was no thrombotic event in any of the three groups of patients.(6)The results showed that recombinant human thrombopoietin and Herombopag Olamine could improve the platelet implantation rate of malignant hematological disease patients after haploidentical hematopoietic stem cell transplantation,with comparable efficacy and good safety.

Objective To systematically review the safety of budesonide inhalation aerosol in the treatment of chronic obstructive pulmonary disease.Methods PubMed,Cochrane Library,Web of Science,Embase,CNKI,WanFang Data,VIP and SinoMed databases were electronically searched to collect randomized controlled trials on budesonide inhalation aerosol in the treatment of chronic obstructive pulmonary disease from inception to June 30,2024.Two reviewers independently screened literature,extracted data,and assessed the risk of bias of included studies.The Meta-analysis was performed by using RevMan 5.3 software.Results A total of 52 studies with 16,768 patients were included.Meta-analysis results showed that the incidence of respiratory system adverse drug reaction(ADR)was higher in the budesonide group than in the control group,with a statistically significant difference[OR=1.18,95％CI(1.10,1.27),P＜0.001].There was no statistically significant difference in the incidence of overall ADR,central and peripheral nervous system ADR,gastrointestinal system ADR,skin ADR,urinary system ADR,infectious disease ADR,and musculoskeletal system ADR between the two groups of patients(P＞0.05).According to the subgroup analysis of the treatment course,in the 3-6 month subgroup,the incidence of ADR was higher in the budesonide group than in the control group,with a statistically significant difference[OR=1.43,95％CI(1.18,1.74),P＜0.001].In the studies with a treatment duration of more than 6 months,the incidence of ADR was higher in the budesonide group than in the control group,with a statistically significant difference[OR=1.54,95％CI(1.16,2.03),P=0.002].In the study of unknown treatment course,the incidence of ADR in the budesonide group was lower than that in the control group,and the difference was statistically significant[OR=0.39,95％CI(0.20,0.76),P=0.005].In the studies with a treatment duration of less than 3 months,the incidence of ADR in the budesonidegroup was comparable to that in the control group(P＞0.05).Conclusion The incidence of respiratory ADR caused by budesonide is higher than that of the control group.Although the incidence of overall ADR is not statistically significant,the occurrence of systemic adverse reactions should continue to be closely monitored in the future treatment of chronic obstructive pulmonary disease.

Objective:To investigate the associations between thyroid homeostasis and risk of carotid plaque in healthy euthyroid individuals.Methods:In this retrospective cohort study, 4 726 healthy euthyroid adults who received health examination two times or more in the Health Promotion Center of the First Affiliated Hospital with Nanjing Medical University from January 2018 to December 2024 and had no carotid artery plaques at the baseline were selected. Data encompassed demographics, physical and laboratory tests, carotid ultrasound, and calculated thyroid sensitivity indices [thyroid feedback quantile-based index (TFQI), thyroid hormone resistance index (TT4RI), thyroid-stimulating hormone index (TSHI), and free triiodothyronine (FT3)/free thyroxine (FT4) ratio] were recorded. The participants were divided into two groups based on whether they had newly-developed carotid plaques. The associations between thyroid homeostasis indices and risk of carotid plaque were analyzed using Cox proportional hazards regression and restricted cubic splines.Results:During 11 459 person-years of follow-up, the cumulative incidence of carotid plaque was 16.84%, with an incidence density of 6.95 cases per 100 person-years. After multivariable adjustment, FT3 ( HR=0.79, 95% CI: 0.68-0.93) and FT4 ( HR=0.95, 95% CI: 0.91-0.98) were inversely associated with risk of carotid plaque. TSH and thyroid hormone sensitivity showed no significant association with the occurrence of carotid plaques. Subgroup analysis showed that there was no significant interaction between thyroid function indicators and risk of carotid plaque among different subgroups, but the decreased FT3 and FT4 levels significantly increased the risk of new-onset carotid plaques in individuals aged<60 years, without diabetes, without hypertension, or without lipid-lowering medication use. Restricted cubic spline analysis indicated that when TFQI (nonlinear P=0.028, node value was 0.29) and TT4RI (nonlinear P=0.014, node value was 54.95) exceeded specific thresholds, their increase were associated with a reduced risk of carotid plaque. Conclusions:The reduction of FT3 and FT4 in healthy euthyroid adults is associated with an increased risk of carotid plaque. When TFQI and TT4RI are higher than specific thresholds, their increase are associated with a reduced risk of new-onset plaques.

Malignant hyperthermia(MH)is a rare perioperative disease with autosomal dominant inheritance,and its pathogenesis involves specific gene mutations.Its clinical feature is that conventional anesthetics can trigger abnormally high metabolic reactions in skeletal muscles.Although the incidence of this disease is low,the condition is dangerous,progresses rapidly,and has a high mortality rate;Its treatment relies on early diagnosis,timely application of the specific drug Dantrolene Sodium,and rapid and orderly comprehensive symptomatic supportive treatment.MH is a critical perioperative emergency that can occur during surgery.It presents with symptoms such as hyperpyrexia,metabolic acidosis,rhabdomyolysis,and dysfunction of multiple organ systems.If not treated promptly,it can quickly lead to life-threatening arrhythmias and cardiac arrest.This condition serves as an essential teaching example in anesthesia crisis resource management.As an effective teaching method,scenario simulation exercises can comprehensively enhance medical staff's personal technical,non-technical,and teamwork abilities through simulating emergency scenarios,teaching assessments,and retrospective discussions,especially suitable for comprehensive management training of fatal diseases.Many countries internationally have incorporated simulation exercises for MH into their routine teaching and training systems.The effectiveness of teaching and training for anesthesiologists in MH and their ability to handle anesthesia crisis events have been continuously improved through a periodic training model.This article systematically reviews the research progress and practical experience of scenario simulation exercises in emergency training for MH,with a focus on exploring how to establish a scenario simulation exercise plan for emergency application and comprehensive symptomatic support treatment of Dantrolene Sodium based on the actual situation in China,providing reference for improving the teaching and training quality of MH and other clinical crisis events.

Objective:To summarize the experience of designing hair transplantation for post-cleft lip repair moustache defects based on the morphological and tissue characteristics of the upper lip.Methods:A retrospective analysis was conducted on the clinical data of male patients treated at the Hair Transplantation Center of Plastic Surgery Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College from January 2011 to July 2024. Based on the morphology and tissue texture of the upper lip after cleft lip repair, the position and shape of the moustache were designed. Hair follicles were harvested from the mid-occipital region and/or the jaw shadow area using either the strip harvesting method or the follicular unit excision (FUE) technique. Needles of 21 or 22 gauge were used to create incisions in the recipient sites down to the superficial subcutaneous layer. The hair shafts were clamped with micro-forceps and then transplanted into the recipient sites to restore the moustache shape. Postoperatively, the density, shape, direction of the moustache, and the condition of the donor site scars were observed and followed up.Results:A total of 47 male patients, aged 23-43 years (mean 28.7 years), were included. Among them, 29 had undergone lower triangular flap repair, 13 received the Millard technique, and 5 were treated with other surgical methods for cleft lip repair. For hair follicle extraction, the strip method was used in 7 cases, and FUE in 40 cases. The donor sites included the jaw shadow area (9 cases), mid-occipital region (23 cases), and a combination of both (15 cases). The number of transplanted follicular units ranged from 33 to 500 (mean 217). Follow-up duration ranged from 9 months to 10 years (mean 3.5 years). Postoperative complications included folliculitis in 6 cases, and 4 cases required additional transplantation due to insufficient density after one year. The remaining patients exhibited satisfactory hair growth, with natural mustache shape and direction. The graft survival rate was approximately 80%, and donor site scarring was minimal.Conclusion:When performing hair transplantation to treat post-cleft lip repair moustache defects, the design should prioritize the morphological and tissue characteristics of the upper lip, followed by consideration of overall position and bilateral symmetry of the moustache. Only by fully considering the characteristics of the recipient area can optimally repair outcomes be achieved.

Objective The protective effect and molecular mechanism of electroacupuncture combined with massage on cartilage damage in knee Osteoarthritis rats were explored.Methods 50 SD rats were selected and divided into a control group,a model group,an electroacupuncture group,a massage group,and an electroacupuncture+massage group of 10 rats each using an odd even number method.The rats in the control group were not treated with modeling,and the rats in the other groups were injected with Papain into the articular cavity to build the right knee Osteoarthritis(KOA)model.The massage and electroacupuncture groups were intervened with electroacupuncture and massage for 2 weeks.The improved Le-quesne MG scale was used to evaluate the behavioral performance of rats.The pressure pain value and heat pain threshold of rats before and after intervention were calculated.After eight weeks,the rats were euthanized and abdominal aortic blood was collected.The serum levels of pain mediators(K+),5-hydroxytryptamine(5-HT),and dopamine(DA)were measured by ELISA in each group of rats.The right knee joint of rats was taken for HE staining Mankin score,TUNEL chondrocyte apoptosis analysis.Toluidine blue staining was used to observe the changes of cartilage matrix polysaccharide;Western blot was used to detect the concentration and expression of cartilage matrix factor type Ⅱ collagen(Collagen Ⅱ),C-terminal peptide(CTX Ⅱ),type Ⅱ collagen C-precursor peptide(CPⅡ),apoptosis related proteins Bax,Bcl-2,Cleared caspase-3,YAP,p-YAP in the knee joint tissues of rats in each group.Results After the establishment of the KOA model,the Lequesne MG score of rats increased significantly(P<0.05).Compared with the model group,the Lequesne MG scores of rats in the electroacupuncture group and massage group decreased significantly(P<0.05).The combination of the two can further reduce the Lequesne MG score significantly(P<0.05).Compared with the control group,the pain threshold(TWL,MWT)of the model group was downregulated significantly,and the levels of serum pain factors(K+,DA,5-HT)were upregulated significantly(P<0.05).Compared with the model group,the pain thresholds(TWL,MWT)of the electroacupuncture group and the massage group were upregulated significantly,while the levels of serum pain factors(K+,DA,5-HT)were downregulated significantly(P<0.05).The combination of the two reflected the highest pain threshold(TWL,MWT)and the lowest serum pain factor(K+,DA,5-HT)levels.The model group rats had significant joint cartilage fissures,cartilage matrix staining and staining area decreased,and Markin score and cell apoptosis ability were improved(P<0.05).Compared with the model group,the arrangement degree of the cartilage layer,staining degree of the cartilage matrix,and staining area of the electroacupuncture group and massage group were all improved,while the Markin score and cell apoptosis rate all decreased significantly(P<0.05).The combination can further reduce the Markin score and the degree of chondrocyte apoptosis(P<0.05).Compared with the control group,the expression of Collagen Ⅱ,CPⅡ,Bcl-2,and p-YAP proteins in the model group decreased significantly,while the expression of CTX Ⅱ,Bax,and Cleared caspase-3 proteins increased significantly(P<0.05).Conclusion The combination of electroacupuncture and massage intervention can significantly improve the pathological state of cartilage injury in rats with osteoarthritis,reduce knee joint pain,inhibit chondrocyte apoptosis,and play a protective role in cartilage injury.Its molecular mechanism may be related to the activation of Hippo YAP signaling pathway,which is worth further research in clinical practice.

Objective To systematically review the safety of budesonide inhalation aerosol in the treatment of chronic obstructive pulmonary disease.Methods PubMed,Cochrane Library,Web of Science,Embase,CNKI,WanFang Data,VIP and SinoMed databases were electronically searched to collect randomized controlled trials on budesonide inhalation aerosol in the treatment of chronic obstructive pulmonary disease from inception to June 30,2024.Two reviewers independently screened literature,extracted data,and assessed the risk of bias of included studies.The Meta-analysis was performed by using RevMan 5.3 software.Results A total of 52 studies with 16,768 patients were included.Meta-analysis results showed that the incidence of respiratory system adverse drug reaction(ADR)was higher in the budesonide group than in the control group,with a statistically significant difference[OR=1.18,95％CI(1.10,1.27),P＜0.001].There was no statistically significant difference in the incidence of overall ADR,central and peripheral nervous system ADR,gastrointestinal system ADR,skin ADR,urinary system ADR,infectious disease ADR,and musculoskeletal system ADR between the two groups of patients(P＞0.05).According to the subgroup analysis of the treatment course,in the 3-6 month subgroup,the incidence of ADR was higher in the budesonide group than in the control group,with a statistically significant difference[OR=1.43,95％CI(1.18,1.74),P＜0.001].In the studies with a treatment duration of more than 6 months,the incidence of ADR was higher in the budesonide group than in the control group,with a statistically significant difference[OR=1.54,95％CI(1.16,2.03),P=0.002].In the study of unknown treatment course,the incidence of ADR in the budesonide group was lower than that in the control group,and the difference was statistically significant[OR=0.39,95％CI(0.20,0.76),P=0.005].In the studies with a treatment duration of less than 3 months,the incidence of ADR in the budesonidegroup was comparable to that in the control group(P＞0.05).Conclusion The incidence of respiratory ADR caused by budesonide is higher than that of the control group.Although the incidence of overall ADR is not statistically significant,the occurrence of systemic adverse reactions should continue to be closely monitored in the future treatment of chronic obstructive pulmonary disease.

Objective:To summarize the experience of designing hair transplantation for post-cleft lip repair moustache defects based on the morphological and tissue characteristics of the upper lip.Methods:A retrospective analysis was conducted on the clinical data of male patients treated at the Hair Transplantation Center of Plastic Surgery Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College from January 2011 to July 2024. Based on the morphology and tissue texture of the upper lip after cleft lip repair, the position and shape of the moustache were designed. Hair follicles were harvested from the mid-occipital region and/or the jaw shadow area using either the strip harvesting method or the follicular unit excision (FUE) technique. Needles of 21 or 22 gauge were used to create incisions in the recipient sites down to the superficial subcutaneous layer. The hair shafts were clamped with micro-forceps and then transplanted into the recipient sites to restore the moustache shape. Postoperatively, the density, shape, direction of the moustache, and the condition of the donor site scars were observed and followed up.Results:A total of 47 male patients, aged 23-43 years (mean 28.7 years), were included. Among them, 29 had undergone lower triangular flap repair, 13 received the Millard technique, and 5 were treated with other surgical methods for cleft lip repair. For hair follicle extraction, the strip method was used in 7 cases, and FUE in 40 cases. The donor sites included the jaw shadow area (9 cases), mid-occipital region (23 cases), and a combination of both (15 cases). The number of transplanted follicular units ranged from 33 to 500 (mean 217). Follow-up duration ranged from 9 months to 10 years (mean 3.5 years). Postoperative complications included folliculitis in 6 cases, and 4 cases required additional transplantation due to insufficient density after one year. The remaining patients exhibited satisfactory hair growth, with natural mustache shape and direction. The graft survival rate was approximately 80%, and donor site scarring was minimal.Conclusion:When performing hair transplantation to treat post-cleft lip repair moustache defects, the design should prioritize the morphological and tissue characteristics of the upper lip, followed by consideration of overall position and bilateral symmetry of the moustache. Only by fully considering the characteristics of the recipient area can optimally repair outcomes be achieved.

Objective The protective effect and molecular mechanism of electroacupuncture combined with massage on cartilage damage in knee Osteoarthritis rats were explored.Methods 50 SD rats were selected and divided into a control group,a model group,an electroacupuncture group,a massage group,and an electroacupuncture+massage group of 10 rats each using an odd even number method.The rats in the control group were not treated with modeling,and the rats in the other groups were injected with Papain into the articular cavity to build the right knee Osteoarthritis(KOA)model.The massage and electroacupuncture groups were intervened with electroacupuncture and massage for 2 weeks.The improved Le-quesne MG scale was used to evaluate the behavioral performance of rats.The pressure pain value and heat pain threshold of rats before and after intervention were calculated.After eight weeks,the rats were euthanized and abdominal aortic blood was collected.The serum levels of pain mediators(K+),5-hydroxytryptamine(5-HT),and dopamine(DA)were measured by ELISA in each group of rats.The right knee joint of rats was taken for HE staining Mankin score,TUNEL chondrocyte apoptosis analysis.Toluidine blue staining was used to observe the changes of cartilage matrix polysaccharide;Western blot was used to detect the concentration and expression of cartilage matrix factor type Ⅱ collagen(Collagen Ⅱ),C-terminal peptide(CTX Ⅱ),type Ⅱ collagen C-precursor peptide(CPⅡ),apoptosis related proteins Bax,Bcl-2,Cleared caspase-3,YAP,p-YAP in the knee joint tissues of rats in each group.Results After the establishment of the KOA model,the Lequesne MG score of rats increased significantly(P<0.05).Compared with the model group,the Lequesne MG scores of rats in the electroacupuncture group and massage group decreased significantly(P<0.05).The combination of the two can further reduce the Lequesne MG score significantly(P<0.05).Compared with the control group,the pain threshold(TWL,MWT)of the model group was downregulated significantly,and the levels of serum pain factors(K+,DA,5-HT)were upregulated significantly(P<0.05).Compared with the model group,the pain thresholds(TWL,MWT)of the electroacupuncture group and the massage group were upregulated significantly,while the levels of serum pain factors(K+,DA,5-HT)were downregulated significantly(P<0.05).The combination of the two reflected the highest pain threshold(TWL,MWT)and the lowest serum pain factor(K+,DA,5-HT)levels.The model group rats had significant joint cartilage fissures,cartilage matrix staining and staining area decreased,and Markin score and cell apoptosis ability were improved(P<0.05).Compared with the model group,the arrangement degree of the cartilage layer,staining degree of the cartilage matrix,and staining area of the electroacupuncture group and massage group were all improved,while the Markin score and cell apoptosis rate all decreased significantly(P<0.05).The combination can further reduce the Markin score and the degree of chondrocyte apoptosis(P<0.05).Compared with the control group,the expression of Collagen Ⅱ,CPⅡ,Bcl-2,and p-YAP proteins in the model group decreased significantly,while the expression of CTX Ⅱ,Bax,and Cleared caspase-3 proteins increased significantly(P<0.05).Conclusion The combination of electroacupuncture and massage intervention can significantly improve the pathological state of cartilage injury in rats with osteoarthritis,reduce knee joint pain,inhibit chondrocyte apoptosis,and play a protective role in cartilage injury.Its molecular mechanism may be related to the activation of Hippo YAP signaling pathway,which is worth further research in clinical practice.