Objective:Dehydrated hereditary stomatocytosis (DHS), a rare condition caused by pathogenic PIEZO1 or KCNN4 mutations, remains under-recognized. This study aimed to improve the diagnosis and management of DHS in China by retrospectively analyzing clinical and genetic characteristics.Methods:Patients diagnosed with DHS at Peking Union Medical College Hospital between 2018 and 2024 were included. All had suspected congenital hemolytic anemia and were confirmed by next-generation sequencing to harbor pathogenic PIEZO1 or KCNN4 mutations. Clinical features, genotypes, iron overload, and treatment outcomes were collected and analyzed.Results:Twenty-three DHS patients were identified, including 21 with PIEZO1 and 2 with KCNN4 mutations. Common manifestations were splenomegaly, jaundice, cholelithiasis, and secondary iron overload. Mild and moderate anemia occurred in 8.7% and 17.4% of patients, respectively, and stomatocytes were present in only 17.4%. The median time from symptom onset to diagnosis was 11 years. The predominant PIEZO1 variants were c.7367G > A and c.6328C > T, with nine novel mutations identified. Both KCNN4 mutations involved c.1055G > A. No clear genotype–phenotype correlation was observed. Secondary hepatic iron overload was found in 21.7% of patients.Conclusion:DHS is often underdiagnosed or misdiagnosed. Genetic testing enables accurate identification, and regular monitoring for secondary hepatic iron overload is crucial for long-term management.

Psoriasis arthritis (PsA) is a chronic inflammatory musculoskeletal disease intricately linked to psoriasis (PsO), with a multifaceted etiology encompassing genetic, environmental, and immunological factors. Characterized by complex clinical manifestations, PsA often follows a protracted course with a propensity for relapses, potentially culminating in joint deformity and disability. The condition is further complicated by associated comorbidities such as inflammatory bowel disease, uveitis, cardiovascular disease, and metabolic syndrome, which significantly diminish patients′ quality of life. Early detection and screening of PsA are crucial for its management and prevention of adverse outcomes. However, in China, there is a notable deficiency in the recognition and early diagnosis of PsA, with missed or incorrect diagnoses being relatively common. The consensus comprises four overarching statements and sixteen detailed recommendations, with the overarching goal of enhancing the early diagnosis and treatment of PsA by clinical physicians, thereby improving patient outcomes.

Objective:Dehydrated hereditary stomatocytosis (DHS), a rare condition caused by pathogenic PIEZO1 or KCNN4 mutations, remains under-recognized. This study aimed to improve the diagnosis and management of DHS in China by retrospectively analyzing clinical and genetic characteristics.Methods:Patients diagnosed with DHS at Peking Union Medical College Hospital between 2018 and 2024 were included. All had suspected congenital hemolytic anemia and were confirmed by next-generation sequencing to harbor pathogenic PIEZO1 or KCNN4 mutations. Clinical features, genotypes, iron overload, and treatment outcomes were collected and analyzed.Results:Twenty-three DHS patients were identified, including 21 with PIEZO1 and 2 with KCNN4 mutations. Common manifestations were splenomegaly, jaundice, cholelithiasis, and secondary iron overload. Mild and moderate anemia occurred in 8.7% and 17.4% of patients, respectively, and stomatocytes were present in only 17.4%. The median time from symptom onset to diagnosis was 11 years. The predominant PIEZO1 variants were c.7367G > A and c.6328C > T, with nine novel mutations identified. Both KCNN4 mutations involved c.1055G > A. No clear genotype–phenotype correlation was observed. Secondary hepatic iron overload was found in 21.7% of patients.Conclusion:DHS is often underdiagnosed or misdiagnosed. Genetic testing enables accurate identification, and regular monitoring for secondary hepatic iron overload is crucial for long-term management.

Psoriasis arthritis (PsA) is a chronic inflammatory musculoskeletal disease intricately linked to psoriasis (PsO), with a multifaceted etiology encompassing genetic, environmental, and immunological factors. Characterized by complex clinical manifestations, PsA often follows a protracted course with a propensity for relapses, potentially culminating in joint deformity and disability. The condition is further complicated by associated comorbidities such as inflammatory bowel disease, uveitis, cardiovascular disease, and metabolic syndrome, which significantly diminish patients′ quality of life. Early detection and screening of PsA are crucial for its management and prevention of adverse outcomes. However, in China, there is a notable deficiency in the recognition and early diagnosis of PsA, with missed or incorrect diagnoses being relatively common. The consensus comprises four overarching statements and sixteen detailed recommendations, with the overarching goal of enhancing the early diagnosis and treatment of PsA by clinical physicians, thereby improving patient outcomes.

Objective:To explore the application effect of preoperative autologous blood localization method in laparoscopic resection of gastric stromal tumors in unfavorable areas of the stomach.Methods:A retrospective analysis was conducted on the case data of 40 patients with gastric stromal tumors in unfavorable locations admitted to Jinjiang Hospital from January 2019 to December 2022. The patients were divided into a control group (intraoperative endoscopic localization method) and an autologous blood localization group according to different intraoperative lesion localization methods, with 20 cases in each group. The surgical time, intraoperative blood loss, hospitalization time, postoperative exhaust time, and adverse reactions were compared between the two groups.Results:The surgery time of the autologous blood localization group was shorter than that of the control group [(92.30±8.80)min vs (108.20±14.87)min, P<0.05]. There was no statistically significant difference in intraoperative bleeding, hospitalization time, and postoperative exhaust time between the two groups (all P>0.05). Two groups of patients did not show an increase in inflammatory indicators such as white blood cells and C-reactive protein on the day after surgery. Both groups of patients did not experience adverse reactions such as fever, abdominal pain, or postoperative complications. Conclusions:The autologous blood injection localization method provides a safe, simple, and effective method for preoperative localization of gastric stromal tumors in unfavorable areas of the stomach under laparoscopy, and is worthy of clinical promotion and use.

Objective:To explore the differences on clinical characteristics, concomitant diseases and treatment status between psoriasis and psoriatic arthritis (PsA), and provide clues for the early diagnosis and treatment of PsA.Methods:Data were collected by in-person interview of 225 patients with psoriasis and 299 patients with PSA who visited the department of rheumatology and Immunology and Department of Dermatology in People′s Hospital of Peking University from November 2020 to May 2021. After informed consent, the questionnaire was completed on site. The differences of clinical characteristics, concomitant diseases, mental health evaluation and treatment status between patients with arthritis (PsA) and patients with psoriasiswere analyzed and compared. Enumeration data were described by frequency. Chi square test was used to compare categorical variables. Multivariate Logistic regression analysis was used to determine the independent risk factors. P value of less than 0.05 was considered statistically significant. Results:Dactylitis [ OR(95% CI)=8.439(4.677,15.226), P<0.001], hip pain [ OR(95% CI)=3.442(1.829,6.480), P<0.001], heel pain [ OR(95% CI)=2.621(1.652,4.157), P<0.001] and low back pain [ OR(95% CI)=1.924(1.156,3.203), P=0.012] may be closely related to the progression of PsA ( P<0.05). The three most common concomitant diseases of patients with PsA and psoriasis both were overweight [43.1%(129/299)、29.3%(66/225)], fatty liver [(28.4%(85/299)、23.1%(52/225)]and hypertension[24.1%(72/299、13.3%(30/225)]. The proportion of osteoporosis in PsA group at the age of 30-39 and 40-49 years old was significantly higher than those in psoriasis group (30-39 years old:12.5%(10/80) vs 1.5%(1/65), χ2=6.14, P=0.013; 40~49 years old: 19.2%(15/78) vs 2.0%(1/51), χ2=8.46, P=0.004]. The proportion of hypertension in PsA group was also higher than that in psoriasis group at the age of 40~49 years old[7.0% (21/78) vs 2.7%(6/51), χ2=4.99, P=0.026)]. And the proportion of fatty liver in PsA group was also higher than that in psoriasis group at the age ≥60 years old [(46.0%(23/50) vs 29.1(7/24), χ2=4.99, P=0.025)]. Among 299 PsA patients, 47.1%(141/299) had anxiety tendency, 45.2%(135/299) had sleep disorder and 41.8%(125/299) had depression tendency. Among 225 psoriasis patients, 44.4%(100/225) had anxiety tendency, 40%(90/225) had sleep disorder and 36.9%(83/225) had depression tendency, there was no significant difference in above-mentioned situations between the PsA and psoriasis patients ( P>0.05). Conclusion:More attention should be paid to the management of concomitant diseases and psychological intervention in patients with PsA. When psoriasis patients occur with heel pain, dactylitis, low back pain and hip pain, the risk of development into PsA should be considered.

Pulmonary blast injury has become the main type of trauma in modern warfare, characterized by externally mild injuries but internally severe injuries, rapid disease progression, and a high rate of early death. The injury is complicated in clinical practice, often with multiple and compound injuries. Currently, there is a lack of effective protective materials, accurate injury detection instrument and portable monitoring and transportation equipment, standardized clinical treatment guidelines in various medical centers, and evidence-based guidelines at home and abroad, resulting in a high mortality in clinlcal practice. Therefore, the Trauma Branch of Chinese Medical Association and the Editorial Committee of Chinese Journal of Trauma organized military and civilian experts in related fields such as thoracic surgery and traumatic surgery to jointly develop the Clinical treatment guideline for pulmonary blast injury ( version 2023) by combining evidence for effectiveness and clinical first-line treatment experience. This guideline provided 16 recommended opinions surrounding definition, characteristics, pre-hospital diagnosis and treatment, and in-hospital treatment of pulmonary blast injury, hoping to provide a basis for the clinical treatment in hospitals at different levels.

Objective:To elucidate the correlation between peripheral blood levels of pentraxin 3 (PTX3) and fibroblast growth factors 2 (FGF2) and clinical manifestations, immunological indexes and disease activity of systemic lupus erythematosus (SLE) patients.Methods:The correlation between peripheral blood levels of PTX3 and FGF2 and clinical manifestations, immunological indexes and disease activity of SLE pa-tients was determined. T test, Mann-Whitney U test and Spearman's rank correlation coefficient were analyzed statistically. Results:Plasma PTX3 levels were significantly higher in SLE patients than in healthy controls (3 191±2 423) pg/ml vs (755±432) pg/ml, t=5.595, P<0.01) . The titer of PTX3 in patients with hematologic in-volvement was higher than that in the patients without [(3 810±2 840) pg/ml vs (2 493±1 830) pg/ml, t=2.008, P=0.049). Plasma PTX3 concentration in SLE patients was positively correlated not only with the level of 24 h urine protein ( r=0.498 6, P=0.005 9), but also with ESR ( r= 0.376, P=0.007) and systemic lupus erythematosus disease activity index (SLEDAI) scores ( r=0.405, P=0.003). On the contrast, plasma PTX3 concentration in SLE patients was negatively correlated with complement 3 ( r=-0.405, P=0.005). Increased serum PTX3 levels accompanied by increased serum FGF2 levels was observed. Plasma FGF2 concentration in SLE patients was positively correlated with SLEDAI scores ( r=0.326, P=0.019), but negatively correlated with level of comple-ment 3 ( r=-0.414, P=0.004) and complement 4 ( r=-0.451, P=0.007). Levels of FGF2 were higher in patients with positive anti-NuA antibody [(138±91) pg/ml vs (59±68) pg/ml, t=2.996, P=0.004 2), anti-dsDNA antibody [(120±96) pg/ml vs (56±58) pg/ml, t=3.583, P=0.000 7] and anti-rRNP antibody (151±109) pg/ml vs (63±61) pg/ml, t=3.757, P=0.000 4) than in patients with negative of these antibodies. Conclusion:The levels of PTX3 and FGF2 in peripheral blood may play a role in determining the disease activity and clinical phenotype of SLE, and can help doctors to make diagnosis and treatment decisions.

Objective To explore the status of glucocorticoid application in patients with systemic lupus erythematosus (SLE) in China.Methods Epidemiological survey was used.The SLE patients who met the 1997 classification criteria of American College of Rheumatology were enrolled.The usage of glucocorticoid and related adverse reactions were recorded and analyzed.Results A total of 400 SLE patients were enrolled,including 35 men and 365 women.The average age was (37.4 ± 14.0) years old,and the average duration of disease was (6.7 ± 5.8) years.There were 310 patients using glucocorticoid as maintenance.Sixty-one percent (n =244) patients started using medium dose (prednisone 30-＜ 60 mg/d) as the initial treatment of glucocorticoid,which lasted for (37 ± 11) days.The time of drug duration in patients with low dose prednisone (7.5-＜30 mg/d)and high dose (60-100 mg/d) was(92 ± 20)and (17 ± 3) days respectively (P ＜ 0.05 between 3 groups).However,patients receiving different initial dosage were of no discrepancy in the maintenance therapy.During maintenance,even though 51.0％ (n =158) patients were on prednisone 2.5-5 mg/d,the duration of drug use in ＞ 5-10 mg/d groupwas longer [(29.9 ± 3.3) months].Patients with involvement of internal organs had a higher tendency to use 60-100 mg/d prednisone or pulse-dose therapy in the initial treatment,nevertheless these two groups had no difference of maintenance dosage.Among all 400 patients,62 patients withdrew glucocorticoid,including 17 patients with disease remission (4.3％),44 by self-withdrawal and one with adverse reaction.Conclusion In China,the medium dosage of glucocorticoid is the most common initial treatment in patients with SLE.Prednisone 2.5-5 mg/d was the most common choicefor maintenance therapy.Currently,the proportion of glucocorticoid withdrawal remains low in SLE patients achieving remission.

Objective To survey the female sexual status and its influence factors during the second trimester of pregnancy.Methods The Chinese Version of Female Sexual Function Index (FSFI) and self-administered questionnaires were used to collect the data of 304 women during the second trimester of pregnancy.The investigation results were analyzed.Results Most women reported a decrease in sexual desire(74.7％,227/304),intercourse frequency(90.8％,276/304) and sexual satisfaction(41.1％,125/304).FSFI all dimensions mean scores of these 304 women during the second trimester of pregnancy were less than the critical value,and total FSFI was (20.82±3.74) scores.The female sexual status during the second trimester of pregnancy were affected by age,duration of marriage,education,the way of pregnancy,F=21.974,12.288,3.345,7.748,the differences were statistically significant,P＜0.05.Conclusion Pregnant women are in a low level sexual function during the relative safety stage for sexual behavior during the second trimester of pregnancy,so providing some sexual education is necessary to improve couples' sexual health.