A 20-year-old male patient presented to the Department of Dermatology of Peking Union Medical College Hospital with complaints of an 8-year history of facial scarring, swelling of the lower limbs, and a 4-year history of scalp thickening. Physical examination showed thickening furrowing wrinkling of the skin on the face and behind the ears, ciliary body hirsutism, blepharoptosis, and cutis verticis gyrate. Both lower limbs were swollen, especially the knees and ankles. The skin of the palms and soles of the feet was keratinized and thickened. Laboratory examination using bone and joint X-ray showed periostosis of the proximal middle phalanges and metacarpals of both hands, distal ulna and radius, tibia and fibula, distal femurs, and metatarsals.Genetic testing revealed two variants in SLCO2A1, c.1658T > A and c.96+4A > C.Through multidisciplinary consultation, we confirmed the diagnosis of pachydermoperiostosis.

OBJECTIVE: To explore the current status, evolution, hot topics, and future research trends in the field of burn-related sepsis research through a visual analysis of literature. METHODS: A bibliometric method was employed to retrieve articles related to burn-related sepsis from January 1, 1994, to May 16, 2024, in the China National Knowledge Infrastructure (CNKI) and the Web of Science database. The CiteSpace 6.3.R1 software was used to analyze the retrieved literature. The number of publications, authors, countries, and institutions in both Chinese and English literature was statistically analyzed. Co-occurrence analysis, clustering analysis, and co-citation analysis of keywords were performed. RESULTS: A total of 1 090 articles from the CNKI database and 1 143 articles from the Web of Science database were retrieved. Over the past 20 years, the volume of Chinese publications has remained stable, although there has been a slight decline in the past two years. In contrast, the number of English publications, after a period of growth, showed a sharp decline over the past three years. In Chinese literature, 1 457 authors published articles on burn-related sepsis as first authors, with 14 core authors publishing four or more articles. In English literature, 98 authors published articles on burn-related sepsis as first authors. Research on burn-related sepsis was conducted by 76 countries, with the United States having the most collaborations and publications. Globally, 1 349 institutions published articles on burn-related sepsis, with the top institutions being the First Affiliated Hospital of the PLA General Hospital (8 articles) for Chinese literature and the University of Texas Medical Branch (57 articles) for English literature. In the co-occurrence analysis, 208 Chinese keywords and 211 English keywords were included. Excluding keywords related to search terms, the top five most frequent keywords in Chinese literature were burn, sepsis, infection, severe burn, and procalcitonin; the top five most frequent keywords in English literature were sepsis, septic shock, mortality, injury, and burn injury. Chinese keyword analysis identified six clusters, with the largest being sepsis, followed by procalcitonin, infection, and severe burn. English keyword analysis identified seven clusters, with the largest being expression, followed by epidemiology, inhalation injury, and acute kidney injury. The persistent clusters in Chinese literature were procalcitonin, with recent emerging nodes being severe burn, inflammatory response, platelets, and predictive value. In English literature, the persistent clusters were inhalation injury and nitric oxide, with recent emerging nodes being continuous renal replacement therapy, hemorrhagic shock, and early enteral nutrition. The longest-lasting emergent keyword in Chinese literature was delayed resuscitation (2003-2010), with the highest emergent strength being severe burn. In English literature, the longest-lasting emergent keywords, each lasting five years, were nitric oxide (2007-2012), management (2019-2024), and impact (2019-2024), with the highest emergent strength being thermal injury. CONCLUSIONS Research on burn-related sepsis has shifted from focusing on early studies on pathogenesis and mortality to focus on prevention, treatment, and early diagnosis. Future research is expected to focus on early diagnosis and risk factors of burn-related sepsis.
Burns/complications* ; Sepsis/etiology* ; Humans ; Bibliometrics ; China

[Objective]To summarize Professor CHEN Hua's experience in treating children with recurrent respiratory tract infection(RRTI)based on"Yin deficiency,blood stasis and heat accumulation".[Methods]Through following Professor CHEN's clinical treatment and summarizing the cases information,the clinical experience and prescription characteristics of Professor CHEN in the treatment of children with RRTI were summarized and sorted out through clinical evidence.[Results]Professor CHEN considers that children with RRTI is caused by wind,phlegm,blood stasis and deficiency,and the pathogenesis is deficiency of the lung and spleen,phlegm and blood stasis obstructing venation,extending and not healing,and accumulation of blood stasis and heat.He puts forward the differentiation and treatment of RRTI from Yin deficiency,blood stasis and heat accumulation,and summarizes the diagnosis and treatment experience of paying attention to wind evil,dispelling wind and relieve symptoms;adjusting measures in accordance with three categories,nourishing Yin and clearing blood stasis;detailed differentiation of deficiency and substance,appropriate use of harmonious method;taking care of middle-Jiao and strengthening the spleen and stomach.The case involved a patient with RRTI,which was diagnosed as a condition of Yin deficiency with concurrent phlegm and blood stasis obstruction.Professor CHEN implemented a staged treatment plan,focusing on nourishing Yin,clearing stasis,which spanned the middle and later stages of the disease.During the prolonged stage,the treatment aimed at nourishing Yin and reducing fever,resolving phlegm and dispelling stasis.In the recovery stage,the focus shifted to nourishing Yin and reducing fever,invigorating the spleen and replenishing Qi.The therapeutic effect was significant.[Conclusion]Professor CHEN inherits the characteristics of pediatrics of Zhejiang school of traditional Chinese medicine,distinguishing and treating children with RRTI from"Yin deficiency,blood stasis and heat accumulation",and the curative effect is remarkable,which has high practical value in guiding clinical practice.

ObjectiveTo explore the characteristics and associated risk factors of diabetes in patients with schizophrenia living in communities, and to provide a basis for the prevention of diabetes comorbidity in this population. MethodsA stratified cluster sampling was used to randomly select patients with schizophrenia in Shanghai who participated in the free health examination provided by the National Basic Public Health Services in 2020. Statistical methods were employed to analyze the general demographic data, clinical characteristics, and laboratory test results of the study subjects. ResultsThe study included 3 374 individuals with schizophrenia, among which the prevalence of diabetes was 17.01%. Statistically significant differences were observed in terms of age, education level, urban area type, marital status, employment status, duration of illness, blood pressure, body mass index, total cholesterol, and triglyceride levels (all P<0.05). Multivariate logistic regression analysis revealed that living in non-central urban areas (OR=1.76, 95%CI: 1.33‒2.32), disease duration of 6‒ years (OR=2.60, 95%CI: 1.07‒6.32), disease duration of 11‒ years (OR=2.72, 95%CI: 1.17‒6.35), disease duration of 16‒ years (OR=3.38, 95%CI: 1.54‒7.42), hypertension(OR=1.73, 95%CI: 1.27‒2.36), obesity (OR=1.52, 95%CI: 1.15‒2.00), and elevated triglyceride levels (OR=2.78, 95%CI: 2.22‒3.49) were risk factors for diabetes in patients with schizophrenia. ConclusionThe prevalence of diabetes in community-dwelling patients with schizophrenia is higher than that in the general population. It is recommended that appropriate health education and rehabilitation guidance be provided as part of community-based mental health services.

Objective:To determine the clinical features and genetic characters of patients with chronic enteropathy associated SLCO2A1 gene (CEAS). Methods:Five CEAS patients diagnosed at Peking Union Medical College Hospital from January 2012 to December 2019 were enrolled in this study. The clinical manifestations, laboratory test, radiological and endoscopic findings, gene detections, treatments and prognosis of these patients were reviewed and analyzed.Results:Five male patients presented gastrointestinal symptoms after puberty, including abdominal pain, diarrhea, intermittent melena or hematochezia, incomplete bowel obstruction, anemia, hypoalbuminemia and hypokalemia. The whole gastrointestinal tract except esophagus could be involved, especially the stomach and ileum. Intestinal lesions were characterized by multiple shallow ulcers with stenosis in the layers of mucosa and submucosa. Five patients were all accompanied with primary hypertrophic osteoarthropathy (PHO), and 1 with myelofibrosis and thoracic duct dysplasia. All patients were homozygous or compound heterozygous mutations of SLCO2A1 gene. Conventional treatment of inflammatory bowel disease and COX-2 inhibitors were ineffective. Conclusions:CEAS is an autosomal recessive genetic disease which widely involves the gastrointestinal tract, and can be associated with skin and bone involvement. There is no effective treatment for CEAS at present. CEAS is a different entity from other inflammatory gastrointestinal diseases.

Objective:To compare the efficacy between Multiloc humeral intramedullary nail system (Multiloc) and proximal humerus internal locking system (PHILOS) in the treatment of adult proximal humeral fractures.Methods:A retrospective analysis was conducted of the 83 adult patients with proximal humeral fracture who had been treated at Trauma Center, The Second People's Hospital of Changzhou from August 2018 to March 2021. They were 33 males and 50 females, with 45 left sides and 38 right sides injured. By the Neer classification, there were 48 two-part, 22 three-part and 13 four-part fractures. The patients were assigned into 2 groups according to different treatments: an observation group ( n=41) receiving treatment by Multiloc and a control group ( n=42) receiving treatment by PHILOS. The 2 groups were compared in terms of humeral neck shaft angles and visual analogue scale (VAS) pain scores preoperation and one month postoperation, Neer shoulder function score at the last follow-up, and incidence of adverse reactions. Results:The preoperative general data showed no statistically significant differences between the 2 groups, indicating the groups were comparable ( P>0.05). The humeral neck shaft angle (127.4°±3.6°) was recovered significantly better and the VAS pain score (1.3±0.3) was significantly lower in the observation group than in the control group (129.6°±4.5°,2.1±0.3) one month after operation ( P<0.05). The excellent to good rate by Neer score at the last follow-up (90.24%, 37/41) was significantly higher and the incidence of adverse reactions (2.44%) significantly lower in the observation group than in the control group (71.43%, 30/42 and (19.05%, 8/42) ( P<0.05). Conclusion:In the treatment of adult proximal humeral fractures, compared with PHILOS, the Multiloc system is more effective in improving shoulder joint function, promoting shoulder motion recovery and reducing pain.

Objective:To explore the feasibility and optimization effect of modifying the Henry Ford Hospital (HFHS) RapidPlan model for stereotactic body radiation therapy planning based on local requirements.Methods:The following changes were made based on Henry Ford Health System(HFHS) Rapid Plan Lung SBRT model, taking the latest clinical guideline evidence and local clinical practice into account: Internal gross target volume(IGTV) and organ at risk(OAR) structure, lung, were added and set corresponding parameters.The upper value of planning target volume (PTV) was adjusted from 109% to 125%. The original training library was replaced with 73 local historical simultaneous integrated boosting plans, and statistical verification and outlier cleaning of the initial trained model were performed using Model Analytics software. Totally 10 cases not included in the model library were selected for independent verification, and automatic optimization result of the models before and after modifying were compared under the same beam condition. The following dosimetric parameters were compared after target dose normalization: conformal index (CI) of target volume, the mean doses, maximum doses and dose-volume parameters of OARs.Results:The " tail" of the PTV′s DVH and the " shoulder" and " tail" of the IGTV′s DVH of model M (local) validation plan (M (local)_P) performs higher than the original model HFHS (HFHS_P). The PTV_CI (1.07±0.13) of M local_P were significantly smaller than HFHS_P (1.25±0.24) ( Z=-2.497, P<0.05). Except for Heart_ D15 cm 3 and Heart_ Dmax, most of the M local_P dosimetric parameters of OARs were lower than HFHS_P, and the standard deviation was smaller. However, the difference of between two plans was no more than 3.06%. 10 HFHS_P plans don′t satisfy dose parameters requirement, two of which PTV_CI values are 1.52 and 1.74, far beyond the clinically acceptable range. Conclusions:Commercial model HFHS could be localized by replacing training library and adjusting parameters. Moreover, plans optimized by the modified model are local clinical acceptable in the aspects of target volume conformity and hotspots, and have a better performance in terms of OAR sparing and plan consistency.

Objective:To analyze and compare the radiation dose and image quality of kilo-voltage cone beam CT systems on different Varian accelerator platforms, providing data to support clinical decisions on selecting optimal protocols for image-guided radiotherapy based on cost-effective ratio (image quality / radiation dose).Methods:The radiation dose and image quality of various CBCT systems and scanning protocols on Varian Edge, Truebeam and ix (new and old) LINACs were obtained using a CT dose index (CTDI) phantom combined with a CT ionization chamber and a Catphan604 phantom, respectively. Figure of merit (FOM) was used to evaluate the cost-effective ratio of the image guidance schemes.Results:Considerable inter-system varieties of FOMs were observed, varying from 0.65 (Image Gently-full trajectory) to 48.46 (Image Gently-half trajectory). The inter-protocol varieties were also large, where the mean±SD was 22.14±13.47.Conclusions:Considering the explicit inter-system and inter-protocol varieties, it is clinically favorable to evaluate the image guidance schemes based on machine-specific measurement. For instance, parameters and equipment with low CTDI w can be beneficial for dose-sensitive patients. High CNR regimen favors patients with high image quality requirements. For ordinary patients, cost-effective ratio in terms of FOM can be very helpful to guide the decision-making of clinical image-guided radiotherapy.

Objective To investigate the clinical characteristics and prognosis of patients with acute aortic dissection (AAD) and hypertension,and explore other related prognostic factors in AAD.Methods The present study enrolled consecutive patients diagnosed with AAD who were admitted to Peking University People's Hospital between January 2000 to December 2015.Patients diagnosed with AAD by CT angiography,aortography or magnetic resonance imaging within 14 days of onset were included.Patients with infectious diseases,haematological diseases,malignancies,autoimmune diseases and patients without clearly clinical diagnosis or incomplete data were excluded.The patients were initially divided into two groups based on their history of hypertension,and their clinical characteristics were compared and analyzed.We further divided AAD patients into survival group and death group according to their in-patient outcomes,and factors related to their prognoses were analyzed.Logistic regression analysis was applied to analyze the independent risk factors related to hospital death in AAD patients with P＜0.05 as the significant value.Results The hypertensive group contained 237/346 cases included (68.45％),patients in this group were generally older than their non-hypertensive counterparts,accompanied by increased prevalence of comorbidities (coronary heart diseases or diabetes) and a statistical significant elevated admission blood pressures (systolic and diastolic,P＜0.05).No significant difference were found between the groups in terms of white blood cell and platelet count,D-dimer,neutrophil to lymphocyte ratio(NLR),fibrinogen,serum creatinine and serum lipid profiles (P＞0.05).Hypertensive patients were less likely to receive surgical treatment compared with those without hypertension(P＜0.05),with increased risk of in-hospital mortality (P＞0.05).Further logistic regression analysis revealed the presence of hypertension did not independently predict in-hospital mortality of AAD patients.Factors such as age,Stanford classification of the AAD,NLR and platelet counts were found to have independent predictive values for in-hospital mortality (P＜0.05).Conclusion AAD patients with hypertension are generally older,have more comorbidities such as coronary heart diseases and diabetes.The presence of hypertension itself is not directly associated with in-hospital mortality in AAD patients,while the Stanford classification,age,NLR and platelet counts are independent risk predictors.

Objective: To investigate the clinical characteristics, coagulation function and associated prognostic factors of acute aortic dissection. Methods: The clinical data of 119 patients with acute aortic dissection (AAD) admitted to Beijing University People's Hospital from November 2008 to January 2016 were analyzed. All the participants were confirmed by computed tomography angiography, and the onset time was less than 14 days. Data of blood routine test, coagulation function at the first admission were collected, and surgical intervention and prognosis were recorded. All the patients, according to the prognosis, or whether disseminated intravascular coagulation (DIC) occurred, were divided into two groups, and the differences between the two groups were compared. Logistic regression analysis was applied to analyze independent risk factors related to in-hospital death in AAD patients. Results: In 119 patients with AAD, the average age was (52.9±14.2) years, with a male/female ratio of 5.3:1. Pain was the most common clinical manifestation in patients with AAD, accounting for more than 90.0%. The nature of pain was mostly expansible and/or transitive pain. Dominant DIC occurred in 13 cases (10.9%), and 7 patients died (53.8%). There were significant differences between the DIC group and non-DIC group in neutrophil/lymphocyte ratio (NLR), platelet count, fibrinogen, D-dimer, FDP, PT, APTT and mortality rate (P<0.05). All the 16 patients in the death group were type A AAD, among which, 7 patients (43.8%) developed with DIC. There were significant differences between the death group and survival group in NLR, platelet count, fibrinogen, D-dimer, FDP, PT, APTT, DIC proportion and surgical operation rate (P<0.05).Logistic regression analysis showed that NLR and platelet count were independent risk factors of in-hospital death (P<0.05). Conclusions D-dimer has a high diagnostic and prognosis value for AAD. The mortality increased with the activation and depletion of platelet. Once DIC occurs, the prognosis is extremely poor. NLR and platelet count are independent risk factors for in-hospital death in patients with AAD.