OBJECTIVE: To summarize the clinical features and management of two brothers affected with X-linked inhibitor of apoptosis protein (XIAP) deficiency. METHODS: This study retrospectively analyzed the clinical presentations, treatment, and follow-up of two brothers with XIAP deficiency diagnosed at Shanghai Children's Hospital in 2020, and summarized similar cases recorded in databases such as PubMed, Wanfang, Chinese Medical Association Journals, and WIP from January 2006 to November 2024. This study was approved by the Medical Ethics Committee of our hospital (Ethics No.: 2025R128-E01). RESULTS: Patient 1 was the younger brother, who presented at 8 years of age with growth retardation, folliculitis, erythema nodosum, and perineal abscess. Sequencing revealed that he has carried a hemizygous c.566T>C (p.Leu189Pro) variant of the XIAP gene, which was inherited from his mother. He was allergic to infliximab treatment and underwent allogeneic stem cell transplantation (HSCT) in January 2021. During a follow-up of 3 years and 10 months post-transplantation, he showed no gastrointestinal symptoms and had a good outcome. Patient 2 was the elder brother, who presented at 10 years and 6 months of age with growth retardation, rash, and anal fistula. Genetic testing revealed the same variant. He was treated with oral azathioprine but did not have regular follow-ups. At 14-years-and-6-months of age, he had developed severe gastrointestinal infection and hemophagocytic lymphohistiocytosis, which was alleviated after treatment with antibiotics, glucocorticoids, immunoglobulin, and rituximab. He is currently being prepared for HSCT. A total of 13 publications were retrieved, which involved 64 patients from 23 families, with 23 different variants identified. The main clinical manifestations included splenomegaly (34 cases, 53.1%), hemophagocytic lymphohistiocytosis (27 cases, 42.2%), and inflammatory bowel disease or colitis (20 cases, 31.8%). There were significant phenotypic differences among patients from the same family. Thirteen patients (20.3%) underwent HSCT, with a survival rate of 61.5%. CONCLUSION For male children with early onset, poor treatment response, especially those with unexplained splenomegaly and IBD-like symptoms, early genetic testing is recommended. HSCT is a safe and effective treatment for XIAP deficiency. For patients with developmental delay, early onset, and severe IBD phenotype, early transplantation is recommended.
Humans ; Male ; X-Linked Inhibitor of Apoptosis Protein/deficiency* ; Child ; Genetic Diseases, X-Linked/therapy* ; Phenotype ; Siblings ; Retrospective Studies ; Hematopoietic Stem Cell Transplantation

Radiochemotherapy-induced oral mucositis (OM) is a common oral complication in patients with tumors following head and neck radiotherapy or chemotherapy. Erosion and ulcers are the main features of OM that seriously affect the quality of life of patients and even the progress of tumor treatment. To date, differences in clinical prevention and treatment plans for OM have been noted among doctors of various specialties, which has increased the uncertainty of treatment effects. On the basis of current research evidence, this expert consensus outlines risk factors, clinical manifestations, clinical grading, ancillary examinations, diagnostic basis, prevention and treatment strategies and efficacy indicators for OM. In addition to strategies such as basic oral care, anti-inflammatory and analgesic agents, anti-infective agents, pro-healing agents, and photobiotherapy recommended in previous guidelines, we also emphasize the role of traditional Chinese medicine in OM prevention and treatment. This expert consensus aims to provide references and guidance for dental physicians and oncologists in formulating strategies for OM prevention, diagnosis, and treatment, standardizing clinical practice, reducing OM occurrence, promoting healing, and improving the quality of life of patients.
Humans ; Chemoradiotherapy/adverse effects* ; Consensus ; Risk Factors ; Stomatitis/etiology*

Objective To explore the effects of behavior change intervention based on the multi-ple-theory model on patients with dyslipidemic ischemic stroke.Methods A total of 93 patients with dyslipidemic ischemic stroke who were hospitalized in the vascular neurology ward of Beijing Tiantan Hospital,Capital Medical University from January to August 2024 were selected as the study subjects using the convenience sampling method.They were randomly divided into control group(n=49)and intervention group(n=44)using the envelope-drawing method.Patients in the control group re-ceived routine stroke health education,while those in the intervention group underwent a 3-month be-havior change program guided by the multiple-theory model.The levels of healthy behaviors,body mass index(BMI),total cholesterol(TC),triglycerides(TG),high-density lipoprotein(HDL-C),and low-density lipoprotein(LDL-C)were compared between the two groups.Results There were no statistically significant differences in general information and disease-related data between the two groups(P＞0.05).At 1-,3-,and 6-month after the intervention,the level of healthy behaviors in the intervention group was higher than that in the control group,with statistically significant differ-ences(P＜0.05).There was a statistically significant difference in BMI between the two groups at 6 months after the intervention(P＜0.05).The TC levels in the intervention group at 3 and 6 months after the intervention were lower than those in the control group,with statistically significant differences(P＜0.05).The HDL-C level in the intervention group at 6 months after the intervention was high-er than that in the control group,with a statistically significant difference(P＜0.05).The LDL-C levels in the intervention group at 1-,3-,and 6-month after the intervention were lower than those in the control group,with statistically significant differences(P＜0.05).There were no statistically significant differences in TG levels between the intervention group and the control group at different time points after the intervention(P＞0.05).Conclusion The behavior change intervention pro-gram based on the multiple-theory model can effectively improve and maintain healthy behaviors and improve blood lipid levels in patients with dyslipidemic ischemic stroke.

Objective To develop an active monitoring model for adverse drug events(ADEs)related to antimicrobial use in children based on the China hospital pharmacovigilance system(CHPS).Methods Trigger items for the active monitoring model were initially drafted through a review of relevant literature,adverse reaction databases,and drug label warnings,and subsequently refined using the Delphi method.A retrospective analysis was performed on pediatric inpatients who received antibiotics at Anning First People's Hospital between January 1 and December 31,2024.The detection rate and positive predictive value(PPV)of the active monitoring model were calculated and compared with spontaneous ADE reports from the same period.Risk factors for ADEs were further analyzed using logistic regression.Results 25 trigger items were established for the active monitoring model.Among 1,784 cases,233 ADEs were identified,yielding a detection rate of 13.06%(233/1,784).The spontaneous reporting rate of adverse events during the same period was 1.85%(33/1,784).The difference between the two was statistically significant(P<0.001).There were 727 positive trigger events,with 299 cases of ADE detected,resulting in an overall PPV of 41.13%(299/727).Logistic regression revealed that antibiotic use exceeding 3 days(OR=1.454,95%CI:1.012-2.088)was significantly associated with ADE occurrence.Conclusion Compared with conventional spontaneous reporting,the active monitoring model can significantly improve the detection rate of ADEs of pediatric antimicrobial drugs and achieve active and real-time monitoring of drug adverse events.

The Yin-Fire theory is the core content of Li Dongyuan's academic thought and has profound and extensive clinical guidance value.This paper analyzes the connotation and background of the Yin-Fire theory,and proposes that the core pathogenesis of Yin-Fire syndrome is qi deficiency without movement,and stagnation turning into fire;qi deficiency without rise,and dampness and turbidity flowing downward;qi deficiency without astringent,and Xiang-Fire rushing upward;qi deficiency and fire hyperactivity,which hurts body fluid and burns blood.This article summarizes the key points of diagnosis and treatment of various diseases by sorting out the commonly used prescriptions and medicines of Yin-Fire theory and its clinical application in miscellaneous internal medicine diseases,and deepens the understanding of the connotation of Yin-Fire syndrome.

Objective:To test the reliability and validity of the general procrastination scale (GPS) in the application of middle school students.Methods:The Chinese version of GPS, the irrational procrastination scale(IPS), and the Maslach burnout inventory(MBI) were utilized to survey 10 825 middle school students in Harbin City through stratified random sampling, and 4 498 students were retested after 4 weeks. Statistical analysis was performed using SPSS 27.0 and Mplus 8.0.Results:The entries were well differentiated.Exploratory and confirmatory factor analysis indicated that GPS was composed of two factors, including active avoidance and lack of planning.The model fit was good (CFI=0.914, TLI=0.901, RMSEA=0.069, SRMR=0.072). GPS was positively correlated with the total scores of IPS and MBI ( r=0.753, 0.677, both P<0.001). The Cronbach's α coefficient of GPS was 0.864, the folded half reliability was 0.870, and the retest reliability after 4 weeks was 0.756. Conclusion:The GPS has good reliability and validity among middle school students, which provides a standard for measuring the procrastination level of middle school students and carrying out related research.

Objective This paper aims to address the challenges of hospital equipment management by implementing the life cycle management system in the medical laboratories of public hospitals.Methods Based on the three principles of standard-ization,refinement,and dynamism,this paper conducted data tracking and managed equipment analysis with specifications dur-ing the processes of planning,discussion,procurement,acceptance,operation,maintenance,performance evaluation,scrap-ping,and updating.Results After implementing life cycle management,the average risk level significantly decreased compared to pre-implementation(P＜0.05).Budget implementation efficiency increased by 14.53％from 2019 to 2023.The investment payback period was controlled within 2 years.Conclusion The application of the life cycle management system to equipment management in medical laboratories of public hospitals is of great significance for the high-quality development of hospitals.

OBJECTIVE To explore the effect of volatile oil of Ligusticum chuanxiong on the transdermal properties and cytotoxicity of triptolide in vitro. METHODS The chemical constituents of the volatile oil of L. chuanxiong were analyzed by gas chromatography-mass spectrometry. The lower abdominal skin of KM mice was separated and divided into triptolide group， triptolide in compatibility with volatile oil of L. chuanxiong groups at 1∶10， 1∶50， 1∶100 （hereinafter referred to as “compatibility 1∶10”“compatibility 1∶50”“compatibility 1∶100” groups）. After the skin of mice in each group was fully exposed to 0.2 g of the corresponding cream for 24 h， the cumulative transdermal dose （Qn） of triptolide in the receiving solution of each group was determined by high-performance liquid chromatography， and the transdermal absorption rate （Jss） was calculated. Human immortalized keratinocytes （HaCat） were used as a model， the CCK-8 method was used to detect the cell survival rate of different concentrations of the volatile oil of L. chuanxiong and triptolide before and after compatibility. RESULTS A total of 62 chemical constituents of the volatile oil of L. chuanxiong were identified， including Z-ligustilide， senkyunolide， and β-selinene. The Qn （P＜ 0.01） and Jss of triptolide increased within 24 h in the compatibility 1∶10 and 1∶50 groups， while the Qn （P＜0.05） and Jss decreased in the compatibility 1∶100 group as compared with the triptolide group. Compared with the triptolide group， the cell survival rate of HaCat was significantly increased in the compatibility 1∶10 and 1∶50 groups when the triptolide concentrations were 36， 72 and 144 ng/mL （P＜0.05 or P＜0.01）； while the cell survival rate of HaCat was decreased in the compatibility 1∶100 group， but the difference was not statistically significant （P＞0.05）. CONCLUSIONS When the compatibility ratio of triptolide and volatile oil of L. chuanxiong was 1∶10 or 1∶50， it can promote the transdermal absorption of triptolide and reduce the cytotoxicity of triptolide to HaCat.

A close relationship between fatty acid metabolism and cancer development is well-established.The hydroxyacyl-coenzyme a dehydrogenase(HADH),a key enzyme in fatty acid beta-oxidation,has recently been identified as an anti-oncogenic factor in various cancers and an oncogenic factor in conditions like acute myeloid leukemia.In cancer cells,HADH not only directly catalyzes fatty acid beta-oxidation but also indirectly influences multiple signaling pathways such as PPAR,TNF-α,JAK-STAT3,PI3K/Akt,IFN-γ,MAPK,and non-canonical Wnt signaling pathways,affecting cancer cell proliferation and migration.HADH shows promise as a potential tumor biomarker for diagnosis,treatment,and prognosis in different cancer types,holding significant clinical value.

Objective To explore the value of dynamic nomogram constructed by multi spiral computed tomography(MSCT)features combined with inflammatory indicators in predicting the status of microvascular invasion(MVI)of hepatocellular carcinoma(HCC)before surgery.Methods The clinical and imaging data of 137 patients with postoperative pathologically confirmed HCC were analyzed retrospectively.According to the status of the MVI,they were divided into positive group(44 cases)and negative group(93 cases).Multivariate logistic regression analysis was used to screen independent risk factors for predicting the MVI status of HCC patients,and a joint prediction model was constructed,which was displayed in the form of a dynamic nomogram.The receiver operating characteristic(ROC)curve,calibration curve and Hosmer-Lemeshow test were used to evaluate the diagnostic efficiency,calibration and goodness of fit of the model,Akaike information criterion(AIC)and Bayesian information criterion(BIC)were used for comparison between the models,and a 5-fold cross-validation and decision curve analysis(DCA)were also used to evaluate the stability and clinical applicability of the model.Results Multivariate logistic regression analysis showed that necrosis and delayed-phase enhancement(DEd),and alkaline phosphatase to lymphocyte ratio(ALR)were independent risk factors for predicting MVI status in HCC patients.The area under the curve(AUC)of the dynamic nomogram was 0.721,with the sensitivity of 0.705 and the specificity of 0.656.The AIC and BIC values were 152.372 and 158.212,respectively.The calibration curve and the Hosmer-Lemeshow test showed that the model had a high degree of calibration and goodness of fit(χ2=2.372,P=0.967),the average AUC of the 5-fold cross-validation was 0.787,and the DCA showed that the nomogram model had a good clinical applicability.Conclusion The dynamic nomogram model constructed by MSCT features combined with inflammatory indicators is feasible to predict the MVI status of HCC patients before surgery,and the dynamic nomogram can directly generate the prediction results of different individuals.