Schimke immuno-osseous dysplasia (SIOD)caused by SMARCAL1 gene mutations is a rare genetic disorder characterized by multi-system involvement, including T-cell dysfunction, skeletal dysplasia, disproportionate short stature, nephrotic syndrome, and kidney failure. This multidisciplinary consultation involved a 9-year-old boy with intrauterine growth retardation, presenting with short stature, T-cell lymphopenia, proteinuria, and degenerative bone and joint disease. Treatment primarily focused on symptomatic and supportive care. Through the multidisciplinary rare disease consultation, holistic support was provided to the patient, offering comprehensive care from various specialtiesx.We also aim to use this case report to enhance clinicians′ under-standing of SIOD and improve the management and treatment of rare diseases.

Objective To evaluate the safety and efficacy of transcatheter arterial chemoembolization(TACE)combined with lenvatinib and camrelizumab in the treatment of advanced hepatocellular carcinoma(HCC).Methods The clinical data of a total of 63 patients with advanced HCC,who received TACE combined with lenvatinib and camrelizumab(triple therapy)or TACE combined with lenvatinib(dual therapy)at the Jingmen Municipal People's Hospital of China between April 2020 and December 2021,were retrospectively analyzed.Triple therapy group had 30 patients,and dual therapy group had 33 patients.The post-treatment tumor response,disease progression-free survival(PFS),overall survival(OS),and the incidence of adverse drug reactions were recorded.Results The median follow-up period of the two groups was 14 months(range of 4-26 months).Compared with the dual therapy group,in the triple therapy group the objective response rate(ORR)was remarkably higher(83.3%vs.57.6%,P=0.026),the disease control rate(DCR)was obviously higher(93.3%vs.69.7%,P=0.039),the median PFS was significantly longer(8.0 months vs.5.0 months,P＜0.01),and the median OS was strikingly longer(24.0 months vs.12.0 months,P=0.004).No statistically significant difference in the incidence of adverse drug reactions existed between the two groups(P＞0.05).Conclusion For the treatment of advanced HCC,TACE combined with lenvatinib and camrelizumab is clinically safe and effective.(J Intervent Radiol,2024,32:57-62)

Objective To investigate the knowledge and behavioral status of foot care among patients with type 2 diabetes in Xinjiekou Community in Beijing and analyze its influencing factors. Methods A convenient sampling method was used to select 278 diabetes patients from Xinjiekou Community in Beijing for questionnaire surveys, which included general information, foot care knowledge, and behaviors of the patients. Results The average score of foot care knowledge among the 278 patients was (71.65±14.59), indicating a moderate level overall. The average score of foot care behavior was (48.83±8.83), indicating a poor level overall. Multivariate stepwise regression analysis showed that gender, duration of diabetes, and whether the patients had received foot care education or not were independent influencing factors for both the foot care knowledge score and the foot care behavior score among community diabetes patients (P < 0.05). Pearson correlation analysis showed that a positive correlation between foot care knowledge and foot care behavior among community diabetes patients was observed(r=0.593, P < 0.001). Conclusion The level of foocare knowledge among diabetes patients in this community in Beijing is moderate, while the status of foot care behaviors is not optimistic. Community healthcare professionals should strengthen health education on foot care, improve patients' knowledge of foot care and compliance with foot care behaviors, and pay attention to male patients and those with a shorter duration of diabetes to reduce the occurrence of diabetic foot.

Objective:To study the remote multi-disciplinary team (MDT) model in diagnosis and treatment of plague, in order to provide scientific basis for clinical treatment of plague.Methods:A retrospective analysis was made on the diagnosis and treatment process of a case of bubonic plague, a sudden imported Class A infectious disease, which was secondary to septicemic plague, involving a remote MDT team consisting of the Infectious Diseases Department, Intensive Care Unit, Respiratory and Critical Care Department, Cardiology Department, Pharmacy Department, and Nosocomial Infection Department of the General Hospital of Ningxia Medical University.Results:The patient was a middle-aged female who was engaged in herding work on the grassland. The first symptom was a sudden pain in the left lower abdomen for three days, accompanied by chest tightness and shortness of breath. After hospitalization, blood culture indicated Yersinia, abdominal CT indicated left lower abdominal lymph node enlargement, and lymph node puncture fluid was positive for Yersinia pestis nucleic acid. Combined with clinical symptoms and signs, the patient was diagnosed as bubonic plague secondary to septicemic plague, and was isolated for treatment. After remote MDT consultation, comprehensive treatment was given, including anti-infection treatment of streptomycin and ciprofloxacin, short-term application of hormones, nutritional support, and local application of chloramphenicol ointment, etc. Secondary acute pancreatitis occurred during the course of the disease, which improved after symptomatic treatment. Finally, after 20 days of treatment, MDT expert group assessed that it met the discharge criteria. No abnormalities were found in follow-up visits outside the hospital. Conclusion:The remote MDT is effective in the treatment of bubonic plague secondary to septicemic plague, which is worth popularizing.

Objective:To explore the prognostic predictive value of neutrophil/lymphocyte ratio (NLR) combined with prognostic nutritional index (PNI) in patients with hepatitis B virus-related acute-on-chronic liver failure (HBV-ACLF).Methods:Clinical data from 149 HBV-ACLF patients admitted to the infectious diseases Department of the General Hospital of Ningxia Medical University were retrospectively analyzed. Demographic data of the enrolled patients and the initial clinical-related data after admission were collected. Patients were divided into survival (93 cases) and death groups (56 cases) according to their prognostic condition 90 days after discharge. Demographic and clinical differences were compared between the two groups data. Receiver operating characteristic (ROC) curves were plotted to determine the optimal cutoff values for NLR and PNI in predicting the 90-day mortality rate of HBV-ACLF patients. The COX regression model was used to conduct univariate and multivariate analyses to investigate the correlation between NLR and PNI and the prognosis of HBV-ACLF patients. Kaplan-Meier survival analysis was used to explore the effects of NLR and PNI on the survival of HBV-ACLF patients.Results:The death group NLR was higher than that of the survival group, while the PNI was lower than that of the survival group, with a statistically significant difference. The area under the receiver operating characteristic curve (0.842, 95% CI: 0.779-0.906) showed patients with adverse prognosis assessed by NLR combined with PNI had a superior prognosis than that of the Model for End-Stage Liver Disease (MELD) and its combined serum sodium (MELD-Na) and Child-Turcotte-Pugh (CTP) scores. COX regression analysis showed that NLR≥3.03 and MELD score were independent risk factors affecting the prognosis of HBV-ACLF patients. PNI > 36.13 was a protective factor for evaluating the prognosis of HBV-ACLF patients. Conclusion:NLR combined with PNI can enhance the prognostic predictive value of HBV-ACLF.

Objective:To evaluate the efficacy and safety of simultaneous integrated boost intensity-modulated radiation therapy (SIB-IMRT) for rectal cancer with lateral lymph node metastasis (LLNM).Methods:From January 2016 to December 2022, 103 rectal cancer patients with LLNM were enrolled. The patients were divided into SIB-IMRT group (52 cases) and conventional chemoradiotherapy (CRT) group (51 cases) using the random number table method. The dose was 50 Gy for the pelvis with 60 Gy of SIB-IMRT for the LLNM in the SIB-IMRT group. The dose was 50 Gy for the pelvis in the CRT group. The primary endpoint was the lateral recurrence rate. The efficacy and adverse reactions of the two groups were compared.Results:The adverse reactions and surgical complications after neoadjuvant radiotherapy were comparable between the two groups. The response rates of LLNM treatment were 76.9% and 56.9%, respectively, in the two groups ( χ2=4.69, P=0.03). The SIB-IMRT group and CRT group had a local recurrence rate of 7.7% and 25.5% ( χ2=5.92, P=0.015), respectively, and a lateral recurrence rate of 3.8% and 23.5% ( χ2=8.49, P=0.004), respectively. Univariate analysis showed that the SIB-IMRT, short axis of lateral lymph nodes <5 mm after radiotherapy, and negative result in the postoperative lymph node pathological examination were factors associated with lateral recurrence. Multivariable regression analysis demonstrated that the SIB-IMRT ( HR=6.42, 95% CI: 1.40-29.49) and short axis of lateral lymph nodes <5 mm after radiotherapy ( HR=0.17, 95% CI: 0.04-0.66) were independent factors associated with lateral recurrence. The two groups had a 3-year disease-free survival of 73.25% and 62.6% ( P>0.05), respectively, and a 3-year overall survival of 87% and 82.5% ( P>0.05), respectively. Conclusions:The SIB-IMRT is safe and effective for rectal cancer with LLNM. The short axis of lateral lymph nodes <5 mm after neoadjuvant radiotherapy and SIB-IMRT is an independent risk factor for lateral recurrence.

Objective:To compile the post quality standard scale for wound, ostomy and continence nurses in China and to test the reliability and validity of the scale.Methods:The first draft of the scale was formed through literature review, semi-structured interview and Delphi expert letter consultation. Using the purposive sampling method, a total of 796 wound, ostomy and continence nurses from 31 provinces, municipalities and autonomous regions of China were selected from April to May 2019 to test the reliability and validity of the scale. A total of 796 questionnaires were sent out and 693 were effectively received, with effective recovery of 87.1%.Results:The post quality standard scale for wound, ostomy and continence nurses included 3 first-level items and 77 second-level items. Three common factors were extracted from exploratory factor analysis and the cumulative variance contribution rate was 72.466%. The correlation coefficient between the first-level items was 0.709-0.898 and the correlation coefficient between the first-level item and the total scale was 0.859-0.937. The Cronbach's α coefficient of the total amount table was 0.992 and the half-fold reliability coefficient was 0.917 ( P<0.01) . Conclusions:The post quality standard scale for wound, ostomy and continence nurses has good reliability and validity, which can be used to evaluate the post quality of wound, ostomy and continence nurses.

Cost data caliber governance is key to fine cost management. To tackle the troubles in cost data management at multiple campuses of one hospital, the authors built a multi-campus cost data caliber governance mode. By means of enhanced top-level design, the mode carried out data governance by such measures as the establishment of data dictionary mapping library, standardizing department names and caliber, classification of charging items of medical services, precisely matching between fixed assets and charging items, interconnecting the management system of charging library and the procurement library of consumables, as well as precisely matching surgical disease types and charging items. These measures accomplished the consistency and comparability of cost data across campuses, building an automated, streamlined, standardized and integrated data governance mode for reference of hospitals with multiple campuses in need of cost management.

Objective:To construct a position responsibilities index system of wound, ostomy and continence nurses (WOCN) based on the Delphi method.Methods:Through literature review, semi-structured interviews, and investigation and research on the current working situation of WOCN, the expert inquiry questionnaire was self-designed with reference to opinions of the expert coordination group. From January to April 2019, a total of 45 senior experts including professional doctors, WOCN representatives, representatives of international Enterostomal Therapist Training Program (ETNEP) and chairmen of various provincial professional committees in China were invited to conduct 3 rounds of Delphi expert consultation, and construct the position responsibilities index system of WOCN.Results:The positive coefficients of the 3 rounds of expert consultation were respectively 100.0% (45/45) , 93.3% (42/45) and 100.0% (42/42) , and the authority coefficient of the experts was (0.92±0.16) . The final position responsibilities index system of WOCN included 5 first-level indexes such as clinical practice, education, research, coordinated management and professional promotion and 33 second-level indexes.Conclusions:The index system constructed in the study clarifies the position responsibilities of WOCN, which helps to provide a certain theoretical basis for the work, performance and classification of specialist nurses.

Objective:To establish a post quality standard index system for wound, ostomy and continence nurses (WOCN) .Methods:An expert coordination group was established in January 2019. Through literature search, semi-structured interviews, WOCN work status surveys, and concerning the opinions of the expert coordination group, the WOCN post quality standard index system was initially constructed. The convenient sampling method was adopted to conduct three rounds of Delphi expert letter consultation among 45 senior experts from all over China from January to April 2019.Results:The expert positive coefficients of three rounds of expert letter inquiries were respectively 100.0%, 93.3% and 100.0%, the expert authority coefficient was (0.92±0.16) , Kendall coordination coefficient was 0.241-0.383 ( P<0.01) and the coefficient of variation was all less than 0.25. The final WOCN post quality standard index system includes 5 first-level indicators (clinical practice ability, education ability, research ability, coordination management ability and professional promotion ability) and 79 second-level indicators. Conclusions:Clarifying WOCN post quality standards can promote the formulation of human resources decisions in various aspects, such as retraining and promotion of specialist nurses, and improve the scientific and effective management of specialist nurses.