Neurofibromatosis type 1 (NF1) presents with a diverse range of symptoms that can affect the skin, bones, eyes, central nervous system, and other organs. This article reports the diagnosis and treatment process of a patient with NF1 complicated by bilateral severe-to-profound sensorineural hearing loss. Genetic testing revealed a heterozygous variant of NF1 NM_ 000267.3: c.4054_ 4058del(p.Ser1352LeufsTer20, supporting the diagnosis of NF1. After thorough evaluation, a right cochlear implantation was performed, yielding satisfactory postoperative results. This case suggests that NF1 patients may exhibit phenotypic heterogeneity and atypicality, providing a reference for clinicians in the diagnosis and treatment of such patients.

Liver diseases have become a major challenge threating the global health, posing a heavy burden on both social and personal well-being. In recent years, the development of the gut-liver axis theory has provided new research perspectives and intervention strategies for the prevention and treatment of liver diseases. Natural products, recognized as biological molecules with diverse sources, rich activities, and minimal side effects, demonstrate great potential in regulating intestinal flora and improving liver health. Studies have shown that natural products such as saponins, polyphenols, polysaccharides, and alkaloids can regulate the composition and metabolites of intestinal flora, thereby intervening in liver diseases. In this paper, we systematically review the role of natural products in the regulation of the intestinal flora-gut-liver axis and summarize recent research progress in the prevention and treatment of liver diseases. Furthermore, we outline the challenges and limitations currently facing the study in this field. Finally, this paper makes an outlook on the clinical application of natural products in treating liver diseases and discusses future research directions, aiming to give new insights into the mechanisms by which natural products regulate the intestinal flora-gut-liver axis and the applications of these products in the prevention and treatment of liver diseases.
Humans ; Gastrointestinal Microbiome/drug effects* ; Liver Diseases/prevention & control* ; Biological Products/pharmacology* ; Polyphenols/pharmacology* ; Saponins/pharmacology* ; Intestines/microbiology* ; Alkaloids/pharmacology* ; Polysaccharides/pharmacology* ; Liver

The Ehlers-Danlos syndrome(EDS)is a rare inherent connective tissue disorder.The prev-alence of EDS in the population is estimated at one out of ten thousand to one out of a hundred thousand.The vascular EDS(vEDS)are rare among the subtypes but are the worst in prognosis.The article reports a case of vEDS admitted to the hospital.The patient was a young man complaining of a sudden onset of aphasia in right hemiparalysis and severe left abdominal pain for unknown reasons.The diagnosis was made after the genetic testing.The patient suffered from vEDS.Then,the multi-disciplinary team(MDT)made a treatment plan tailored to this young patient.The complexity in classification and delusive presentations of the EDS make the correct diagnosis very challenging.This article hopes to report this case and to share the experiences to the bet-ter understanding of this disease.

This study summarized the nursing experience of 2 pediatric heart transplant surgeries.Perioperative care included preoperative maintenance of cardiac function,volume management,infection prevention measures,and psychological preparation.Early postoperative care involved circulatory monitoring,management of right heart dysfunction,use of immunosuppressants,observation of rejection reactions,control of postoperative infections,nutritional support,psychological care,and home continuity care.The postoperative rehabilitation effect of the children is satisfactory after multi-team special treatment and nursing care.The postoperative hospitalization time for the 2 patients were 20 and 30 days.After discharge,2 patients showed good recovery during follow-up.

Objective:To observe any effect of percutaneous tibial nerve stimulation (PTNS) combined with electro-acupuncture on detrusor overactivity after a spinal cord injury.Methods:Forty spinal cord injury survivors with neurogenic detrusor overactivity were randomly assigned to a control group or an observation group, each of 20. Both groups received routine bladder training and electro-acupuncture modulating 3 sacral spinal nerves. The observation group also received 20 minutes of bilateral PTNS five times a week for 8 weeks. The frequency was 10Hz with a pulse width of 200μs. Before and after the treatment, both groups′ urination frequency, incontinence and average daily urine volume were assessed using a urodynamics analyzer, bladder diaries and an incontinence quality of life questionnaire (I-QOL).Results:After treatment, the average involuntary detrusor contraction volume (IDCV), maximum detrusor pressure at filling time (P det·max), bladder compliance (BC), residual volume and the TL value of the electromyogram of the urethral sphincter (LgTLR) had all improved significantly in both groups. The 1st IDCV, BC and LgTLR of the observation group were then significantly better than in the control group, on average, with the average P det·max and residual volume significantly lower than in the control group. The average daily single urine output and I-QOL score of both groups had increased significantly, while the average daily urination frequency and frequency of urinary incontinence had decreased significantly. Both were again significantly better in the observation group. Conclusion:Combining percutaneous electrical stimulation of the tibial nerves with electro-acupuncture can effectively inhibit detrusor overactivity after a spinal cord injury, reducing urinary incontinence.

OBJECTIVE To explore the grouping efficacy of diagnosis related group （DRG） and the influential factors of hospitalization cost in diabetes cases， and to provide theoretical support for improving DRG payment system， reducing medical cost and enhancing the efficiency of medical insurance funds. METHODS The information of 4 368 diabetic patients who were hospitalized in a 3A hospital in Xi’an from January 1， 2021 to June 30， 2023 was retrospectively analyzed， and DRG grouping of them was summarized； the hospitalization costs of patients in different DRG groups were analyzed by using one way ANOVA and Bonferroni multiple comparison. Coefficient of variation （CV） was used for evaluation within the group， and the influential factors of hospitalization costs were analyzed by one-way linear regression analysis and multi-factor linear regression analysis. RESULTS & CONCLUSIONS The CV values of the four DRG groups were all lower than 0.8， indicating good grouping results and good consistency within the group； the difference of hospitalization cost among the four groups was statistically significant （P＜0.05）， and the hospitalization cost of China Healthcare Security-DRG version 1.1 FW11 group was significantly higher than those of other three groups （P＜0.05）. Length of stay， drug cost， the number of other diagnoses， test cost and payment method have significant positive effects on the hospitalization cost of diabetic patients. Whether there is pharmacist intervention has a significant negative influence on the hospitalization cost of patients. Under the DRG payment method， medical institutions can consider multidisciplinary linkage and incorporate a variety of management and service tools， including pharmacist’s intervention， to develop refined management measures， to reduce the economic burden of patients’ families and society.

Radiation pneumonitis and pulmonary fibrosis are important dose-limiting side effects of radiotherapy that influence the prognosis and quality of life of patients with thoracic cancer. The disorder of the immune system plays a key role, especially macrophages. With the discovery of underlying molecular mechanisms, including the chemotaxis and polarization of macrophages, the infiltration of inflammatory cells, the release of inflammatory and pro-fibrotic factors, extracellular matrix deposition and the remodeling of lung structure, increasing strategies are under investigation to facilitate the prevention or treatment of lung injury via targeting macrophages. In this paper, the role of macrophages in the development of radiation pneumonitis, pulmonary fibrosis, and potential drug use strategies were reviewed.

Objective To study and estimate the public dose constraint and the atmospheric radiation capacity of an isotope industrial park, and to provide a basis for management of radioactive effluent discharge in isotope industrial parks. Methods We collected the natural environment data, meteorological data, and population distribution and dietary data as well as the source terms of radioactive airborne effluents of an isotope industrial park. The public dose constraint value of the isotope industrial park was determined based on the natural environmental radiation hazard level. The atmospheric environmental radiation capacity of the industrial park was estimated by the all-pathways method. Results The public dose constraint value of the industrial park was 0.2 mSv/a. At the end of industrial park planning, the maximum individual effective dose to the public caused by airborne effluent discharge was 1.07×10⁻⁶ Sv/a, which accounted for 0.54% of the public dose constraint value. The proportions of discharges of airborne radionuclides to respective environmental radiation capacities ranged from 0.04% to 4.34%. Conclusion The determined public dose constraint value of the isotope industrial park can be used as the total radiation capacity of the regional atmospheric environment to constrain the discharge of radioactive effluents. The environmental radiation capacity of each radionuclide can be estimated by the all-pathways method considering the comprehensive impact of radionuclide discharges, which is suitable for the control and management of isotope production enterprises in industrial parks.

Objective To investigate the radiation dose to operators in the process of ²⁴¹Am-Be neutron source logging, and discuss neutron source management and protective measures for operators in well logging. Methods Through on-site observation and measurement of ²⁴¹Am-Be neutron source logging in a company, we obtained the surface γ dose rate and neutron dose rate of the neutron source, as well as the operating time and distance of various processes including source taking, transfer, and loading, calculated the radiation dose to operators in various processes, and analyzed the source and proportion of the personal effective dose to operators. Results The effective doses of neutron irradiation and γ irradiation were 94.17 μSv and 2.72 μSv, respectively, for the combined processes of source tank inspection, transfer, and detection; 36.66 μSv and 24.08 μSv, respectively, for source loading and unloading; and 130.83 μSv and 26.80 μSv, respectively, for the whole neutron source logging process. The total annual effective dose of neutron source logging was 15.78 mSv, as estimated by logging 100 times per year. Conclusion In the process of ²⁴¹Am-Be neutron source logging in the company, the effective dose to operators mainly arises from neutron irradiation. Therefore, it is necessary to strengthen neutron source management and take effective protective measures against neutron radiation.

Objective To explore the radiation shielding optimization plan for a medical proton cyclotron developing and commissioning building at various commissioning stages. Methods According to the maximum source termsat different commissioning stages, we used the empirical formula to estimate the instantaneous dose rate at the point of interest outside the shield of the building, and optimized the building’s shielding ateach commissioning stage. Results When adding 1.0 m mobile concrete shielding blocks (“blocks” below) each to wall 3 and wall 4 at the cyclotron commissioning stage, 1.0 m blocks to wall 4 and 1.25 m blocks to wall 5 at the beam transport line commissioning stage, and 1.0 m blocks to wall 9 and 0.4 m blocks to the ceiling at the simulated treatment room commissioning stage, the dose rates at the points of interest outside the shield could meet the dose rate limit requirements. Conclusion The application of mobile concrete shielding blocks not only meets the shielding requirements, but also has economical and space-saving advantages, conforming to the principle of shielding optimization. This can be an approach to the optimization of radiation shielding for high-energy particle accelerators or similar scientific projects.