Hereditary angioedema (HAE) is a rare genetic disorder, typically managed with on-demand medications during acute attacks. Clinical trials and real-world studies have demonstrated the efficacy and safety of icatibant in treating acute HAE attacks. This article aims to summarize research findings on icatibant in HAE treatment, providing a reference for its clinical application.

The clinical phenotype heterogeneity of epilepsy patients with ADGRV1 gene mutation is significant, ranging from self limiting febrile seizures to developmental epileptic encephalopathy, even causing sudden epileptic death. A case of infantile epileptic spasms syndrome with a novel heterozygous variant of the ADGRV1 gene c.4100C>A (p.Thr1367Lys) was reported in this article. The site of this variant had not been reported yet, and the clinical manifestations of the child mainly included epileptic spasms (first onset at 4 months old), mild growth and development delay, highly irregular video electroencephalogram, and effective treatment with adrenocorticotropic hormone.

Nuclear medicine plays a pivotal role in diagnosis and treatment of tumors.The development of radionuclide probes with high affinity and specificity is an effective approach to achieve precise diagnosis and treatment of tumors.With high binding affinity,rapid tissue penetration and excellent pharmacokinetic properties,bicyclic peptide-based radionuclide probes demonstrate remarkable advantages in diagnosis and targeted therapy of tumors.The structural characteristics and design strategies of bicyclic peptide-based radionuclide probes were reviewed in this article,mainly focused on research progresses in targeting tumor-associated receptors.

We report the clinical course from birth to adolescence of a patient carrying a compound heterozygous variation in the ectonucleotide pyrophosphatase/phosphodiesterase family member 1(ENPP1) gene. The patient was diagnosed with generalized arterial calcification of infancy shortly after birth, and subsequently with autosomal recessive hypophosphatemic rickets type 2 at the age of 11 years. Following effective blood pressure control, treatment with neutral phosphate, calcitriol, and vitamin D was initiated. During follow-up, no progression of vascular calcification was observed. Through this case report and a review of relevant literature, we aim to enhance clinicians′ understanding of this rare condition.

Objective:To introduce the cultivation model of the scientific research talent echelon in the university-affiliated specialized hospital, and talent cultivation system of Research-Life-Cycle based on this model, summarize its role in promoting discipline development, and propose future measures, providing references for the talent team building and discipline development of specialized hospitals and departments of general hospitals.Methods:This study examined and assessed the effectiveness of the cultivation and the contribution of talents to the advancement of academic disciplines by quantitatively analyzing and comparing the scientific research output and discipline development of personnel trained through the talent echelon policy before and after its implementation for over a decade, and integrating theories related to talent development.Results:Since the implementation of the cultivation model, 116 research talents had completed the cultivation process, accounting for 18% of the hospital′s overall research personnel, and the academic papers echelon talents published as main authors also accounted 60%, which indicated the majority of echelon talents have become the key talents to discipline development and national strategies. The growth rate of the hospital′s papers had significantly increased before and after the policy implementation, with the data rising from 0.16% to the current 1.01% globally.Conclusions:The scientific research talent echelon system has effectively strengthened the cultivation of medical science and technology innovation talents, promoted the formation of the group effect in the hospital, and provided strong support for the development of disciplines. According to the limitations we analyze to the scientific research talent echelon cultivation model, suggestions, for achieving better results in the cultivation of talent echelons, are proposed that we should continuously follow up on national policies to optimize systems, firmly serve for the central task of discipline development, promote the implementation of the comprehensive evaluation to scientific talents, systematically provide young talents with ″real combat″ opportunities, and continuously pursuit the transition from extensive management to refined and personalized management.

OBJECTIVES: To analyze the efficacy and safety of concomitant left atrial appendage clipping during heart valve surgery for valvular heart disease patients with atrial fibrillation. METHODS: Fifty-eight patients who underwent concomitant left atrial appendage clipping during cardiac valve surgery in the Second Affiliated Hospital of Nanchang University from January 2017 to June 2023 were included in the analysis, including 1 case who underwent aortic valve replacement, 49 cases who underwent mitral valve replace-ment (or valvuloplasty)+tricuspid valvuloplasty, and 8 cases who underwent double valve replacement+tricuspid valvuloplasty (3 cases combined with coronary artery bypass grafting). The patients were followed up for 3-36 months [(16.69±6.61) months] after operation, and the changes of cardiac function and the occurrence of serious adverse complications were evaluated. RESULTS: The cardiopulmonary bypass time ranged from 75 to 145 min [(102.50±21.03) min], and the aortic cross-clamp time ranged from 35 to 80 min [(58.02±14.63) min]. The length of postoperative intensive care unit stay was 1 to 5 days [(2.47±0.82) d], and the length of postoperative hospital stay was 7 to 22 days [(10.84±2.69) d]. Cardiac ultrasound indicated complete closure of the left atrial appendage in all cases. During the follow-up, New York Heart Association (NYHA) functional classifications were improved in 54 patients. No left atrial appendage-related bleeding events or other perioperative complications were observed; and no cerebral infarction, limb embolism events, or mortality cases occurred during the follow-up. CONCLUSIONS For valvular heart disease patients with atrial fibrillation, concomitant left atrial appendage clipping during cardiac valve surgery demonstrates efficacy and safety, with no severe adverse events during a medium-term follow-up.
Humans ; Atrial Appendage/surgery* ; Atrial Fibrillation/complications* ; Male ; Female ; Heart Valve Diseases/complications* ; Aged ; Middle Aged ; Heart Valve Prosthesis Implantation/methods* ; Treatment Outcome ; Cardiac Surgical Procedures/methods* ; Mitral Valve/surgery*

Objective:To explore the effect of preoperative gamma-glutamyl transferase to albumin ratio (GAR) on prognosis of patients with Barcelona clinic liver cancer (BCLC)stage 0-A hepatocellular carcinoma (HCC) after microwave ablation (MWA).Methods:The clinical data of 201 patients with BCLC stage 0 to A who underwent MWA at two centers of Jiaxing Second Hospital and Shengjing Hospital of China Medical University from January 2011 to December 2018 were retrospectively analyzed, including 152 males and 49 females, aged (57.5±9.6) years. X-tile software was used to divide patients into a low GAR group ( n=84, GAR<0.9) and a high GAR group ( n=117, GAR≥0.9). Kaplan-Meier method was used for survival analysis, and log-rank test was used for survival comparison. Univariate and multivariate Cox regression analysis was used to evaluate the relationship between GAR and prognosis. Results:The cumulative survival rates at 1, 3 and 5 years postoperatively were 98.7%, 93.8%, 78.5% for the low GAR group, which were superior to that 97.2%, 87.1%, 70.2% for the high GAR group, and the difference was statistically significant ( χ2=11.89, P=0.001). The recurrence-free survival rates at 1, 3 and 5 years after surgery between the two groups was no significant difference ( χ2=1.70, P=0.192). Multivariate analysis revealed that high GAR ( HR=2.723, 95% CI: 1.508-4.914, P=0.001) was independent risk factors for overall survival after MWA in patients with BCLC stages 0 to A HCC. Male gender ( HR=1.959, 95% CI: 1.127-3.305, P=0.017) and tumor diameter ≥2 cm ( HR=1.547, 95% CI: 1.008-2.373, P=0.046) were independent risk factors for recurrence after MWA in patients with BCLC stages 0 to A HCC. Univariate analysis, GAR≥0.9 was not associated with postoperative recurrence ( HR=1.315, 95% CI: 0.869-1.989, P=0.195). Conclusion:Preoperative GAR (≥0.9) is an independent risk factor affecting overall survival in patients with BCLC stages 0 to A HCC after MWA.

Objective To understand the clinical applicability and implementation of expert consensus on the implementation and removal of protective restraints in psychiatry,and to provide references for promoting the standardized practice of psychiatric protective restraints and updating the consensus.Methods By the convenience sampling method,a questionnaire survey was conducted among nurses from 480 hospitals in 30 provinces from June 15 to July 15,2024.The survey was conducted using the instrument for evaluating clinical applicability of guide-lines(version 2.0)and a self-compiled questionnaire on the clinical implementation of the restraint consensus.Results A total of 7,844 valid questionnaires were collected,with a valid questionnaire recovery rate of 93.78％.The results of clinical applicability scoring showed that the consensus had the lowest availability score(64.72％)and the highest acceptability score(76.74％).The results showed that nurses' receiving training and the level of their hospitals were the main influencing factors for scores in various dimensions(P＜0.05).4,774 participants(87.42％)believed that the application of consensus could enhance the standardization of nurses' restraint operations.The safety rate of the restraint consensus was 79.51％,and the economic ratio was 76.87％.Among the evaluators,1,739(22.17％)believed that there were implementation obstacles in the consensus.Conclusion The clinical applicability of the consensus is relatively good,and the application of the consensus helps to improve the standardization of clinical operations.In the future,efforts should be made to strengthen the promotion and training of the consensus,develop hierarchical promotion strategies according to the characteristics of medical institutions,and improve the quality of evidence for the consensus,so as to further enhance the clinical application effect of the consensus.

Objective To investigate the clinical efficacy of the traditional Chinese medicine(TCM)throat opening and brightening method in treating unilateral vocal cord paralysis(UVCP).Methods Sixty patients with UVCP were prospectively collected and randomly assigned to two groups:the Chinese herbal medicine group(trea-ted with Buyang Huanwu Decoction,n=30)and the throat opening and brightening method group(treated with TCM throat opening and brightening method,n=30).The clinical studies that had utilized injection laryngoplasty for the treatment of UVCP(historical control group).Evaluation indicators included the voice handicap index-10(VHI-10),GRBAS-G,objective acoustic measurements of voice(vocal intensity,F0,shimmer,jitter,HNR),and aerodynamic measurements(maximum phonation time,MPT).Results Before treatment,no significant differences were observed between the two groups in all the evaluation indicators(P＞0.05).Post-treatment,the throat open-ing and brightening method group demonstrated significant improvements in VHI-10,GRBAS-G,shimmer,jitter,HNR,and MPT compared to pre-treatment values(P＜0.01),and these improvements were superior to those in the Chinese herbal medicine group.Pre-treatment VHI-10,GRBAS-G,and shimmer scores in the throat opening and brightening method group were significantly higher than those in the historical literature group(P＜0.01).Af-ter treatment,no significant differences were noted in any assessed parameters between the two groups(P＞0.05).Conclusion The TCM throat opening and brightening method significantly enhances phonatory function and quality of life in patients with UVCP,showing comparable efficacy to injection laryngoplasty.