Objective:To compare the preoperative presentation, intraoperative findings, and postoperative outcomes between middle ear cholesteatoma with tympanosclerosis （MECwTS） and middle ear cholesteatoma without tympanosclerosis （MECw/oTS）, thereby investigating the clinical characteristics of MECwTS. Methods:A retrospective analysis was conducted on the clinical data of 120 patients with middle ear cholesteatoma. Patients were divided into two groups based on the presence or absence of concomitant tympanosclerosis: the MECwTS group （n=49） and the MECw/oTS group （n=71）. All patients underwent preoperative evaluations including temporal bone CT, otoscopic examination, pure-tone audiometry, tympanometry, and assessment using the Zurich Chronic Middle Ear Inventory （ZCMEI-21） quality of life scale. All patients underwent canal wall down mastoidectomy with tympanoplasty. Concurrent ossicular chain reconstruction was performed: partial ossicular replacement prosthesis （PORP） in 83 cases and total ossicular replacement prosthesis （TORP） in 37 cases. Intraoperative disease severity was assessed using the Cholesteatoma Comprehensive Score Scale （CCSS）. Postoperative follow-up lasted at least one year and included pure-tone audiometry, otoscopic examination, and the ZCMEI-21 scale administered at ≥1 year post-surgery. Preoperative and postoperative air-bone gap （ABG） and ZCMEI-21 scores were compared between the MECwTS and MECw/oTS groups. Additionally, surgical efficacy was defined as a postoperative ABG ≤20 dB; the hearing improvement efficacy of PORP versus TORP was compared based on this criterion. Results: ①Preoperative ABG showed no significant difference between the MECw/oTS and MECwTS groups（P>0.05）. Postoperative ABG was （18.65±10.21） dB in the MECw/oTS group versus （22.55±9.53） dB in the MECwTS group, demonstrating a statistically significant intergroup difference （P<0.05）. ②Intraoperative CCSS scores were significantly higher in the MECwTS group （8.04±2.18） compared to the MECw/oTS group （5.93±1.44） （P<0.05）. ③Preoperative ZCMEI-21 scores showed no significant difference between groups （P>0.05）. Postoperative ZCMEI-21 scores were （22.24±8.11） in the MECw/oTS group versus （27.02±7.21） in the MECwTS group, indicating a statistically significant difference （P<0.05）. ④Postoperative ABG ≤20 dB was achieved in 54 patients （65.06%, 54/83） in the PORP group and 16 patients （43.24%, 16/37） in the TORP group. This difference in efficacy rates was statistically significant （P<0.05）. The overall efficacy rate for ossiculoplasty was 58.33% （70/120）. Conclusion: Patients with MECwTS exhibit more severe middle ear and mastoid pathology compared to those with MECw/oTS, resulting in poorer postoperative hearing levels and quality of life outcomes. Both PORP and TORP implantation can improve postoperative hearing to some extent; however, PORP appears to offer superior hearing improvement efficacy compared to TORP.
Humans ; Cholesteatoma, Middle Ear/complications* ; Retrospective Studies ; Tympanoplasty ; Myringosclerosis/surgery* ; Female ; Male ; Adult ; Middle Aged ; Ossicular Replacement ; Ossicular Prosthesis ; Young Adult ; Ear, Middle ; Treatment Outcome ; Mastoidectomy ; Audiometry, Pure-Tone ; Adolescent ; Quality of Life

Hereditary angioedema(HAE)is a rare autosomal dominant disorder characterized by recur-rent,unpredictable episodes of skin and mucosal edema,which may affect the face,extremities,respiratory tract,gastrointestinal tract,and genitals,with a global prevalence of approximately 1 in 50 000.This case re-port presents a young female patient with a history of recurrent abdominal pain and multisite edema.During an acute episode,laboratory tests revealed decreased complement C4 levels along with reduced concentration and function of C1 esterase inhibitor.Computed tomography(CT)demonstrated bowel wall edema and pelvic effu-sion.Previously undiagnosed,the patient was admitted for this acute attack and was ultimately diagnosed with HAE following a multidisciplinary treatment(MDT)team discussion at our hospital.The rapid diagnosis and treatment of this case highlight the critical role of MDT in the management of complex and rare diseases.

Objective This study employs bibliometrics and visualization analysis techniques to systematically review and analyze the current status and evolutionary trends of research on Dysosma versipellis,both domestically and internationally.The discussion encompasses the current state of research on Dysosma versipellis as well as the latest hot topics in the field.Methods The literatures related to Dysosma versipellis and meeting the inclusion criteria were searched through China Knowledge Network(CNKI)and Web of science(WOS)databases,and the core author groups,cooperative relationships among research institutions and key words were analyzed by CiteSpace software.Results Eventually,1793 Chinese documents and 771 English documents were included.The author with the highest number of Chinese articles is Zeng Kang;The authors with the highest number of articles in English are Arora Rajesh;The number of articles published in China generally increases first and then decreases;The Shanghai Institute of Pharmaceutical Industry is the institution that studies Dysosma versipellis the most,and the exchanges and cooperation among institutions in the research field need to be further deepened.The keyword analysis shows that the research content of Dysosma versipellis mainly focuses on anti-tumor application,extraction and synthesis of derivatives.Conclusion At present,the extraction and synthesis of lignans and their related derivatives from Dysosma versipellis and their clinical application in the treatment of condyloma acuminatum and anti-tumor are the research hotspots of Dysosma versipellis.The toxicological mechanisms of damage caused by Dysosma versipellis and its active components,as well as potential therapeutic approaches,are expected to become a focal point of future research.

The female reproductive system is essential for sustaining reproductive endocrine homeostasis,however,its vulnerability to various endogenous and exogenous insults,including pathological conditions,pharmacological agents,genetic predispositions,and environmental factors,often results in compromised fertility.The existing protective approaches(including surgical interventions,hormonal replacement therapies,and assisted reproductive techniques)are constrained by several limitations,such as adverse therapeutic effects,technical complexities,and their incapacity to reverse ovarian senescence.Artemisinin and its derivatives(ARTs),characterized by their unique endoperoxide bridge configuration,have exhibited outstanding therapeutic performance across multiple domains including malaria treatment,anticancer therapy,inflammation modulation,and parasitic infection control.Emerging research has identified their novel protective capabilities against various reproductive system pathologies.This comprehensive review systematically elucidates the molecular mechanisms underlying artemisinin-based interventions in reproductive pathologies and evaluates their clinical translation prospects,thereby proposing innovative strategies for the development of next-generation fertility-protective agents with enhanced safety and efficacy profiles.

Hereditary angioedema(HAE)is a rare autosomal dominant disorder characterized by recur-rent,unpredictable episodes of skin and mucosal edema,which may affect the face,extremities,respiratory tract,gastrointestinal tract,and genitals,with a global prevalence of approximately 1 in 50 000.This case re-port presents a young female patient with a history of recurrent abdominal pain and multisite edema.During an acute episode,laboratory tests revealed decreased complement C4 levels along with reduced concentration and function of C1 esterase inhibitor.Computed tomography(CT)demonstrated bowel wall edema and pelvic effu-sion.Previously undiagnosed,the patient was admitted for this acute attack and was ultimately diagnosed with HAE following a multidisciplinary treatment(MDT)team discussion at our hospital.The rapid diagnosis and treatment of this case highlight the critical role of MDT in the management of complex and rare diseases.

Objective This study employs bibliometrics and visualization analysis techniques to systematically review and analyze the current status and evolutionary trends of research on Dysosma versipellis,both domestically and internationally.The discussion encompasses the current state of research on Dysosma versipellis as well as the latest hot topics in the field.Methods The literatures related to Dysosma versipellis and meeting the inclusion criteria were searched through China Knowledge Network(CNKI)and Web of science(WOS)databases,and the core author groups,cooperative relationships among research institutions and key words were analyzed by CiteSpace software.Results Eventually,1793 Chinese documents and 771 English documents were included.The author with the highest number of Chinese articles is Zeng Kang;The authors with the highest number of articles in English are Arora Rajesh;The number of articles published in China generally increases first and then decreases;The Shanghai Institute of Pharmaceutical Industry is the institution that studies Dysosma versipellis the most,and the exchanges and cooperation among institutions in the research field need to be further deepened.The keyword analysis shows that the research content of Dysosma versipellis mainly focuses on anti-tumor application,extraction and synthesis of derivatives.Conclusion At present,the extraction and synthesis of lignans and their related derivatives from Dysosma versipellis and their clinical application in the treatment of condyloma acuminatum and anti-tumor are the research hotspots of Dysosma versipellis.The toxicological mechanisms of damage caused by Dysosma versipellis and its active components,as well as potential therapeutic approaches,are expected to become a focal point of future research.

The female reproductive system is essential for sustaining reproductive endocrine homeostasis,however,its vulnerability to various endogenous and exogenous insults,including pathological conditions,pharmacological agents,genetic predispositions,and environmental factors,often results in compromised fertility.The existing protective approaches(including surgical interventions,hormonal replacement therapies,and assisted reproductive techniques)are constrained by several limitations,such as adverse therapeutic effects,technical complexities,and their incapacity to reverse ovarian senescence.Artemisinin and its derivatives(ARTs),characterized by their unique endoperoxide bridge configuration,have exhibited outstanding therapeutic performance across multiple domains including malaria treatment,anticancer therapy,inflammation modulation,and parasitic infection control.Emerging research has identified their novel protective capabilities against various reproductive system pathologies.This comprehensive review systematically elucidates the molecular mechanisms underlying artemisinin-based interventions in reproductive pathologies and evaluates their clinical translation prospects,thereby proposing innovative strategies for the development of next-generation fertility-protective agents with enhanced safety and efficacy profiles.

Objective:To explore the predictive and prognostic value of prognostic nutritional index(PNI)for patients with locally advanced esophageal squamous cell carcinoma(ESCC)undergoing induction chemotherapy combined with immune sequential radiotherapy.Methods:A retrospective analysis was conducted on clinical data from 126 locally advanced ESCC patients who had undergone induction chemotherapy combined with immune sequential radiotherapy at Zhejiang Cancer Hospital between May 2019 and August 2023.Receiver operating characteristic(ROC)curves were used to determine optimal PNI cutoff values within 1 week before induction chemoimmunotherapy,within 1 week before radiotherapy,and at 4±1 weeks after radiotherapy initiation,with subsequent patient stratification.The Kaplan-Meier method was used to generate survival curves and the log-rank test was used to compare overall survival(OS)and progression-free survival(PFS)between groups.Cox regression analysis was employed to identify factors affecting the prognosis of locally advanced ESCC patients undergoing induction chemoimmunotherapy combined with sequential radiotherapy.Results:A total of 126 locally advanced ESCC patients,118 males and 8 females,with a median age of 65 years(44-78 years)were included.The optimal critical values of PNI before induction chemoimmunotherapy,before radiotherapy and during radiotherapy identified using ROC curves were 46.2,48.3 and 37.9.The median OS and PFS were 47.3 and 28.2 months in the group with PNI≥48.3 before radiotherapy,and 18.7 and 15.2 months in the group with PNI<48.3 before radiotherapy,respectively(P<0.01,P<0.05).The median OS was not reached and the median PFS was 25.7 months in the group with PNI≥37.9 in radiotherapy,and the median OS and PFS were 17.0 and 12.5 months in the group with PNI<37.9 in radiotherapy,respectively(P<0.01,P<0.05).The median OS was not reached and the median PFS was 28.4 months in the group with elevated PNI after induction chemoimmunization;the median OS and PFS were 20.4 and 16.0 months in the group with reduced PNI(P<0.01,P<0.05).Multifactorial analysis showed that PNI in radiotherapy[HR=2.292,95%CI(1.264,4.159),P<0.05],and change in PNI after induction of chemoimmunization[HR=2.120,95%CI(1.007,4.463),P<0.05]were factors affecting OS.Conclusion:PNI during radiotherapy and changes in PNI after induction chemoimmunity correlate with patients'treatment efficacy and prognosis,and can be used as important indicators to predict the benefits of induction chemoimmunization combined with sequential radiotherapy for ESCC.

Objective:To report five families of familial periodic paralysis.Methods:The clinical and genetic data of 5 families with familial periodic paralysis caused by SCN4A gene mutation who visited the First Affiliated Hospital of Henan University of Science and Technology from 2017 to 2022 were analyzed retrospectively.Results:The probands carried heterozygous missense mutations of SCN4A gene c. 3395G>A p. Arg1132Gln (Case 1), c. 2015G>A p. Arg672His (Case 2 and case 3), c. 2006G>A p. Arg669His (Case 4), c. 2111C>T p. Thr704Met (Case 5), respectively. Among them, four probands were diagnosed as hypokalemic periodic paralysis, one patient considered normal blood potassium periodic paralysis, and the treatment of acute attack was mainly potassium supplement. The main treatment for acute attacks was potassium supplementation, which was administered through intravenous infusion of potassium chloride combined with oral potassium chloride sustained-release tablets in the hospital. Simultaneously blood potassium levels and electrocardiogram monitoring were closely monitored. The main approach outside the hospital was to adopt a reasonable lifestyle and avoid triggering factors.Conclusions:The clinical manifestations caused by SCN4A gene mutation are diverse, and special attention should be paid in diagnosis, treatment and genetic counseling. Gene sequencing is an important molecular genetic diagnostic method.

Objective:To report five families of familial periodic paralysis.Methods:The clinical and genetic data of 5 families with familial periodic paralysis caused by SCN4A gene mutation who visited the First Affiliated Hospital of Henan University of Science and Technology from 2017 to 2022 were analyzed retrospectively.Results:The probands carried heterozygous missense mutations of SCN4A gene c. 3395G>A p. Arg1132Gln (Case 1), c. 2015G>A p. Arg672His (Case 2 and case 3), c. 2006G>A p. Arg669His (Case 4), c. 2111C>T p. Thr704Met (Case 5), respectively. Among them, four probands were diagnosed as hypokalemic periodic paralysis, one patient considered normal blood potassium periodic paralysis, and the treatment of acute attack was mainly potassium supplement. The main treatment for acute attacks was potassium supplementation, which was administered through intravenous infusion of potassium chloride combined with oral potassium chloride sustained-release tablets in the hospital. Simultaneously blood potassium levels and electrocardiogram monitoring were closely monitored. The main approach outside the hospital was to adopt a reasonable lifestyle and avoid triggering factors.Conclusions:The clinical manifestations caused by SCN4A gene mutation are diverse, and special attention should be paid in diagnosis, treatment and genetic counseling. Gene sequencing is an important molecular genetic diagnostic method.