BACKGROUND:Vertigo is closely related to clinical neurological disorders.When neurons are damaged or dead,it may lead to abnormalities in the vestibular system and trigger vertigo symptoms.Therefore,it is necessary to explore and analyze the hotspots related to vertigo that are common in clinical neurology. OBJECTIVE:To analyze the vertigo-related histopathological changes in clinical neurology and the research hotspots worldwide using bibliometric methods. METHODS:The WanFang database and Web of Science core set database were searched by the first author to retrieve the research-related literature published from 2014-2023 on the treatment of common vertigo in clinical neurology.A bibliometric analysis of the number of publications,country/region,institution,keywords,co-cited literature,and highly cited literature was peformed using VOSviewer_1.6.19 software to summarize the research hotspots in this research field. RESULTS AND CONCLUSION:Web of Science core set database had the highest number of 174 publications in this field in 2022,and WanFang database had the highest number of 133 publications in this field in 2020.The top 3 countries with the highest number of publications are the United States,Germany,and China.The University of Munich,Germany is the international institution with the highest number of publications in this field,while Chengdu University of Traditional Chinese Medicine is the Chinese institution with the highest number of publications in this field.The results of keyword analysis showed that the research hotspot diseases in this field in China are mainly Meniere's disease,cervical vertigo,senile vertigo,benign paroxysmal positional vertigo,isolated vertigo,and hypertensive vertigo,and the treatments include acupuncture,rehabilitation,medication(gastrodin,Banxia Baizhu Tianma Tang),and manipulative reduction.International research hotspot diseases in this field mainly include benign paroxysmal positional vertigo,vestibular disorders in new coronavirus cases,Meniere's disease,vestibular schwannoma,acoustic neuromas,and vestibular migraines,etc.,and the hotspot treatments are antivertiginous medications,antidepressant and anxiolytic treatments,and microsurgery.The results of literature co-citation analysis showed that for acute vestibular syndrome with persistent vertigo as the main symptom,three-step bedside ophthalmoscopy(HINTS:Head-Impact-Nystagmus-Strabismus Test)is more sensitive than early MRI in the diagnosis of combined strokes in patients with acute vestibular syndrome,which is the most peer-recognized method of detecting strokes in vestibular syndrome,whereas hormonal therapy is more effective to treat vestibular neuritis patients with paroxysmal vertigo as the main symptom.The results of highly cited literature analysis showed that,in the hot literature included in WanFang database in the past 10 years,acupuncture at Fengchi point and the acupuncture method of inducing resuscitation to improve posterior circulation ischemic vertigo have achieved certain results.The literature published in the past 3 years has indicated that Ginkgo biloba leaf extract+gastrodin,acupuncture+Banxia Baizhu Tang,betahistine+gastrodin,vestibular rehabilitation training+Epley Maneuver,all can improve the vertigo symptoms to different degrees.While there were no featured anti-vertigo drugs indicated in the literature in the Web of Science core set data in the recent 10 years,and most of them are based on traditional anti-vertigo drugs and microsurgery.However,there are a few case reports in the international literature in the last 3 years that found that COVID-19 infection may lead to vestibular neuritis and vertigo symptoms.The onset and progression of vertigo may be closely related to neuronal damage and regeneration.For example,viral infections,inflammatory stimuli,or other pathologic factors may lead to neuronal damage or death,thereby affecting the function of the vestibular system.Vertigo-related diagnosis and treatment standardization guidelines have been published both domestically and internationally.Currently,international guidelines recommend the combination of vestibular rehabilitation and physical rehabilitation for the treatment of vertigo,and Chinese guidelines recommend the combination of Chinese and Western medicine,reduction and acupuncture.However,the level of evidence is not very high,so a large number of large-sample,multicenter randomized controlled trials on anti-vertigo treatment are needed in the future.

Objective:To investigate the therapeutic efficacy of venetoclax combined with avapritinib in treatment of refractory/relapsed acute myeloid leukemia (AML) with KIT gene mutation.Methods:The clinical data of 2 AML patients with KIT gene mutation who received venetoclax combined with avapritinib admitted to Canglang Hospital of Suzhou in October 2022 and November 2022 were retrospectively analyzed, and the relevant literature was reviewed.Results:Both patients with high-risk relapsed/refractory AML and KIT gene mutation were females; the one was 53 years and the other was 17 years. Case 1 was diagnosed with AML-M 2, and genetic testing revealed positive mutations in ASXL1, KIT, and RUNX1. The patient relapsed after transplantation and then was treated with venetoclax combined with avapritinib achieving morphologic leukemia-free status (MLFS). Case 2 was diagnosed with AML, and RUNX1-RUNX1T1 (AML1-ETO) fusion gene and KIT and DX15 gene mutations were detected. The patient was treated with venetoclax combined with avapritinib regimen after relapse, and the treatment regimen significantly reduced the tumor load. Complete remission was achieved after bridging to allogeneic hematopoietic stem cell transplantation. Conclusions:AML with KIT gene mutation is heterogeneous and some patients are difficult to treat with very poor prognosis. Bridging (secondary) hematopoietic stem cell transplantation can be the better treatment choice for relapsed patients achieving MLFS or complete remission after venetoclax combined with avapritinib treatment regimen.

Objective:To compare the medium-term clinical effects of arthroscopic double row repair between traumatic and degenerative medium supraspinatus tear.Methods:A retrospective study was conducted to analyze the clinical data of 23 patients who had been treated for traumatic or degenerative medium supraspinatus tear by the same arthroscopic double row repair and postoperative rehabilitation at Sports Medicine Center, The First Hospital Affiliated to Army Medical University between January 2015 and August 2020. They were assigned into 2 groups according to different tears. In the traumatic group of 8 cases of traumatic medium supraspinatus tear, there were 5 males and 3 females with an age of (46.1±4.3) years and a tear size of (1.3±1.0) cm 2. In the degenerative group of 15 cases of degenerative medium supraspinatus tear, there were 4 males and 11 females with an age of (59.9±8.1) years and a tear size of (4.1±1.1) cm 2. At preoperation and the last follow-up, the shoulder pain was evaluated by visual analogue scale (VAS), and the shoulder function by American Shoulder and Elbow Surgeons (ASES) score, Constant-Murley score and Simple Shoulder Test (SST); the improvements in active range of motion (ROM) of the shoulder were recorded at the last follow-up. Results:The 2 groups were comparable because there was no significant difference between them in the general clinical data ( P>0.05). The traumatic and degenerative groups were followed up for (40.3±11.2) and (36.4±12.4) months, respectively. At the last follow-up, the improvements in range of anterior flexion and internal rotation vertebral rank in the degenerative group [55.3°±33.6° and (4.1±1.3) ranks] were significantly greater than those in the traumatic group [27.5°±22.5° and (2.3±1.9) ranks] ( P<0.05). At the last follow-up, the VAS, ASES, Constant-Murley, and SST scores in the degenerative group were improved respectively by (3.7±0.8), (40.9±14.0), (38.4±9.4), and (6.5±1.4) points compared with their preoperative values, significantly greater than those in the traumatic group [(2.3±0.7), (19.6±14.6), (19.2±7.9), and (3.8±0.7) points] ( P<0.05). Conclusion:Arthroscopic double row repair can achieve significant medium-term improvements in shoulder function for both traumatic and degenerative medium supraspinatus tears, but the improvements may be grater for the degenerative ones.

The Ly-6 and uPAR (LU) domain-containing proteins represent a large family of cell-surface markers. In particular, mouse Ly-6A/Sca-1 is a widely used marker for various stem cells; however, its human ortholog is missing. In this study, based on a systematic survey and comparative genomic study of mouse and human LU domain-containing proteins, we identified a previously unannotated human gene encoding the candidate ortholog of mouse Ly-6A/Sca-1. This gene, hereby named LY6A, reversely overlaps with a lncRNA gene in the majority of exonic sequences. We found that LY6A is aberrantly expressed in pituitary tumors, but not in normal pituitary tissues, and may contribute to tumorigenesis. Similar to mouse Ly-6A/Sca-1, human LY6A is also upregulated by interferon, suggesting a conserved transcriptional regulatory mechanism between humans and mice. We cloned the full-length LY6A cDNA, whose encoded protein sequence, domain architecture, and exon-intron structures are all well conserved with mouse Ly-6A/Sca-1. Ectopic expression of the LY6A protein in cells demonstrates that it acts the same as mouse Ly-6A/Sca-1 in their processing and glycosylphosphatidylinositol anchoring to the cell membrane. Collectively, these studies unveil a novel human gene encoding a candidate biomarker and provide an interesting model gene for studying gene regulatory and evolutionary mechanisms.
Humans ; Membrane Proteins/genetics* ; Pituitary Neoplasms/genetics* ; Biomarkers

Objective:To explore the application of venetoclax in transplantation of patients with refractory acute myeloid leukemia (AML).Methods:The diagnosis and treatment process of a patient with refractory AML who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) under venetoclax and hypomethylating agents bridging myeloablative preconditioning regimen after induction therapy failure in the First Affiliated Hospital of Soochow University in March 2020 were retrospectively analyzed.Results:The patient was a 28-year-old female who was diagnosed with refractory AML. The patient was initially given induction chemotherapy with IA (idarubicin+cytarabine) (3+7) regimen, but the disease did not relieve, then the induction chemotherapy with CLAG (cladribine+cytarabine+granulocyte colony stimulating factor) regimen was given, but the disease still did not relieve. After chemotherapy with venetoclax and hypomethylating agents bridging myeloablative preconditioning regimen, salvage haploid allo-HSCT was performed. Re-examination of bone marrow showed remission, and implantation was successful. The patient was followed up for 100 days and had sustained remission, and no transplantation complications occurred.Conclusion:For refractory AML patients who have failed primary induction therapy, the use of venetoclax and hypomethylating agents bridging myeloablative preconditioning regimen can be used as a preferred solution for salvage allo-HSCT.

Objective:To investigate the incidence, risk factors and survival of cGVHD patients in combination of a haploidentical donor supported with an unrelated umbilical cord blood for hematopoietic stem cells transplantation (haplo-cord-HSCT).Methods:300 hematological malignancies individuals who received dual transplantation were enrolled in the study between January 2012 and July 2016 at the department of Hematology in the First Affiliated Hospital of Soochow University. The clinical diagnosis and scoring the severity of cGVHD syndromes according the National Institutes of Health (NIH) consensus conference in the 2014 update. Cox proportional hazards regression was used to identify risk factors associated with transplant outcomes.Results:During follow-up with a median time of 26.4 months (range 0.2-61.8) post transplantation, the 1-year, 3-year and 5-year cumulative incidence of cGVHD was 26.3 % (95 % confidence interval [CI], 23.5 %~29.1 %), 30.3 % (95 % CI, 27.3 %~33.3 % ) and 32.2 % (95 % CI, 28.7 %~35.7 %). For all 73 patients with cGVHD, first-line or second-line treatment were given. During the follow-up period, 53 patients survived, and 20 patients died. In multivariate analysis, the cGVHD overall survival (GOS) were associated with thrombocytopenia(<100×109/L)(HR=0.103, 95 % CI 3 %-36.1 %, P<0.001). Conclusions:Our data suggest that, the 5-year cumulative incidence of cGVHD was 32.2 % after haplo-cord-HSCT with hematological malignancies. Thrombocytopenia (<100×109/L)was independent risk factors for GOS.

Objective:This study aims to investigate the efficacy and safety of chimeric antigen receptor (CAR) T-cell bridging allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the treatment of recurrent and refractory acute B-lymphocytic leukemia (R/R B-ALL) .Methods:A total of 50 R/R B-ALL patients who underwent CAR T-scell therapy to bridge allo-HSCT in the First Affiliated Hospital of Soochow University from January 2017 to May 2019 were retrospectively analyzed. The overall survival (OS) rate, event-free survival (EFS) rate, cumulative recurrence rate (CIR) , and transplant-related mortality (TRM) of patients with different bone marrow minimal residual disease (MRD) levels were analyzed before and after CAR T-cell infusion and before allo-HSCT.Results:The response rate of CAR T-cell therapy and the incidence rate of severe cytokine release syndrome were 92% and 28% , respectively. During 55 infusions, no treatment-related deaths occurred in any of the patients. The median time of CAR T-cell infusion to allo-HSCT was 54 (26-232) days, the median follow-up time after CAR T-cell infusion was 637 (117-1097) days, and the 1-year OS and EFS rates were (80.0±5.7) % and (60.0±6.9) % . The 1-year CIR and TRM after allo-HSCT were (28.0±0.4) % and (8.0±0.2) % . After CAR T-cell infusion and before allo-HSCT, patients with bone marrow MRD<0.01% had a significantly longer EFS [ (70.0±7.2) % vs (20.0±12.6) % , P<0.001; (66.7±7.5) % vs (36.4±14.5) % , P=0.008]and lower CIR [ (25.0±0.5) % vs (70.0±2.6) % , P<0.001; (23.08±0.47) % vs (45.45±2.60) % , P=0.038]. Conclusion:CAR T-cell therapy bridging allo-HSCT is safe and effective for recurrent and refractory B-ALL.

Objective:To summarize the clinical characteristics of an early death in patients with de novo acute promyelocytic leukemia (APL) , analyze the risk factors and direct causes of early death, and perform survival analysis.Methods:The clinical data of 368 patients with de novo APL in three centers (First Affiliated Hospital of Soochow University, Soochow Guangci Hospital, and Soochow Hopes Hospital of Hematology) during January 2011-December 2017 were retrospectively analyzed. The clinical characteristics of patients who suffered hemorrhagic early death and non-hemorrhagic early death were compared. The risk factors for early death, survival, and prognosis of patients with APL were analyzed.Results:Among the 368 de novo APL patients, 31 died early with an early mortality rate of 8.4%. The median time from diagnosis to death was 7 (0-29) d. On comparison of the clinical characteristics of patients with early death and non-early death and subsequent multivariate analysis using a logistic regression model, it was observed that age ≥50 years and WBC ≥10×10 9/L were independent risk factors for early death ( P<0.01) . A total of 27 (87.1%) of the 31 early deaths was directly attributed to hemorrhage as the immediate cause of early death. Hemorrhage was the only cause of death in patients <50 years old and the major cause of death in patients ≥50 years old. A comparison of the clinical characteristics of patients with hemorrhagic early death and patients with non-hemorrhagic early death suggested that the median age and indirect bilirubin concentration of patients with hemorrhagic early death were lower than those with non-hemorrhagic early death ( P<0.05) . The median follow-up time for all patients was 41.0 (0.3-101.4) months. The 2-year overall survival (OS) rate was (93.5±1.3) %, and the 5-year OS rate was (91.0±1.5) %. The 2-year disease-free survival (DFS) rate was (98.8±0.6) %, and the 5-year DFS rate was (97.1±0.9) %. The 2-year OS rate of patients ≥50 years old and patients <50 years old was 79.3% vs 94.2%, P=0.000; the 2-year DFS rate was 92.3% vs 98.1%, P=0.023. The respective 2-year OS rates of high-risk and non-high-risk patients were 77.3% and 96.7% ( P=0.000) and the respective 2-year DFS rates were 94.0% and 98.4% ( P=0.139) . Conclusion:Age and WBC are independent prognostic factors for early death. We observed a difference in early mortality between high-risk and low-risk APL, but no difference in DFS rate.

Objective: To compare the outcomes between haploidentical donor hematopoietic stem cell transplantation (haplo-HSCT) and matched-sibling donor transplantation (MSD-HSCT) for paroxysmal nocturnal hemoglobinuria (PNH) . Methods: The clinical data of 40 PNH patients received HSCT (haplo-HSCT=25, MSD-HSCT=15) from July 2007 to May 2018 were analyzed retrospectively to compare the outcomes between haplo-HSCT and MSD-HSCT groups. Results: There were no differences in terms of gender, age, patients of PNH-AA and median time from diagnosis to transplantation between the 2 groups (P>0.05) . The median values of absolute mononuclear cell counts and CD34⁺ cells infused were 10.74 (4.80-22.86) ×10⁸/kg and 12.19 (5.14-17.25) ×10⁸/kg (P=0.866) , 3.57 (0.68-7.80) ×10⁶/kg and 4.00 (3.02-8.42) ×10⁶/kg (P=0.151) respectively, in haplo-HSCT and MSD-HSCT groups. All patients attained complete engraftment, no patient occurred graft failure. The median durations for myeloid and platelet engraftment were 12 (range, 9-26) and 11 (range, 7-15) days (P=0.065) , 19 (range, 11-75) and 13 (range, 11-25) days (P=0.027) respectively, in haplo-HSCT and MSD-HSCT groups. During a median follow-up of 26 (4-65) months in haplo-HSCT and 36 (4-132) months in MSD-HSCT groups (P=0.294) , the incidences of grade Ⅰ-Ⅳ acute graft-versus-host disease (aGVHD) were 32.0% and 20.0% (P=0.343) , grade Ⅱ-Ⅳ aGVHD were 16.0%, 13.3% (P=0.759) , chronic GVHD were 30.7% and 24.6% (P=0.418) , moderate-severe chronic GVHD were 12.7% and 7.1% (P=0.522) respectively, in haplo-HSCT and MSD-HSCT groups. The incidences of infection were 32.0% (8/25) and 26.7% (4/15) (P=1.000) respectively, in haplo-HSCT and MSD-HSCT groups. No patients occurred early death and relapse. Three-year estimated overall survival (OS) were (86.5±7.3) % and (93.3 ±6.4) % (P=0.520) , GVHD-free and failure-free survival (GFFS) were (78.3±8.6) % and (92.9±6.9) % (P=0.250) respectively, in haplo-HSCT and MSD-HSCT groups. Conclusion The preliminary results indicated that haplo-HSCT was a feasible choice for PNH with favorable outcomes, haplo-HSCT and MSD-HSCT produced similar therapeutic efficacy.

Objective: To compare the outcomes of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for paroxysmal nocturnal hemoglobinuria (PNH) with paroxysmal nocturnal hemoglobinuria-aplastic anemia (PNH-AA) syndrome. Methods: The outcomes of 46 patients who received allo-HSCT (16 PNH patients, 30 PNH-AA patients) from July 10, 2007 to June 2, 2018 were analyzed retrospectively. The conditioning regimen was busulfan, cyclophosphoramide, and ATG in haploidentical donors and unrelated donors. Patients with matched sibling donors were treated with the fludarabine, cyclophosphamide, and ATG regimen. Results: There were no differences of baseline data between the 2 groups except gender distribution and the numbers of haploidentical donor transplantation. The median values of absolute nucleated cell counts were 10.58 (3.83-13.83) ×10⁸/kg in the PNH group and 10.81 (3.96-33.40) ×10⁸/kg in the PNH-AA group (P=0.668) . The median doses of CD34⁺ cells infused were 5.00 (3.14-8.42) ×10⁶/kg and 3.57 (1.97-6.17) ×10⁶/kg (P=0.002) , respectively. All patients obtained complete engraftment. The median time for myeloid engraftment were 11 (7-14) days in the PNH group and 12 (10-26) days in the PNH-AA group (P=0.003) . The median time for platelet engraftment were 13 (11-16) days and 18 (12-75) days (P=0.002) , respectively, after a median follow-up of 36 (4-132) months in the PNH group and 26 (4-75) months in the PNH-AA group (P=0.428) . There were no differences of incidence rates of acute graft-versus-host disease (aGVHD) , chronic GVHD and infection between PNH and PNH-AA groups (P>0.05) . No patient occurred early death and relapse. The estimated 3-year overall survival (OS) of PNH and PNH-AA groups were (100.0±0.0) % and (85.7± 6.6) % (P=0.141) , GVHD-free and failure-free survival (GFFS) were (100.0±0.0) %, (78.7±7.7) % (P=0.067) . Conclusions allo-HSCT is effective for patients with PNH and PNH-AA syndrome. The preliminary results indicate that myeloid and platelet engraftment in PNH group were faster than PNH-AA group. There were no differences in OS and GFFS between PNH group and PNH-AA group.