Objective:To analyze the incidence and impact on the auditory prognosis of vertigo/dizziness in sudden sensorineural hearing loss patients with moderately severe hearing loss and above. Methods:Clinical data of patients with unilateral sudden sensorineural hearing loss hospitalized from January 2008 to December 2022, aged 18-60 years, PTA≥50 dB HL, and within 14 days of onset were selected. Based on the clinical records of sudden sensorineural hearing loss patients, we determined whether they were accompanied by vertigo/dizziness. The degree of hearing loss is referenced to the 2021 WHO grading criteria and divided into the moderately severe, severe, profound, and total deafness groups. The SPSS 22.0 software was applied to analyze the difference in the auditory prognosis between sudden sensorineural hearing loss patients with moderately severe hearing loss and above who have dizziness/vertigo and those who do not. Results:A total of 697 patients with moderately severe hearing loss and above were collected, including 382 males and 315 females, with an average age of（40.8±11.0） years. The proportions of sudden sensorineural hearing loss patients with dizziness/vertigo among those with moderately severe to total deafness hearing loss were 18.4%, 35.7%, 47.9%, and 76.4% respectively. Compared to the moderately severe, severe, profound, and total deafness groups, the difference was statistically significant（P<0.001）. The complete recovery rates of sudden sensorineural hearing loss patients with moderately severe to total deafness hearing loss were 28.2%, 25.2%, 18.2%, and 1.9% respectively, and the total effective rates were 72.8%, 83.5%, 86.7%, and 78.0% respectively. There were statistically significant differences in complete recovery rate（P<0.001）, significant efficiency rate（P<0.001）, effective rate（P=0.026）, and no recovery rate（P=0.022） among the moderately severe, severe, profound, and total deafness groups. The differences in complete recovery between sudden sensorineural hearing loss patients with and without vertigo/dizziness were statistically significant in the moderately severe, severe, profound, and total deafness groups（P<0.05）, while the total effective rates were only statistically significant in the profound group compared to those without vertigo/dizziness（P<0.05）. After inpatient treatment, sudden sensorineural hearing loss patients with dizziness/vertigo had statistically significant final hearing thresholds at 4 000 and 8 000 Hz for moderately severe hearing loss patients, 2 000-8 000 Hz for severe hearing loss patients, 500-8 000 Hz for profound hearing loss patients, and 2 000-8 000 Hz for total deafness patients compared to those without dizziness/vertigo（P<0.05）. Conclusion:The higher the degree of hearing loss in patients with moderately severe hearing loss and above, the higher the proportion of accompanied vertigo/dizziness. Patients with vertigo/dizziness have poorer recovery of high-frequency hearing, and the complete recovery rate is significantly lower than the patients without vertigo/dizziness.
Humans ; Male ; Female ; Hearing Loss, Sudden/complications* ; Adult ; Dizziness/complications* ; Prognosis ; Middle Aged ; Hearing Loss, Sensorineural/complications* ; Vertigo/complications* ; Young Adult ; Adolescent

Objective:To investigate the differential impact of ultra-high-resolution photon-counting detector CT (UHR PCD-CT) and energy-integrating detector CT (EID-CT) on image quality and calcified plaque-induced luminal stenosis in coronary CT angiography (CCTA).Methods:This retrospective analysis was conducted on patients who underwent both EID-CT and UHR PCD-CT CCTA at the Geriatric Hospital of Nanjing Medical University between January 2021 and November 2024. A total of 141 patients were included in the study, within 46 patients having scans within a 12-month interval. Image quality of all coronary artery segments was subjectively evaluated. Patients with paired scans (interval≤12 months) were included for calcified plaque analysis. Subjective visualization of calcified plaques evaluated. The blooming artifact was calculated as an objective evaluation index for assessing the calcified plaques. Additionally, the degree of coronary artery lumen stenosis resulting from calcified plaques was assessed, along with the measurement of plaque volume and the Agatston score. Changes in lumen stenosis between the two scans were also evaluated. The Wilcoxon signed-rank test was used to compare the subjective scores of coronary artery image quality and calcified plaques between the two groups, and paired-sample t-tests were used to compare the blooming artifact and lumen stenosis degree. Results:The PCD-CT image quality score was significantly higher than that of EID-CT [PCD-CT : 5 (4,5), EID-CT: 4 (4,5); Z=-21.38, P<0.001]. Compared to EID-CT, PCD-CT reduced the blooming artifact (PCD-CT: 38.88%±9.09%, EID-CT: 50.11%±11.52%; t=-12.97, P<0.001), significantly improving the subjective score for visualization of calcified plaques [PCD-CT: 5 (4,5), EID-CT: 3 (2,3); Z=-9.68, P<0.001], and the measured lumen stenosis was notably lower in PCD-CT(PCD-CT:34.88%±18.20%, EID-CT:45.31%±23.42%; t=-9.93, P<0.001). Among 129 analyzed calcified plaques, luminal stenosis was reduced on PCD-CT in 110 plaques (85.3%) and increased in 19 (14.7%), including 4 plaques that had unclear boundaries with the adjacent lumen in EID-CT CCTA images, making the stenosis difficult to assess. Conclusion:Compared to EID-CT, UHR PCD-CT for CCTA significantly improves coronary artery image quality, provides clearer visualization of calcified plaques and adjacent lumen details, and it can reduce the overestimation of coronary artery caleified plaque stenosis.

Objective:To investigate the differential impact of ultra-high-resolution photon-counting detector CT (UHR PCD-CT) and energy-integrating detector CT (EID-CT) on image quality and calcified plaque-induced luminal stenosis in coronary CT angiography (CCTA).Methods:This retrospective analysis was conducted on patients who underwent both EID-CT and UHR PCD-CT CCTA at the Geriatric Hospital of Nanjing Medical University between January 2021 and November 2024. A total of 141 patients were included in the study, within 46 patients having scans within a 12-month interval. Image quality of all coronary artery segments was subjectively evaluated. Patients with paired scans (interval≤12 months) were included for calcified plaque analysis. Subjective visualization of calcified plaques evaluated. The blooming artifact was calculated as an objective evaluation index for assessing the calcified plaques. Additionally, the degree of coronary artery lumen stenosis resulting from calcified plaques was assessed, along with the measurement of plaque volume and the Agatston score. Changes in lumen stenosis between the two scans were also evaluated. The Wilcoxon signed-rank test was used to compare the subjective scores of coronary artery image quality and calcified plaques between the two groups, and paired-sample t-tests were used to compare the blooming artifact and lumen stenosis degree. Results:The PCD-CT image quality score was significantly higher than that of EID-CT [PCD-CT : 5 (4,5), EID-CT: 4 (4,5); Z=-21.38, P<0.001]. Compared to EID-CT, PCD-CT reduced the blooming artifact (PCD-CT: 38.88%±9.09%, EID-CT: 50.11%±11.52%; t=-12.97, P<0.001), significantly improving the subjective score for visualization of calcified plaques [PCD-CT: 5 (4,5), EID-CT: 3 (2,3); Z=-9.68, P<0.001], and the measured lumen stenosis was notably lower in PCD-CT(PCD-CT:34.88%±18.20%, EID-CT:45.31%±23.42%; t=-9.93, P<0.001). Among 129 analyzed calcified plaques, luminal stenosis was reduced on PCD-CT in 110 plaques (85.3%) and increased in 19 (14.7%), including 4 plaques that had unclear boundaries with the adjacent lumen in EID-CT CCTA images, making the stenosis difficult to assess. Conclusion:Compared to EID-CT, UHR PCD-CT for CCTA significantly improves coronary artery image quality, provides clearer visualization of calcified plaques and adjacent lumen details, and it can reduce the overestimation of coronary artery caleified plaque stenosis.

CAPOS syndrome is an autosomal dominant neurological disorder caused by mutations in the ATP1A3 gene. Initial symptoms, often fever-induced, include recurrent acute ataxic encephalopathy in childhood, featuring cerebellar ataxia, optic atrophy, areflflexia, sensorineural hearing loss, and in some cases, pes cavus. This report details a case of CAPOS syndrome resulting from a maternal ATP1A3 gene mutation. Both the child and her mother exhibited symptoms post-febrile induction,including severe sensorineural hearing loss in both ears, ataxia, areflexia, and decreased vision. Additionally, the patient's mother presented with pes cavus. Genetic testing revealed a c. 2452G>A（Glu818Lys） heterozygous mutation in theATP1A3 gene in the patient . This article aims to enhance clinicians' understanding of CAPOS syndrome, emphasizing the case's clinical characteristics, diagnostic process, treatment, and its correlation with genotypeic findings.
Humans ; Child ; Female ; Cerebellar Ataxia/diagnosis* ; Talipes Cavus ; Hearing Loss, Sensorineural/diagnosis* ; Optic Atrophy/diagnosis* ; Mutation ; Phenotype ; Sodium-Potassium-Exchanging ATPase/genetics* ; Foot Deformities, Congenital ; Reflex, Abnormal

Objective:To investigate the effect of subacute exposure of Di(2-ethylhexyl)phthalate(DEHP)on endometrial decidualization and early pregnancy miscarriage in mice.Methods:CD1 mice were orally administrated with 300(low-dose group),1000(medium-dose group),or 3000 mg·kg-1·d-1 DEHP(1/10 LD50,high-dose group)for 28 days,respectively.An early natural pregnancy model and an artificially induced decidualization model were established.The uterine tissues were collected on D7 of natural pregnancy and D8 of artificially induced decidualization,respectively.The effects of a subacute exposure to DEHP on the decidualization of mice were detected by HE staining,Masson staining,TUNEL assay,and Western blotting.A model of spontaneous abortion was constructed in mice after subacute exposure to 300 mg·kg-1·d-1 DEHP,and the effect of impaired decidualization on pregnancy was investigated by observing the pregnancy outcome on the 10th day of gestation.Results:Compared with the control group,the conception rate was significantly decreased in the high-dose DEHP subacute exposure group(P＜0.05).HE staining showed that,compared with the control group,the decidual stromal cells in the low-and medium-dose exposure groups were disorganized,the nuclei of the cells were irregular,the cytoplasmic staining was uneven,and the number of polymorphonuclear cells was significantly reduced.Masson staining showed that compared with the control group,the collagen fibers in the decidua region of the DEHP low-dose group and the medium-dose group were more distributed,more abundant and more disorderly.TUNEL assay showed increased apoptosis in the decidua area compared to the control group.Western blotting showed that the expression of BMP2,a marker molecule for endometrial decidualization,was significantly reduced(P＜0.05 or P＜0.01).The abortion rate and embryo resorption rate were increased,and the number of embryos,uterine wet weight,uterine area and placenta wet weight were decreased in DEHP low-dose group compared to the control group stimulated by mifepristone,an abortifacient drug(P＜0.05 or P＜0.01).Conclusion:Subacute exposure to DEHP leads to impaired endometrial decidualization during early pregnancy and exacerbates the risk of adverse pregnancy outcomes in mice.

Objective:To compare the safety of radial artery puncture in elderly patients aged 75 years and older who are undergoing neurointerventional procedures.Methods:A single-center retrospective study was conducted, involving 350 elderly patients aged 75 years and older who received neurointerventional treatment at Beijing Tiantan Hospital, Capital Medical University, from June to December 2022.The participants were divided into two groups based on the puncture site: femoral artery puncture and radial artery puncture.The safety indicators compared between the two groups included puncture failure, changes in puncture site, general puncture complications(such as subcutaneous bleeding, puncture site hematoma, and vasospasm), severe puncture complications(including distal limb ischemia and pseudoaneurysm), and lower limb venous thrombosis.Multivariate Logistic regression analysis was conducted to evaluate the impact of different puncture methods on the occurrence of complications.Results:Among the 350 patients, 280 underwent femoral artery puncture, while 70 underwent radial artery puncture.There were no statistically significant differences in baseline characteristics between the two groups(all P>0.05).The proportions of patients using antiplatelet drugs prior to surgery, puncture failure rates, rates of change in puncture sites, and the incidence of severe complications-including distal limb ischemia and pseudoaneurysm-were not significantly different between the two groups( χ2=2.051, 0.075, 0.588, 3.175; P=0.152, 0.784, 0.443, 0.075).In the femoral artery puncture group, 20.4%(57 cases)of patients experienced general puncture complications(including subcutaneous bleeding, puncture site hematoma, and vasospasm), whereas only 8.6%(6 cases)in the radial artery puncture group experienced such complications, revealing a statistically significant difference between the two groups( χ2=5.720, P=0.022).Multivariate Logistic regression analysis indicated that, compared to femoral artery puncture, radial artery puncture was associated with a reduced risk of all complications( OR=0.272, 95% CI: 0.139-0.532, P<0.001), general puncture complications( OR=0.375, 95% CI: 0.153-0.919, P=0.032)and lower limb venous thrombosis( OR=0.219, 95% CI: 0.050-0.954, P=0.043). Conclusions:In elderly patients aged 75 years and older who are undergoing neurointerventional procedures, radial artery puncture is associated with a reduced incidence of general puncture complications and lower limb venous thrombosis when compared to femoral artery puncture, indicating a superior safety profile.

Objective:To investigate the clinical effect of CAG stimulating regimen for refractory adult early T cell precursor acute lymphoblastic leukemia (ETP-ALL) complicated with fusarium infection and the clinical features as well as antifungal strategy of cutaneous fusarium infection.Methods:The diagnosis and treatment of 1 adult patient diagnosed as ETP-ALL complicated with cutaneous fusarium infection in the First Hospital of Jilin University in September 2020 were retrospectively analyzed, and related literatures were reviewed.Results:VICP chemotherapy regimen showed no effectiveness in this patient who was presented with persistent agranulocytosis complicated with cutaneous fusariosis infection. After amphotericin B therapy for infection, he achieved the stable disease and successfully underwent CAG stimulating regimen salvage treatment. The minimal residual disease turned into negative after consolidation chemotherapy based on the myeloid regimen. Finally this patient survived from haploid allogeneic hematopoietic stem cell transplantation after consolidation chemotherapy and fusarium was under the control by using posaconazole as secondary prevention therapy.Conclusions:CAG stimulating regimen can be recommended as reinduction therapy for relapsed/refractory ETP-ALL. Sequential therapy of amphotericin B followed by posaconazole can be a useful antifungal strategy for fusarium infection.

Objective:To investigate the distribution of pathogenic bacteria of bloodstream infection after chemotherapy in patients with acute leukemia (AL), to analyze the risk factors for the occurrence of adverse events and to construct a nomogram model to predict the occurrence of adverse events.Methods:The clinical data of 313 AL patients with bloodstream infection who were admitted to the First Hospital of Jilin University from January 2018 to December 2020 were retrospectively analyzed, and the incidence, fatality and distribution characteristics of pathogenic bacteria after chemotherapy in AL patients were analyzed; the occurrence of adverse events (death or infectious shock) in patients with different clinicopathological characteristics were compared. Unconditional logistic binary regression model multifactor analysis was used to screen independent risk factors for the occurrence of adverse events in AL patients with bloodstream infection after chemotherapy; the nomogram model for predicting the occurrence of adverse events was developed by using R software; the Hosmer-Lemeshow test was used to verify the predictive effect of the model.Results:Of the 313 AL patients, the overall fatality rate was 4.2% (13/313), the all-cause fatality rate of bloodstream infection was 3.5% (11/313). Of the 313 cases, 254 cases (81.1%) were Gram-negative bacteria infection, mainly including 115 cases (45.3%) of Escherichia coli, 80 cases (31.5%) of Klebsiella pneumoniae, and 29 cases (11.4%) of Pseudomonas aeruginosa, and 10 cases (3.9%) died; 51 cases (16.3%) were Gram-positive cocci infection, mainly including 22 cases (43.1%) of Streptococcus spp., 20 cases (39.2%) of Staphylococcus spp., 7 cases (13.7%) of Enterococcus faecalis, and 0 case died; 8 cases (2.6%) were fungal infection, including 4 cases (1.3%) of Candida tropicalis, 2 cases (0.6%) of Candida subsmoothis, 1 case (0.3%) of Candida smooth, 1 case (0.3%) of new Cryptococcus, and 3 cases (37.5%) died. The differences in the occurrence rates of adverse events were statistically significant when comparing different treatment stage, risk stratification, timing of sensitive antibiotic use, total duration of fever, and glucocorticoid use in chemotherapy regimen, infecting bacteria carbapenem resistance, and leukemia remission (all P < 0.05). The results of logistic binary regression analysis showed that the use of glucocorticoid in chemotherapy regimen, the total duration of fever ≥7 d, the timing of sensitive antibiotic use ≥24 h, and carbapenem resistance of the infecting bacteria were independent risk factors for the occurrence of adverse events in AL patients with bloodstream infection after chemotherapy (all P < 0.05). A nomogram prediction model for the occurrence of adverse events in AL patients with bloodstream infection was established, and the nomogram model was calibrated and validated with good calibration and discrimination. Conclusions:The pathogenic bacteria of bloodstream infection after chemotherapy in AL patients is mainly Gram-negative bacteria, and the presence of glucocorticoid in chemotherapy regimen, long total duration of fever, poor timing of sensitive antibiotics, and infecting bacteria carbapenem resistance are risk factors for the occurrence of adverse events in AL patients with bloodstream infection after chemotherapy, and the nomogram prediction model based on these factors has a reliable predictive ability for the occurrence of adverse events.

Objective:To analyze the genetic counseling characteristics and interpretation of pathogenic variants for mitochondrial hearing loss.Methods:We analyzed a total of 513 unrelated families from Chinese Deafness Genome Project (CDGP), in which previous gene testing had found no pathogenic mutations with hearing loss (HL) related to the nuclear genes. We used targeted testing and complete mtDNA sequencing for the families′ available members.Results:Among the first individuals of the families to be tested, 20 cases (16 probands and 4 normal hearing consultants) were discovered with variants in mtDNA, including m.1095T>C, m.1310C>T, m.1494C>T and m.1555A>G in MT-RNR1, m.7445A>G, m.7505T>C, m.7510T>C and m.7511T>C in MT-TS1, and m.3243A>G in MT-TL1. We identified MT-RNR1 and MT-TS1 variants occurred as homoplasmic changes, while MT-TL1 variants occurred as heteroplasmic changes. Most mitochondrial hearing loss were characterized by slope or flat moderate to profound sensorineural HL. HL associated with the mtDNA pathogenic variant was variable onset age, severity and audiometric configuration. The penetrance for HL in individuals with the m.1095T>C variant might be low. Progression in the severity of HL was caused by the m.7445A>G pathogenic variant and the patients with m.7505T>C had cookie bite HL. MT-TL1 m.3243A>G was a common spot for pathogenic variants associated with nonsyndromic HL as well as syndromic HL with diabetes mellitus. Conclusions:Individuals with the mtDNA variants and their maternal relatives have a higher risk of HL and phenotypic heterogeneity in the age of onset, progression, and level of HL. The medical history should include review of any audiological testing of the probands and their family members on a maternal mtDNA background. The process of genetic evaluation needs rule out nuclear gene mutations and analyzes the genetic load of mitochondrial pathogenic variants in the probands or consultants. In these cases, we rely on clinical and genetic evaluation molecular analysis to appropriately counsel families for whom an exact cause of HL and to help them make informed medical decisions.

Objective:This investigation aims to assess the impact of CSF3R mutations and the presence of measurable residual disease (MRD) on the prognosis of patients with CEBPA double mutations who have acute myeloid leukemia (AML) .Methods:The prognostic significance of these two factors was examined in the present study, which included 66 patients with complete genetic mutations and sequential MRD information.Results:Following the second course of chemotherapy, the MRD status and CSF3R mutations of these patients were linked to their long-term prognosis. CSF3R mutated patients showed inferior relapse-free survival (RFS) (5-year RFS: 15.2% vs 38.7% , P=0.006) and overall survival (OS) (5-year OS: 18.2% vs 60.6% , P=0.038) compared with those with wild-type CSF3R. After the second course of chemotherapy, patients with negative MRD had an RFS of 64 months and an OS of not reaching, which was significantly longer than that of patients with positive MRD (15 and 48 months, and the P value were 0.004 and 0.050, respectively) . CSF3R mutations ( HR=0.317, 95% CI 0.129-0.779, P=0.012) , WT1 mutations ( HR=0.304, 95% CI 0.115-0.804, P=0.016) , and NRAS mutations ( HR=0.153, 95% CI 0.061-0.385, P<0.001) were all independently associated with a poor prognosis for RFS, and CSF3R mutations and positive MRD tended to be independently associated with a poor prognosis for OS, according to the results of a Cox proportional-hazards model analysis ( P values were 0.071 and 0.088, respectively) . The patients were divided into three groups based on their CSF3R mutation status and MRD status following treatment: wide-type CSF3R and negative MRD, mutated CSF3R or positive MRD, and mutated CSF3R and positive MRD, which showed significantly different RFS ( P<0.001) and OS ( P=0.006) . Conclusion:Both CSF3R mutations and positive MRD were associated with poor outcome in AML patients with CEBPA double mutations. An integrity model based on these two factors may be beneficial for accurately evaluating the prognosis of these patients.