Objective To study the vestibular function in patients with sudden deafness .Methods Retrospec-tive analysis of 436 cases of patients with sudden deafness ,being divided into two groups according to whether the merger of vertigo .147 cases with vertigo group ,75 cases of which were mild to moderately severe hearing loss .In-clud the rise type of 28 cases ,26 cases of flat type ,21cases of decline type .72 cases of which were severe hearing loss .In 289 cases without vertigo ,157 cases had mild moderate and severe hearing loss .Includ the rise type of 36 cases ,57 cases of flat type ,64 cases of decline type .One hundred and thirty two cases of which were severe hearing loss .Use video electronystagmogram (VNG) to test the vestibular function .Process the data with SPSS 13 .0 soft-ware .Results One hundred and twenty three(83 .7% )cases of the vertigo group suffered from vestibular function decline ,with 51 cases of mild to moderately severe hearing loss ,72 cases of severe and above .165 (57 .1% )cases of the without vertigo group suffered from vestibular function decline .There were 45 cases of mild to moderately se-vere hearing loss ,120 cases of severe and above .35 patients with vertigo patients with BPPV ,28 cases of which happened within 1 week in sudden deafness occured and 80% of which were vestibular function decline .Conclusion Patients with sudden deafness with vertigo suffered from vestibular function damage .Patients with sudden deafness without vertigo also suffered from vestibular function damage .And the more serious the hearing loss ,the higher the vestibular function decline opportunities .

Objective:To analyze the competence assessment examinations of registered dietitians in China during 2017-2021 to inform the training of dietetic professionals.Methods:A systematic review was conducted about the composition and changing trends of examination participants during 2017-2021. Participants were divided into subgroups based on gender, age, education, professional background and affiliations and the qualification rate was compared across different subgroups.Results:The number of examination participants showed an increasing trend overall, with the majority being females and aged 26-35 years. The ratio of participants with bachelor's: master's: doctor's degree was about 15: 9: 1. The proportion of participants majoring in nutrition science decreased from 32.1% to 20.3%, while that of participants majoring in other medical and food sciences increased. Among participants from healthcare institutions, a decreasing proportion of participants were with a major in nutrition science, while this proportion among participants from companies were stable at 38.8% to 50.2%. The average qualification rate was 35.7%, showing no significant differences among different gender and age groups. But the qualification rate varied significantly across subgroups with different education level, professional background, and affiliations ( P < 0.05). Subgroups with more advanced degrees showed higher qualification rate and the best rate was observed in the subgroups with professional background in nutrition science and from nutrition-related institutions. Conclusion:The training of dietetic professionals in China should be improved, lifelong learning should be promoted in nutrition practitioners, and the sustainable development of dietitians should be impelled.

OBJECTIVE To explor e the characteristics of spectral oscillatory power of resting-state electroencephalography(EEG) in subjective tinnitus patients and to lay the foundation for study of central mechanism of tinnitus.METHODS 31 subjects(15 subjects with subjective tinnitus and 16 age matching healthy subjects) underwent a 128-channel resting-state EEG analyses. After a series of preprocessing, data were segmented into 8 frequency bands, including δ(0.5～3.5 Hz), θ(4～7.5 Hz), α1(8～10 Hz), α2(10～12 Hz), β1(13～18 Hz), β2(18.5～21 Hz), β3(21.5～30 Hz) and γ(30.5～44 Hz). The group differences of spectral power were analyzed by independent t test. Correlation between spectral power of each frequency band and tinnitus subjective symptoms were also analyzed. RESULTS Significant higher spectral power of the α1, β and γ bandwere found in the left and right temporal areas of tinnitus group compared with that of normal group.Tinnitus subjects also had higher spectral powerof the δ and θ band in temporo-parietal areas than that of the normal group. There was no significant difference of spectral power in other frequency bands. Moreover, signif icant positive cor relation were found between tinnitus loudness and spectralpower of right anterior lateral(R=0.66, P =0.007) and right anterior medial(R=0.58, P =0.031) areas. CONCLUSION Tinnitus subjects have higher spectral oscillatory power on right and left temporal lobe and temporo-parietal area. A positive correlation exsit between tinnitus loudness andspectral power of right anterior lateral and medial areas in tinnitus subjects, whichindicate that central reorganizationexsit in tinnitus reorganization andγ band maybe considered as a possible biomarkerforthe tinnitus subjective symptom.

Objective:To investigate the expression of Toll-like receptor 8 (TLR8) in diffuse large B-cell lymphoma (DLBCL) and its correlation with clinicopathological characteristics and prognosis of patients.Methods:The data in the Oncomine database was used to analyze the difference of TLR8 mRNA expression between DLBCL tumor tissues and normal lymphocytes, and the result was verified in two independent subsets GSE 25638 and GSE 32018 of the NCBI-GEO database. The OSDLBCL online survival analysis tool was used to analyze the correlation of TLR8 mRNA relative expression level with overall survival (OS) and progression-free survival (PFS) of DLBCL patients. Gene ontology bioprocess (GO_BP) enrichment analysis was performed by using GSEA software. The correlation of TLR8 mRNA expression with tumor immune cell infiltration degree and immune checkpoint-related molecule expression was analyzed by TIMER online tool website. A total of 53 DLBCL patients who underwent lymph node biopsy in Yancheng No. 1 People's Hospital from June 2020 to June 2021 were selected. Immunohistochemistry was used to detect the expression of TLR8 protein, and its relationship with the clinicopathological characteristics of patients was analyzed.Results:The analysis result of data from Oncomine and GEO databases showed that the relative expression levels of TLR8 mRNA in tumor tissues of patients with DLBCL or activated B cell-like DLCBL were higher than those in normal lymphocytes (all P < 0.001). The results of OSDLBCL online survival analysis indicated that the OS ( P = 0.020) and PFS ( P = 0.004) in DLBCL patients with high TLR8 mRNA expression were worse than those in patients with low TLR8 mRNA expression. The level of TLR8 was related to the abnormal function of immune response, cytokine metabolism and DNA damage monitoring; the result of TIMER online analysis showed that the expression level of TLR8 mRNA was positively related to the degree of neutrophil infiltration ( r = 0.78, P < 0.001) and the expression of immunosuppressive molecules [HAVCR2 ( r = 0.85, P < 0.001), LAG3 ( r = 0.63, P < 0.001), CD274 ( r = 0.77, P < 0.001), TIGIT ( r = 0.32, P = 0.037), and C10ORF54 ( r = 0.34, P = 0.029)]. Among 53 DLBCL patients, 29 patients (54.7%) had low expression of TLR8 protein and 24 patients (45.3%) had high expression of TLR8 protein. There were statistical differences in the expressions of TLR8 protein in DLBCL patients with different serum lactate dehydrogenase and β 2-microglobulin levels (both P < 0.05). Conclusions:TLR8 is highly expressed in DLBCL patients, and TLR8 may be a prognostic marker of DLBCL.

Objective:To investigate the familial inheritances, clinical features, treatments and outcomes of familial Waldenstrom macroglobulinemia (WM) patients.Methods:The clinical manifestations, laboratory examinations, diagnosis and treatments, and follow-up data of 6 familial WM patients who were admitted to Yancheng No.1 People's Hospital from June 2002 to July 2019 were retrospectively analyzed, and the literature was reviewed.Results:Among 6 WM patients, 4 patients had dizziness and fatigue at the onset, 1 patient had recurrent low-grade fever and abnormal sweating as the first manifestations, 1 patient was hospitalized due to pulmonary infection, and WM was found later. Two brothers of the patients were diagnosed with WM, another 2 brothers of the patients had IgM-type monoclonal gammopathy of undetermined significance (MGUS) during the physical examination. All the 6 patients were middle-aged/elderly men, with a median age of 63 years old (51-70 years old). The median follow-up time were 71.5 months (4-217 months), and by the end of the follow-up (June 2020), 2 cases died of pulmonary infection, and 1 of them developed acute myeloid leukemia; the other 4 cases were in regular chemotherapy. Two IgM-MGUS patients were followed up without symptoms.Conclusions:WM patients have familial aggregation, and their clinical manifestations are highly heterogeneous. Patients with family history may have poor prognosis. It is necessary to strengthen the awareness of WM and family history screening.

The incidence of tinnitus is very high, which can affect the patient's attention, emotion and sleep, and even cause serious psychological distress and suicidal tendency. Currently, there is no uniform and objective method for tinnitus detection and therapy, and the mechanism of tinnitus is still unclear. In this study, we first collected the resting state electroencephalogram (EEG) data of tinnitus patients and healthy subjects. Then the power spectrum topology diagrams were compared of in the band of δ (0.5-3 Hz), θ (4-7 Hz), α (8-13 Hz), β (14-30 Hz) and γ (31-50 Hz) to explore the central mechanism of tinnitus. A total of 16 tinnitus patients and 16 healthy subjects were recruited to participate in the experiment. The results of resting state EEG experiments found that the spectrum power value of tinnitus patients was higher than that of healthy subjects in all concerned frequency bands. The
Attention ; Brain ; Electroencephalography ; Humans ; Parietal Lobe ; Tinnitus

Objective:To investigate the clinicopathological features, diagnosis, differential diagnosis and treatment of nodal marginal zone lymphoma (NMZL) with elevated monoclonal IgM.Methods:The clinical data of one NMZL patient with elevated monoclonal IgM treated at Yancheng No.1 People's Hospital in July 2020 were retrospectively analyzed, and the related literature was analyzed.Results:The patient was a 57-year-old female and the main clinical manifestations were fatigue and bone pain in left rib. Serum immunofixation electrophoresis showed IgM-κ type M proteinemia, bone marrow cytology showed a few plasmacytoid lymphocytes, bone marrow biopsy and immunohistochemistry showed B-cell non-Hodgkin lymphoma, bone marrow genetic testing showed MYD88 L265p and CXCR4 were both negative, postoperative pathology result of retroperitoneal lymph node biopsy was marginal zone lymphoma (mature small B type, prone to NMZL),and immunohistochemistry results: CD3, CD5, CD138, κ, λ, CD10, Cyclin D1 were negative, CD20, Pax-5, CD23 (FDC), bcl-2 were positive; Ki-67 positive index < 5%. The final diagnosis was NMZL with elevated monoclonal IgM. Partial remission was achieved after 8 cycles of reduced-dose CHOP regimen; thalidomide was used in the maintenance treatment, the disease condition was stable until August in 2021 and the follow-up was continuing.Conclusions:NMZL with elevated monoclonal IgM is relatively rare. Its diagnosis should be differentiated from Waldenstr?m macroglobulinemia and other inert B-cell lymphomas. Currently, there is no standard treatment and following the principle of individualized treatment can improve the prognosis of patients.

Objective:To explore the risk factors of bortezomib-related peripheral neuropathy (BIPN) and the clinical and electrophysiological characteristics of patients in treatment of multiple myeloma (MM).Methods:The clinical data of 71 newly diagnosed MM patients treated with BD (bortezomib + dexamethasone) regimen in Yancheng First People's Hospital from March 2016 to December 2019 were retrospectively analyzed. The bone marrow morphology, immunology, cytogenetics, molecular biology (MICM), routine electrophysiological examination before and after treatment were performed. All patients were divided into the peripheral neuropathy (PN) group and the non-PN group according to the presence or not of BIPN, and the clinicopathological differences of both groups were also compared; a binary logistic regression model was used to analyze the factors affecting the occurrence of PN. The electrophysiological characteristics were summarized and fluorescence in situ hybridization (FISH) was used to detect karyotype of BIPN patients.Results:Among 71 MM patients, there were 40 cases (56.3%) of PN and 31 cases (43.7%) of non-PN. The proportion of patients at international staging system (ISS) staging Ⅲ, and the levels of IgA, IgG, IgM, serum creatinine, β 2-microglobulin (β 2-MG) in the PN group were higher than those in the non-PN group, and hemoglobin (Hb) level in the PN group was lower than that in the non-PN group, and the differences were statistically significant (both P < 0.05). Binary logistic regression analysis showed that increased IgA ( OR = 1.151, 95% CI 1.012-1.309, P = 0.033), increased IgG ( OR = 1.055, 95% CI 1.000~1.112, P = 0.049), increased IgM ( OR = 1.010, 95% CI 1.001-1.018, P = 0.022), increased serum creatinine ( OR = 1.037, 95% CI 1.011~1.065, P = 0.005), increased β 2-MG ( OR = 1.564, 95% CI 1.039-2.354, P = 0.032) were risk factors for BIPN. Among 40 patients with BIPN, 33 cases (82.5%) of sensory nerve conduction velocity (SCV) were abnormal, 23 cases (57.5%) of motor nerve conduction velocity (MCV) were abnormal; 31 cases (77.5%) showed demyelination damage, 9 cases (22.5%) had axonal damage. Among 40 patients with BIPN, 24 cases underwent FISH detection, including 19 cases (79.2%) with chromosomal mutations, of which 12 cases (50.0%) were mixed subtype abnormal. Conclusions:MM patients with high levels of β 2-MG, IgA, IgG, IgM and serum creatinine are more prone to PN when treated with bortezomib. The electrophysiology of patients with BIPN is mainly characterized by demyelination of sensory nerves.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disease caused by abnormal expression of glycosylphosphatidylinositol (GPI) on the cell membrane due to mutations in the phosphatidylinositol glycan class A(PIGA) gene. It is commonly characterized by intravascular hemolysis, repeated thrombosis, and bone marrow failure, as well as multiple systemic involvement symptoms such as renal dysfunction, pulmonary hypertension, swallowing difficulties, chest pain, abdominal pain, and erectile dysfunction. Due to the rarity of PNH and its strong heterogeneity in clinical manifestations, multidisciplinary collaboration is often required for diagnosis and treatment. Peking Union Medical College Hospital, relying on the rare disease diagnosis and treatment platform, has invited multidisciplinary clinical experts to form a unified opinion on the diagnosis and treatment of PNH, and formulated the Expert Consensus of Multidisciplinary Diagnosis and Treatment for Paroxysmal Nocturnal Hemoglobinuria (2024), with the hope of promoting the standardization of PNH diagnosis and treatment.