Thrombocytopenia 2 (THC2) is an autosomal dominant hematologic disorder caused by germline mutations in the ANKRD26 gene, characterized primarily by persistent thrombocytopenia and a predisposition to myeloid neoplasms. Owing to nonspecific clinical presentation and limited disease awareness, THC2 is frequently underdiagnosed or misdiagnosed, potentially leading to inappropriate interventions. This article systematically outlines the clinical manifestations, pathogenesis, and strategies for the precise diagnosis and treatment of THC2, aiming to provide a theoretical basis and practical guidance for its clinical management and for the in-depth investigation of its pathogenesis.

OBJECTIVE To evaluate cerebrospinal fluid(CSF)flow dynamics and volume changes of pulsatile tinnitus(PT)patients caused by sigmoid sinus wall dehiscence(SSWD)with different intracranial pressure via MRI.METHODS Prospective enrolled 35 SSWD-PT patients with intracranial hypertension,25 SSWD-PT patients with normal intracranial pressure and 35 age-,sex-matched healthy controls.Demographic characteristics were recorded.Intracranial pressure was assessed by the index of transverse sinus stenosis(ITSS)and morphology changes.CSF flow dynamics were evaluated via phase-contrast magnetic resonance imaging(PC-MRI)and CSF volume were evaluated via three-dimensional T1-weighted turbo field echo(3D T1-TFE)sequence and ITK-SNAP software.Compared the differences of each index between three groups.RESULTS The mean flux and regurgitant fraction were significantly different among the three groups(P<0.05).The intracranial hypertension group presented significantly decreased mean flux(MF)and significantly increased regurgitant fraction(RF)compared to controls(P<0.017).There were no significant differences in MF and RF of normal intracranial pressure group compared with intracranial hypertension group and control group(P>0.017).There were no statistical differences in age,sex,body mass index,forward flow volume,backward flow volume,mean velocity,peak velocity,stroke volume and CSF volume(P>0.05).CONCLUSION SSW D-PT patients have abnormal changes in CSF,and those with increased intracranial pressure are more obvious.These changes may be associated with abnormal hemodynamics in the sigmoid sinus and the occurrence of PT.

The sound generation and conduction of pulsatile tinnitus(PT)caused by sigmoid sinus wall dehiscence(SSWD)are closely related to abnormal blood flow and intracranial pressure,sigmoid plate and temporal bone air cells.Clarifying the mechanism of tinnitus is the premise of targeted treatment.This article reviews the research progress on the mechanism of SSWD-PT.

OBJECTIVE To observe the changes of static functional connectivity(sFC) and dynamic functional connectivity(dFC) of amygdala subregions in patients with bilateral idiopathic tinnitus and hearing loss(TINHL). METHODS The resting-state functional magnetic resonance imaging(fMRI) data of 30 patients with bilateral tinnitus and hearing loss and 37 normal controls(NCs) were collected to analyze the intensity changes of sFC and dFC in 8 subregions of amygdala and the whole brain in TINHL patients. RESULTS There were no significant differences in age,sex and education between the two groups. Compared with the NCs group,the sFC intensity of the right basolateral subregion and right cerebellar peduncle 1 region,the left basolateral subregion and left orbital medial frontal gyrus and left angular gyrus in TINHL group was significantly decreased. The dFC intensity of left amygdalostriatal subregion and left precuneus in TINHL group was increased,but the dFC intensity was reduced in the left basolateral subregion and right angular gyrus as well as between the right superficial subregion and right medial orbital of superior frontal gyrus. CONCLUSION Among the 8 subregions of amygdala,the bilateral basolateral subregions,the left amygdalostriatal subregion and the right superficial region shown abnormal functional connectivity with other regions of the brain,which are the important parts of emotional abnormalities in TINHL.

OBJECTIVE To analyze the clinical characteristics of pulsatile tinnitus induced by sigmoid sinus abnormalities in high and normal intracranial pressure states.METHODS There were 66 patients of pulsatile tinnitus induced by sigmoid sinus abnormalities,including 55 cases of sigmoid sinus wall dehiscence and 11 cases of sigmoid sinus diverticulum.The index of transverse sinus stenosis(ITSS)was used to assess intracranial pressure in magnetic resonance venography(MRV).We obtained 41 cases in intracranial hypertension group and 25 cases in normal intracranial pressure group.The age,gender,handedness,tinnitus lateralization,mean arterial pressure(MAP),body mass index(BMI),tinnitus duration,tinnitus frequency,tinnitus loudness,tinnitus handicap inventory(THI)and blood biochemical examination were recorded to analyze.RESULTS The BMI was 24.98 kg/m2(22.87 kg/m2,28.46 kg/m2)and 24.01 kg/m2(20.34 kg/m2,25.03 kg/m2)and THI score was 45.59±23.47 and 33.84±20.13 in intracranial hypertension group and normal intracranial pressure group,respectively.Compared with normal intracranial pressure group,the BMI and THI of intracranial hypertension group were significantly increased(P was 0.047 and 0.042 respectively).No significant difference were found in other indicators.CONCLUSION There are some different characteristics in pulsatile tinnitus induced by sigmoid sinus abnormalities in high and normal intracranial pressure states,which manifest obviously increased BMI and THI score in pulsatile tinnitus patients with intracranial hypertension.

To provide basis for prevention and treatment by analyzing the clinical features, emotional and cognitive states and their correlations of idiopathic tinnitus. Cross-sectional study was used. Thirty-six right, 44 left, and 46 bilateral idiopathic tinnitus patients diagnosed in Beijing Tongren Hospital were prospectively enrolled from October, 2020 to February, 2022. The clinical data was recorded and the THI, DBI, STAI, and MoCA were evaluated. The clinical features and the incidence of severe tinnitus, hearing lose, anxiety, and cognitive impairment were compared by one-way ANOVA, Kruskal-Wallis H, and chi-square test. The correlation between tinnitus or hearing and emotional and cognitive states were evaluated by multivariable correlation analysis. There was no significant difference in age, BMI, years of education, tinnitus duration, and the incidence of hearing loss among groups ( F=0.730, P=0.484; F=1.535, P=0.219; F=1.506, P=0.226;χ2=4.242, P=0.120;χ2=6.672, P=0.083). In right, left, and bilateral tinnitus patients, the number of severe tinnitus was 12, 7, and 20 cases and the incidence was 33.3%, 15.9%, and 43.5%; the number of depression was 13, 14, and 26 cases and incidence was 36.1%, 31.8%, and 53.5%; the number of trait anxiety was 3, 2, and 10 cases and the incidence was 8.3%, 4.5%, and 21.7%. Compared with left tinnitus patients, the incidence of severe tinnitus, depression, and trait anxiety was higher in bilateral tinnitus patients (χ2=8.139, P=0.004;χ2=5.558, P=0.018;χ2=5.753, P=0.007). The incidence of state anxiety and cognitive impairment were no significant difference among groups (χ2=0.142, P=0.931;χ2=1.338, P=0.512). The overall incidence of state anxiety and cognitive impairment were 16.7%(21/126) and 37.3%(47/126) respectively. There was positive correlation between THI score and BDI, S-AI, and T-AI scores ( r=0.529, P=0.001; r=0.649, P<0.001; r=0.483, P=0.003) and negative correlation between THI and MoCA scores ( r=-0.364, P=0.029) in right tinnitus group. The positive correlation was found between THI score and BDI, S-AI, and T-AI scores in left tinnitus group ( r=0.508, P<0.001; r=0.506, P<0.001; r=0.357, P=0.017). The positive correlation between THI score and BDI, S-AI, and T-AI scores ( r=0.753, P<0.001; r=0.527, P<0.001; r=0.536, P<0.001) and the positive correlation between tinnitus duration and MoCA score( r=0.334, P=0.023) were also found in bilateral tinnitus group.

To provide basis for prevention and treatment by analyzing the clinical features, emotional and cognitive states and their correlations of idiopathic tinnitus. Cross-sectional study was used. Thirty-six right, 44 left, and 46 bilateral idiopathic tinnitus patients diagnosed in Beijing Tongren Hospital were prospectively enrolled from October, 2020 to February, 2022. The clinical data was recorded and the THI, DBI, STAI, and MoCA were evaluated. The clinical features and the incidence of severe tinnitus, hearing lose, anxiety, and cognitive impairment were compared by one-way ANOVA, Kruskal-Wallis H, and chi-square test. The correlation between tinnitus or hearing and emotional and cognitive states were evaluated by multivariable correlation analysis. There was no significant difference in age, BMI, years of education, tinnitus duration, and the incidence of hearing loss among groups ( F=0.730, P=0.484; F=1.535, P=0.219; F=1.506, P=0.226;χ2=4.242, P=0.120;χ2=6.672, P=0.083). In right, left, and bilateral tinnitus patients, the number of severe tinnitus was 12, 7, and 20 cases and the incidence was 33.3%, 15.9%, and 43.5%; the number of depression was 13, 14, and 26 cases and incidence was 36.1%, 31.8%, and 53.5%; the number of trait anxiety was 3, 2, and 10 cases and the incidence was 8.3%, 4.5%, and 21.7%. Compared with left tinnitus patients, the incidence of severe tinnitus, depression, and trait anxiety was higher in bilateral tinnitus patients (χ2=8.139, P=0.004;χ2=5.558, P=0.018;χ2=5.753, P=0.007). The incidence of state anxiety and cognitive impairment were no significant difference among groups (χ2=0.142, P=0.931;χ2=1.338, P=0.512). The overall incidence of state anxiety and cognitive impairment were 16.7%(21/126) and 37.3%(47/126) respectively. There was positive correlation between THI score and BDI, S-AI, and T-AI scores ( r=0.529, P=0.001; r=0.649, P<0.001; r=0.483, P=0.003) and negative correlation between THI and MoCA scores ( r=-0.364, P=0.029) in right tinnitus group. The positive correlation was found between THI score and BDI, S-AI, and T-AI scores in left tinnitus group ( r=0.508, P<0.001; r=0.506, P<0.001; r=0.357, P=0.017). The positive correlation between THI score and BDI, S-AI, and T-AI scores ( r=0.753, P<0.001; r=0.527, P<0.001; r=0.536, P<0.001) and the positive correlation between tinnitus duration and MoCA score( r=0.334, P=0.023) were also found in bilateral tinnitus group.

Objective To investigate the effect of endovascular treatment to initial and long-term outcomes of postpartum deep venous thrombosis (DVT) . Methods The clinical data, venography and 3-yaer follow up data of 30 female patients with acute or subacute deep vein thrombosis in the postpartum period who received endovascular therapy consisting of catheter-directed thrombolysis with angioplasty (stenting for some patients with iliac vein compression syndrome (IVCS)) were retrospectively evaluated. Clot removal was graded as grade Ⅲ(>95%), grade Ⅱ(50% to 95%), and grade Ⅰ(<50%). Grade Ⅱ, Ⅲconsidered effective. Data from onset time, vaginal delivery or by elective, patients ages, IVCS or not and stenting or not were included in this analysis. And the χ2 test and survival analysis was used for statistical analysis of initial and long-term patency respectively. Results In 30 cases, the initial result included 14 cases(46.7%) of gradeⅢ, 12 cases(40.0%) of gradeⅡand 4 cases(13.3%) of gradeⅠ. Patients' onset time, vaginal delivery or by elective, patients ages and IVCS or not have no significant statistical difference to initial patency(χ2=0.88, 1.28, 1.15, 3.08,P>0.05).For 18 patients with IVCS, there was significant statistical difference between patients implanting stents(initial patency was 100.0%(7/7) and whom not
implanting stents(initial patency was 63.6%,7/11) (χ2=5.14, P<0.05). 1, 3, 6 months and 1, 2, 3 years follow-up showed: The primary patency of postoperative 1, 3, 6 months respectively were 86.7%, 70%(21/30),66.7%(20/30), and postoperative 1, 2, 3 years were all 66.7%. There was significant statistical difference between patients with IVCS implanting stents and whom not implanting stents (χ2=6.562, P<0.05), patients with IVCS not implanting stents and whom without IVCS (χ2=3.377, P<0.05). There was no significant statistical difference among patients with IVCS implanting stents and whom without IVCS(χ2=1.932, P>0.05). Conclusions Endovascular therapy consisting of catheter-directed thrombolysis with angioplasty could be considered as a primary therapeutic procedure in patients with acute or subacute postpartum DVT. Stenting for treatment of iliac compression which contributed to postpartum DVT is very valuable to ensuring higher long-term patency rate.

Objective To discuss the application of spiral CT angiography in postoperative re-examination of lower limb artery stent implantation. Methods During the period from March 2012 to March 2014 at the Affiliated Nanjing Hospital of Nanjing Medical University, CT angiography was carried out in 67 patients who had received stent implantation for arteriosclerotic occlusion of lower limb. The diseases involved 78 lower limbs, and a total of 85 stents were employed. The volume rendering (VR), maximum intensity projection (MIP), multi-planar reformation (MPR) and curved surface reconstruction (CSR) were performed to stereoscopically display the lesion’s anatomy as well as the implanted stents, and the imaging manifestations were compared with the clinical symptoms and DSA findings. Results Successful examination was accomplished in 65 patients (81 stents in total), and clear images were obtained. Of the 81 stents, no stenosis was seen in 43, Ⅱ - Ⅳ grade stenosis in 32 and complete occlusion in 6. The results were closely correlated with the clinical symptoms. The CT angiography manifestations in 31 patients (34 stents in total) were compared with their DSA performed in two weeks, and the results showed that the stenotic degrees of three stents judged by CT angiography were not consistent with those judged by DSA. The consistent rate of CT angiography was 91.2% when taking DSA as the standard. Conclusion Lower limb arterial CT angiography examination is a safe and non-invasive technique, it can clearly display the stent inner canal. Therefore, this technique is of great value in postoperative re- examination of lower limb artery stent implantation.

Objective To investigate the effects of advanced glycation endproducts (AGEs) on proliferation of pericytes of bovine retinal capillary vessels and expression of transforming growth factor ?(TGF-?). Methods [WTBZ]The proliferation of pericytes detected by methyl thiazolyl tetrazolium (MTT) colorimetric assay, cellular cycle of pericytes was analyzed by flow cytometry was used to analyze cell, and TGF-? protein expression of pericytes was observed by immunofluorescent staining. Results AGEs inhibited the proliferation of pericytes of bovine retinal capillary vessels, stopped the cellular cycle of pericytes in synthesis phase (S phase), increased the number of apoptotic cells obviously (P