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Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting both upper and lower motor neurons (MNs) with large unmet medical needs. Multiple pathological mechanisms are considered to contribute to the progression of ALS, including neuronal oxidative stress and mitochondrial dysfunction. Honokiol (HNK) has been reported to exert therapeutic effects in several neurologic disease models including ischemia stroke, Alzheimer's disease and Parkinson's disease. Here we found that honokiol also exhibited protective effects in ALS disease models both in vitro and in vivo. Honokiol improved the viability of NSC-34 motor neuron-like cells that expressed the mutant G93A SOD1 proteins (SOD1-G93A cells for short). Mechanistical studies revealed that honokiol alleviated cellular oxidative stress by enhancing glutathione (GSH) synthesis and activating the nuclear factor erythroid 2-related factor 2 (NRF2)-antioxidant response element (ARE) pathway. Also, honokiol improved both mitochondrial function and morphology via fine-tuning mitochondrial dynamics in SOD1-G93A cells. Importantly, honokiol extended the lifespan of the SOD1-G93A transgenic mice and improved the motor function. The improvement of antioxidant capacity and mitochondrial function was further confirmed in the spinal cord and gastrocnemius muscle in mice. Overall, honokiol showed promising preclinical potential as a multiple target drug for ALS treatment.
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Objective:To analyze the case characteristics and outcomes of 12 idiopathic giant cell myocarditis(IGCM)cases after heart transplantation(HT).Methods:From June 2004 to May 2022, clinical data were retrospectively reviewed for 12 cases with pathologically confirm IGCM after HT at Fuwai Hospital.General characteristics, clinical manifestations, pathological examinations and postoperative follow-ups are recorded.Results:From June 2004 to May 2022, a total of 1 143 HT operations are performed at Fuwai Hospital and 12 cases of IGCM(1.05%)are confirmed by postoperative pathology.The age is(47.6±7.3)years.There are 5 boys and 7 girls.Initial presenting manifestations are congestive heart failure(7 cases, 58.3%)and arrhythmia(4 cases, 33.3%). Median time from symptom onset to HT is 6 months.All of them are undiagnosed pre-operation.And dilated cardiomyopathy(5 cases, 41.7%)and arrhythmogenic right ventricular cardiomyopathy(3 cases, 25%)are confirmed.The follow-up period is(4~142)months post-HT.One death occurred during perioperative period and another is due to heart failure at 68 months post-HT.Only 1 case of grade 1R transplant heart rejection occurrs at 9 years post-HT and there is no case of recurrence.According to Kaplan-Meier survival analysis, cumulative survival rates of 1/5/10 years post-HT in IGCM patients are 91.7%, 91.7% and 73.3% respectively.No significant difference exist in survival rate for other etiologies post-HT(Log-rank P=0.265). Conclusions:HT is efficacious for end-stage IGCM.Regular and sufficient postoperative immunosuppression is vital for preventing heart transplant rejection and recurrent IGCM.Most IGCM patients have a decent prognosis post-HT.
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Background@#The dietary agent sulforaphane (SFN) has been reported to reduce diabetes-induced renal fibrosis, as well as inhibit histone deacetylase (HDAC) activity. Bone morphologic protein 7 (BMP-7) has been shown to reduce renal fibrosis induced by transforming growth factor-beta1. The aim of this study was to investigate the epigenetic effect of SFN on BMP-7 expression in diabetes-induced renal fibrosis. @*Methods@#Streptozotocin (STZ)-induced diabetic mice and age-matched controls were subcutaneously injected with SFN or vehicle for 4 months to measure the in vivo effects of SFN on the kidneys. The human renal proximal tubular (HK11) cell line was used to mimic diabetic conditions in vitro. HK11 cells were transfected to over-express HDAC2 and treated with high glucose/palmitate (HG/Pal) to explore the epigenetic modulation of BMP-7 in SFN-mediated protection against HG/Pal-induced renal fibrosis. @*Results@#SFN significantly attenuated diabetes-induced renal fibrosis in vivo. Among all of the HDACs we detected, HDAC2 activity was markedly elevated in the STZ-induced diabetic kidneys and HG/Pal-treated HK11 cells. SFN inhibited the diabetes-induced increase in HDAC2 activity which was associated with histone acetylation and transcriptional activation of the BMP-7 promoter. HDAC2 over-expression reduced BMP-7 expression and abolished the SFN-mediated protection against HG/Pal-induced fibrosis in vitro. @*Conclusion@#Our study demonstrates that the HDAC inhibitor SFN protects against diabetes-induced renal fibrosis through epigenetic up-regulation of BMP-7.
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Objective To improve the understanding of Danon disease and the efficacy of heart transplantation by multi-disciplinary team (MDT) pattern. Methods Prior to heart transplantation, MDT consultation was performed on one case of Danon disease, an extremely rare X-chromosome dominant genetic disease. The content of consultation included surgical indication, preoperative preparation and postoperative precaution, as well as the role of MDT in the treatment of Danon disease was summarized. Results Preoperative echocardiography showed that the patient presented withdilated-phase of hypertrophic cardiomyopathy complicated with heart failure, which was considered as Danon disease with end-stage heart failure. After MDT consultation, the patient received the preoperative treatment including anti-heart failure, reduction of pulmonary artery pressure, intra-aortic balloon pump (IABP) assistance, liver protection, strengthening nutritional support, etc. The patient underwent orthotopic heart transplantation after a suitable donor was matched. The patient developed muscle weakness in the limbs 5 days after operation, which was gradually mitigated after reducing the dose of glucocorticoid. At postoperative 48 days, the patient was discharged in good condition and continually treated with triple immunosuppressive regimen after discharge. Reexamination at postoperative 6 months revealed that the patient was in good health without any abnormality in electrocardiogram and echocardiography images. Conclusions Danon disease is extremely rare and MDT pattern may enhance the efficacy and safety of treatment by heart transplantation.
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OBJECTIVE：To comp are cytotoxicity and anti-inflammatory effects of raw Aconitium kusnezoffii and A. kusnezoffii processed with Terminalia chebula . METHODS ：Using H 9c2 cardiomyocytes isolated from rat as subjects ，CCK-8 assay was used to detect the effects of 0.5，1，2，4，6，8，10 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula on cell inhibition rate after cultured for 4，8，12，24 h. Hoechst 33258 staining was used to observe the effects on cell morphology characteristics after treated with 2，4，6 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula . Using macrophages RAW264.7 cells as subjects ，CCK-8 assay was used to detect the effects of 0.05，0.1，0.25，0.5，0.75，1，1.5，2 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula on cell survival rate after cultured for 24 h. ELISA assay was used to detect the effects of 0.05，0.1，0.25，0.5 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula on the release of NO ， TNF-α and IL-6 in RAW 264.7 inflammation cells induced by LPS. RESULTS ：When the mass concentration was 0.5，1 mg/mL， neither raw A. kusnezoffii and A. kusnezoffii processed with T. chebula had no inhibitory effect on H 9c2 cells. When the mass concentration was 2 mg/mL，the inhibitory effects of A. kusnezoffii processed with T. chebula on H 9c2 cells was higher than that of raw A. kusnezoffii （P＜0.05 or P＜0.01）；fluorescence intensity of cells treated for 24 h was stronger than that of raw A. kusnezoffii，but its nucleus was intact. When the mass concentration was 4-10 mg/mL，the inhibitory rate of A. kusnezoffii processed with T. chebula on H 9c2 cells at different time points （except for 24 h culture of 8，10 mg/mL）was significantly lower than raw A. kusnezoffii （P＜0.05 or P＜0.01）. The characteristics of cell morphology also showed that the fluorescence intensity of raw A. kusnezoffii group at 4，6 mg/mL was stronger than that of A. kusnezoffii processed with T. chebula group，and the cell nucleus fragmentation was more serious in the raw A. kusnezoffii group. 0.05-0.5 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula had no toxicity to RAW264.7 cells. 0.25，0.5 mg/mL raw A. kusnezoffii and 0.1，0.25，0.5 mg/mL A. kusnezoffii processed with T. chebula showed significant inhibitory effect on the release of NO ，0.05，0.1，0.25，0.5 mg/mL raw A. kusnezoffii and A. kusnezoffii processed with T. chebula showed significant inhibitory effect on the release of TNF-α and IL-6 in RAW 264.7 cell（P＜0.05 or P＜ 0.01）. The inhibitory effects of A. kusnezoffii processed with T. chebula at the same concentration on the release of NO was better than that of raw A. kusnezoffii ，but poorer than raw A. kusnezoffii in the inhibitory effects on the release of TNF-α and IL-6. CONCLUSIONS：The toxicity of A. kusnezoffii can be reduced after processed with T. chebula ，especially the toxicity of it in medium or high concentration and short-term use is lower than that of raw A. kusnezoffii . At the same time ，the anti-inflammatory effect of A. kusnezoffii processed with T. chebula is comparable to that of raw A. kusnezoffii at the same concentration.
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Objective@#To evaluate the sensitivity and specificity of immunohistochemistry (IHC) in the classification of cardiac amyloidosis on endomyocardial biopsy (EMB) and heart allograft.@*Methods@#Twenty cardiac tissues from 19 patients at Fuwai Hospital from January, 1990 to April, 2017 with histopathologic features of amyloidosis and Congo red staining positivity were included. IHC was performed with monoclonal antibodies against AA amyloid and polyclonal antibodies against transthyretin (ATTR), λ-light chain (AL-λ), κ-light chain (AL-κ), ApoAⅠ, ApoAⅡ, ApoA Ⅳ and β2-microglobin. The extent of interstitial staining was evaluated by light microscopy, and three patterns were recognized; these included diffuse pericellular pattern, discrete pericellular pattern, and nodular pattern. Two patterns of vascular deposition were also noted, including arterial pattern and venous pattern. Endocardial involvement was also assessed and recorded.@*Results@#Nineteen cases were divided into three groups according to the pattern of proteins expression in specimens. The first group (5 cases) only showed single protein expression on EMB. The second group (6 cases) showed more than one protein expression, but one of them was intensely stained or any staining of any protein together with ApoA Ⅳ co-staining. The third group (8 cases) also showed more than one protein expression and all of them had intense staining. Amyloid deposits were successfully subtyped as AL-λ, ATTR, AL-κ and ApoAⅠby IHC in the former two groups with the sensitivity of 11/19. In the third group, amyloid deposits could not be subtyped by immunohistochemistry due to their poor specificity. The pericellular pattern tended to favor AL over ATTR amyloidosis and vascular deposition tended to favor ATTR.@*Conclusions@#Amyloid deposits can be reliably subtyped in diagnostic cardiac specimens using IHC. The co-deposition of chaperon proteins, the distribution of amyloid proteins and clinical features are also auxiliary to subtype cardiac amyloidosis.
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Objective To observe the protective and regeneration-promoting effects of Ruyi Zhenbao Pill (RZP) on nerve injury in zebrafish.Methods The zebrafish model of central nervous injury was induced by mycophenolate mofetil,the model of peripheral motor nerve and axonal injury was induced by ethanol,and the model of myelin damage was induced by ethidium bromide.The variations of central nerve,axon and myelin sheath fluorescence intensity,and peripheral motor nerve length in zebrafish,which exposed to the different concentration (10.0,33.3,and 100.0 μ.g/mL) of RZP,were observed with fluorescence microscope.The effective protection rates of RZP on zebrafish central nerve and axone,and the regeneration-promoting effect on peripheral motor nerve and myelin sheath were analyzed and calculated with the image processing software NIDS-Element's.Results In 10.0,33.3,and 100.0 μg/mL RZP groups,the zebrafish central nervous injury protective rates were 2％,24％,and 50％ (P ＜ 0.001),respectively,the peripheral nerve regeneration promoting rates were 44％ (P ＜ 0.05),49％ (P ＜ 0.01),and 93 ％ (P ＜ 0.001),the axonal injury recovery rates were 3％,29％ and 48％ (P ＜ 0.05),and the myelin sheath regeneration promoting rates were 36％ (P ＜ 0.01),37％ (P ＜ 0.001),and 41％ (P ＜ 0.001),compared with model group.Conclusions RZP could not only protect the central nervous and axonal injury,but also promote the regeneration of peripheral nerve and myelin sheath in zebrafish.
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Objective@#To investigate the clinical features and surgical treatment effect of patients with cardiac cavernous hemangioma.@*Methods@#Clinical data of 9 patients (5 male, aged from 4 to 53 years old) with cardiac cavernous hemangioma, who underwent surgical treatment from November 2002 to March 2015 and the diagnosis of cardiac cavernous hemangioma was confirmed by postoperative histological examination, were retrospectively analyzed. Effects of surgical treatment were analyzed.@*Results@#Four patients were asymptomatic (heart murmur presented in 3 patients during physical examination). Three patients presented with palpitation, chest distress, and short of breath. One patient presented with epigastric discomfort and another patient presented with intermittent fever for more than 10 months. ST and T wave changes of electrocardiogram were found in 2 patients, cardiac mass was detected in the right heart chamber in 5 patients by echocardiography, and no cardiac mass was detected the rest 4 patients. Cardiac masses were resected en bloc, then the adjacent tissues were repaired in 7 patients, and mass was partially resected due to the involvement with adjacent heart structure. No cardiac mass was found during operation in 1 case, impaired mitral valve structure was excised and postoperative pathologically confirmed as cardiac valve cavernous angioma on the excised mitral valve structure. No signs of recurrence or enlargement of cardiac cavernous hemangioma were found during the 11(10, 11)years follow up.@*Conclusions@#There is no specific clinical feature for patients with cardiac cavernous hemangioma. It is difficult to detect the cardiac valve cavernous angioma by echocardiography before surgery. Individualized surgical treatment is associated with good clinical outcome in this patient cohort. However, the clinical features and surgical treatment effect of patients with cardiac cavernous hemangioma still need to investigate in large sample trial.
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Objective To evaluate the clinical application of high quality for super-selective renal artery embolization (SRAE) in treating bleeding after percutaneous nephrolithotomy (PCNL).Methods 134 patients received percutaneous nephrolithotomy were divided into control group (67 patients) and observation group (67 patients).All of patients with serious bleeding after PCNL were given SRAE in the Second Affiliated Hospital of Kunming Medical College from June 2010 to June 2015.At the same time,we gave high quality nursing to observation group.The patients in control group received routine nursing.The effect of nursing was observed.Results The degree of hematuria disappear of the patients in observation group was higher than that in control group (P＜ 0.05).There were fewer complications in observation group.In the sixth month after discharge,none of them had obvious renal impairment.No recurrence of hematuria,pus kidney and urinary cyst was tested.All cases were satisfied with the treatment.Conclusion It's the key to prevent serious complications and cure successfully with effective and timely supervision and high quality nursing care during the perioperation of SRAE in treating bleeding after PCNL.
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<p><b>OBJECTIVE</b>To assess the pathologic markers for evaluation of reversibility in pulmonary hypertension (PAH) related to congenital heart disease.</p><p><b>METHODS</b>Twenty-eight patients with congenital heart disease complicated by PAH were subclassified into reversible pulmonary hypertension (RPAH) and irreversible pulmonary hypertension (IPAH), according to post-operative mean pulmonary artery pressure (MPAP). Pulmonary vascular lesion was analyzed according to Ruan's method. Mean medium thickness percent, mean medium area percent and pulmonary arteriolar density were measured by quantitative morphometry. Immunohistochemical study for transgelin and filamin A was carried out.</p><p><b>RESULTS</b>Amongst the 28 cases studied, 24 were RPAH and 4 were IPAH. Of the 24 patients with RPAH, 13 (54.2%, 13/24) had pulmonary vascular lesion of grade 0, 9 (37.5%, 9/24) of grade 1 and 2 (8.3%, 2/24) of grade 2. Of the 4 patients with IPAH, 1 had lesion of grade 1, 1 of grade 2 and 2 of grade 3. Both preoperative and postoperative MPAP were higher in IPAH patients than that in RPAH patients[(53.3±23.4) mmHg versus (34.1±12.7) mmHg, P=0.020 and (35.0±8.8) mmHg versus (17.8±3.9) mmHg, P<0.01]. Compared to patients with pulmonary vascular lesion of grades 0 and 1, the preoperative MPAP in patients with grades 2 and 3 showed no significant difference, but the postoperative MPAP was higher (P<0.05 or 0.01). Compared to control group, mean medium thickness percent and mean medium area percent were significantly higher in RPAH and IPAH categories (12.0±3.5, 8.5±2.0 versus 5.7±1.0, P<0.01 and 55.8±11.1, 49.0±9.4 versus 34.0±5.5, P<0.01). Mean medium thickness percent was significantly higher in IPAP group than that in RPAH group (12.0±3.5 versus 8.5±2.0, P=0.001). Correlation analysis demonstrated that mean medium thickness percent and mean medium area percent had positive correlation with preoperative and postoperative MPAP. There was no correlation between grading of pulmonary vascular lesion and reversibility. Transgelin and filamin A had stronger staining in pulmonary vascular smooth muscle cells in IPAH than those in RPAH and controls(P<0.05).</p><p><b>CONCLUSIONS</b>Pathologic assessment of lung biopsy remains the gold standard for evaluation of the reversibility in PAH related to congenital heart disease. Mean medium thickness percent, mean medium area percent and immunoreactivity for transgelin and filamin A are useful parameters.</p>
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Humans , Biomarkers , Metabolism , Biopsy , Filamins , Metabolism , Heart Diseases , Pathology , Hypertension, Pulmonary , Diagnosis , Pathology , Lung , Pathology , Microfilament Proteins , Metabolism , Muscle Proteins , MetabolismABSTRACT
Objective To study the ultrastructural features of myocytes in uterine junctional zone (JZ).Methods From August 2010 to August 2013,there were 16 pre-menopause patients who suffered from cervical neoplasm to be performed hysterectomy.Samples of JZ and outer myometrium (OM) of hysterectomy specimens were collected.There were 8 specimens from the proliferative-phase and 8 specimens from the secretory-phase of endometrium.Ultrastructural features of JZ and OM were examined by using transmission electron microscopy and the related indices of myocytes were compared by using Student's t test.Results At JZ,there were more cytoplasmic process in the myocytes.The myocytes of JZ exhibited significant difference compared with those of OM.Firstly,the contractile structural components,such as the dense patches,dense bodies and the myofilaments were less abundant.In contrast,the perinuclear cell organelles were more distinct.The mitochondria,endoplasmic reticulum and Golgi apparatus were more prominent,denoting active protein synthesis.Secondly,the mean diameter of cell and nuclei demonstrated cyclic change.In proliferative phase of endometrium,the cell diameters of JZ and OM were (4.70_±0.52) and (4.69± 1.20) μm,respectively,which there were no significant difference(P=0.987).While in secretory phase,the cell diameters of JZ and OM were (3.75±0.36) and (4.92±0.51) μm,which there were significant difference (P=0.006).In proliferative phase,the nuclei diameters of JZ and OM were (3.24±0.41) and (2.90±0.62) μm,and in secretory phase,the nuclei diameters of JZ and OM were (2.44±0.27) and (2.92±0.44) μm.There were no significantly different in both phases (P=0.374,P=0.097).The diameters of cell and nuclei had cyclical changes (P ＜ 0.05).However,the cyclical changes were absent in OM (P＞ 0.05).Thirdly,the myofilaments/cytoplasm ratio of JZ in proliferative and secretory phases were 0.27±0.04 and 0.34±0.03,which were significantly less than those of OM in respective phases (0.49±0.03and 0.54±0.03;P=0.000,P=0.000).The myofilaments/cytoplasm ratio exhibited cyclical changes in JZ (P=0.029),but in OM,the cyclical changes were absent (P=0.083).Conclusions Compared with OM,ultrastructures associated with synthetic organelles are prominent,whereas the contractile organelles are reduced.And there are the cyclical changes in ultrastructural characteristics.The ultrastructural features of JZ are the basis of its physiology.
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Objective: To dynamically evaluate left ventricular perfusion, global and local functional changes during left ventricular aneurysm (LVA) formation and to explore the relationship between the size of LVA and LVEF, LVESV, LVEDV by gated99mTc-MIBI SPECT (GSPECT) and gated18F-FDG PET metabolic (GPET) imaging in experimental pigs. Methods: LVA model was established by occlusion of left circumlfex artery (LCX) and placing an Ameroid constrictor at the proximal end of left anterior descending artery (LAD) in a total of 16 Chinese mini-pigs. At the 1st, 4th and 8th weeks of surgery, the changes of total perfusion defect (TPD), LVA formation and LVEF, LVESV, LVEDV were dynamically evaluated by GSPECT and GPET; the relationships between the size of LVA and LVEF, LVESV, LVEDV were analyzed respectively.Results: There were 5 pigs died in surgery and 2 died at the 1st week of modeling. According to golden (pathological) standard, 9 animals successfully ifnished the dynamic imaging study. At the 1st week of (basic) modeling, 4 animals formed large LVA, 2 formed small LVA at the apex and 3 without LVA formation. At the 4th and 8th weeks of modeling, dynamic imaging presented that the animals with large LVA had gradually increased range and degree of perfusion defect, LVEDV, LVESV, while gradually decreased LVEF; the above indexes were relatively stable in animals with small or none LVA. In addition, the size of LVA was related to LVEF (r=-7.26), LVEDV (r=0.855) and LVESV (r=0.825), allP<0.05. Conclusion: In experimental pigs, at the beginning of LVA formation, large range and severe perfusion defect may cause large aneurysm, the LV functional damage and remodeling may gradually increase and the prognosis is poor; in contrast, the animals with small or none LVA have better prognosis and usually without ventricular remodeling; which implies that in acute phase of LVA formation, the size of aneurysm may predict the trend of global LV systolic function and remodeling at the early stage.
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Objective: To describe the clinical characteristics with long-term prognosis in patients with mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHCM). Methods: A total of 66 MVOHCM patients treated in our hospital were retrospectively studied for their morbidity, clinical characteristics and mortality. The cumulative survival rate was calculated by Kaplan-Meier method; the risk factors for cardiac death and cardiovascular events were analyzed by uni- and multivariate Cox proportional hazard model. Results: There were 66 (2.74%) patients suffering from MVOHCM among 2413 patients of hypertrophic cardiomyopathy and the average diagnostic age was (40.16 ± 14.64) years. With (7.30 ± 6.25) years of follow-up study, the cardiovascular mortality was 13.6% and unexplained syncope (HR=13.37, 95% CI: 1.65-114.46, P=0.015) was the independent predictor for cardiovascular death. There were 45.45% (30/66) patients experienced at least 1 time of cardiovascular event and the most frequent one was non-sustained ventricular tachycardia (NSVT); 19.70% (13/66) of patients combined with apical aneurysms, and they were more inclined to experience NSVT. Conclusion: MVOHCM patients usually have unfavorable prognosis with the higher incidence of cardiovascular events, some patients may develop apical aneurysm. The early diagnosis of MVOHCM is important for appropriate treatment.
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Objective To research the influences of massive hemorrhage on spatial learning and memory ability in elderly SD rats .Methods Fifty six aged SD rats were randomly divided into 3 groups:blank group (B group ,n=8) ,control group (C group , n=24) ,and hemorrhage group (H group ,n=24) .B group was not given any intervention .C group received femoral artery ligation and was sutured under general anesthesia .H group underwent femoral artery puncture phlebotomy ,and then the rats were sutured . Morris water maze was used to test the spatial working ability .Results The escape latency of H group on postoperative day 1 ,3 and 7 were(29 .93 ± 7 .93)s ,(34 .56 ± 6 .74)s and (15 .47 ± 6 .42)s respectively .Compared with B group(12 .56 ± 3 .08)s ,these re‐sults indicated the spatial learning and memory of H group was destroyed after surgery 1 d and 3 d (P0 .05) .The escape latent periods to platform observed in C group rats on day1 ,3 and 7 after operation were(15 .74 ± 5 .86)s ,(15 .61 ± 2 .56)s and (13 .56 ± 4 .61)s .Compared with B group [(12 .56 ± 3 .08)s] ,these results indicated that there was no significant difference 7 (P>0 .05) .Conclusion The findings of this study indicate that massive hemorrhage of old rats may destroy the spatial learning and memory .
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<p><b>OBJECTIVE</b>To study the clinical and pathologic features of primary cardiac inflammatory myofibroblastic tumor.</p><p><b>METHODS</b>A total of 4 patients with primary cardiac inflammatory myofibroblastic tumor were encountered during the period from 1993 to 2013 in National Center for Cardiovascular Disease. The clinical features, imaging findings and outcomes of the 4 patients were evaluated. ALK protein expression and ALK gene status were studied using the archival tumor tissues.</p><p><b>RESULTS</b>There were 1 female and 3 male patients. The age of patients ranged from 5 months to 30 years (mean = 16 years). The tumor was located in right ventricle (n = 2), right atrium (n = 1) or pericardium (n = 1). Histologic patterns included 2 cases of fibrous histiocytoma type, 1 case of granulomatous type and 1 case of sclerosing type. Immunohistochemical study showed that 2 cases expressed ALK protein. Fluorescence in-situ hybridization however did not reveal any ALK gene rearrangement.</p><p><b>CONCLUSIONS</b>Inflammatory myofibroblastic tumor of the heart is rarely encountered and easily misdiagnosed. It carries distinctive clinical and pathologic features. ALK protein expression is helpful in arriving at the correct diagnosis.</p>
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Adolescent , Adult , Child , Female , Humans , Infant , Male , Biomarkers, Tumor , Genetics , Metabolism , Diagnosis, Differential , Granuloma, Plasma Cell , Pathology , Heart Neoplasms , Pathology , Histiocytoma, Benign Fibrous , Pathology , Immunohistochemistry , In Situ Hybridization, Fluorescence , Receptor Protein-Tyrosine Kinases , Genetics , MetabolismABSTRACT
<p><b>OBJECTIVE</b>To investigate the histopathological features of primary restrictive cardiomyopathy (PRCM).</p><p><b>METHODS</b>Nine extransplanted hearts from heart transplantation recipients were examined. Gross and histopathological findings were observed, photographed and final pathological diagnosis was compared to clinical diagnosis. The myocardial ultrastructure changes were determined using transmission electron microscopy.</p><p><b>RESULTS</b>The hallmark pathologic feature of PRCM was distinguished by myocardial cell degeneration and hyperplastic collagen fibrils around the myocardial cells.Fibrosis was severer in left ventricle free wall than in ventricular septum and right ventricle. The degree of myocardial cell degeneration and poloidal disorder were severer in patients with reduced ejection fraction (EF) than in patients with preserved EF. Transmission electron microscope evidenced severe interstitial fibrosis, myofibrillar changes of sarcomere structure, abnormalities both on intercalated disc number and distribution.</p><p><b>CONCLUSIONS</b>PRCM is characterized by hyperplastic collagen fibrils around the cardiomyocytes. Fibrosis is severer in left ventricle than in right ventricle. Sarcomere dysplasia is the main cause of PRCM, and ultrastructural examination is helpful for PRCM diagnosis.</p>
Subject(s)
Humans , Cardiomyopathy, Restrictive , General Surgery , Fibrosis , Heart Transplantation , Heart Ventricles , Myocardium , Pathology , Myocytes, Cardiac , SarcomeresABSTRACT
Objective To explore the two different anesthesia methods on hemodynamics and inflammatory cytokines in elderly patients during peroperative period.MethodsFifty elderly patients with Knee Replacement( ASA Ⅰ,Ⅱ )were randomly divided into general anesthesia group( group A,n =25 ) and combined general and epidural anesthesia group( group B,n =25 ).The changes of mean arterial pressure(MAP) and heart rate ( HR ) were monitored before induction of anesthesia ( T1 ),at intubation ( T2 ),during skin incision ( T3 ) and at the time of extubation ( T4 ),at 30 min after extubation ( T5 ).Blood samples were taken from artery for determination of plasma TNF-α,IL-6,IL-10 concentrations before tourniquet inflation ( T5 ),10 min after tourniquet deflation(T6),30 min after tourniquet deflation (T7)and 30 min after operation (T8)by enzymelinked immunosorbent assay(ELISA).ResultsThe MAP and HR of patients in two groups at T2,T3,T4 were all increased when compared with T1 [ group A:HR:( 94.3 ± 10.4 ) bpm,( 96.4 ± 12.7 ) bpm,(93.3 ± 11.1 )bpm vs(62.6 ±7.3)bpm;MAP:( 18.8 ±3.4)kPa,( 19.6 ±3.4)kPa,( 17.8 ±2.0)kPa vs ( 14.5 ± 1.5)kPa,P＜0.05;group B:HR(76.2 ±6.5)bpm,(70.1 ± 9.7) bpm,(71.5 ± 8.3) bpm vs(64.6 ± 8.4) bpm;MAP:( 16.3 ± 2.5 ) kPa,( 15.3 ± 1.2) kPa,( 14.8 ± 1.4) kPa vs ( 14.1 ± 1.3 ) kPa,P ＜ 0.05 ].There was significant difference on MAP and H R between group A and group B( F =11.957,9.745;P ＜ 0.05 ).The level of plasma TNF-α,IL-6 and IL-10 were significantly increased at T6 to T8 compared with T5 in both groups[ groupA:TNF-α:(4.36 ±0.18) ng/L,(7.54 ± 1.23) ng/L,(10.35 ±2.21 )ng/L vs (2.26 ±0.16) ng/L; groupA:IL-6:(4.32 ±0.21 ) ng/L,( 8.35 ± 1.26 ) ng/L,( 10.23 ± 2.23 ) ng/L vs ( 1.36 ± 0.08 ) ng/L; groupA:IL-10:(5.32±1.10) ng/L,(7.56 ± 1.36) ng/L,(8.63 ± 2.21) ng/L vs (1.25 ± 0.03) ng/L; groupB:TNF-α:(3.43 ±0.06)ng/L,(5.24 ±2.10) ng/L,(7.68 ± 1.43) ng/L vs(2.22 ±0.15) ng/L;groupB:IL-6:(3.41 ±0.08 ) ng/L,(5.34 ± 1.34 ) ng/L,( 8.54 ± 2.03 ) ng/L vs ( 1.28 ± 0.04 ) ng/L; groupB:IL-10:( 7.28 ± 1.22 )ng/L,( 10.53 ± 2.14)ng/L,( 12.45 ± 2.03 )ng/L vs( 1.31 ± 0.04)ng/L,P ＜ 0.05 ].And there was significant difference on TNF-α,IL-6 and IL-10 between group A and group B( F =7.889,3.554,5.443,respectively,P ＜0.05).ConclusionCompared with general anesthesia group,combined general and epidural anesthesia group can ensure hemodynamic stability of elderly patients during peroperative period very well and can reduce the releasing of inflammatory cytokins,it is a viable and an ideal method.
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ObjectiveTo explore two different anesthesia methods on hemodynamics and the quality of palinesthesia in elderly patients during peroperative period.Methods Sixty elderly patients with Hip Replacement( ASA,Ⅰ,Ⅱ ) were randomly divided into general anesthesia group ( group A,n =30 ) and combined general and epidural anesthesia group( group B,n =30).The changes of mean arterial pressure(MAP)and heart rate( HR ) were monitored before induction of anesthesia( T1 ),at intubation( T2 ),during skin incision (T3) and at the time of extubation ( T4 ),at 30 min after extubation ( T5 ) and at the same time,the dosage of general anesthetics and each index's time after operation to awake were recorded of the patients in both groups.ResultsThe MAP and HR of patients in two groups at T2,T3,T4,T5 were all increased when compared with T1.And the increasing degree of MAP and HR in group A were higher than that in group B ( MAP:within group F =17.352,interaction F =4.326,between groups F =8.652; HR:within group F =11.561,interaction F =5.241 between groups F =7.248; P ＜ 0.05 ).The dosage of general anesthetics was significantly different between two groups[ sevoflurane:(1.40 ± 0.30)MAC vs (1.00 ± 0.12 )MAC,t =0.37,P＜0.05 ; fentanyl:(0.34 ±0.08)mg vs(0.18 ±0.03) mg,t =0.21,P ＜0.05 ; vecuronium:(6.20 ±0.32) mg vs(4.10 ±0.31 ) mg,t =1.24,P ＜0.05 ; propofol:(448 ±24) mg vs(393 ±26) mg,t =3.46,P ＜0.05].There was significant difference on gag reflex time [ ( 18.00 ± 1.27 ) min vs ( 12.31 ± 2.54 ) min,t =2.74,P ＜ 0.05 ],time to extubation [ ( 24.03 ± 2.42 ) min vs ( 16.05 ± 1.20 ) min,t =3.68,P ＜ 0.05 ],fully awake time [(29.54±5.24)min vs(19.25±2.64)min,t=1.35,P＜0.05] between these two groups.ConclusionThe two different anesthesia methods can ensure haemodynamic stability of elderly patients undergoing hip replacement during peroperative period.But compared with general anesthesia group,combined general and epidural anesthesia group can reduce the dosage of general anesthetics and shorten the time of extubation significantly,it is a viable and an ideal method.
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objective To analyze the correlation between the level of serum uric acid and the clinical and pathological features of IgA nephropathy.Methods Totally 148 patients diagnosed as IgA nephropathy by renal biopsy in our hospital from January 2007 to December 2010 were divided into hyperuricaemic group(41 cases)and non-hyperuricaemic group(107 cases)according to the level of serum uric acid.The clinical parameters and renal pathology grade were compared.Results There were significant differences between hyperuricaemic group and non-hyperuricaemic group in the incidences of hypertension(63.4%vs 38.3%),disease duration[(18.90±10.12)months vs(9.46±3.91)months]and body mass index[(22.81±3.60)kg/m2vs(15.32±2.54)kg/m2](all P0.05).The blood urea nitrogen(BUN)[(8.93±4.28)mmol/L vs (5.21±2.18)mmol/L],creatinine(Cr)[(155.96±107.72)μmol/L vs(79.52±40.01)μmol/L],serum triglycerides[(2.11±1.06)mmoVL vs(1.86±1.20)mmol/L]and 24-hour urine protein amount [(4328.16±1434.25)mg/24 h vs(2885.10±1388.15)mg/24 h]were significantly different between the two groups(all P<0.05).The percentage of Lee's grade I+Ⅱin hyperuricaemic group was 12.2%,and IV+V grade was 39.0%,while percentage of Lee's grade I+Ⅱin non-hyperuricaemic group was 25.2%,and IV+V grade was 16.9%(P<0.05).Tubulointerstitial lesions(TIL)gradeⅢ+IV was more in hyperuricaemic group,which was 68.3%,while TIL grade II was more in non-hyperuricaemic group,which was 76.6%.Renal artery damage grade II+Ⅲ was more in hyperuricaemic group.which was 73.2%,while renal artery damage grade 0+1 was more in non-hyperuricaemic group,which was 69.2%.Conclusion The level of serum uric acid was related with 24-hour urine protein amount,blood pressure and kidney function in IgA nephropathy,and Lee's grade,TIL grade and renal artery damage grade were severe in hyperuricaemic group.
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Objective To analyze the surgical and pathologic features of isolated ascending aortitis without evidence of rheumatologic or autoimmune diseases by comparing that of Takayasu's arteritis.Methods Consecutive 965 excised ascending aortas were reviewed and 40 cases with aortitis were selected from the pathological archives of past 20 years in Fuwai Hospital.The clinical history,laboratory and angiographic data,surgical findings,steroid therapy and followup results of these 40 cases were reviewed.Pathological parameters included the thickness of aortic wall and its various layers,inflammation activity,multinuclear giant cells,inflammatory necrosis,laminar necrosis and atheroselerosis.The t test and Chi square analysis were used to compare the means and the rates between the two groups.Results Twenty-five of 40 cases were diagnosed with isolated aortitis and its prevalencei was 2.6%(25/965),while 8 cases with Takayasu's arteritis and 7 cases with other vasculitis were confirmed.The age of isolated ascending aortitis was older than that of Takayasu's arteritis[(46±12)y vs(34±14)y,t=2.30,P>0.05] while the male/female ratio was similar in both groups(1.1 vs 1.0,t=0.01,P>0.05).Their main clinical manifestation was aortic aneurysm with a bigger aortic diameter in isolated aortitis than that in Takayasu's arteritis [(59±18)vs(46±12)mm,P>0.05].The asymptomatic cages mostly were found in isolated ascending aortitis (28%vs 0,x2=2.98,P>0.05).The erythrocyte sedimentary rate was normal in isolated aortitis but increased in Takayasu's arteritis[(15±17)mm/1 hvs[27±25)mm/1 h,U=48.50,P<0.05 ].Inflammatory edema,thickening and calcification were noted in more than half cases of both groups.but slighter thickening of aortic wall was foand in isolated ascending aortitis in contrast to the remarkable thickening in Takayasu artefifis[(2506±493)vs (3300±430)μm,t=-3.00,P<0.01].Giant cells,inflammatory necrosis,laminar necrosis and accompanied atherosclerosis were more common in isolated ascending aortitis.Aortic regurgitation was common but inflammatory invasion wege found in few aortic valves. Steroid was prescribed in 5 cases with isolated ascending aortitis and none of them was reported with complications while aortic perivalvular leakage occurred in 3 of 20 cases without steroids therapy. In the Takayasu's arteritis cases, new aneurysm of abdominal aorta was found in 1 of 3 cases with steroid therapy. Meanwhile, perivalvular leakage and subclavian artery stenosis occurred in 2 of 5 cases without steroid. Conclusion Isolated aortitis is more common than Takayasu's arteritis in ascending aortic diseases and there is some definite difference in clinical and pathological aspects between these two diseases. Since the effect of steroid therapy is uncertain and in order to avoid overtreatment, we suggest that steroid should not be given to cases with isolated ascending aortitis after surgery and clinical and angiographic follow-up should be emphasized.