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find Keyword "肌病" 72 results
  • Surgical Treatment of Adenomyosis:Report of Clinical Evaluation of 97 Cases

    目的:研究子宫腺肌病局部病灶切除术的可行性,方法及手术疗效的评价。方法:对2002年3月至2006年3月97例子宫腺肌病保留子宫仅作局部病灶切除术的患者进行随访观察。结果:97例子宫腺肌病手术后痛经治愈率:轻度,100%,中度37.8%,重度36%,痛经缓解率,中度及重度分别为62.2%及64%。月经过多治愈率为100%。妊娠分娩率原发不孕57.6%,继发不孕52.9%。结论:子宫腺肌病局部病灶切除术既治愈和改善了患者的临床症状又保留生殖器官的完整性及生理生殖功能可且有助于妊娠率的提高,是值得使用的方法。

    Release date:2016-09-08 10:01 Export PDF Favorites Scan
  • EFFECTS OF MECHANICAL STIMULATION ON EXPRESSION OF AUTOANTIGENS IN MYOBLASTS

    Objective To explore the effects of mechanical stimulation on the expression of autoantigens in myoblasts. Methods According to different processing methods, C2C12 cells were divided into the experimental group and control group; the experimental group was divided into 4 subgroups: 2-, 4-, and 6-day and 1-day stretch groups. In 2-, 4-, and 6-day stretch groups, mechanical loading was added on the C2C12 cells at a stretching frequency of 0.25 Hz and cellular deformation amplitude of 10%, 2 hours a day for 2, 4, and 6 days respectively by Flexercell 5000 strain unit, and at a stretching frequency of 1 Hz and cellular deformation amplitude of 15% for 1 hour in 1-day stretch group. In the control group, the cells were routinely cultured for 1, 2, 4, and 6 days (1-, 2-, 4-, and 6-day control). The cells were observed by inverted phase contrast microscope. The cell proliferation was detected by flow cytometry; the expressions of autoantigens were detected by Western blot method, including the Ku/the catalytic subunit of DNA-dependent protein kinase (DNA-PKcs), U1-70 (A part of ATP-dependent DNA helicase II), histidyl tRNA synthetase (HRS), and Mi-2 (reconfigurable components deacetylase complexes of NuRD). Results The exfoliated cells were found in 1-day stretch group, but no exfoliated cell was seen in the control group for 1-day culture. The cells proliferated more obviously in 2-day stretch group than in the control group for 2-day culture; cell differentiation was found in 4-day stretch group, and cell fusion in 6-day stretch group, which were similar to those in the control group for 4- and 6-day culture. After single stretching, cell apoptosis was found in 1-day stretch group, showing no significant difference in the relative DNA proliferation index (DPI) when compared with DPI of control group for 1-day culture (t=0.346, P=0.747). After cyclic stretching, DPIs of 2- and 4- day stretch groups were significantly increased when compared with those of the control group for 2- and 4-day culture (P lt; 0.05), but no significant difference was found between control group for 6-day culture and 6-day stretch group (t=1.191, P=0.303). Compared with the control group for 2-day culture, the relative protein expression of autoantigens (DNA-Pkcs, Mi-2, HRS, and U1-70) in 2-day stretch group decreased significantly (P lt; 0.05), but no significant difference was found between control group for 4-day culture and 4-day stretch group (P gt; 0.05). The relative protein expressions of autoantigens in 4-day stretch group significantly increased when compared with those of 2-day stretch group (P lt; 0.05), but the relative protein expressions of autoantigens in the control group for 4-day culture significantly decreased when compared with those of the control group for 2-day culture (P lt; 0.05). Conclusion Short-term mechanical stimulation can inhibit the expressions of autoantigens in myoblasts, but with the time prolonging, cell differentiation and fusion and adaptation to mechanical stimulation would result in diminished inhibitory effect.

    Release date:2016-08-31 04:12 Export PDF Favorites Scan
  • 线粒体脑肌病的护理体会一例

    Release date:2016-09-08 09:27 Export PDF Favorites Scan
  • 同种异体原位心脏移植六例

    目的总结6例行原位心脏移植术患者的外科治疗经验。方法采用Shumway和Stanford方法行心脏移植,供心保护采用经主动脉根部灌注4℃心脏停搏液;术后免疫抑制治疗采用环孢素A、骁悉和强的松联合治疗,根据血环孢素A浓度及心内膜活检调整环孢素A用量。结果6例患者均康复,无手术及术后死亡,术后未发生超急性或急性排斥反应。结论受者-供者选择、供心保护、吻合技术、合理应用免疫抑制剂和围术期并发症的处理是手术成功的关键。

    Release date:2016-08-30 06:25 Export PDF Favorites Scan
  • EFFECT OF BMSCs TRANSPLANTATION ON CARDIAC FUNCTION OF DIABETES MELLITUS RATS

    Objective To observe the effect of BMSCs on the cardiac function in diabetes mellitus (DM) rats through injecting BMSCs into the ventricular wall of the diabetic rats and investigate its mechanism. Methods BMSCs isolated from male SD rats (3-4 months old) were cultured in vitro, and the cells at passage 5 underwent DAPI label ing. Thirty clean grade SD inbred strain male rats weighing about 250 g were randomized into the normal control group (group A), the DM group (group B), and the cell transplantation group (group C). The rats in groups B and C received high fat forage for 4 weeks and the intraperitoneal injection of 30 mg/kg streptozotocin to made the experimental model of type II DM. PBS and DAPI-labeledpassage 5 BMSCs (1 × 105/μL, 160 μL) were injected into the ventricular wall of the rats in groups B and C, respectively. After feeding those rats with high fat forage for another 8 weeks, the apoptosis of myocardial cells was detected by TUNEL, the cardiac function was evaluated with multi-channel physiology recorder, the myocardium APPL1 protein expression was detected by Western blot and immunohistochemistry test, and the NO content was detected by nitrate reductase method. Group C underwent all those tests 16 weeks after taking basic forage. Results In group A, the apoptosis rate was 6.14% ± 0.02%, the AAPL1 level was 2.79 ± 0.32, left ventricular -dP/dt (LV-dP/dt) was (613.27 ± 125.36) mm Hg/s (1 mm Hg=0.133 kPa), the left ventricular end-diastol ic pressure (LVEDP) was (10.06 ± 3.24) mm Hg, and the NO content was (91.54 ± 6.15) nmol/mL. In group B, the apoptosis rate was 45.71% ± 0.04%, the AAPL1 level 1.08 ± 0.24 decreased significantly when compared with group A, the LVdP/ dt was (437.58 ± 117.58) mm Hg/s, the LVEDP was (17.89 ± 2.35) mm Hg, and the NO content was (38.91±8.67) nmol/mL. In group C, the apoptosis rate was 27.43% ± 0.03%, the APPL1 expression level was 2.03 ± 0.22, the LV -dP/dt was (559.38 ± 97.37) mm Hg/ s, the LVEDP was (12.55 ± 2.87) mm Hg, and the NO content was (138.79 ± 7.23) nmol/ mL. For the above mentioned parameters, there was significant difference between group A and group B (P lt; 0.05), and between group B and group C (P lt; 0.05). Conclusion BMSCs transplantation can improve the cardiac function of diabetic rats. Its possible mechanismmay be related to the activation of APPL1 signaling pathway and the increase of NO content.

    Release date:2016-09-01 09:08 Export PDF Favorites Scan
  • 脂质沉积性肌病

    脂质沉积性肌病是一组根据病理命名的疾病,主要表现为骨骼肌纤维内脂质异常沉积。尽管近年来关于脂质沉积性肌病的分子学研究较为广泛,但目前根据发现的致病基因诊断的脂质沉积性肌病仅包括4种:原发性肉碱缺乏症(PCD),多种脂酰辅酶A缺乏症(MADD),中性脂肪贮存伴鱼鳞病(NLSDI)和中性脂肪贮存性肌病(NLSOM)。由于这类疾病的临床特点缺乏特异性,所以特殊的实验室检查包括基因学检查对于部分可治性肌病显得尤为重要,如PCD患者对口服大剂量左旋肉碱有效,而近来越来越多的报道也证实携带有ETFDH基因突变的MADD患者对单独核黄素治疗有明显的疗效,这种情况在我国尤为显著。因此,本文对以上各种亚型的脂质沉积性肌病的临床特征、生化检查及基因学的研究进行概述。

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  • Result of surgical treatment of hypertrophic obstructive cardiomyopathy with coronary heart disease

    ObjectiveTo summarize the perioperative management strategies and early results of modified Morrow expanded operation and coronary artery bypass grafting (CABG) in patients with hypertrophic obstructive cardiomyopathy (HOCM) and coronary atherosclerotic heart disease.MethodsBetween January 2012 and December 2017, in the Second Inpatient Department of Fuwai Hospital, 32 patients (20 females and 12 males) underwent modified expanded Morrow operation and CABG. The median age was 53.7±8.7 years (interquartile range 37 to 67 years). Preoperative chest distress symptom was found in 24 patients, chest pain symptom was found in 14 patients, history of syncope in 6 patients. Cardiac echocardiography, electrocardiogram, chest X-ray, magnectic resonance imaging (MRI) were performed routinely after operation and follow-up to analyze structure and function of heart and mitral valve.ResultsAll patients underwent modified and expanded Morrow combined with CABG. The preoperative left ventricular outflow tract peak pressure difference (LVOTG) was 40 to 152 (79.6±28.7) mm Hg. Four patients underwent myocardial bridge releasing in the same period, mitral valve replacement in 2 patients, mitral valve angioplasty in 3 patients, Maze operation in 2 patients and tricuspid valveoplasty in 3 patients. There was no hospital mortality. CABG surgery in patients with branches included anterior descending artery in 26 patients, diagonal branch in 16 patients, left circumflex in 8 patients, right coronary artery in 11 patients. There were 15 patients with one coronary artery (CA) bypass graft, 5 patients with two CA bypass grafts, and 12 patients with 3 CA bypass grafts. The average of CA bypass grafts was 1.9±0.6. The postoperative ICU time ranged from 1–13 (4.1±2.8) days and postoperative hospital stay ranged from 7 to 30 (12.6±5.5) days. No severe postoperative complications were found and 1 patient had postoperative incision healing. The postoperative new arrhythmia included left bundle branch block in 6 patients. Compared with the preoperative values, postoperative left ventricular outflow tract peak pressure (79.6±28.7 mm Hg vs. 10.8±5.9 mm Hg, P<0.001), interventricular septum thickness (1.9±0.4 cm vs. 1.3±0.5 cm, P<0.001) were decreased obviously. Mitral valve closure is good or only mild reflux, mitral valve forward movement (SAM sign) disappeared. The patients were followed up for 6-68 months, with an average of 38.8±20.6 months. All patients were followed up with symptoms disappeared or only mild symptoms. NYHA classification decreased Ⅰ to Ⅱ grade after surgery, without long-term mortality, complications or reoperation.ConclusionFor patients with hypertrophic obstructive cardiomyopathy with coronary atherosclerotic heart disease, the application of improved expand morrow operation at the same time undergoing coronary artery bypass grafting is safe. It can significantly improve patients' survival and reduce symptoms, play a synergistic effect, and do not increase the patient's surgical complications.

    Release date:2019-01-23 02:58 Export PDF Favorites Scan
  • Automatic detection model of hypertrophic cardiomyopathy based on deep convolutional neural network

    The diagnosis of hypertrophic cardiomyopathy (HCM) is of great significance for the early risk classification of sudden cardiac death and the screening of family genetic diseases. This research proposed a HCM automatic detection method based on convolution neural network (CNN) model, using single-lead electrocardiogram (ECG) signal as the research object. Firstly, the R-wave peak locations of single-lead ECG signal were determined, followed by the ECG signal segmentation and resample in units of heart beats, then a CNN model was built to automatically extract the deep features in the ECG signal and perform automatic classification and HCM detection. The experimental data is derived from 108 ECG records extracted from three public databases provided by PhysioNet, the database established in this research consists of 14,459 heartbeats, and each heartbeat contains 128 sampling points. The results revealed that the optimized CNN model could effectively detect HCM, the accuracy, sensitivity and specificity were 95.98%, 98.03% and 95.79% respectively. In this research, the deep learning method was introduced for the analysis of single-lead ECG of HCM patients, which could not only overcome the technical limitations of conventional detection methods based on multi-lead ECG, but also has important application value for assisting doctor in fast and convenient large-scale HCM preliminary screening.

    Release date:2022-06-28 04:35 Export PDF Favorites Scan
  • Idiopathic inflammatory myopathies associated interstitial lung disease

    Idiopathic inflammatory myopathies are a group of connective tissue diseases characterized by nonsuppurative inflammation of the striated muscle. At present, the diagnostic criteria for polymyositis/dermatomyositis classification proposed by Bohan and Peter in 1976 is mainly used clinically. In clinical observations, it is found that myopathy involves not only skin and muscle but also affects many systems of the body. Interstitial lung disease occupies an important part, and it is an important cause of death of patients with inflammatory myopathy. Patients with idiopathic myositis should be examined as early as possible by high-resolution CT to improve the detection rate of myositis-associated interstitial lung disease and start treatment as soon as possible. At the same time, the patients with myositis have different clinical manifestations due to specific antibodies in the serum; some specific antibodies may indicate poor prognosis and poor treatment response. Timely screening of patients with positive myositis-specific antibodies in patients with the pulmonary interstitial disease can help the patient’s diagnosis and treatment process.

    Release date:2018-12-24 02:03 Export PDF Favorites Scan
  • Retinal manifestations of mitochondrial encephalomyopathy

      Objective To observe the retinal manifestations and classification of mitochondrial encephalomyopathy,and explore the relationship between retinopathy and systemic manifestations.Method The clinical data of 88 inpatients with mitochondrial encephalomyopathy were retrospectively analyzed,in whom 12 patients(24 eyes)with retinal manifestations who diagnosed by ophthalmology consultation and complete medical records were collected. There were nine males and three females aged from 14 to 33 years with the mean age of(20.1±7.0)years. The disease duration ranged from 2.5 to 20 years,with the mean of(9.5±6.8)years. All the patients had the eye symptoms of the different degree,such as limbs weakness,hearing decline and central nervous system symptoms. Ophthalmologic examination including best corrected visual acuity,slit lampa microscope,indirect ophthalmoscopy,noncontact Tonometer,ptosis,ocular movement,pupillary reflex and color fundus photography. Among the patients,three,one,two and five patients had undergone fundus fluorescein angiography(FFA),optical coherence tomography(OCT),lectroretinogram(ERG)and visual field examination respectively. Diabetic retinopathy were divided into “salt and pepper”, retinitis pigmentosa(RP),retinal pigment epithelium(RPE),choroidal capillary atrophy and simplex optic atrophy according to the inspection results.Results All the patients′ both eyes were involved,the disease degree of bilateral eyes was accordant. The ptosis and(or)eye movement limitation were found in nine patients(75.0%),and decreased visual acuity was in six patients(50.0%).“Salt and pepper” was found in six patients(12 eyes),presenting retinal granular pigmentation and depigmentation;the visual acuity was 0.4-1.2;no central nervous system symptoms were found in patients,such as hearing decline,twitch,ataxia and hypophrenia. RP was found in one patient(two eyes),presenting retinal cells sample pigmentation,retinal vessel shrink,optic atrophy;the vision were light perception in both eyes;hypophrenia,hearing decline,bilateral lower limbs pain and onset twitch were also found in them. RPE and choroidal capillary atrophy were found in three patients(six eyes),the choroidal great vessels and flake pigment accumulation surrounding the retina were observed;the visual acuity was hand movement0.7;limbs weakness was found in two patients;hearing decline was found in three patients;barylalia and hypophrenia were found in two patients;somnolence was found in one patient. Simplex optic atrophy was found in two patients(four eyes);the vision was 0.1-0.7;central nervous system symptoms were found in patients,such as limbs weakness,twitch,hypophrenia and headache.Conclusion Retinopathy types is concerned with visual prognosis and central nervous system symptoms.

    Release date:2016-09-02 05:41 Export PDF Favorites Scan
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