Objective To investigate the clinical and pathological features of Miyoshi myopathy(MM) with dysferlin protein deficient. Methods The clinical and pathological data of the 3 patients with MM were analysed. Results 3 patients were onset at youngster.The clinical manifestation were myastheria and myoatrophy in distal of lower limbs.1 case combined myalgia and tumefaction in lower limbs at early stage of onset;1 case showed myathenia in proximal of lower limbs.The level of serum creatine phosphokinase (CK) was significantly ligher in the 3 cases (7543 IU/L, 5657 IU/L, 8721 IU/L respectively). The level of serum lactic dehydrogenase (LDH) was significantly higher in the 2 cases (456 IU/L ,636 IU/L respectively).The result of muscle pathology was showed myogenic damage in all the cases. The expression of dysferlin protain in membrane of muscle cells was completely deficient, although the expression of dystrophin was normal. Inflammatory cells infiltration was found in 1 case's muscle tissue. Conclusions The clinical characters of MM patient are onset at youngster,myasthenia and myoatrophy in lower limbs.The deficit of dysferlin protain can be found by pathology.

Objective To study the expression of intercellular adhesion molecule-1 (ICAM-1), interleukin-1? (IL-1?) and ultrastructure changes in capillaries and to explore whether capillaries may play a role in the pathogenesis of polymyositis (PM). Methods Ten cases of patients with PM were collected. Control group 1 consists of 6 patients with other myopathies,Control group 2 consists of 2 muscle specimens without clinical and histological evidence of a neuromuscular disorder. An immunohistochemical technique was applied to indentify the expression of ICAM-1 and IL-1? in capillaries in the PM group, Control group 1 and Control group 2. The first analysis was a qualitative assessment. For the second analysis, the number of positive mononuclear cells and capillaries of the whole tissue section from each patient and control was estimated for all the immunologic markers investigated. 10 PM patients were also examined by electron microscopy in order to observe capillary changes in muscles. Results We found that the capillaries in tissues from PM (median (64?20)/mm~2 of tissue, ranging 44—106/mm~2) were significantly less than those from controls (median (157?46)/mm~2 of tissue, ranging 93—213/mm~2) (t=-4.523, P=0.003). A significantly higher number of capillaries expressed IL-1? in the PM group than in the Control group 1(median 15/mm~2 of tissue (ranging 6—46/mm~2) vs 3/mm~2 of tissue (ranging 0—16/mm~2)) (t=3.721, P=0.003). There was no significant difference between PM group and Control group 1 in the number of capillaries expressing ICAM-1 (t=0.529,P=0.617). However, ICAM-1 expressions in endothelial cells of capillaries were stronger in PM patients as IL-1?. Electronic microscopy found capillary changes in 6 PM patients, showing as thickening and duplication of basement membrane, swelling of endothelial cells, stenosis or occlusion of vessels.Conclusion Our findings indicate that capillary changes might take a role in the pathogenesis of PM.

Allelic Imbalance ; Genetic Techniques ; Humans

Objective To analyze the clinical features of pandrug-resistant Acinetobacter baumannii (PDR-Ab) in a hospital and compare the efficacy of different antibiotic treatments on patients with pneumonia caused by PDR-Ab.Methods Data were ret- rospectively collected from all isolated PDR-Ab strains in our hospital from February 2004 to March 2005.The clinical features and outcomes were reviewed.Results A total of 77 strains of PDR-Ab were collected, 45 of which were pathogens causing clini- cal infections (35 strains from lower respiratory tract, 6 from bloodstream, 3 from drainage fluid, and 1 from wounds).Lower respiratory tract was the most common source of PDR-Ab.More than 90% of the isolated PDR-Ab strains produced OXA-23 type?-lactamase.Cefoperazone-sulbactam plus minocyeline showed good efficacy for patients with PDR-Ab pneumonia.The total clinical cure rate was 68.4%.Bacterial eradication rate was 42.1%.The factors influencing bacterial clearance were pro- longed mechanical ventilation prior to positive culture (17.5 d vs 5.5 d).mixed infection (100% vs 12.5%) and lower GCS score (9.1?0.7 vs 13.2?2.1).Concomitant septic shock (OR=13.8) and APACHEⅡscore (OR=2.1) were independent factors of clinical outcome.Conclusions Nosocomial infections caused by PDR-Ab are not untreatable.Our analysis suggests that cefoperazone-sulbactam plus minocycline may be an effective treatment for lower respiratory tract infections caused by PDR-Ab in our hospital.

Objective To investigate the effect of neonatal mechanical ventilation on lung function and recurrent respiratory diseases of patients.Methods Lung function and recurrent respiratory diseases were studied in 16 children who had been ventilated at birth and were followed up in 8 years later.Results Fifty percentage of them had recurrent respiratory diseases and 75 % of had lung function abnormalities, in which residual volume/ total lung capacity (RV/ TLC) was 56.25 %,43.7 % (Vmax 25),specific airway resistance (sRaw) and specific airway conductance (sGaw) 50 % respectively. The forced expiratory flow during 25 %-75 % of forced vital capacity (FEF 25 %-75 %) and maximal expiratory flow rate at 50 % FVC (Vmax50) in the high peak respiratory pressure (PIP) group were lower than those in low PIP group (P

Objective: To investigate the mRNA expression of chemokine receptors in human villi and trophoblasts of first trimester gestation . Methods: The authors first obtained villous tissues from fifteen women who had undergone selective termination at 5 - 10 weeks of normal gestation. Total RNA was then extracted, using the TRIzol reagent, from villous tissues or Percoll-gradient purified trophoblasts. Consequently, the expressions of chemokine receptors in villous tissues and trophoblasts were investigated by way of semi-quantitative reverse transcriptase-polymerase chain reaction.Results: The chemokine receptors, CXCR4 and CXCR6, were highly expressed in each villous tissue, while the CCR6, CCR7, XCR1 and CX3CR1 were moderately expressed in villi. The chemokine receptors, CCR1- CCR5, CCR8 - CCR10, CXCR1 -CXCR3, were expressed only in some villous samples, while no CXCR5 mRNA was found in any villous tissue. The authors also found that the freshly isolated and Percoll-purified trophoblasts expressed CCR1, CCR3 - CCR5, CCR8 - CCR9, CXCR1 - CXCR4, CXCR6, XCR1 and CX3CR1 mRNA. Conclusion: A variety of chemokine receptors were expressed in villous tissues and trophoblasts of human first trimester gestation, hence, these receptors may play an important biological role at the materno-fetal interface in normal human pregnancy.

Bone Cysts, Aneurysmal ; diagnostic imaging ; pathology ; surgery ; Cartilage Diseases ; diagnostic imaging ; pathology ; surgery ; Female ; Hamartoma ; diagnostic imaging ; pathology ; surgery ; Humans ; Infant ; Mesoderm ; diagnostic imaging ; pathology ; surgery ; Nasal Cavity ; diagnostic imaging ; pathology ; surgery ; Neoplasm Recurrence, Local ; Nose Diseases ; diagnostic imaging ; pathology ; surgery ; Tomography, X-Ray Computed

Objective To study the association between clinical symptoms,psychosomatic factors and autonomic nervous function in patients with gastroesophageal reflux disease (GERD).Methods Thirty-four patients with GERD diagnosed by reflux disease questionnaire (RDQ) and endoscopy and 15 healthy control subjects were enrolled in this study.All the subjects were divided into two groups,one with normal scores of Zung's self-rating anxiety scale (SAS) and Zung's depression scale (SDS) as GERD (-) and the other with abnormal scores of SAS and SDS as GERD (+).Reflux symptom score was recorded for both groups at the same time.Autonomic nervous function was assessed by their heart rate variability (HRV) on dynamic electrocardiogram (DCG).The time domain parameters analyzed included standard deviation (SD) of average R-R interval during 24 hours (SDNN),SD of average 5-minute sinus heart rate (SDANN),mean square root of the difference of adjoining two R-R interval (rMSSD),and proportion of the heart beats with difference of R-R interval more than 50 ms from the total heart beats (PNN 50),and the frequency domain parameters analyzed included low-frequency (LF),high-frequency (HF) and ratio of LF to HF.Results Average scores of SAS and SDS were significantly higher in patients with GERD than those in healthy controls (48?9 vs 38?6 and 48?11 vs 41?6,respectively,P

Objective:To evaluate the efficacy and safety of multimodal endovascular therapy for acute ischemic stroke in elderly patients.Methods:A total of 263 elderly patients with acute ischemic stroke were enrolled as research subjects from January 2017 to December 2018.Patients were divided into two groups: the intravenous thrombolytic therapy group and the endovascular therapy group.Treatment outcomes were compared by using the National Institutes of Health Stroke Scale(NIHSS)and the modified Rankin Scale(mRS).Results:Of 263 patients, 125 were in the intravenous thrombolytic therapy group and 138 were in the intravascular therapy group.The rate of good/excellent outcomes was higher in the endovascular treatment group than in the intravenous thrombolytic therapy group(97.8% vs.91.2%, χ2=5.713, P<0.05). The NIHSS score and mRS score were lower in the endovascular treatment group than the in intravenous thrombolytic therapy group[(2.3±0.3) vs.(6.0±1.8), (1.1±0.1) vs.(2.3±0.3), t=9.067 and 16.970, P<0.05]. There was no significant difference in the incidence of adverse reactions between the two groups(8.6% vs.8.0%, χ2=0.041, P>0.05). Conclusions:Multimodal mechanical thrombus retrieval is a safe and effective treatment for intracranial large vessel occlusion in acute ischemic stroke patients and should be recommended and promoted.

Objective To investigate the clinical manifestions, pathological features and diagnosing methods of Lafora disease. Methods The chnical and pathological features of 5 patients with Lafora disease who were diagnosed by axillary biopsies were systemically studied. The specimen were stained by HE, PAS and AB-PAS methods. Results Four of 5 cases had an onset during adolescence and 1 during adulthood. All cases presented with progressive generalized tonic-clonic seizure, myoclonus and dementia. Emotional disturbance, dysarthria and ataxia appeared in the early course of the disease. Lafora bodys were identified in myoepithelial cells and duct cells of both eccrine sweat glands and apecrine sweat glands in the biopsies of axillary skin. Conclusions Lafora disease could be confirmed by round and oval periodic acid-Schiff- positive inclusions in skin biopsy specimen combined with the proper clinical settings. Both axillary and other skins can be chosen as the sites of biopsy.