Objective To investigate pathogenesis, clinical and pathological feature of a case of nemaline myopathy and review of relevant researches. Methods A ease of an 8-year-old girl with muscle weakness and her clinical presentation, family history, changes of serum enzymology and EMG, characteristic of light and electron microscopic studies were described. The earlier literature and new genetic findings concerning these muscle abnormalities are also briefly summarized. Results Nemaline myopathy is diagonosed by clinical manifestation and observed result of electron microscopy. Conclusions Muscle biopsy is the only way to diagnose nemaline myopathy and electron microscopy plays an important role in diagnosing it.

Objective To observe the neuroprotective role and its mechanism of estrogens in male Sprague-Dawley rats with focal cerebral ischemia. Methods The rats were randomly divided into three groups: E2-pretreat group, E2+TAM (tamoxifen)-pretreat group and control group. The models of cerebral ischemia were maded by occlusion middle cerebral artery using an intraluminal filament method. 2 and 24 hours after focal cerebral ischemia, the neurological deficit scores and the death rate of rats were evaluated. 2,3,5-triphenyltetrazolium chloride (TTC) stain was used to assess the volume of infarction. Terminal transferase dUTP nick ending labeling (TUNEL) and immunohistochemistry were carried to observe neuron apoptosis and the expressions of Bcl-2, Bax and p53. Results Compared with control group, the volumes of infarction, death rates, neurological deficit scores and the numbers of neuron apoptosis both at 2 and 24 h were significantly decreased in E2-pretreated group (all (P

Objective:To explore features and relevant influencing factor of ecological executive function in children with childhood absence epilepsy (CAE).Methods:Forty children with CAE (CAE group) and 40 healthy children with physical examination (control group) from April 2017 to July 2020 in Anhui Provincial Children′s Hospital were selected. The behavior rating inventory of executive function (BRIEF) parental questionnaire was used to evaluate the executive function of children. The differences of ecological executive function between 2 groups were compared.Results:The BRIEF total score, behavioral regulation index (BRI) and metacognition index (MI) in CAE group were significantly higher than those in control group: (52.03 ± 10.89) scores vs. (44.05±5.06) scores, (49.45 ± 9.93) scores vs. (43.85 ± 4.70) scores and (53.18 ± 11.24) scores vs. (44.95 ± 5.32) scores, and there were statistical differences ( P<0.01). Multiple stepwise regression analysis result showed that inhibit inhibition, shift, emotional control, initiation, working memory, planning, organization, monitoring, MI and total score were correlated with disease control ( P<0.01), and disease control had a negative predictive effect on them ( R2 = 0.174, 0.158, 0.234, 0.325, 0.383, 0.337, 0.378, 0.199, 0.463 and 0.435); BRI was correlated with onset age and disease control ( P<0.01 or <0.05), and onset age and disease control had a negative predictive effect on BRI ( R2 = 0.336). Conclusions:Children with CAE have ecological executive dysfunction. The control of the disease and the onset age are the main factors affecting the ecological executive function.

Objective:To investigate the expression of CXCL1 2 and CXCR4 in adenoid cystic carcinoma(ACC)and to explore its re-lationship with clinicopathologic characteristics and prognosis of the patients.Methods:The expression of CXCL1 2 and CXCR4 in af-fected tissue was detected immunohistochemically in 62 cases of ACC.Both of the two factors and clinicalpathology factors were pro-cessed in accordance with the Kaplan-Meier method and the COX regression model.Results:The positive rates of CXCL1 2 and CX-CR4 expression were 54.8%(34/62)and 77.42%(48/62)respectively.Patients with the 2 factor expression had a shorter survival time than those without them(P<0.05).Multivariate analysis revealed that CXCR4 expression,clinical stage,histological differentia-tion and metastasis/recurrence were independent risk factors for the prognosis of ACC patients.Conclusion:The expression of CXCR4 may be correlated with the malignancy of ACC.CXCR4 expression,clinical stage,metastasis/recurrence and histological differentia-tion can indicate the prognosis of ACC patients.

After percutaneous coronary intervention (PCI) was performed,since CHD risk factors still exist, coronary restenosis rate remains high.Therefore, self-management after PCI is very important.The present article made a review on current condition and research progress of self-management in patients after PCI, aiming at providing reliable evidence for rehabilitation after PCI.

Objective To assess the efficency and safety of non-peritoneal drain versus peritoneal drain after appendectomy of severe appendicitis. Methods Randomized controlled trials (RCTs) or quasi-randomize controlled trials(QRCTs) were searched and identified from CNKI (1994 to Oct 2008) ,CBM (1978 to Oct 2008), VIP (1989 to Oct 2008), Wanfang Data (1997 to Oct 2008), MEDI.INE (1966 to Oct 2008), EMBASE (1974 to Oct 2008),The Cochrane Library (issue3, 2008) and SCI (1974 to Oct 2008), and related journals were also scanned. We evaluated the quality of the included studies by Jadad scale and analyzed the data by Cochrane Collaboration' s RevMan 5. 0. Results We included 15 randomized controlled trials or quais-randomized controlled trials (n = 2809). Meta analysis showed that there were statisticly differences between two groups on the incidence of wound infection [OR = 0.43,95%CI (0.29,0.65)], postoperative intestinal adhesion [OR = 0.26,95%CI(0. 18,0.37)]and the duration of hospital stay [WMD = - 0.38,95%CI(- 4.96, - 1.20)], but no difference was found on the incidence of abscesses [OR = 0. 77,95% CI(0. 39, 1. 51)]. Conclusions The current evidences show that contrast with peritoneal drain, the non-peritoneal drain can significantly reduce the incidence of wound infection and intestinal adhesion, and shorten the duration of hosipital stay. Before draw the conclution into clinical practice, further high-quality, large scale, double-blind randomized controlled trials are still needed.

A 19-year-old Chinese woman presented with progressive ulcers and recurrent fever for 1 year.The lesions originally began with purplish red maculopapules,then rapidly developed into ulcer with severe pain and spread to the trunk and extremities accompanied by intermittent fever.Histopathology revealed lymphoid infiltration of middle and large atypical T cells throughout the dermis and subcutaneous fat laver along with focal epidermotropism.The lymphoid infiltrates were positive for CD3,CD8,T-cell intercellular antigen(TLA)and T-cell receptor β(TCR-β).Gene analysis showed the rearrangement of T-cell receptor gene.According to the above findings,the patient was diagnosed as primary cutaneous aggressive epidermotropic CD8-positive cytotoxic T-cell lymphoma. Combined thempy with cyclophosphamide,vincristine,prednisone and bleomycin resulted in partial remission of skin lesions,but she eventually died 22 months after the onset of disease.

Objective To develop a sensitive and reliable RP-HPLC method for the simultaneous determination of nine nucleosides including uracil, cytidine, guanine, uridine, adenine, guanosine, thymidine, adenosine and 2'-deoxyadenosine from Fritillaria taipaiensis P. Y. Li that had been cultivated in different producing areas; To compare the contents of these nucleosides from different producing areas. Methods The analysis was performed on a Venusil MP C18 (2) column (4.6 mm×250 mm, 5 μm) with a gradient of methanol-water at a flow rate of 1.0 mL/min;the detective wavelength was set at 260 nm; the column temperature was set at 35 ℃. Results Uracil, cytidine, guanine, uridine, adenine, guanosine, thymidine, adenosine and 2'-deoxyadenosine were obtained in the good linear range of 0.269 5–16.17 μg/mL, 0.132 1–7.927 5 μg/mL, 0.095 5–5.73 μg/mL, 1.16–69.6 μg/mL, 0.48–28.8 μg/mL, 0.571 5–57.15 μg/mL, 0.526–52.6 μg/mL, 3.307 5–198.45 μg/mL, 0.530 5–31.83 μg/mL, respectively (r≥0.999 5);the recovery was in the range of 96.49%–101.65%(RSD≤2.92%). Conclusion The contents of the nine nucleosides from different producing areas have differences. Fritillaria taipaiensis P. Y. Li from Xianyi Village, Chengkou County, Chongqing City, whether the cultivated ones or wild ones, contain the highest level of nucleosides. The established method can provide references for the perfection of quality standard for Fritillaria taipaiensis P. Y. Li.

Adult ; Endocarditis ; complications ; Endocarditis, Bacterial ; complications ; Heart Defects, Congenital ; complications ; Humans ; Male ; Pulmonary Valve ; microbiology

Objective To examine aortic dissection by using T1W/TFE sequence and to observe the imaging characteristics of aortic dissection in T1W/TFE sequence. Methods Twenty patients confirmed as aortic dissection by CT and ultrasound received MRI scanning of T1W/TFE sequence on inclined sagittal and SE sequence on transaxial at Philips Gyroscan 1.5 MR imager. The signal changes of aortic dissection in T1W/TFE sequence were observed. Results In T1W/TFE sequence, we observed not only an intimal flap, a double lumen and range of aortic dissection, but also the site of intimal tear and thrombus in the false lumen. Conclusion By using T1W/TFE sequence we obtained the high quality image of aortic dissection and high contrast of imaging. Combined with SE sequence , T1W/TFE sequence can provide reliable information for analyzing and curing aortic dissection in clinic.