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 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 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 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.

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 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.

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

[Objective] To explore the effect of early treatment of Chinese herbal medicine on the long-term prognosis of acute myocardial infarction (AMI) . [Methods] One hundred and fifty-seven AMI patients were divided into two groups according to the treatment during the hospitalization: 129 patients treated with Chinese herbal medicine and western medicine were in group A, and 28 patients treated with western medicine only were in group B. Statistical analysis of age, sex, infarction location, complications and medical history was made in all of the patients. A follow-up survey was made to investigate the subsistence of the patients and the incidences of all the events (including death and severe cardio-cerebrovascular events) . [Results] With the death as the end event, the survival graph of group A was higher than group B (P=0.1166); when with all the severe events as the end event, the survival graph of group A was still higher ( P=0.048) .[Conclusion] The probability of incidences of severe events including death in group A is lower than that in group B.

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.

The pharmacokinetics and content of 3H11-8H-HPD in tumor, liver, skin and muscular of tumor-bearing nude mice were reported and compared with free 3H-H'PD. The conjugated molar ratio of 3Hll-3H-HPD was 1 : 27. The 97% antibody activity of conjugate was retained. After 3Hll-3H-HPD or 3H-HPD iv into tumor-bearing nude mice, the pharmacokinetics parameter of blood-drug concentration indicated that the T1/2 of conjugate group was longer than that of 3H-HPD, the elimination rate constant ( Kel ) of conjugat group was larger than that of 3H-HPD group, but the aparent volume of distribution ( Vd ) of the former was less than that of the latter. Tissue distribution study reveald that the radioactivity in tumor tissue of conjugate was increased average 0.9 fold than that of 3H-HPD group. Tumor/liver ratio in both 3Hll-3H-HPD group and 3H-HPD group were 0.66 ? 0.24 and 0.42?0.20, respectively. Contents of 3Hll-3H-HPD in skin or muscular were decreased average 1 fold than that of free 3H-HPD, respectively. The study result was shown the pharmacokinetics characteristic of large molecule conjugate and the targeting action of monoclonal antibody.