To investigate the features of CT, ultrasonography and fundus fluorescein angiography (FFA) of morning glory syndrome, the data on CT, A/B-scan ultrasonography and fundus fluorescein angiography (FFA) were retrospectively analyzed in 8 cases of morning glory syndrome (MGS). Among those cases, 6 were examined with CT, 4 with FFA and 8 with A/B-scan ultrasonography. Results showed that the characteristics of CT, A/B-scan ultrasonography and FFA in MGS included: (1) The attachment spot of optic nerve became thin and vitreous body protruded to the posterior wall of eyeball with a spherical shape on CT image; (2) in the early period of FFA, hypofluorescence appeared on the optic, the abnormal arteriae and veins around the optic papilla were displayed clearly and in the late period, optic disc was stained with fluorescein; (3) on B-scan ultrasonogram, the vitreous cavity extended to the posterior pole and optic papilla, and projected to the basal part of muscle cones and thus the posterior part of vitreous cavity looked like an upside-down bottleneck. Sometimes the echogenic band of retinal detachment could also be seen. On A-scan ultrasonogram, both vitreous cavity and bottleneck showed no ultrasonic echoes and presented a base line without any evident wave crest. It is concluded that CT, A /B-scan ultrasonography and FFA could show the imageological features of MGS from different aspects, which helps clinicians to differentiate it from other diseases such as optic disc coloboma. CT and A /B-scan ultrasonography, in particular, are considered to be reliable imageological methods for the accurate diagnosis of MGS and are superior to the traditional techniques.

Objective To explore the pathologic changes of the opening blood-brain barrier(BBB) in the early stages of 50% TBSA scalded rabbits.Methods Ten male rabbits inflicted with 50% TBSA of Ⅲ degree were randomized into five groups: control,postburn 1 h,2 h,3 h,4 h.Brain tissues were isolated at different phases and observed in gross,by microscope and transmission electron microscope.Results Endothelial cells swelled and BBB began to open in 2 h after scald.The tight junction of endothelial cells became vague and the opening of BBB increased as lesion time prolonged.Conclusion As the lesion time prolonged,the injury of endothelial cells became more serious and the opening of BBB progressed,which resulted in the formation of brain edema.

In recent years, there is increasing evidence that the pathogenesis of Parkinson's disease (PD) may involve the more generation of reactive oxygen species, and investigations on patients have shown that PD is under a status of oxidative stress. The defense against the toxic effects of reactive oxygen species is an essential task within the brain. An important component of the cellular detoxification of reactive oxygen species is the antioxidant glutathione. Consequently, it seems reasonable to propose that increase of brain concentrations of glutathione including glutathione analogs or precursors could be very effective in diminishing the cumulative effects of oxidative damage, and have been investigated as potential therapeutic targets in the treatment and prevention of PD or other neurodegenerative disease.

Many countries have developed standard strategy under the tendency of the economic globalization.TCM international standardization faces more and more competitions.This article analyzes the competitors and competitive status of TCM international standardization by the research methods of competitive intelligence,competitor analysis and SWOT analysis,and puts forward corresponding strategic measures in details.

To investigate the effects of pyrrolidine dithiocarbamate (PDTC) on the proliferation and PCNA (proliferating cell nuclear antigen) expression of cultured human retinal pigment epithelium cells, human retinal pigment epithelium cells (RPE) were cultured from normal adults who, died accidentally. The effects of PDTC on the proliferation of RPE cells were examined by using methyl thiazlyl tetrazolium (MTT) assay. The effects of PDTC on the PCNA expression of RPE cells were immunohistochemically examined by employing biological image analysis system (BIAS). After treatment with PDTC of various of concentration ranging from 0.062 to 1 g/L for 24 h, or concentrations ranging from 0.031 to 1 g/L, the proliferation of RPE cells decreased in a dose-dependent manner. After treatment with PDTC of concentration varying from 0.062 to 1 g/L for 24 h, the PCNA expression was also suppressed in a dose-dependent manner. It is concluded that PDTC can inhibit the proliferation of RPE cells in vitro in a dose-and time-dependent manner, at least in part, by down-regulating the expression of PCNA. PDTC may be used to prevent and treat the proliferative vitreoretinopathy (PVR).

Abstract: Objective To investigate the clinical phenotype and genotype characteristics of mitochondrial encephalomyopathy (ME) families in children. Methods　The clinical data and genetic test results of eleven ME families who were admitted to the department of pediatrics of three tertiary hospitals in Hainan Province from January 2007 to December 2021 were retrospectively analyzed. Results　A total of 13 cases were diagnosed in eleven ME families, including 6 males (46.15%) and 7 females (53.85%). The age of onset ranged from 6 months to 12 years, the interval from onset to diagnosis was 9 months to 8 years and Morava score was 6-11. Clinical symptoms mainly included abnormal movement, developmental retardation or regression, seizures, stroke-like episodes; among the 13 children, 11 (84.62%) had elevated blood lactic acid and 4 (30.77%) had elevated blood creatine kinase. Cranial MRI mainly involved temporal parietal occipital lobe, cerebellum, brainstem and basal ganglia, some with brain atrophy. Gene detection showed that 8 families (72.72%) were caused by mtDNA mutation, of which 5 families and 6 patients were caused by MT-TL1, m.3243A>G, and 5 asymptomatic carriers of 4 families (80.00%) were detected; MT-ND5, m.13513 G>A was detected in 2 families and 3 patients, and an asymptomatic mutation carrier was detected in a family (50.00%); MT-ND3, m.10191T>C was detected in one family and one patient, and 2 asymptomatic mutation carriers were detected. Three families were caused by nDNA mutations (27.27%). A compound heterozygous mutation of c.751C>T and c.516-2A >G in SURF1 gene was found in one family and one patient, which followed autosomal recessive inheritance. The pathogenic loci were inherited from mother and father, respectively. Two new spontaneous mutations c.1040C>G and c.2060_2062delTAG in DNM1L gene were respectively detected in two families and two patients. All children were given mitochondrial cocktail therapy and symptomatic treatment after diagnosis by genetic testing. Follow-up to June 2022, two families were lost to follow-up and 9 families were followed up regularly; three of the 11 children were still survived. Conclusions　For children diagnosed with ME, genetic testing of family members can screen out early asymptomatic pathogenic mutation carriers, achieve early diagnosis of ME and guide clinical genetic counseling. Two new pathogenic sites of DNM1L gene were found in this study, which expanded the genotype spectrum.

Abstract： Objective　To investigate the clinical characteristics and pathogenic genetic mutation of a case with encephalopathy due to defective mitochondrial and peroxisomal fission-1 (EMPF1). Methods　The clinical data and genetic test results of a patient with EMPF1 admitted to the Department of Pediatrics, the Affiliated Hospital of Xiangya Medical College of Central South University in August 2020 were retrospectively analyzed. Results　An 8-year-old girl, her main clinical features were developmental regression, microcephaly, hypotonia, refractory epilepsy, cranial MRI suggesting brain atrophy and abnormal signals in the right temporal-occipital-parietal cortex, aEEG showing slow wave discharge in the right hemisphere; Whole-exome sequencing of families suggested that the child had a heterozygous missense variant at the c.1040C>G site in the DNM1L gene and the verification results by Sanger sequencing showed that her parents had no variant in this site, which was a novel mutation in accordance with autosomal dominant inheritance; bioinformatics analysis predicted that the mutation was pathogenic. After 2 years of outpatient follow-up, the patient's condition was stable after mitochondrial cocktail therapy and antiepileptic drugs, no epileptic seizure occurred in the past year, mental state and swallowing function improved, and she could be fed orally with occasional nausea and vomiting. Conclusions　The main clinical manifestations of EMPF1 are psychomotor developmental delay or regression, dystonia, limb paralysis, epilepsy and so on. According to the clinical phenotype and genetic test results, the rare disease can be diagnosed early.

Objective Discussing the rationality of psychological intervening therapy in the treatment of early breast neoplasm. Methods By summarizing and reviewing 84 patients suffering from the early breast cancer between January of 2003 and December of 2005, we procured some information of their clinical manifes-tations, clinical therapy and statistic results of psychological assessment. Results By intervening the psycho-logical therapy, the patients' self-efficiency and living quality can be bettered. Conclusion Patients with early breast neoplasm need to have a comprehensive treatment with general or local therapy and individual psychologi-cal intervention. Nevertheless, it is rather practical to enhance the general level of prevention and therapy of breast neoplasm through the research of psychological problems after the operation.

Objective To study the effect of femoral offset reconstruction ON the Harris hip score and the hip ROM (range of motion) after artificial hip replacement in femoral neck fractures. Methods Follow-ups were conducted for 47 patients who had undergone artificial hip replacements for their unilateral femoral neck fractures. We took X-ray pictures to compare the femoral offsets at the affected and unaffected hips. The operated hips were evaluated by the Harris hip score. Statistical analyses were performed with SPSS l2.0 software. Results The Harris hip score of the operated hip was positively related to femoral offset reconstruction(t=7.25,P＜0.001).There was a significant and positive correlation between femoral offset and the hip abductor ROM(r=0.80,P＜0.001).The rate of femoral offset reconstruction had no significant difference between total hip arthroplasty and bipolar hip arthroplasty (χ2=0,P＞0.05).Conclusions Reconstruction of femoral offset after artificial hip replacement in the femoral neck fractures can improve funetional recovery and ROM of the hip. Femoral offset reconstruction is uncorrelated to the selection of total hip arthroplasty or bipolar hip arthroplasty.