0bjective To determine the effect of 2 therapies(early realignment versus delayed open urethroplasty)for posterior urethral injuries associated with pelvic fractures. Methods A sys-tematic review of all the relevant clinical trials was performed.Trials were identified from specialized trials register of Cochrane UGDP Group,the Coehrane library,additional electronic search(mainly MEDLINE、EMBSAE、CBM)and manual searching.Clinical trials comparing early realignment and delayed open urethroplasty of posterior urethral disruption associated with pelvic fractures were includ-ed.Statistics analysis was managed by using Review Manager 4.2. Results Ten clinical trials inclu-ding 470 patients were included.Meta-analysis indicated that odds ratio(OR)of the rate of urethral stricture was 0.27[95%confidence interval(95%CI)0.08,0.86,P=0.03].The OR of the percent-age of strictures needing urethroplasty was 0.25,95%CI 0.08,0.74,P=0.01. Conclusions Early realignment may provide better outcomes than delayed open urethroplasty after posterior urethral dis-ruption.It can decrease urethral stricture formation and the percentage of strictures needing urethro-plasty.But the results might be influenced by the bias of grouping,especially the severity of injuries.

The clinical, neuroelectrophysiological, neuropathological characteristics and outcome of a case of acute sensory axonopathy are reported. The patient was a 32-year-old female. She presented with acute onset sensory abnormalities, sensory ataxia, pseudoathetosis and areflex. Lab testing showed cerebrospinal fluid albumin cytologic dissociation. Electromyography studies and sural nerve pathology were consistent with acute sensory axonopathy. Treatment including intravenous immunoglobulin and steroid therapy was provided. Her symptoms recovered slowly without relapse, so did tibial H-reflex amplitudes.

Objective:To observe the protective effect of Yulangsan polysaccharide ( YLSP) on liver injury induced by cyclophos-phamide(CTX) in mice. Methods:Liver injury induced by CTX in mice was used as the animal model and the mice were randomly di-vided into the normal group, CTX model group, biphenyldicarboxylate ( BPDC) group, YLSP group respectively with high, medium and low dose. Except the normal group, the other groups were injected with CTX, i. p. , for 7 days to make the model. Then the ani-mals in the YLSP groups were intragastrically administered with YLSP for 7 days. The activities of alanine aminotransferase( ALT) , as-partate aminotransferase( AST) in serum, malondialdehyde( MDA) , superoxide dismutase( SOD) , glutathione( GSH) and glutathione peroxidase ( GSH-Px) in liver tissue were investigated. Hematoxylin and eosin ( HE) stain was used to study the changes in hepatic tissue of the pathological mice. Results:Compared with the model group, YLSP could obviously reduce the activities of ALT, AST and the content of MDA, and increase the content of GSH, SOD and GSH-Px (P<0. 05 or P<0. 01). HE staining showed that YLSP had significant protective effect on liver injury induced by CTX. Conclusion:YLSP has protective effect on liver injury induced by CTX.

AIM:To observe the effect of B-HT933, a selective α2-adrenoceptor agonist, on lipopolysaccha-ride ( LPS )-induced TNF-αproduction in neonatal rat cardiomyocytes and to explore the underlying mechanisms . METHODS:The neonatal rat cardiomyocytes were cultured .The localization of α2A-adrenoceptor in the cardiomyocytes was examined by immunofluorescence staining .The cardiomyocytes were exposed to LPS or/and B-HT933 for different time.The level of TNF-αin the supernatants and the mRNA expression of TNF-αwere detected by ELISA and real-time PCR, respectively.In addition, LPS-associated signal molecules in the cardiomyocytes were also examined by Western blotting.RESULTS: Immunofluorescence staining showed that α2A-adrenoceptors were localized in the cardiomyocytes . LPS stimulated TNF-αproduction in the cardiomyocytes in a dose and time-dependent manner .B-HT933 pretreatment sig-nificantly inhibited the expression of TNF-αat mRNA and protein levels in LPS-treated cardiomyocytes .Furthermore, LPS exposure induced IκBαand p38 phosphorylation in cardiomyocytes and only IκBαphosphorylation was prevented by B-HT933 treatment.CONCLUSION:α2A-adrenoceptors are present in neonatal rat cardiomyocytes and its agonist B -HT933 inhibits LPS-induced TNF-αproduction in cardiomyocytes via suppressing IκBαphosphorylation .

OBJECTIVE: To explore the clinical, neuropathological and genetic characteristics of a patient with Perrault syndrome caused by TWNK mutation. METHODS: Potential variation of the TWNK gene was detected by next-generation sequencing (NGS) and verified by Sanger sequencing. RESULTS: The patient has featured primary amenorrhoea and progressive sensorineural hearing loss since childhood. She also had gait anormaly, distal limb atrophy and weakness, and nystagmus. Further study confirmed sensory neuronopathy accompanied with upper and lower motor neuron involvement as well as cerebellum atrophy. NGS has identified two heterozygous variants of the TWNK gene, namely c.794G>A (p.Arg265His) and c.1181G>A (p.Arg394His). Sanger sequencing confirmed that c.1181G>A (p.Arg394His), a known pathogenic variant, was derived from her farther, while c.794G>A(p.Arg265His), a novel variant, was derived from her mother and likely pathogenic according to the ACMG guidelines. CONCLUSION Perrault syndrome is a group of disorders with a high phenotypic heterogeneity. The compound heterozygous variation of c.794G>A (p.Arg265His) and c.1181G>A(p.Arg394His) of the TWNK gene may underlie Perrault syndrome in the patient.
Child ; Female ; Genetic Testing ; Gonadal Dysgenesis, 46,XX ; Hearing Loss, Sensorineural ; Humans ; Pedigree