Aim To investigate whether Ca2+/calmodulin-dependent kinase Ⅱ(CaMKⅡ)contribute to tumor necrosis factor ?(TNF-?)-induced cardiomyocyte hypertrophy.Methods The protein content was assayed with Lowry's method.The cardiomyocytes volumes were measured by computer photograph analysis system.The protein synthesis was assayed with[3H]-lencine incorporation method.[Ca2+]i transient was measured by Till image system by cell-loading Fura-2/AM.The expression of CaMKⅡ?B was determined by Western blot.Results ① TNF-? significantly induced the increase of protein content,[3H]-leucine incorporation and cell size;These responses were significantly suppressed by KN93,a selective CaMKⅡ inhibitor.② TNF-? increased the amplitude of the spontaneous Ca2+ transients in cultured ventricular myocytes from the neonatal rat;CaMKⅡ inhibitor KN93 can suppress the elevation induced by TNF-?.③ TNF-? significantly increased the expression of CaMKⅡ?B.Concluslon CaMKⅡ signal pathway are involved in TNF-?-induced cardiomyocyte hypertrophy in rats.

Aim To investigate whether Ca~(2+)/calmodulin-dependent kinase Ⅱ(CaMKⅡ)contribute to tumor necrosis factor α(TNF-α)-induced cardiomyocyte hypertrophy.Methods The protein content was assayed with Lowry's method.The cardiomyocytes volumes were measured by computer photograph analysis system.The protein synthesis was assayed with[~3H]-lencine incorporation method.[Ca~(2+)]_i transient was measured by Till image system by cell-loading Fura-2/AM.The expression of CaMKⅡδ_B was determined by Western blot.Results ① TNF-α significantly induced the increase of protein content, [~3H]-leucine incorporation and cell size;These responses were significantly suppressed by KN93, a selective CaMKⅡ inhibitor.② TNF-α increased the amplitude of the spontaneous Ca~(2+) transients in cultured ventricular myocytes from the neonatal rat;CaMKⅡ inhibitor KN93 can suppress the elevation induced by TNF-α.③ TNF-α significantly increased the expression of CaMKⅡδ_B.Concluslon CaMKⅡ signal pathway are involved in TNF-α-induced cardiomyocyte hypertrophy in rats.

A method to assess library-holding journals according to their weighted citations was proposed in light of the actual needs of users for core journals, the use of library-holding journals and references by authors in different orders of precedence.The Western journal of rheumatology, highly cited by authors of Second Military Medical U-niversity, were assessed using this method, showing that this method is better than the citation analysis-based tra-ditional method in assessment of journals.

The information commons concept-based stereoscopic resources support system for academic library with brand-new connotations and its application were proposed according to the increasing development and expansion of informationization, networking and digitization in different social fields, the significant change in disseminating methods of information resources, and the need of readers for library service.

SUMMARY Hemophilia A is aninherited bleeding disorder, lack of coagulation factorⅧ( FⅧ) , and if combined with intracranial malignant tumor, the operation risk is very high. Department of Pediatric Sur-gery in Peking University First Hospital used coagulation factor replacement therapy, succeeded in the operation of 2 cases of intracranial malignant tumor with hemophilia A in children, with no abnormal bleeding events. The establishment of the multi subject cooperation group before operation, good preoper-ative preparation, enough alternative factors, and close postoperative monitoring, are the key to the suc-cessful treatment.

Objective To summarize the experience of the ventriculoperitoneal shunt in treating children with methylmalonic aciduria combined with hydrocephalus,and to assess the clinical value.Methods From September 2012 to May 2016,a total of 12 patients with methylmalonic aciduria combined with hydrocephalus in Peking University First Hospital were enrolled,including 7 boys and 5 girls.All the 12 patients underwent ventriculoperitoneal shunt.Drug therapy was performed after surgery.The clinical manifestations and imaging findings were used as the basis for adjusting the pressure of the diverter valve appropriately.The clinical condition of patients were evaluated retrospectively.The patients' clinical symptoms,signs,imaging materials,surgical complications and postoperative prognosis were analyzed.Results All the cases were followed up for 3 to 36 months,no death case and no serious postoperative complications of hydrocephalus occurred.Clinical symptoms of intracranial hypertension were relieved or disappeared.The head circumference progressive enlargement stopped.The anterior fontanelle tension decreased significantly.Setting-sun sign of eyes disappeared.One out of 4 cases with convulsion and epilepsy was relieved after the operation.Seven cases of poor vision or vision loss,postoperative visual acuity were improved though not recover to normal eyesight.One case of the children with hearing loss,postoperative hearing recovered.During the follow-up period,the head CT showed that the ventricle was narrowed significantly,interstitial brain edema improved obviously.Conclusion Ventriculoperitoneal shunt is a effective method for treating children with methylmalonic aciduria combined with hydrocephalus,which is beneficial for patients with these diseases.

Objective:The clinical manifestations, types of gene mutations, therapeutic effects and prognostic characteristics of 15 children with cblC type methylmalonic acidemia (MMA) and hydrocephalus were analyzed to improve the clinical understanding of the disease, so as to provide a basis for the treatment of the disease.Methods:From April 2015 to January 2019, 15 patients with MMA and hydrocephalus in Department of Pediatric Surgery, Peking University First Hospital were enrolled, and all gene detection showed clbC type.All the 15 patients underwent ventriculoperitoneal shunt.After surgery, the clinical manifestations and imaging findings were applied as the basis to adjust the pressure of the diverter valve appropriately.Clinical data and gene mutation characteristics of 15 children with cblC type and hydrocephalus were retrospectively analyzed, and the therapeutic effects and prognosis were summarized and analyzed as well.Results:There were 8 males (53.3%) and 7 females (46.7%), aged from 2 to 33 months.All the cases were followed up from 11 to 55 months, without death case and serious postoperative complications of hydroce-phalus.The head circumference of 3 cases (20.0%) was in the normal range, 1 case (6.7%) was greater than the normal range, and 11 cases (73.3%) were less than the normal range.Four patients (26.7%) were transferred to the pediatric intensive care unit after surgery.c.609G>A mutation was the most common in this study, with 7 cases (46.7%) of c. 609G>A homozygous mutation, and 5 cases (33.3%) of c. 609G>A heterozygous mutation.Clinical symptoms of intracranial hypertension were relieved or disappeared.The head circumference progressive enlargement was stopped.The anterior fontanelle tension greatly decreased, all " setting-sun" sign of eyes disappeared, and vision loss and hearing loss were better compared with the pre-operation.Four cases (26.7%) displayed normal intelligence and exercise, and 11 cases (73.3%) were left with mild to severe psychomotor retardation.During the follow-up pe-riod, the head CT showed that the ventricle was remarkably narrowed, and interstitial brain edema obviously improved.Conclusions:Ventriculoperitoneal shunt in the treatment of cblC type MMA with hydrocephalus has positive effects.The head circumference of most cblC type MMA with hydrocephalus is less than the normal range.c.609 G>A is the most common mutation in cblC type MMA with hydrocephalus.Perioperative " metabolic crisis" can result in serious complications.

OBJECTIVETo study in vitro and in vivo protein expression and biological function of gene pp1158, a hepatocellular carcinoma (HCC)-related gene.

METHODSpp1158 was expressed with fusion expression vector pET-32a in E. Coli-BL21 (DE3), and rabbit anti-pp1158 fusion protein polyclonal antibody was prepared. The biological function and differential expressions of pp1158 were studied by in vitro colony formation assay nude mouse in vivo tumor formation assay of transfected BEL7402 cell line and immunohistochemistry and Western blot. Differential expression of pp1158 in human fetal tissues were examined by Northern blot.

RESULTSIn vitro experiment showed that pp1158 inhibited colony formation rate of transfected BEL 7402 cells, with an inhibition rate of 58.3% (P < 0.01). Tumor formation assay indicated that tumor formation of pCMV-Script-1158 transfected BEL 7402 cell line was significantly inhibited (P < 0.05) as compared with that of the control group. Immunohistochemical assay showed that pp1158 was expressed in human tissue in the following sequence: normal liver >/= noncancerous liver tissue > HCC. Western blot indicated that a 60kD protein (pp1158 protein) was expressed in BEL 7402 cells transfected with pCMV-Script-pp1158 DNA, while it was detected in BEL 7402 cells transfected with pCMV-Script vector DNA. Northern blot showed pp1158 was expressed in the placenta at a very high level, heart, liver, muscle, pancreas and lung but expressed poorly in the brain and kidney.

CONCLUSIONpp1158 is a new candidate tumor suppressor gene of HCC.

Angiopoietin-like 4 Protein ; Angiopoietins ; Animals ; Blotting, Northern ; Blotting, Western ; Carcinoma, Hepatocellular ; genetics ; metabolism ; pathology ; Female ; Gene Expression Regulation, Neoplastic ; Genes, Tumor Suppressor ; Humans ; Intercellular Signaling Peptides and Proteins ; Liver Neoplasms ; genetics ; metabolism ; pathology ; Male ; Mice ; Mice, Inbred BALB C ; Molecular Sequence Data ; Neoplasm Proteins ; genetics ; metabolism ; Neoplasm Transplantation ; Neoplasms, Experimental ; genetics ; metabolism ; pathology ; Proteins ; genetics ; RNA, Messenger ; genetics ; metabolism ; Transplantation, Heterologous ; Tumor Cells, Cultured

Objective To analyze the categories of co-existed nervous system anomalies in children with meningomyelocele in order to consummate the treating strategy for meningomyelocele.Methods Sixty-nine children who had complete clinical data diagnosed as meningomyelocele in Department of Pediatric Surgery,Peking University First Hospital from Jan.2005 to Jun.2013,were retrospectively analyzed.Preoperative spinal MRI was routinely performed.Ultrasonagraphy,CT,MRI or combination of the 2 measures were adopted to evaluate the brain.Microscopically excision of the sac,exploration of the spinal canal,brisement of the tethered cord and meningomyelocele repair were routine operation procedures.Other lesions detected during operation were treated accordingly.MRI findings and intraoperative findings were also compared.Results Preoperative spinal MRI found 59 cases of cone bit,24 cases of syringomyelia,20 cases of mild syringomyelus,14 cases of diastematomyelia,6 cases of intraspinal cyst,6 cases of intraspinal lipoma.Nineteen cases of hydrocephalus were diagnosed by iconography,among which 5 cases complicated with caudal herniation of cerebellar tonsils.Seventeen cases of diastematomyelia,8 cases of intraspinal lipoma,6 cases of intraspinal cyst were detected and treated during operation.Three cases of intraspinal lipoma and 3 cases of osseous diastematomyelia were ignored by preoperative MRI reports.Two cases of intraspinal epidermoid cyst were misdiagnosed as general cyst.One case of teratoma who was not found on MRI was detected by operation and resected.Syringomyelus or syringomyelia was found in all the children complicated with hydrocephalus.Sixty-one cases(88.4％) among the 69 cases complicated with other nervous system anomalies.All of patients were followed up for 4-71 months,averaged 23.8 months.All of patients had no new symptom.Conclusions Children with meningomyelocele often complicate with multiple nervous system anomalies.Preoperative brain and entire spinal iconography,together with thorough intraoperative exploration,are very important so as to avoid omission the co-existed lesions during operation.

In the past 30 years, with the advancement of functional neurosurgery, neuroelectrophysiology and neuroimaging, deep brain stimulation (DBS), as a new tool for the treatment of dyskinesia, has been considered to have underwent the fastest development in this field.Many patients with dyskinesias have significantly improved their main clinical symptoms after treatment with DBS, some of the improvement are even dramatic.Due to its minimally invasive characteristics, reversibility and adjustability, DBS therapy has been increasingly used in the treatment of dystonia in children.Hereditary dystonia is the most common type of dyskinesia in children, and there is no effective treatment yet.Recently, some dyskinesia at home and abroad centers have carried out DBS treatment for pediatric hereditary dystonia and achieved some encouraging results.Now, the effect of DBS in the treatment of hereditary dystonia in children and the main process of DBS treatment were mainly discussed, and shared the experience based on the clinical practices of Multidisciplinary Collaborative Diagnosis and Treatment Center for Children′s Motor Disorders, Peking University First Hospital.