Analysis of gene characteristics and therapeutic effect of cblC type methylmalonic acidemia with hydrocephalus / 中华实用儿科临床杂志

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.

Synthesis and cytotoxicity study of cembrane triazole derivatives / 中国药科大学学报

A series of triazole derivatives were designed and synthesized based on a natural product cembrane separated from Croton laevigatus Vahl which showed potential antitumor activity against HeLa cells.Twelve novel compounds were synthesized and their structures were characterized by 1H NMR, 13C NMR and HRMS.Their cytotoxicities in vitro were evaluated for HeLa,K562 and K562/A02 cells by MTT assay.The results showed that some cembrane derivatives possessed antitumor activities.Substituted triazole connected to cembrane derivatives exhibited potent activity toward drug-resistant K562/A02 cells.

Methylmalonic aciduria combined with hydrocephalus treated with ventriculoperitoneal shunt / 中华实用儿科临床杂志

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.

A comparative study of anterolateral thigh perforator flap with and without sensory nerve graft for sensation reconstruction in head and neck region / 中华显微外科杂志

Objective To compare the operation effects of sensation restoration after tumor resection in the head and neck defects reconstruction by anterolateral thigh flap with or without sensory nerve transplantation.Methods The application of 76 cases anterolateral thigh flaps in head and neck defects reconstruction after tumor resection from May,2011 to May,2015 were observed.35 patients underwent simultaneous reconstruction with sensory nerve anastomosed perforator based anterolateral thigh flap and 41 patients underwent simultaneous reconstruction without sensory nerve graft based anterolateral thigh flap,which was named as group A and group B,respective ly.The treatment effectiveness of the two groups was compared and graded into First,Second and Third rate.Results All the 76 flaps survived.Satisfying morphology for the maxillofacial region and stage-one healing for the donor site was available.During the 24-week follow-up,group A were 65.71％ (First rate),28.57％ (Second rate),5.72％(Third rate) respectively,and the condition of group B were 31.70％,26.84％,41.46％.The treatment effectiveness of group A was better than that of group B.Postoperative complications were less than group B(P ＜ 0.05).Conclusion Compared with the loss of nerve transplantation of anterolateral thigh perforator flap,sensory nerve anastomosed perforator based anterolateral thigh flap is of great value to reconstruct the defect after resection of tumor in head and neck region and restore the sensation of flap.

The inspiration of American pharmacists training system / 药学实践杂志

Taking Pharm .D .degree education in School of Pharmacy ,University of Pittsburgh as an example ,this pa-per introduced the American pharmacists training system ,degree setting for service-oriented pharmacy students ,training objec-tive ,course design ,teaching staff and other aspects .This paper also reviewed pharmacy education and professional develop-ments for licensed pharmacists in China .Based on the successful experience abroad ,proposals were made to modify the domes-tic pharmacy education .The process of personnel training should be emphasized to improve the quality and capability of licensed pharmacists in China .

Peri-operation treatment for patients with hemophilia A in children with intracranial malignant tumor:a report of 2 cases / 北京大学学报(医学版)

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.

Analysis of the co-existed nervous system anomalies in children with meningomyelocele / 中华实用儿科临床杂志

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.

Design push-pull osmotic pump tablets of famotidine based on an expert system for the formulation design of osmotic pump of poor water-soluble drug / 药学学报

The purpose of this study is to design push-pull osmotic pump (PPOP) tablets of famotidine using the expert system for the formulation design of osmotic pump of poor water-soluble drug which had been established by the authors. Firstly, the parameters which were requisite of the system input were obtained from literatures and experimental tests. Then the parameters were input into the system, and the program was run. The system displayed the designed formulations sequential. Finally, famotidine PPOP was prepared according to the designed formulations and the in vitro dissolution was carried out. It was found out that the target formulation of famotidine PPOP which could release for 24 hours was obtained in a very short period. Meanwhile, the practicability of the established expert system was proved.

Identification method with significant specificity of volatile oil of Pogostemon cablin / 中国中药杂志

<p><b>OBJECTIVE</b>To develop an identification method with significant specificity for patchouli oil.</p><p><b>METHOD</b>The fingerprint was performed by gas chromatography with patchouli alcohol and pogostone as chemical markers.</p><p><b>RESULT</b>The similarity of 12 samples were higher than 0.9 and it can be used to identify the characteristics of patchouli oil.</p><p><b>CONCLUSION</b>The GC fingerprint can be used for identification of patchouli oil.</p>

Surgical treatment for huge hepatoblastoma in children / 中华普通外科杂志

Objective To discuss the surgical treatment for huge hepatoblastoma in children,and the technique of hepatectomy without blockade of the blood supply to the remained liver lobes.Methods We reviewed 12 cases of huge hepatoblastorna who had been operated from July 2001 to January 2009 in our hospital.The mean age of the children was 3.2 years(range,11 months to 12 years).The diameter of the tumor was from 10 to 23 cm.3～7 cycles of chemotherapy was routinely administrated before operation.When the tumor reduced to a certain size that radical resection could be performed safely,regular hepatectomy was conducted.Hepatoblastoma resection without blocking the blood supply to the remained liver lobes was performed in every patient.Results The operations were successfully accomplished in all the 12 children.5 cases received right trihepatectomy (segment Ⅳ,Ⅴ～Ⅷ),4 cases received right hemihepatectomy(segment Ⅴ～Ⅷ),and the other 3 cases received Ⅳ,Ⅶ,Ⅷ segmentectomy,right Ⅴ,Ⅵ segmentectomy,and left hemihepatectomy respectively.The intraoperative hemodynamic parameters were stable,and there was no perioperative mortality.Postoperative chemotherapy wag routinely administrated.The follow-up period varied from 2 to 92 months.11 children survived and were disease free,among those 6 children have survived for more than 5 years.One child had brain and lung metastasis 5 months post operation,and died 7 months post operation. Conclusion Preoperative chemotherapy administrated to children with huge hepatoblastoma can reduce the tumor size and render tumor reseetable.Hepatoblastoma resection without blocking the blood supply to the remained liver lobes is a safe and feasible surgical technique.

Finite element simulation of bone grafting process for avascular necrosis of femoral head / 生物医学工程学杂志

Based on the unilateral CT images of a clinical patient in stage III for avascular necrosis of femoral head, three-dimensional finite element models of proximal femur are established, including normal model, necrosis model and prothetic model. Based on the same set of CT images, the material properties are assigned to the three finite element models respectively. Finally, the process of bone grafting for avascular necrosis of femoral head is simulated with finite element analysis. The results indicate that when the necrosis parts of femoral head are removed, the stress and displacement of proximal femur increase accordingly, and after the surgical operation of bone grafting, the stress and displacement of proximal femur can decrease effectively and become closer to the normal state. All the results indicate that the bone grafting operation is effective and reliable.

Wolff's law-based continuum topology optimization method and its application in biomechanics / 生物医学工程学杂志

A new method for the simulation of the mass distribution of cancellous bone is presented on the basis of finite element analysis (FEA). In this method,the process of bone remodelling is considered as a process of the topology optimization of a corresponding continuum structure. Fabric tensor is used to express the microstructure and the constitutive properties of cancellous bone. The effective volume fraction or the relative density of a point in the design domain is expressed by the invariables of the fabric tensor. A reference strain interval, which is corresponding to the strain dead zone of a bone in biomechanics, is applied to detect the the final topology of the structure. By the present approach, several numerical results are given, i. e., the simulation on the shape of the coronal plane of vertebrae, the predictions of the mass distributions of the two-dimensional and the three-dimensional proximal femurs. The validity and feasibility of this new method are verified by the comparison between the results of the present work and those in the published literatures.

An Elementary Introduction to Education of the Specialized Master of Public Health Degree / 中华医学教育探索杂志

Applying the specialized Master of Public Health (MPH) degree education is one of the most important parts of our nation's medical degree system innovation. It has a positive effect on the development of health care in our country. To ensure the quality of the MPH degree, we should be more careful with course studies, do better in field and social practice, be stricter with degree dissertation rejoining, and supervise and control the process of the training.