Hutchinson-Gilford progeria syndrome (HGPS) is an early onset severe premature aging disorder due to a point mutation in LMNA gene which encodes nuclear lamin A/C. The mutation activates a cryptic splice site within exon 11 of LMNA, resulting in a 50-amino acid in-frame deletion in prelamin A. However, it is not clear how the mutation in a structural protein under the nuclear envelope could give rise to premature aging phenotypes. Recent studies showed that various abnormalities have been found in nuclear structures and functions of HGPS cells, mainly including progerin accumulation and nuclear morphology abnormalities, altered nuclear mechanical properties, changes of histone methylation patterns and epigenetic control, gene misregulation, p53 signalling activation, and increased genomic instability. Two hypotheses recently emerged in the explanation of the pathogenic mechanisms contributing to HGPS. No effective clinical intervention has been developed so far for HGPS. Several fascinating therapeutic strategies have recently been provided, such as farnesyltransferase inhibitors, antisense oligonucleotides and RNA interference. HGPS has been considered to be a model for studying the mechanisms responsible for normal aging. This study will help to elucidate the physiological functions of lamin A and nuclear envelope, together with their roles in normal aging process and diseases.

Epigenetics refers to non-coding sequence changes such as DNA methylation, histone modifications, chromosome remodeling and non-coding RNA regulation.Histone modifications include acetylation, phosphorylation, methylation, ubiquitination and ADP ribosylation.The combinations of different histone modifications, known as "histone code", are dynamic during development and differentiation and play important roles in the regulation of gene expressions in spatial-temporal manners.The modification on a particular residue in a histone affects not only the modifications at different residues in its own protein but also other histones.The histone modifications is a complicated network and the regulation remains elusive.

Objective To evaluate the possibility and clinical value of total spondylectomy for the treatment of spinal tumors and to investigate a reliable method of spinal reconstruction after total spondylectomy. Methods Twenty seven cases of spinal tumors which eroded both vertebral body and its attachments were treated with total spondylectomy and internal fixation as reconstruction techniques. There were benign, malignant and metastatic tumors, which involved different levels from upper cervical to lower lumbar spines. One to 3 spinal vertebrae were removed. Results Twenty three cases were followed up for 7 to 96 months (with an average of 25 months). Among them, 1 case of L 5 malignant neurofibroma and 1 case of C6,7 giant cells tumor recurred in 10 and 12 months after operation, but the patients refused further treatment. One case of C2-4 chordoma recurred 1 year after operation, after second surgery, the result was satisfactory. No recurrent signs were found in the rest of 20 cases. Among 25 cases with neurological lesions, obvious improvement were obtained in 23 after operation. Conclusion For patients with involvement of spinal vertebra eroded by tumors, total spondylectomy is an effective procedure. After total spondylectomy, spinal stability can be reconstructed by stable internal fixation system.

Cell aging,the fundamental unit of biological decay,is responsible for senile disease. With the development of research,the mechanism of cell aging has been investigated in molecular level. Two hypotheses have emerged to explain the reason that lamins contribute to cell aging,one is the mechanical stress hypothesis,the other is the gene expression hypothesis. The latter proposes that mutations in A-type lamins lead to abnormal tissue-specific gene regulation. In recent years,the molecular mechanisms of lamins causing cell aging primarily include gene mutation,Zmpste24 mutation,CAAX mutation and other mutations. These mutations in the gene that encode nuclear lamins cause nuclear lamina damage directly and result in cell aging,which have been associated with several degenerative disorders.

Objective To discuss the application effect of health care failure mode and effect analysis in quality control of polysomnngraphy.Methods The health care failure mode and effect analysis were applied to analyze the process of polysomnngraphy,process reengineering was carried out according to potential risk factors and causes affecting the results of polysomnography in order to ensure the continuous improvement of quality of polysomnography.Results After the improvement of the key link affecting the quality of polysomnography,the number of the patients underwent polysomnography increased from 345 in 2007 to 657 in 2010,the success rate increased from 93％ to 98％ and the doctors' trust level about polysomnography increased from 90％ to 96％.Conclusions Applying the health care failure mode and effect analysis to analyze of key link affecting the quality of polysomnography prospectively and adopting the pertinent countermeasures can effeetively improve the quality of polysomnngraphy.

Objective To assess the usefulness of the MSCTA with 3D reconstruction to epigastric tumors in interventional treatment.Methods MSCTA were performed in 27 cases of epigastric tumors before and after interventional treatment.3D reconstruction were taken for blood vessels with technique of MIP(maximum intensity projection)and VRT(volume rendering technique).Results 42 times MSCTA and 3D reconstruction were performed in 27 cases of epigastric tumors.We discovered 5 feeding arteries with mutation in abdominal cavity,7 collateral branches formed after embolization and 2 abnormal courser of celiac artery.The display of vasculative with MSCTA was better than that of DSA.Conclusion 3D-MSCTA for epigastric tumors not only can show directly and clearly the stereo-anotomic images of the tumor feeding vessels but also provide guidance for planning the treatment.

Objective To investigate the incidence and clinical significance of “no flow” (NF) phenomena after primary percutaneous coronary intervention (PCI) in the treatment of acute myocardial infarction (AMI). Methods ninety nine consecutive AMI patients underwent primary PCI were devided into NF group (18 cases) and non NF group (81 cases). The incidence of NF, clinical characteristics, blush grade, ST T segment elevation, peak CPK, left ventricular function and cardiac events were analysed. Results Eighteen patients demonstated NF, the incidence of NF was 18.2%. Compared with non NF, previous MI、diabetes、anterior MI and multivessel disease were more frequently observed in the cases with NF ( P 0.05). Conclusions The incidence NF after primary PCI in the treatment of AMI is 18.2%. The patients with NF show malperfusion in the myocardial microcirculation, larger infarct size, ventricular dysfunction and poor prognosis.

Objective Harvest of autogenous bone from iliac crest has long been held as the gold standard for anterior lumbar arthrodesis. This technique is accompanied by an added morbidity to the pelvis. The present study was to validate the experimental result showing that harvesting bone tissue from an adjacent vertebral body for bone graft followed by replacing the defect by ?-tricalcium phosphate (TCP) plug could be a feasible procedure in lumbar interbody fusion procedure. Methods 20 patients underwent single- or multiple-level circumferential lumbar fusion with anterior cages and posterior pedicle screws in one institution. All cages were filled with cancellous bone harvested from the adjacent vertebral body, with the resulted vertebral body defect filled with a TCP plug. Results Bone grafts were successfully harvested from 28 vertebral bodies in all the patients. There was no major vascular injury, and blood loss of the anterior procedure averaged 250ml (50-350ml). One TCP plug was broken during its insertion, and one endplate was broken, both due to wrong surgical technique. These incidences did not affect the final outcome. At the last follow-up (mean 28 months), a solid lumbar spine fusion was demonstrated in all the patients. At 2-year follow up, ODI and VAS were remarkably improved (P

[Objective] To study the application and clinical result of extended trochanteric osteotomy in hip joint revision.[Methods]Thirteen hips in 13 patients who were treated with extended trochanteric osteotomy in hip joint revision from 2003 to 2007 were followed-up for an average time of 32.3 months.The revision reasons,Harris score,osteotomed length,and the oeteotomed union time were analyzed.[Results]The average preoperative Harris score of all patients was 38(24~68),the average postoperative Harris score was 77(57-100),with the improvement of 39 points.The average osteotomed length from the tip of great trochanter to distal osteotomy site was 12.4 cm(9~15.1 cm).The osteotomy sites were healed in 11 patients at 3 months and 2 patients at 6 months after operation.[Conclusion]Extended trochanteric osteotomy is useful in hip joint revision.After osteotomy femoral prosthesis and cement could be taken out easily,and the osteotomy site could be healed easily.

10 points or if the progressing osteoarthritis was proved by the T nnis grade.And the remaining patients would join in the satisfied group.The differences of preoperative clinical and radiographic factors between unsatisfied group and satisfied group were identified.[Result]Ten hips in the unsatisfied group had significantly more WOMAC pain score(t=3.969,P