Late onset methylmalonic acidemia (MMA) is a rare genetic metabolic disease.This case is a 46 year old adult patient with MMA complicated with hyperhomocysteinemia.It starts with progressive limb weakness and mental abnormality, and has dysuria and respiratory failure.Neurological examination showed decreased muscle strength of limbs and pyramidal tract sign.The levels of blood homocysteine and urinary methylmalonic acid increased significantly.Head, neck, thoracolumbar magnetic resonance imaging showed abnormal signals in the spinal cord from the level of foramen magnum to the level of lumbar 1 vertebral body.Two heterozygous variants of mmachc were found by gene detection: c: 609G>A, c: 349G>A, consistent with cobalamin C deficiency.Treat with L-carnitine, vitamin B12 and betaine.The patients′ mental symptoms, limb muscle strength and respiratory failure were improved, and the level of blood homocysteine also decreased significantly.

Anesthesia modalities for endovascular treatment of acute ischemic stroke include general anesthesia and conscious sedation.Several recent studies have shown that the general anesthesia is associated with the poor outcome of endovascular treatment in patients with acute ischemic stroke.The underlying reason of causing worse outcome is unknown.It may be associated with hemodynamic instability and hypotension.Currently,it is considered that both general anesthesia and conscious sedation have their advantages and disadvantages.We must choose individualize anesthesia modalities for each patient before we obtain highquality evidence.

Objective To evaluate the clinical effect of mandibular osteomuscular flap pedicled with temporalis and radial forearm free flap to repair palatomaxillary defects. Methods From March 2008 to March 2011,nine patients with palatomaxillary defects following malignant tumor ablation,were repaired with mandibular osteomuscular flap pedicled with temporalis and radial forearm free flap. According to Brown's classification for the maxillectomy defect,eight cases were type Ⅱ B and 1 case was type Ⅱ C. There were 6 males and 3 females with an average age of 57 years(range,34-68 years). Results All the 9 osteomuscular flaps and forearm flaps survived.The patients were followed up for 12 to 24 months with an average of 14 months. The patients acquired satisfactory appearance and complete functional restoration except 1 case of chondrosarcoma of recurrence was encountered 6 months after operation. Conclusions Palatomaxillary defects were reconstructed by mandibular osteomuscular flap pedicled with temporalis and radial forearm free flap immediately.It is easy to elevate,safe,and there were no donor site problems.This is an ideal method of repairing the palato maxillary defects.

Apoptosis of neutrophils controls the duration and the intensity of an inflammatory response and therefore the extent of neutrophil- mediated tissue damage, disturbance of neutrophil apoptosis has been associated with many diseases, underlying mechanism is not elucidated. C5a is a complement fragment that has multifunctional properties, which induces neutrophil chemoattraction, an oxidative burst, enhancement of phagocytosis, release of granule enzymes, and suppress neutrophil apoptosis. Several studies have reported calpain is involved in both neutrophil functions and apoptosis and it might play a more specific role in the regulation of neutrophil apoptosis. Diffenrent isoform of calpains is activted by diffenrent stimuli through different transduction pathway. It was reported previously that calpain is required for neutrophil migration and chemotaxis induced by C5a. In addition, autophagy is a ubiquitous physiological process that occurs in all eukaryotic cells and is considered to be a survival mechanism. Atg5 promotes autophagy and is indispensable to autophagosome formation. Upon calpain activation, Atg5 is cleaved and the resulting 24 ku Atg5 mediates apoptosis while losting the property of autophagy. Therefore, Atg5 represents a molecular switch between autophagy and apoptosis. The interaction among the C5a, calpain and Atg5 was introduced and new direction for further research was provided.

The present new situation is calling for our advancement with the time and the development of the hospital in the spirit of the time at an unprecedented rate. To achieve this goal,the author brings forward"three musts" as follows:the spirit of the time must be entertained in following the trend of development;scientific method of adhering to self-reliance by drawing on others'strong points must be upheld;the prinlciple of making breakthroughs by proceeding from the actual situation must be maintained.

The present paper explores how to build a stronger contingent of scientific and technical cadres in the new situation by acting on the important thought of "Three Represents".The accomplishment of this aim requires straightening out "three outlooks"(world outlook,life outlook and value outlook),professional ethics and matter of fact attitude,and putting one's talent in a right relationship to the hospital's cultivation.

In the new situation,party leaders and cadres should take the lead in culturing communist accomplishment,and must reinforce theory study,strengthen communist belief,culture moral quality,culture noble trait,create a positive image.

Objective:To explore the factors which lead to pharyngo-cutaneous fistula after total laryngectomy.Method:A retrospective study of 125 patients who underwent total laryngectomy during a 16-year period was carried out.Result:The rate of post-laryngectomy pharyngo-cutaneous fistula is 16.0% (20/125),it appears reduced tendency.With single-factor analysis,the factors effect on the rate of post-laryngectomy pharyngo-cutaneous fistula include clinical tumor stage,tumor types,pre-operative radiotherapy dose,intra-operative blood transfusion,operative duration.With all varible logistic model analysis,the factors effect on the rate of pharyngo-cutaneous fistula are clinical tumor stage and pre-operative radiotherapy dose,but pre-operative radiotherapy is very possible to be a risk factor (P=0.0566).With logistic model stepwise regression analysis,the factors effect on the rate of pharyngo-cutaneous fistula are clinical tumor stage and operative duration.Conclusion:Pharyngo-cutaneous fistula appears easily in patients with T3、T4 tumor,and have pre-operative radiotherapy, especially have large dose preradiotherapy.For these high risk patients,the duration of operation should be as short as possible to reduce the rate of post-laryngectomy pharyngo-cutaneous fistula.

Objective To investigate the relationship between leprosy and some correlative immunomolecules in peripheral blood.Methods lymphocytes from peripheral blood in 16 leprosy patients in different periods were immunologically labeled by monoclonal antibodies from mouse CD 3/CD 4/CD 8,CD 3/CD (16+56) (NK) and CD 3/HLA-DR were determined by using flow cytometry(FCM).Results The levels of CD 3 +/CD 4 +,CD 3 +/HLA-DR and CD 4/CD 8 ratio in active period were lower in 16 patients than those of normal group,while CD 3 +/CD (16+56) +,CD 3 +/HLA-DR + were higher,NK was in the normal range.In stable period 2～4 week after treatment CD 3 +/CD 4 +,CD 3/HLA-DR - and CD 4/CD 8 were gradually increased,CD 3 +/CD (16+56) +,CD 3 +/CD 8 + and CD 3 +/HLA-DR + were increased slightly or no change ,and NK was increased slightly in 13 cases in unstable period.In other 3 case,CD 3 +/CD 4 +,CD 3 +/HLA-DR and CD 4/CD 8 were decreased than that in stable period,while the CD 3 +/CD (16+56) +,CD 3 +/HLA-DR + and NK were double increased than that in stable period.Conclusions Dynamic determination of associated immunlogical molecules in peripheral blood of leprosy patients is helpful to evaluate the change of illness state,it also play an important role in the treatmant of the disease.

Objective To observe the relationship between cerebral border zone infarction and cerebral artery stenosis or occlusion.Methods 45 patients admitted to our hospital in 2002 were studied retrospectively.They were diagnosied as acute cerebral border zone infarction and classified into anterior border infartion,posterior border infartion and internal border infartion by diffusion weighted MR imaging(DWI). The stenosis or occlusion of cerebral arteries was evaluated by magnetic resonance angiography(MRA) or transcranial doppler(TCD) examinations.Results Cerebral artery stenosis or occlusion was founded in 32 patients(71.1%).The lesions were most located in middle cerebral artery(47.6%),next in internal carotid artery(31%), relatively seldom in anterior cerebral artery and posterior cerebral artery(11.9%). No difference of artery abnormity was found between the patients with single border infarction and the patients with mutiple border infarction.But middle cerebral artery stenosis was more frequently seen in the patients with internal border infartion( P