32 patients with old atlantoaxial dislocation with bulb-spinal cord compression were treated surgically from 1971 to 1989. Their diagnoses were confimed mainly by X-ray examination and measurement. The average time of follow-up after operation was 6 years. The rate fo excellence and good was 87.5% per cent. The causes of atlantoaxial dislocation, operative techniques involved and results are discussed. The authors think that the key to the increase of the therapeutic effects is early diagnosis and surgical treatment before the irreverisible changes of bulb-spinal cord compression occur.

Objective To investigate the clinical and molecular pathological features of limb-girdle muscular dystrophy 2A (LGMD2A) of Chinese patients. Methods Thirty cases of LGMD with excluding LGMD2B were included in this study. The muscle specimens were performed by a standard series methods of histochemistry, enzymohistochemistry, immunohistochemistry and Western blot. The clinical and molecular pathological features of LGMD2A were retrospective analyzed. Results Five cases with no or only trace expression of calpain-3 protein were diagnosed as calpainopathy (LGMD2A) by Western blot analysis. The age of onset of these 5 patients ranged from 10 to 45 years and the duration of the disease were about 2-10 years. Proximal muscles weakness and atrophy of lower limbs were predominantly involved. In all patients,symptoms progressed slowly. The ambulation could be retained for many years but running and jumping were impaired early. The serum creatine kinase level was elevated moderately to markedly. Electromyography showed myopathic patterns in all cases. Two siblings had similar symptoms indicating autosomai recessive inherited pattern. Pathologically, there was marked variation in fibre size and most small fibres were round. Some necrotic and regenerating fibers were seen. Fibres with centrally placed nuclei can be found frequently. No infiltrations of inflammatory cells were seen. Lobulated fibers were observed in 2 patients by NADH-TR stain. The expression of dystrophin, caveolin-3, α-, β-, γ- and δ-sarcoglycan protein were normally staining of 5 LGMD2A patients' specimens by immunohistochemistry. Two patients had reduced staining of dysferlin by immunohistochemistry study. Conclusions Clinical and pathological characteristics of our 5 LGMD2A patients are consistent with typical muscular dystrophy features reported in other countries. Identification of calpian-3 deletion by Western blot is essential for the diagnosis of calpainopathy.

A new method for the determination of organic acids from mainstream cigarette smoke using ultra low temperature solvent extraction-comprehensive two-dimensional gas chromatography/time-of-flight mass spectrometry(GC×GC-TOF/MS)was established. The mainstream smoke was directly trapped by ethyl ether with ultra low temperature solvent extraction device, and cleaned up with liquid-liquid extraction. The concentrated extracts were further derived by N,O-bis(trimethylsily)trifluoroacetamide (BSTFA). The good separation of silanized product was achieved by the column set of DB-1 (30 m × 0. 25 mm, 1. 0 μm) as the 1st column and DB-wax (1. 5 m × 0. 1 mm, 0. 1 μm) as the 2nd column with modulation period of 6 s and scan range of m/z 45-450 . The results showed that linearity correlation coefficients were larger than 0 . 99 , and the average recoveries were between 80. 17% and 107. 81% with the relative standard deviations (RSD) in the range of 0 . 4%-12 . 1% ( n=5 ) . The detection limit and the quantitation limit were 1 . 3-24 . 5 μg/kg and 4. 1-77. 1 μg/kg, respectively.

Objective:To investigate the extracellular signal-regulated kinase 5 (ERK5) and matrix metallo proteinase-9 (MMP-9) expres-sion levels in osteosarcoma tissues and their clinical significance. Methods:The ERK5 and MMP-9 expression levels in 71 specimens of osteosarcoma tissue and 40 specimens of normal bone tissue were detected by immunohistochemistry. The relationship between ERK5 and MMP-9 expression levels, their clinical characteristics, and prognosis of patients with osteosarcoma were analyzed. Results:The positive expression of ERK5 and MMP-9 in osteosarcoma tissues was 85.9%(61/71) and 74.65%(53/71), respectively, which were significantly higher than those in normal bone tissues at 12.5%(5/40) and 10.0%(4/40) (all P<0.05). The positive expression of ERK5 and MMP-9 was associated with Enneking stage and metastasis (all P<0.05). Kaplan-Meier analysis showed that the survival duration of patients with positive ERK5 and MMP-9 expression levels was shorter than those of the patients in the negative expression groups (all P<0.05). Univariate analysis of COX proportional hazards regression model revealed that tumor size, Enneking stage, metastasis, and positive ERK5 and MMP-9 expression levels are relevant to the overall survival of patients with osteosarcoma (all P<0.05). Multi-variate analysis of COX proportional hazards regression model confirmed that Enneking stage, metastasis, and positive ERK5 and MMP-9 expression levels can act as independent prognostic factors for osteosarcoma patients (all P<0.05). Conclusion:The ERK5 and MMP-9 expression levels are high in osteosarcoma tissues and are related to the clinical characteristics and prognosis of patients with osteo-sarcoma. Thus, ERK5 and MMP-9 expression levels may play important roles in osteosarcoma development and progression.

Objective To investigate the expressions of CD147,MMP-9 and TIMP-1 in human gliomas and analyze the correlations.Methods Expressions of CD147,MMP-9 and TIMP-1 were assessed in paraffin-embedded specimens collected from 78 gliomas and 12 benign brain lesion tissues by immunohistochemistry.Real time PCR was performed to detect CD147 mRNA expression.Results The positive rates of CD147,MMP-9 and TIMP-1 expression were 62%(48/78),71%(55/78),59%(46/78) respectively.We found a significant positive correlation between CD147,MMP-9,TIMP-1 expressions and poor gliomas differentiation by Spearman analysis(rs=0.2671-0.5631,Ps＜0.01).There was also a significant positive correlation between CD147 and MMP-9 expression(rs =0.3576,P＜0.01).In addition,the expressions of CD147(47% vs.80%,x2=9.510),MMP-9(56% vs.89%,x2=10.702),and TIMP-1(49% vs.71%,x2=4.138) were significantly higher in advanced gilomas than early gliomas(Ps＜0.05).The relative expression levels of CD147 mRNA in gliomas of Ⅰ to Ⅳ pathological grades were 0.15,0.27,0.46,0.78 respectively.Conclusion The expressions of CD147,MMP-9 and TIMP-1 were important characteristic of gliomas,which may serve as biomarkers in the glioma prognostic prediction.