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Objective To investigate the clinical features, electrophysiological characteristics and treatment of stiff-person syndrome (SPS). Methods Medical records were retrospectively collected from 8 SPS patients to analysis their clinical features, laboratory studies, electromyography characteristics and treatment effect. Results All 8 patients presented with classic SPS, experienced progressive muscle stiffness, rigidity and spasm with paroxysmal exacerbation, which most frequently involved the thoracolumbar paraspinal muscles and bilateral lower limbs and other parts of body including thoracic and abdominal wall, upper limbs, neck, head and face. Five patients underwent electromyography and the results showed continuous motor unit activity (CMUA) in the involved muscles at rest. CMUA reduced markedly in 2 cases after intravenous diazepam. Anti-glutamic acid decarboxylase (GAD) antibody testing was positive in one of 5 tested cases. All 8 patients experienced partially symptomatic relief for their muscle rigidity and spasm after benzodiazepines. Combined immunotherapy further attenuated the symptoms in two cases receiving intravenous immunoglobulin (IVIG) and one case receiving glucorticosteroids, respectively. Symptoms were completely relieved following thymectomy in 2 cases with thymoma. Conclusion SPS is characterized by progressive muscle stiffness, rigidity and spasm with paroxysmal exacerbation affecting the axial trunk and bilateral lower limbs most frequently. Electromyography indicates CMUA in these involved muscles at rest. Treatment with benzodiazepines combined with immunotherapy can improve the neurological manifestations. Thymectomy can completely relieve symptoms of SPS in patiens with thymoma.
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Objective To explore the expression of Tiaml in clear cell renal cell carcinoma and analyze its correlations to pathology of disease and prognosis.Methods The expressions of Tiam1 protein in 107 specimens of human clear cell renal cell carcinoma and 20 specimens of normal renal tissues were detected by immunohistochemical staining and its clinical significance was then analyzed.Results The expression of Tiam1 protein was higher in renal cancers than in the adjacent normal tissues ( P ＜ 0.01 ).Tiam1 protein expression rates were 47.6％ and 72.7％ in Ⅰ - Ⅱ and Ⅲ - Ⅳ tumors,while 49.3％ and 76.5％ in T1 - T2 and T3 - T4 tumors,respectively ( P ＜ 0.01 ).Expression of Tiam1 protein was higher in lymph node positive renal carcinoma tissues than in lymph node negative renal carcinoma tissues ( 71.7％ versus 47.5％,P ＜ 0.05 ).The expression of Tiam1 in carcinoma tissues showed a positive relationship with tumor vascular invasion (81.3％ versus 48.0％,P ＜ 0.01 ).In patients followed-up 5 - 8 years,Kaplan-meier analysis and the log-rank test showed that the 5-year survival was significantly different between the group of lower and higher Tiaml expression groups ( 84.4％ versus 46.8％,P ＜ 0.05 ).Conclusions The expression of Tiaml protein was higher in human primary renal carcinoma than in normal renal tissues.The positive rate of Tiam1 protein expression was related to classification,TNM stage,lymph node metastasis and vascular invasion.The detection of the expression of Tiaml protein may be helpful in the diagnosis and prognosis of renal carcinoma.
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Objective To investigate the feasibility, rationality and efficacy of the autologous dermal-fat composite tissue used as filling materials in the repair of nasal dorsum collapse.Methods The dermal fat composite tissue block (7.0 cm× 1.0 cm × 1.0 cm) was removed from the buttocks or abdoman as filling materials. 13 cases of nasal dorsum and nasal-shaped misfits were treated by using an umbrella graft of the auricular cartilage plus dermal-fat composite tissue graft to reconstruct natual shape of nasal dorsum and tip. Results The nasal dorsum and tip were repaired in 13 patients with collapsed nasal dorsum. The dermal-fat composite tissue survived well, and the incisions were healed in stage Ⅰ and the incision trace was not obvious. After follow-up for one year, the shape of nose was much satisfactory. Conclusion It is a well-accepted and easy-going procedure to repair collapsed nasal dorsum with autologous dermal-fat or a combination of composite ear cartilage tissue graft, with satisfactory effect and no rejection reactions.
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BACKGROUND:The mesenchymal stem calls (MSCs) have multiple-direction differentiation potential.How to induce human mesenchymal stem cells (hMSCs) into chondrogenic in vitro is an advanced researching direction.OBJECTIVE: Experimental study on chondrogenic differentiation of mesenchymal stem cells from human bone marrow in study culture medium so as to observe the characteristic of the cells in the continuous progressive process,such as culture of the primary generation and passage culture.DESIGN: A single sample and open study.SETTING : Department of Orthopaedics, Children's Hospital Affiliated to Soochow University.MATERIALS:A total of 5 children with single bone cyst were selected from Children's Hospital Affiliated to Soochow University from August 2002 to September 2003,including 3 boys and 2 girls aged from 3 to 12 years.All of them donated 5 Ml bone marrow, which was added with 1 Ml (20 U/Ml) heparinization, from which MSCs was obtained. Main reagents were detailed as follows: F-12 (Gibco, USA), fetal calf serum (Sijiqing Bioengineering Company, Hangzhou), trypsin (Sigma, USA), transforming growth factor-β1 (TGF-β1) (Sigma, USA) and vitamin C (the Third Medical Company,Nanjing; batch number: 021017).METHODS:① Standard culture medium:0.1 volume fraction of fetal calf serum,100 U/Ml penicillin,100 U/Ml streptomycin, 5.8 g/L Hepes, 2 g/L NaHCO3 and 0.3 g/L glutamine were added into F-12 culture medium (Ph 7.2-7.4). ②Study culture medium: 50 μg/L vitamin C and 1 μg/L TGF-β1 were added into standard culture medium (Ph 7.2-7.4). ③Cell culture of the primary generation: 12 Ml Hanks which contained 1 Ml of heparinization (20 μ/Ml) and 5 Ml bone marrow was centrifugated. Then the cells which had nucleolus were rinsed. Then cells were cultured in a 50 Ml plastic culture bottle at the density of 3×109 L-1 and were cultured in 6-well culture plate at the density of 1×109 L-1. One bottle and 3 well was used study culture medium,the other bottle and 3 well was used standard culture medium.The cells were cultured in incubator containing 0.05 volume fraction of CO2 and saturated tumidity at 37℃. The medium was changed every 48 hours. The cells were observed under inverted microscope. ④ Passage culture: When the primary generation cells had been cultured with standard culture medium for 10-14 days, we would find that the cells covered 80％ of the bottle bottom. Then 2.5 g/L of trypsin containing 0.2 g/L of EDTA was used to digestion and passage. The third generation cells were separated at the ratio of 1:1, and were cultured in two bottles. One bottle used standard culture medium, the other used study culture medium. At the same time, the cells in 6-well plate which contains coverslip were deal with in the same way. 3-wells used standard culture medium, and the others used study culture medium. Culture time was7 days.The cells were observed under inverted microscope.⑤ Index detection:Alcian blue staining was used to show the expression of the glycosaminoglycan of the primary generation and the third generation. Type- Ⅱ collagen immunohistochemical staining was used to show the express of the type- Ⅱ collagen of the primary generation and thethird generation.MAIN OUTCOME MEASUREMENTS:① Morphological observation; ② secretion of glycosaminoglycan; ③ expression of type- Ⅱ collagen.RESULTS: ① Observation under inverted microscope: Most of the hMSCs in primary culture were spindle; a few of them were wide, flat and polygon. After passage, the cells were found in a uniform spindle shape. The cells, which were induced in TGF-β1 and vitamin C, became round or oval shape. ② The positive rate of primary generation was 82.4％, the 3rd generation which were cultured in study culture medium was 76.3％ (x2 =1.14,P＞ 0.05), and the 6th generation was 68.5％(x2 =5.22, P ＜ 0.05). The cells, which were culturedinstandardculturemedium, were negative. ③ The 3rd generation and 6th generation cells which were cultured in study culture medium, were found that brown-yellow granules distributed in cytoplast. It was the positive reaction. The cells, which were cultured in standard culture medium, were negative.CONCLUSION:MSCs derived from human bone marrow may be inducedinto hondrocytes under study culture medium which contained TGF-β1 and vitamin C in vitro.The express of the glycosaminoglycan and type Ⅱ collagen show the characteristic of chondrocyte.
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Objective To investigate the effectiveness of Ender nail fixation in the management of pediatric femoral shaft fractures. Methods A total of 24 children with femoral shaft fracture underwent small incision Ender nail internal fixation followed by 4 weeks of monolateral hip spica-cast immobilization. Results Follow-up observations for 6~24 months revealed no non-union or delayed union. The affected limbs were found within 1 cm shorter or longer than the contralateral in 4 cases. None became lame, and the functions of lower limbs were completely recovered. Conclusions So far as indications and contra-indications of Ender nail fixation are strictly followed, this treatment for femoral shaft fractures in children of age 5~10 years gives characters of small incision, anatomic reduction, unimpaired periosteum, short hospital stay, and quick functional recovery.