Slipped upper femoral epiphysis with hemophilia A

A 13-year-old boy who had hemophilia A was reported with pain in the left thigh and hip on walking. He had no history of trauma. Severe hemophilia A is diagnosed with a Factor VIII level of <1 iu/dl. The presumptive diagnosis was that of a spontaneous bleed into the hip joint. Factor VIII mutational analysis revealed a C to G substitution at nucleotide 6683 which results in a cystine change at codon 2194. However, the symptoms persisted and an X-ray demonstrated the presence of an acute on chronic slip of the upper femoral epiphysis. The patient was transferred to the center treating his hemophilia where the hip was pinned in situ under cover with Factor VIII. This case demonstrates the need to be aware of a possible traumatic diagnosis of hip pain in a hemophiliac child with a longstanding history of spontaneous bleeding into joints.     

成纤维细胞生长因子23在血液透析患者磷和维生素D代谢中的作用

目的 探讨成纤维细胞生长因子23（FGF-23）在维持性血液透析（MHD）患者磷和维生素D代谢中的作用及相关调控机制。 方法 采用酶联免疫分析法（ELISA）对59例MHD患者（血透组）及20例健康志愿者（对照组）进行血清全段FGF-23测定，同时应用放免法测定血清1，25-二羟维生素D（1,25(OH)2VitD）水平。血透组患者测定血清白蛋白（Alb）、血红蛋白（Hb）、血肌酐（Scr）、尿素氮（BUN）、钙（Ca）、磷（P）及全段甲状旁腺激素（iPTH）等指标。 结果 血透组血清FGF-23水平明显高于对照组[（215.23±123.55）比（28.72±11.49） ng/L，P < 0.01]，而血清1,25(OH)2VitD水平明显低于对照组[（13.25±8.73）比（42.24±12.45） μg/L，P < 0.01]。Pearson相关分析显示，血透组血清FGF-23水平与血清P、Scr、Ca、iPTH及透析疗程时间呈正相关（P < 0.05）；与血清1,25(OH)2VitD水平和年龄呈负相关（P < 0.05）；而与性别、血压、血清Alb、Hb、BUN等指标无相关。多元回归分析显示，血清P、Ca、Scr、iPTH和1,25(OH)2VitD是影响血清FGF-23的主要变量，5者组成的模型解释了总变异的约62%（R2=0.623，P < 0.01）。 结论 MHD患者血清全段FGF-23水平明显增高，而1,25(OH)2VitD水平明显降低。FGF-23的调控是由复杂的多种因素共同作用的结果，血清P、Ca、Scr、iPTH和1,25(OH)2VitD是影响血清FGF-23水平的主要调控因子。     

低蛋白饮食对环孢素A肾病大鼠肾间质纤维化的作用

Objective To investigate whether low-protein diet has protective effect on the progression of renal interstitial fibrosis in rats with cyclosporine A (CsA)-induced nephropathy. Methods Eighteen male Sprague-Dawley rats were randomly divided into three groups (6 rats in each group). The rats in control group (C group) received common diet; in model group (M group) low-salt diet; in intervention group (Ⅰ group) low-salt and low-protein diet. After diet adaptation period of one week, the rats in C group received subcutaneous injection of olive oil 1 mg/kg daily for 5 weeks, while M group and Ⅰ group subcutaneous injection of CsA (diluted into 25 g/L with olive oil) 1 ml/kg for 5 weeks. All the rats were sacrificed at the end of the 5th week. The food-intake and body weight were measured daily. The creatinine clearance (Ccr) was examined before rats were sacrificed. The semi-quantitative pathological analysis on kidney sections was performed. The mRNA and protein expression of transforming growth factor-β1 (TGF-βI) and type Ⅰ collagen (Col Ⅰ) in kidney tissue was determined with real time PCR and immunohistochemical staining, respectively. Results The food-intake and body weight of rats in M and I groups were significantly lower than those in C group (P<0.05). Compared with C group, the Ccr levels in M and Ⅰ groups were significantly reduced [(0.65±0.15) ml/min, (0.40+0.13) ml/min vs (1.55±0.29) ml/min, P<0.05], the relative fibrosis areas of kidney interstitium in M and I groups were significantly increased (3.60%±0.46%, 3.26%±0.75% vs 0.44%±0.24%, P<0.05), the mRNA and protein expression of TGF-β1 in M and I group was significantly up-regulated (by 2.6 and 3.1 times in mRNA and by 1.5 and 1.6 times in protein, respectively, P<0.05), and the mRNA and protein expression of Col Ⅰ in M and I groups was also significantly up-regulated (by 3.0 and 3.5 times in mRNA and by 2.3 and 2.1 times in protein, respectively, P<0.05). There were no significant differences between M and I groups in every parameters above-mentioned except the rat body weight and Ccr. Both the body weight and Ccr in Ⅰ group were significantly lower than those in M group (P<0.05). Compared with C group, the urine osmotic pressure in M group and in I group were deceased (for M group, P>0.05; for I group, P<0.05). Compared with C group, the serum cholesterol levels in M and I groups were significantly increased (P<0.05), and the serum phosphorus level in I group was significantly decreased (P<0.05). The levels of serum albumin and serum calcium of all three groups had no statistical differences (P>0.05). Conclusion Low-protein diet has no renoprutective effects on the rat model of cyclosporin A nephropathy, on the contrary, may induce body weight loss.     

安氏Ⅱ类错(牙合)畸形对青春期面部骨轮廓发育的影响

目的:研究安氏II类错畸形对青春期面部骨轮廓发育的影响。方法:收集安氏II类错畸形的成人患者和青少年患者共362例。摄取头颅定位侧位片及头颅定位正位片。对面高、面宽等19项测量项目做比较分析及t检验。结果:①安氏II类青少年患者与正常青少年相比,下颌基骨长度、下颌长度较小,但无统计学差异。②安氏II类成年患者与正常成年人相比,下颌基骨长度、下颌长度有显著性差异。③安氏II类患者下颌骨在青春发育期有向前、向上旋转的趋势。结论:安氏II类错畸形患者经过青春期发育,使面部畸形程度更加严重。本研究得出安氏II类错畸形对青春期面部骨轮廓发育的影响,对正颌外科和正畸治疗具有参考意义。     

骨髓间充质干细胞促进骨-肌腱结合部愈合的初步观察

[目的]观察骨髓间充质干细胞对骨-肌腱结合部愈合的影响.[方法]采用骨髓穿刺、全骨髓培养法获取兔骨髓间充质干细胞.36只18周龄新西兰大白兔随机分为2组,实验组将骨髓间充质干细胞与Pluronic F-127载体材料结合后,植入兔髌骨部分切除模型,对照组只进行手术,不植入细胞.在术后6、12、18周进行X线片、大体和组织学观察评估.[结果]X线片显示术后6、12、18周实验组骨-肌腱结合部新生骨面积显著大于对照组（P＜0.01）.大体观察可见实验组骨-腱结合部愈合较早.HE染色显示实验组术后6周有大量的纤维母细胞和类软骨细胞增生,并可见新骨形成;12周,髌腱与松质骨接触面软骨细胞移行带形成;18周,纤维软骨移行带排列更有序.Safranin 0染色显示实验组术后6周,基质染色较深,部分骨-腱结合处可见相对浓染区;12周,基质染色范围明显减少,沿类软骨细胞走行分布;18周,类软骨细胞集中的区域染色较12周有所减退.组织学检查显示实验组术后6、12、18周骨-腱结合部组织愈合明显,较对照组迅速.[结论]骨髓间充质干细胞可以促进骨-肌腱结合部细胞增生,促进其早期愈合.     

通用型脊柱内固定系统在胸腰椎不稳定性骨折的应用

[目的]分析通用型脊柱内固定系统Ⅱ型(GSS-Ⅱ)治疗胸腰椎不稳定性骨折的疗效。[方法]对33例胸腰椎不稳定性骨折的患者应用GSS—II固定系统进行复位固定,并对疗效进行总结。[结果]31例获得6～32个月随访,脊髓神经功能有不同程度的恢复,无螺钉松动及断钉现象,无伤椎高度丢失。[结论]GSS-Ⅱ固定系统治疗胸腰椎不稳定性骨折具有操作简单、短节段固定、三维稳定性、恢复并维持脊柱生理曲度的优点。适合于治疗胸腰椎不稳定性骨折。