不同骨延长器治疗肢体畸形并大段骨缺损

[目的]利用Ilizarov支架、Orthofix肢体重建系统（Orthofix LRS）及Hybrid固定系统（Hybrid Fixation System）与Orthofix LRS的组合，对不同的肢体畸形并大段骨缺损进行矫形及骨延长治疗，同时观察其疗效。[方法]自2000年8月-2004年3月分别用Ilizarov支架、Orthofix LRS及Hybrid支架与Orthofix LRS的组合进行骨痂牵开／骨段滑移治疗合并肢体畸形的大段骨缺损。畸形处采用线形／楔形截骨。畸形愈合并骨短缩者楔形截骨后进行骨痂牵开骨延长术，骨不连并畸形及短缩者接合点加压与截骨矫形骨段滑移延长同时进行。[结果]矫正股骨短缩畸形7cm1例，胫骨6例，内翻畸形2例，后成角畸形2例，混合畸形2例。平均延长5．3cm（4．5—7cm），平均延长时间3．5个月，平均延长后外固定时间7个月，无神经血管损伤，膝踝关节活动未受影响。[结论]Ilizarov支架、Orthofix LRS、Hybrid固定系统与Orthofix LRS的组合用于骨痂牵开／骨段滑移治疗合并肢体畸形的大段骨缺损均能达到矫形及骨延长的治疗目的。Orthofix LRS及Hybrid固定系统与Orthofix LRS的组合较Ilizarov支架操作简便，安全可靠，患者乐于接受。     

Cholinesterases and peanut agglutinin binding related to cell proliferation and axonal growth in embryonic chick limbs

Embryonic cholinesterases are assigned important functions during morphogenesis. Here we describe the expression of butyrylcholinesterase and acetylcholinesterase, and the binding of peanut agglutinin, and relate the results to mitotic activity in chick wing and leg buds from embryonic day 4 to embryonic day 9. During early stages, butyrylcholinesterase is elevated in cells under the apical ectodermal ridge and around invading motoraxons, while acetylcholinesterase is found in the chondrogenic core, on motoraxons and along the ectoderm. Peanut agglutinin binds to the apical ectodermal ridge and most prominently to the chondrogenic core. Measurements of thymidine incorporation and enzyme activities were consistent with our histological findings. Butyrylcholinesterase is concentrated near proliferative zones and periods, while acetylcholinesterase is associated with low proliferative activity. At late stages of limb development, acetylcholinesterase is concentrated in muscles and nonexistent within bones, while butyrylcholinesterase shows an inverse pattern. Thus, as in other systems, in limb formation butyrylcholinesterase is a transmitotic marker preceding differentiation, acetylcholinesterase is found on navigating axons, while peanut agglutinin appears in non-invaded regions. These data suggest roles for cholinesterases as positive regulators and peanut-agglutinin-binding proteins as negative regulators of neural differentiation.     

Ischemic stroke in infancy,childhood, and adolescence

The authors studied 34 patients with juvenile ischemic cerebrovascular disease over a 15-year period. Of the 34 patients, 23 had intracranial occlusions attributed to cerebral thrombosis or embolism and 11 had occlusions resulting from moyamoya disease. Clinicopathological features were evaluated in the 23 cases with ischemic stroke, but not those with moyamoya disease. The cause of the arterial occlusion remained undetermined in 11 patients and was found to be an embolism based on congenital heart disease in 8, on trauma in 3, and on infection in 1. Cerebral angiography was performed in 21 patients. Of these, 17 had stenoses or occlusions corresponding to their symptoms. CT scans were performed in 10 patients; the lesion in question showed no stenosis or occlusion with cerebral angiography. With regard to prognosis, patients with unknown etiology had good outcomes compared with those with congenital heart disease. With respect to acute infantile hemiplegia, 10 patients had convulsive seizures and 4 had a history of an earlier infection. Angiography and CT scans in patients with congenital heart disease demonstrated arterial occlusive sites in the middle cerebral artery region. Three patients had abscesses after their ischemic lesions.     

病房康复延伸护理对脑卒中迟缓性偏瘫患者三维步态时空参数的影响

目的:观察病房康复延伸护理对脑卒中迟缓性偏瘫患者三维步态时空参数的影响。方法:将60例脑卒中迟缓性偏瘫患者随机分为常规护理组(A组,n=30)、病房康复延伸护理组(B组,n=30)两组,予以相应的方法治疗8周,评估治疗前后两组患者三维步态时空参数。结果:与治疗前相比,治疗后两组患者的步长、步幅、步速、步频、支撑相、双支撑相等参数及B组摆动相均有不同程度的改善(P<0.05); A组摆动相和步宽及B组步宽的变化差异无统计学意义(P>0.05)。与治疗后A组比较,治疗后B组的步长、步幅、步速、步频、支撑相、双支撑相等参数改善较为明显(P<0.05),步宽和摆动相的改善差异无统计学意义(P>0.05)。结论:病房康复延伸护理能改善脑卒中迟缓性偏瘫患者三维步态时空参数,具有临床意义。     

The regulative potential of the limb region in 11.5-day rat embryos following the amputation of the fore-limb bud

Summary The regulative potential of the fore-limb region following the removal of the limb bud was investigated in 11.5-day rat embryos. Fore-limb buds were amputated from a total of 54 embryos. Five embryos were immediately examined via scanning electron microscopy (SEM) to assess the quality of the operation and the reproducibility of the technique. In all cases, the forelimb bud and adjacent tissues extending down to the celomic cavity at the same level were completely removed. The remaining 49 operated embryos were cultured in vitro and examined at different time intervals. Gross inspection of embryos which had been cultured for 24 h, revealed that 24 out of 37 had developed a pair of limb bud-like protrusions at the operation site. Twelve formed only a single protrusion, while nothing was found in the remaining embryo. These protrusions were examined in greater detail under SEM and light microscopy. SEM observations showed that these protrusions were covered with an intact layer of ectoderm. In embryos with a pair of protrusions, these outgrowths developed opposite somites 7 to 13. The failure of either one of these two outgrowths to form, produced our second type of experimental embryo, those which had just a single protrusion. Histological examination revealed that an apical ectodermal ridge (AER) was discernible on the protrusions of 36% of the embryos. Finally, we have established how these protrusions were constructed from SEM observations of operated embryos cultured for 6 h and 10 h.     

中国人肢带型肌营养不良症粘附蛋白基因突变鉴定

目的肢带型肌营养不良症（ＬＧＭＤ）是常见的常染色体遗传肌肉病，特点为髋肩带肌无力、萎缩，进行性发展至远端肌肉，男女均可受累，有明显的遗传异质性，国外研究发现重型ＬＧＭＤ分别涉及１３ｑ１２、１７ｑ２１、４ｑ１２、５ｑ３３区域编码γ－、α－、β－、δ－糖蛋白的４个基因座位。中国人群中ＬＧＭＤ的致病基因研究尚无报道，拟通过粘附蛋白（ａｄｈａｌｉｎ）基因突变的检测，鉴定其致病基因。方法用ＰＣＲ－ＳＳＣＰ及ＤＮＡ测序法对ａｄｈａｌｉｎ基因第２、３外显子进行了突变检测及鉴定。结果发现１例散发的９岁女患者ａｄｈａｌｉｎ第２、３外显子存在纯合Ａｒｇ７７Ｃｙｓ突变，此突变在本实验的４０条野生染色体中未发现。结论发现中国人群中重型ＬＧＭＤ（ＤＬＭＤ）存在ａｄｈａｌｉｎ基因突变。提示１７ｑ２１区的ａｄｈａｌｉｎ基因是中国人ＬＧＭＤ的一个致病基因。     

The effects of high dose of parathyroid hormone on fetal osteoclasts and their precursors in vivo: An ultrastructural-cytochemical study

Background: It is not well known how the immediate precursors of osteoclast develop into osteoclasts in the fetus. This ultrastructural-cytochemical study was designed to clarify the formation process of the osteoclasts and their increased activities in the fetal mouse limb buds after administration of high dose parathyroid hormone (PTH). Methods: Twenty-four or forty-eight hours after the high doses of PTH were injected into amniotic fluid of the pregnant C₃H mice, the femoral limb buds of embryos were dissected out. Tartrate-resistant acid phosphatase (TRAP) reactions were performed while preparing specimens for electron microscopy. Results: Both control and PTH-given preosteoclasts and osteoclasts exhibited TRAP-positivities in dense bodies and vesicles. As effects of PTH, a binucleated preosteoclast of tandem fashion was observed. More osteoclastic hyperactivities were observed in the diaphyseal bone marrow. An osteoclast with a large cytoplasm exhibited two sets of clear zones and ruffled borders. Some osteoclasts demonstrated prominent amoeboid figures, while other osteoclasts developed large cytoplasmic vacuoles, which contained pieces of calcified chondroid bars. Conclusions: Our results revealed the progression of maturation from young preosteoclasts to osteoclasts. An existence of a peculiar binucleated preosteoclasts suggested one of the processes for multinucleation of the osteoclast. Quite remarkable osteoclastic hyperactivities were obviously the effects of high dose PTH. Our results also indicated the endophagocytic ability of the osteoclast. How PTH affected the osteoclasts and their precursors in the diaphyseal bone marrow can be speculated. © 1995 Wiley-Liss, Inc.     

The dynamics of isometric bimanual coordination

Eight right-handed subjects performed rhythmic isometric applications of torque in the directions of pronation and supination of the forearm, in single limb and bimanual conditions. Bimanual movements were executed in either in-phase (homologous muscles simultaneously active) or anti-phase (non-homologous muscles active simultaneously) modes of coordination, in self-paced and frequency-scaled conditions. In the inphase (frequency-scaled) condition, subjects were required to synchronise (applications of torque) with each beat of a metronome, either in the direction of pronation or supination. In the anti-phase (frequency-scaled) condition, subjects were required to synchronise (applications of torque) with each beat of the metronome, either to the left or to the right. Departures from the anti-phase mode of coordination were observed as pacing frequency was increased. However, these departures were of short duration and the anti-phase mode was always re-established. These findings are in marked contrast to those obtained when there is free motion of the limbs. There also existed systematic differences between the stability of the pronation and supination phases of torque application. These differences were, in turn, modified through coincidence with the pacing signal. These results are discussed with reference to the constraints imposed upon the coordination dynamics by the intrinsic properties of the neuromuscular-skeletal system.     

Control strategies in directing the hand to moving targets

Summary We have evaluated the use of visual information about the movement of a target in two tasks tracking and interceptions — involving multi-joint reaching movements with the arm. Target velocity was either varied in a pseudorandom order (random condition) or was kept constant (predictable condition) across trials. Response latency decreased as target velocity increased in each condition. A simple model that assumes that latency is the sum of two components — the time taken for target motion to be detected, and a fixed processing time — provides a good fit to the data. Results from a step-ramp experiment, in which the target stepped a small distance immediately preceding the onset of the ramp motion, were consistent with this model. The characteristics of the first 100 ms of the response depended on the amount of information about target motion available to the subject. In the tracking task with randomly varied target velocities, the initial changes in hand velocity were largely independent of target velocity. In contrast, when the velocity was predictable the initial hand velocity depended on target velocity. Analogously, the initial changes in the direction of hand motion in the interception task were independent of target velocity in the random condition, but depended on target velocity in the predictable condition. The time course for development of response dependence was estimated by controlling the amount of visual information about target velocity available to the subject before the onset of limb movement. The results suggest that when target velocity was random, hand movement started before visual motion processing was complete. The response was subsequently adjusted after target velocity was computed. Subjects displayed idiosyncratic strategies during the catch-up phase in the tracking task. The peak hand velocity depended on target velocity and was similar for all subjects. The time at which the peak occurred, in contrast, varied substantially among subjects. In the interception task the hand paths were straighter in the predictable than in the random condition. This appeared to be the result of making adjustments in movement direction in the former condition to correct for initially inappropriate responses.     

Limb preference after unilateral pyramidotomy in adult and neonatal rats

As previously reported, unilateral pyramidotomy in newborn rats results in the development of an aberrant ipsilateral corticospinal tract that originates from the intact side. In the present study, limb preference after unilateral pyramidotomy in adult and neonatal rats was examined in search of differences that might correlate with this aberrant tract. However, no significant differences were observed between the two groups. Postoperatively, adult animals preferred the limb corresponding to the intact corticospinal tract in spite of a pre- and postoperative testing bias toward the opposite limb. Similarly, the animals that sustained neonatal lesion followed by testing at maturity also preferred the limb corresponding to the normal crossed corticospinal tract.