Factors affecting cognitive, motor, behavioral and executive functioning in children with phenylketonuria

We administered measures of cognitive, frontal lobe (executive), behavioral and motor functioning to 18 children with classical phenylketonuria, aged 12–101 months, in order to determine the relationship of age, current and lifetime average phenylalanine levels, and individual variation (standard deviation of lifetime average levels) to these functions. On measures of cognitive function, in children ≥ 3 y of age lower current phenylalanine levels were associated with higher cognitive functioning. On a behavioral temperament scale designed for normal children, we found that higher current and average phenylalanine levels correlated with more difficult temperament. Motor function was also poorer in children with phenylketonuria, and was most impaired in children with current phenylalanine levels >360μmol/l. We also identified a previously unreported correlation between increased individual variation and poorer executive function performance, a finding that may raise new management concerns about level fluctuations. Maintenance of phenylalanine levels μ 360 μml/l may be necessary for optimal performance in children with phenylketonuria.     

Umstellungsosteotomien bei unikondylärer Gonarthrose

Trauma und Berufskrankheit - Die Umstellungsosteotomie bei unikondylärer Gonarthrose dient einerseits der Verbesserung der Lebensqualität der Betroffenen, indem sie Schmerzen nimmt und...     

前列腺癌的分期:是否需采用磁共振成像?——决策分析探讨

目的 :确定磁共振成像在前列腺癌术前分期的正确使用。方法 :文献综述是通过使用循证医学原理和医学技术评估来完成。决策分析模型用于比较策略Ａ(在临床分期基础上进行前列腺癌根治术 )与策略Ｂ(临床分期基础上认为可手术的患者而通过磁共振成像探查到其包膜外疾病而不适合前列腺癌根治术 )。结果 :通过文献复习 ,专家组的意见不推荐用磁共振进行分期。尚未发现有关疗效的研究结果。决策分析模型表明包括有磁共振分期的方案降低了费用 (磁共振成像＄ 10 5 68;前列腺癌根治术＄ 11669)且有几乎相同的预期寿命 (磁共振成像12 .5 9年 ;前列腺…     

程序升温毛细管气相色谱的精油成分保留指数研究

本文采用线性程序升温毛细管气相色谱法对200多种天然存在和人工合成的挥发油、香精成分,分别在极性柱(DB-WAX)和非极性柱(DB-1)上进行了Kovats保留指数的测定。归纳了部分化合物的保留指数规律;考察了气体平均线速,程序初温及升温速率对保留指数的影响。并结合我们所开发的色谱数据库管理系统进行了香精的定性分析。     

247 对环境污染,农药与癌症预防的误解

引起癌症的主要原因胃：吸烟，不平衡膳食，慢性传染病和激素因素，最近的研究表明：（１）啮合类动物的致癌剂并不罕见，在标准的大剂量动物致癌试验中测试的化学物质，不论是天然的还是人工合成的，半数都是“致癌剂”；（２）啮齿类动物致癌试验中的致癌剂量很高，与低剂量的人类无关，动物试验所用剂量很高；正是使许多所试化学物质得出阳性结果的原因据；（３）尽管在人类摄入的化学物质中有９９．９％都是天然的，但是管理政策     

Annexins I to VI: quantitative determination in different human cell types and in plasma after myocardial infarction.

Concentrations of annexins I to VI were quantitatively determined in extracts of placenta and different human cell types. They were detectable in all extracts studied, but lymphocytes/monocytes, endothelial cells and fibroblasts had very high annexin contents. The results indicate cell type specific annexin-repertoires. Annexins are intracellular proteins lacking signal sequences but which are detectable in trace amounts in plasma of healthy humans. The majority of plasma samples drawn from 14 patients suffering from myocardial infarction had elevated annexin III, IV and V concentrations. Shortly after infarction increased annexin levels were detected, reaching maximal values 24 to 48 h later. In the course of the following days annexin concentrations returned towards normal plasma levels.     

Fractures of the thoracolumbar spine Late results and consequences of dorsal instrumentation

Summary Between January 1989 and July 1992, 76 patients with thoracolumbar fractures were operatively treated at the Department of Trauma Surgery, Hannover Medical School. After a mean of more than 3 years, 56 of 62 patients (90 %) still alive who had their implants removed were examined. According to the ASIF classification 33 patients sustained type A fractures, 13 type B and 10 type C. Three patients with incomplete paraplegia returned to normal; in one case of complete paraplegia no change occurred. In 40 cases the dorsal instrumentation was combined with transpedicular cancellous bone grafting. The mean operative time totaled 3 h. In this series, two complications (3.6 %) were observed: one iatrogenic vertebral arch fracture without consequences and one deep infection. Compared to the preoperative status, our follow-up examinations demonstrated permanent physical and social sequelae: the percentage of individuals able to do physical labor was reduced by half (22 to 11 patients) whereas the share of unemployed or retired patients doubled (4 to 8 patients). At the time of follow-up examination only 21 of 42 patients continued in sports. The assessment of complaints and functional outcome with the ”Hannover Spinal Trauma Score” reflected a significant difference (P < 0.001) between the status before injury (96.6/100 points) and at the time of follow-up (71.4/100 points). The radiographic assessment in the lateral plane (Cobb technique) demonstrated a significant (P < 0.001) mean restoration from an initial angle of –15.6 ° (kyphosis) to + 0.4 ° (lordosis). Serial postoperative radiographic follow-up showed progressive loss of correction; at follow-up examination we found a mean of 10.1 ° (P < 0.001). Compared to the preoperative deformity a mean improvement of 6.1 ° to an average of –9.7 ° at follow-up examination was noted. The addition of transpedicular cancellous bone grafting did not decrease the loss of correction. CT scans after implant removal were performed in 9 cases: only 3 of 9 patients showed evidence of intervertebral fusion. No correlation could be found between ASIF classification and radiographic outcome. However, the preoperative wedge angle of the vertebral body correlated significantly with the postoperative loss of reduction. Due to disappointing results after dorsal stabilization with transpedicular cancellous bone grafting we recommend a combined procedure with dorsal stabilization and ventral fusion in cases of complete or incomplete burst injury of the vertebral body.      

Influence of baclofen upon the alpha-motoneuron in spasticity by means of f-wave analysis

Intrathecal baclofen dramatically improves severe spastic syndromes. This improvement is likely related to reduced excitability of alphamotoneurons. To investigate the influence of baclofen upon the alphamotoneuron, we analyzed F-waves before and after intrathecal baclofen bolus injection (usually 50 μg) as well as after administration of different, constantly delivered doses (60–200 μg/day). Intrathecal baclofen bolus decreased the maximum F-wave amplitude (F_p) from an initial value of 9% of the maximum M amplitude (M_max) (=F/M-ratio) to 2.4% of the M_max after 130–180 min, reduced the mean F-wave amplitude 60% within 150 min, and shortened the mean duration by 40–60% after 130–180 min. Constantly delivered baclofen of 100 μg/day reduced the F/M-ratio from 5% to 2%, the mean F-wave amplitude by 40–80%, and the F-wave mean duration by 40–80%. The minimum F-wave latency did not change after bolus or during steady state administration. The findings indicate that the F-wave mean and maximum amplitude as well as the mean duration are altered in a quantifiable manner following intrathecal baclofen application.© 1995 John Wiley &Sons, Inc.