A Dutch guideline for the treatment of scoliosis in neuromuscular disorders

Background

Children with neuromuscular disorders with a progressive muscle weakness such as Duchenne Muscular Dystrophy and Spinal Muscular Atrophy frequently develop a progressive scoliosis. A severe scoliosis compromises respiratory function and makes sitting more difficult. Spinal surgery is considered the primary treatment option for correcting severe scoliosis in neuromuscular disorders. Surgery in this population requires a multidisciplinary approach, careful planning, dedicated surgical procedures, and specialized after care.

Methods

The guideline is based on scientific evidence and expert opinions. A multidisciplinary working group representing experts from all relevant specialties performed the research. A literature search was conducted to collect scientific evidence in answer to specific questions posed by the working group. Literature was classified according to the level of evidence.

Results

For most aspects of the treatment scientific evidence is scarce and only low level cohort studies were found. Nevertheless, a high degree of consensus was reached about the management of patients with scoliosis in neuromuscular disorders. This was translated into a set of recommendations, which are now officially accepted as a general guideline in the Netherlands.

Conclusion

In order to optimize the treatment for scoliosis in neuromuscular disorders a Dutch guideline has been composed. This evidence-based, multidisciplinary guideline addresses conservative treatment, the preoperative, perioperative, and postoperative care of scoliosis in neuromuscular disorders.     

Aflatoxin contamination of rice in the United Arab Emirates

Many people in the United Arab Emirates store rice in large quantities for long periods of time before use. Five hundred samples of rice were collected from households in Al-Ain city during the summers of 1992-1994. Aflatoxin B₁ was detected in 160 samples (64%) of long grain rice and 81 Samples (32%) of short grain rice at levels ranging from 1.2 to 16.5 μg/kg. The moisture content of samples varied between 5.7% and 15.3%. Species ofAspergillus andPenicillium (includingA. flavus andA. parasiticus) were isolated from discoloured, broken and insect damaged grain and it was confirmed that at least two of the isolates ofA. flavus were aflatoxigenic. These findings demonstrate that rice may contribute to dietary exposure to aflatoxins which are known to be carcinogenic and immunosuppressive.     

特异腐质霉纤维素酶的研究

由腐植土中分离到一株嗜热真菌,经鉴定为特异腐质霉(Humicola insolens Cooney etEmerson)。研究了这株菌纤维素酶的产生条件和一般性质。菌在含麦麸5％、NaNO0.3％的液体培养基(灭菌前pH7.5,灭菌后pH7.2)中,于45℃培养4天,以羧甲基纤维素钠为底物,每ml滤液酶活力为20个单位。酶作用的最适条件为:pH6.0,温度为65—70℃。该纤维素酶是一种耐热酶,热稳定性较强,70℃保温5分钟后,酶活力剩余88％。底物对该酶的热钝化有较强的保护作用,无底物存在条件下,70℃保温6小时后,酶活力仅剩余1％,而在同样的处理温度和时间,在有底物存在条件下,酶活力可剩余30％。该酶在45℃保温15小时的条件下,pH稳定范围为6.0—9.0。     

昆虫弦音器及其声音感受分子机制

弦音器是昆虫类特有的一种机械感受器,亦称弦音感受器或剑梢感受器。它主要具有感知外界声压和体内肌肉运动的听觉功能,研究弦音器的机能结构对揭秘昆虫听觉的神经机制有重要的科学意义。本文从弦音器多样性和进化入手,重点综述了弦音器的微细结构、基因功能定位、声音感受分子机制及其声压增幅分子生物物理学原理,为昆虫听觉仿生学的研究提供了理论依据。     

鸡PPARγ基因的表达特性及其对脂肪细胞增殖分化的影响

为分析鸡PPARγ基因的组织表达特性及其在脂肪细胞增殖和分化过程中的功能,文章以东北农业大学高、低腹脂双向选择品系肉鸡为实验材料,利用Western blotting方法,检测PPARγ基因的组织表达特性及其在高、低脂系肉鸡腹部脂肪组织间的表达差异;采用RNAi技术,在鸡原代脂肪细胞中抑制PPARγ基因的表达后,通过MTT和油红O提取比色的方法,研究鸡PPARγ基因对脂肪细胞增殖和分化的调控作用;利用Real-timePCR和Western blotting技术,分析PPARγ基因表达下调后,其他脂肪细胞分化转录因子以及与脂肪细胞分化相关的重要基因的表达变化情况。结果表明,PPARγ基因在7周龄高脂系肉鸡腹部脂肪组织、肌胃、脾脏、肾脏组织中表达量较高,在心脏中表达量较低,在肝脏、胸肌、腿肌、十二指肠中未检测到表达信号;与高脂系相比,PPARγ基因在5和7周龄低脂系肉鸡腹部脂肪组织中的表达量较低(P<0.05);PPARγ基因的表达量下降后,鸡脂肪细胞的增殖能力增强,分化能力减弱;同时,C/EBPα、SREBP1、A-FABP、Perilipin1、LPL、IGFBP-2基因的表达量均下降(P<0.05)。由此可见,PPARγ基因的表达可能与肉鸡腹部脂肪的沉积有一定的关系,该基因可能是调控鸡脂肪细胞增殖与分化的关键因子。     

11个绵羊品种MSTN基因非翻译区的变异

利用PCR-RFLP技术对特克塞尔羊、夏洛莱羊、小尾寒羊、蒙古羊、乌珠穆沁羊、阿勒泰羊、呼伦贝尔羊、塔什库尔干羊、多浪羊、湖羊和岗巴羊11个品种的345个个体的肌肉生长抑制素(Myostatin, MSTN)基因非翻译区(UTR)的变异进行了多态性分析。结果表明大小为271 bp和1 003 bp的扩增片段经限制性内切酶MboⅡ和BsaⅠ酶切后表现多态, 经卡方检验所有品种在该基因座位均处于平衡状态(P>0.05), 3种基因型在11个绵羊品种中的分布差异极显著(P<0.01)。通过限制性内切酶HpyCH4Ⅳ 酶切实验, 证明我国9个地方绵羊品种不存在特克塞尔绵羊中发现的导致肌肉发达的SNP位点, 并在3′UTR区发现了个别碱基突变位点能够形成miRNA作用的靶基序, 测序表明3′UTR区的突变频率较高。