Symptomatic palliative care for children with neurodisability

Paediatric palliative care and neurodisability are two relatively new, evolving paediatric sub-specialities that have increasing relevance in the current paediatric landscape. For many people palliative care has been synonymous with end of life care, but in paediatrics it encompasses much more and is for all children with life-threatening or life-limiting conditions, from the point of diagnosis. This breadth of focus is demonstrated well through the interface between paediatric palliative care and paediatric neurodisability. In this article we explore this unique interface through the three domains of complex symptom management, advanced care planning and end of life care. We describe the practicalities involved in all three areas and highlight the importance of early referral and the process of “dual” or “parallel” planning. We cover in more depth the specific management of the symptoms: dystonia/abnormalities of muscle tone, seizures, pain, agitation, secretions, respiratory failure, and gut failure.     

69份苦玄参种质SSR遗传多样性及品质性状相关标记分析

目的:采用简单重复序列(SSR)分子标记技术对广西苦玄参主产区69份苦玄参种质样本进行遗传多样性及亲缘关系分析,并筛选与苦玄参苷含量相关联的优良种质基因。为苦玄参种质资源评价、遗传进化分析及分子标记辅助育种等提供依据。方法:基于转录组测序技术,开发20对引物进行批量扩增。利用各标记位点的遗传多态信息,分析69份苦玄参种质的遗传多样性及亲缘关系,并通过一元线性和多元逐步回归分析,筛选与苦玄参苷含量相关联的分子标记。结果:20对SSR引物共扩增出76个等位基因,平均每个位点观测等位基因3.8个,高于有效等位基因数(1.9692个),稀有等位基因率为38.2%,等位基因分布不均匀。等位基因多态率范围为0~59%,平均38.24%,各位点多态率差异较大。各位点多态信息含量(PIC)变化范围为0~0.6211,平均0.3780;Shannon多态性信息指数变化范围为0~1.2401,平均0.7590;Nei’s基因多样性指数(Nei)变化范围为0~0.6823,平均0.4409;以上3个指标最高的为P21,最低为P7,各位点遗传多样性存在较大差异。各位点平均观测杂合度为0.3824,低于平均期望杂合度(0.4425),表现为杂合子缺失;平均遗传分化系数Fst为0.3659;基因流Nm平均值为0.4332,种质遗传分化较大,基因流较小。一元线性回归分析和多元逐步回归分析结果表明,与苦玄参苷IA和IB相关的位点各有5个,其中仅有1个位点与2个成分的含量均相关。结论:20个SSR标记位点遗传多样性存在较大的差异,供试69份种质遗传分化大,基因流较小;从供试20个SSR标记中筛选出9个与苦玄参苷含量相关联的标记位点,试验结果可为苦玄参遗传进化分析及良种选育和繁育等提供依据。     

基于化学模式识别技术提升参威骨痹片的质量标准并监测其生产中质控指标的转移率变化

目的:提升参威骨痹片的质量标准,初步探索其质量控制指标成分在批间含量差异较大的原因。方法:采用HPLC建立参威骨痹片的指纹图谱,以Diamonsil C18(4. 6 mm×250 mm,5μm)为色谱柱,流动相乙腈(A)-0. 1%磷酸水溶液(B)梯度洗脱(0～5 min,10%A;5～15 min,10%～12%A;15～30 min,12%～26%A;30～43 min,26%～31%A,43～50 min,31%～40%A,50～70 min,40%～55%A;70～84 min,55%～72. 5%A),检测波长230 nm。以共有峰为自变量绘制正交偏最小二乘法-判别分析-变量重要性投影(OPLS-DA-VIP)图,将共有峰对该制剂各批次间指纹图谱差异的贡献度量化,寻找差异较大的色谱峰,结合相关文献,筛选出与参威骨痹片临床适应症相关的成分并进行其含量测定的专属性试验,最终选定质控指标。通过HPLC-二极管阵列检测器(DAD)同时对本品及其生产过程中间体中质控指标进行测定,检测波长236,276,230,322 nm,其他条件同HPLC指纹图谱检测方法。结果:HPLC指纹图谱共标定了26个共有峰,各批次样品指纹图谱与对照指纹图谱的相似度均≥0. 950。优选出马钱苷酸、龙胆苦苷、芍药苷、蛇床子素为参威骨痹片的质控指标,四者的平均质量分数分别为161. 02,401. 80,255. 54,80. 68μg·g-1。结论:所建立的指纹图谱及多指标定量分析方法稳定、可靠,可用于参威骨痹片的质量控制。原料药批间质控指标成分含量差异和生产过程中间体的质控方法不够完善是引起该制剂批间质控指标成分含量差异较大的主要原因。     

Antinuclear antibodies positivity is not rare during multiple sclerosis and is associated with relapsing status and IgG oligoclonal bands positivity

IntroductionAs an immune-mediated disease of the central nervous system, multifaceted aspects of a humoral immune response are widely described during multiple sclerosis (MS). However, the prevalence of different auto-antibodies, such as antinuclear antibodies (ANA), during MS is very variable and their clinical relevance remains controversial. Our aim was to evaluate the prevalence and clinical correlations of ANA positivity in South Tunisian MS patients.Material and methodsWe performed ANA screening using indirect immunofluorescence (IIF) on HEp-2 cells (Biosystems®) in 82 MS patients. For ANA positive samples (titer ≥1/160), anti-ds-DNA detection (IIF on Crithidia luciliae (Biosystems®)) and extractable nuclear antigen typing (immunodot (Euroimmun®)) were performed.ResultsANA were positive in 35/82 MS patients (42.7%). The titer was ≥ 1/320 in 16/35 patients. The antigenic specificity of ANA was identified in 7/35 patients. None of the patients had extra-neurological manifestations. No correlation was found between ANA and age, gender, MS course, disease duration, disability, annual relapse rate nor IgG index. ANA positivity was more frequent in patients with IgG oligoclonal bands (OCB) (47.1%) than in patients without IgG OCB (16,6%) (p = 0.049). Regarding disease activity, ANA positivity was significantly more frequent in patients with relapse (52.6%) than in patients in remission (25.9%) (p = 0.031).ConclusionOur results showed that ANA positivity in MS disease is not rare. This positivity was not associated with clinical expression of any connective tissue disease. ANA occurrence in MS was associated with IgG OCB+ profile and relapsing status, probably reflecting an ongoing immune dysregulation.     

Impact des troubles orthopédiques sur la ventilation du sujet IMC/polyhandicapé

Cerebral Palsy motor disorder triggers orthopedic complications. These complications do develop by themselves and gradually degrade the motor function. The functioning of the ventilatory pump is no exception to this process. Other orthopedic impairments are impacting more indirectly ventilation. Some orthopedic treatments used for PC people are likely to worsen this clinical situation, temporarily or more permanently. Any therapeutic intervention must include two essential points: acting previously to realign posture and considering interactions of treatments on different functions.     

经典名方泽泻汤的HPLC指纹图谱及多指标含量测定研究

目的建立经典名方泽泻汤的HPLC指纹图谱,结合化学模式识别技术对其进行分析,并测定泽泻汤中3种成分23-乙酰泽泻醇B、23-乙酰泽泻醇C和白术内酯Ⅲ的含量,为泽泻汤质量控制提供科学依据。方法采用Waters WAT054275C18色谱柱(250 mm×4.6 mm,5μm),以乙腈-水为流动相进行梯度洗脱,建立15批泽泻汤的HPLC指纹图谱,并采用"中药色谱指纹图谱相似度评价系统"、SPSS22.0及SIMCA14.1软件对15批泽泻汤的HPLC指纹图谱进行相似度评价及聚类分析(CA)、偏最小二乘判别分析(PLS-DA)等化学模式识别。同时测定23-乙酰泽泻醇B、23-乙酰泽泻醇C和白术内酯Ⅲ的含量。结果建立了15批泽泻汤的HPLC指纹图谱,相似度均0.94,标定了18个共有峰,指认出3个色谱峰,分别为11号峰白术内酯Ⅲ、15号峰23-乙酰泽泻醇C、16号峰23-乙酰泽泻醇B,CA和PLS-DA将15批泽泻汤样品分为2类,同时15批泽泻汤中23-乙酰泽泻醇B、23-乙酰泽泻醇C和白术内酯Ⅲ的质量分数分别为0.321～0.569、0.075～0.139、0.106～0.142 mg/g。结论 HPLC指纹图谱结合多成分同时测定的方法,快速、简便、重复性好,为泽泻汤及其制剂的质量评价提供参考。     

健康教育在白血病护理中的应用效果

目的评价血液科白血病疾病护理中应用健康教育的临床效果,为白血病护理工作提供参考。方法选择医院收治的白血病患者,总计60例。进行白血病患者随机分组护理,对照组与试验组各30例白血病患者,分别采取常规护理和常规护理+健康教育,比较2组白血病患者的负性情绪、自我效能状况评分以及护理满意度、护理依从率。结果试验组白血病患者干预后SAS、SDS、GSES评分均明显优于对照组,护理满意度与护理依从率均明显高于对照组,指标差异具有统计学意义,P>0.05。结论血液科白血病治疗期间配合落实常规护理、健康教育可以在提高患者自我效能的同时提高患者的依从性,符合患者身心护理需求,整体护理效果显著。     

The impact of frost-damage on the quality and quantity of the secreted antigen-specific IgG repertoire

Freezing of alum-based vaccines drastically alters their colloidal composition and leads to irreversible cluster formation. The loss of stability is well described, but the impact of frost damage on the functionality of the induced and secreted antibody repertoire has not been studied in detail. We therefore applied our single-cell measurement platform to extract the frequencies of Immunoglobulin G-secreting cells in combination with individual secretion rates and affinities. We showed that, frost-damaged or not, the tested vaccine was able to generate similar frequencies of total and antigen-affine IgG-secreting cells. Additionally, the frost-damaged vaccine stimulated a similar T-cell cytokine secretion pattern when compared to the regularly stored vaccine. However, frost-damaged vaccines induced no efficient affinity maturation and a complete collapse of the affinity distribution was observed. This study unveiled the impact of frost-damage to alum-based vaccines on the induced secreted antibody repertoire, and illustrated the power of functional single-antibody analysis.     

白细胞介素35和白细胞介素37与结直肠癌临床病理学特征及预后的相关性研究

背景与目的：结直肠癌（colorectal cancer，CRC）为世界第三常见恶性肿瘤，近年来研究者认为白细胞介素（interleukin，IL）-35和（或）IL-37与CRC的发展有关，但其作用机制尚未阐明。探究IL-35和IL-37在CRC发展中的作用及其可能的机制，分析IL-35和IL-37与CRC患者预后的相关性。方法：收集2013年—2017年在上海交通大学医学院附属同仁医院接受治疗的191例CRC患者手术病理蜡块的肿瘤组织，与其匹配的非癌组织是来源于同一患者的肠癌手术切缘蜡块组织。应用免疫组织化学（immunohistochemistry，IHC）染色法将CRC患者的癌组织与非癌组织染色，并运用Image-Pro Plus将IHC染色阳性部分定量分析，结合随访结果，探讨癌组织与非癌组织中IL-35和IL-37的表达水平与CRC临床病理学特征及预后的相关性。结果：与非癌组织相比，CRC组织中IL-35的表达量减少了50%（P<0.000 1）。CRC组织中IL-37的表达量与非癌组织相比增加了40%（P=0.012）。多因素生存分析显示，癌组织中IL-35（HR=0.39；95% CI：0.16~0.97；P=0.04）的表达水平是CRC患者术后生存的独立预测指标。结论：IL-35和IL-37蛋白的表达水平可能与CRC的发展有关，IL-35的表达水平可能是CRC患者术后生存的独立预测指标。