Pathophysiologic basis for growth failure in children with ichthyosis: an evaluation of cutaneous ultrastructure, epidermal permeability barrier function, and energy expenditure

OBJECTIVE: Because an impaired epidermal permeability barrier is present in many of the ichthyoses, we examined the contribution of barrier failure to caloric requirements in children with ichthyosis and growth failure. STUDY DESIGN: Transepidermal water loss (TEWL) and ultrastructural parameters of the permeability barrier were evaluated in 10 hospitalized children with ichthyosis and growth failure. Nutritional intake, resting energy expenditure, and calories lost as heat of evaporation were determined. RESULTS: Mean basal TEWL rates were markedly elevated in all study patients in comparison to the expected upper limit of normal (39.6+/-20.6 vs 8.7 mL/m(2) per hour). The severity of abnormalities in the ultrastructure of permeability barrier-related structures, assessed semiquantitatively, correlated significantly to mean basal TEWL rates (P <.001). Total body daily TEWL was elevated (746 +/- 468 vs 209 mL/d), resulting in a caloric drain of 433 +/- 272 kcal/d (21 +/- 9.8 kcal/kg per day) through heat of evaporation. Nutrient intake exceeded requirements in all, but resting energy expenditure exceeded predicted in 5 of 6 patients and correlated significantly with mean basal TEWL rates (P <.005). CONCLUSIONS: A defective permeability barrier in children with ichthyosis can result in ample chronic losses of water and calories to impair growth.     

Calculation of resting energy expenditure in obese children

For the calculation of resting energy expenditure, which is the main part of total energy expenditure in children with low physical activity, Fusch et al. have developed an equation.
Conclusion : This equation might be useful for research but not in daily work with obese patients.     

Toxic oxygen products alter calcium homeostasis in an asthma model

After anaphylactic or synthetic leukotriene C4 contractions in guinea pig trachealis muscle, an accelerated initial rate and greater total myorelaxation are induced in these muscle preparations when they are immersed in calcium-free medium, O(Ca++)E. Inhibition of the late phase of anaphylaxis (ANA) by FPL 55712 (10(-5) mol/L) eliminated the post-ANA O(Ca++)E-augmented myorelaxation, suggesting a causal role for SRS-A products. Hypoxia or superoxide dismutase/catalase pretreatment also abolished the post-ANA or leukotriene C4 O(Ca++)E-augmented myorelaxation. The data support the hypothesis that toxic oxygen products generated with SRS-A and/or LTC4 induce an alteration in Ca++ homeostasis in airway smooth muscle. In this model of allergic asthma, airway smooth muscle alteration after ANA may contribute to the pathogenesis of asthma and/or airway hypersensitivity associated with allergic asthma.     

基于局部一致性(ReHo)方法的慢性吸烟静息状态功能磁共振(fMRI)研究

目的:探讨慢性吸烟者静息状态下的脑功能活动情况。方法:采用局部一致性(ReHo)方法,对44名慢性吸烟者和44名与之相匹配的不吸烟的健康志愿者在静息状态下脑功能活动情况进行比较分析。结果:与不吸烟者相比,慢性吸烟者静息状态右侧额下回BOLD信号ReHo降低,左侧顶上回BOLD信号ReHo增强(P<0.05,纠正后,cluster水平)。结论:研究结果提示慢性吸烟会导致额叶和顶叶脑区功能活动的改变。     

基于脑功能网络的抑郁症识别研究

传统基于脑电(EEG)的抑郁症研究将电极视为孤立节点,忽略了它们之间的关联性,难以发掘抑郁症患者异常大脑拓扑改变。为此,本文提出一种基于脑功能网络(BFN)的抑郁症识别框架,为避免容积导体效应,相位延迟指数用于构建BFN;以加权与二值化BFN信息互补为基础,选取"小世界"特性密切相关及最小生成树特定脑区BFN指标,采用递进式指标分析策略寻找抑郁症识别潜在标识物。本文以48名受试者静息态EEG数据用于验证方案,结果表明组间同步性在左颞、右顶枕、右额脑区明显改变;加权BFN最短路径长度和聚类系数,二值化BFN左颞和右额的叶子分数及右顶枕的直径与患者健康问卷9项(PHQ-9)之间具有相关性,且获得最高94.11%的识别率。此外,研究发现相对于健康对照者,抑郁症患者的信息处理能力明显下降。通过上述结论,期望本研究结果可为BFN构建与分析提供新的思路,为抑郁症识别潜在标识物的发掘提供新的方法。     

活动平板运动试验诱发ST段抬高的临床意义

为探讨活动平板运动试验诱发ST段抬高的临床意义 ,分析了 9例无心肌梗死 (简称心梗 )而运动诱发ST段抬高的静息心电图、运动试验及冠状动脉 (简称冠脉 )造影检查结果。结果 :5 0 5 5例行平板运动试验者中 ,有 11例未患心肌梗死而运动诱发心绞痛伴ST段抬高 ,发生率 0 .2 2 %。其中 ,8例患者作了进一步检查 ,冠脉造影显示均有程度不等的血管病变 ,缺血相关血管的狭窄达到 5 0 %～ 10 0 %。ST段抬高导联与缺血相关血管有良好对应关系。另有 1例患者于运动试验 1周后死于心脏性猝死。结论 :无心梗患者运动诱发心电图ST段抬高是冠脉痉挛或冠脉严重狭窄所致心肌局部缺血的标志。     

Early decrease in resting energy expenditure with bedtime insulin therapy

AimsIn type 2 diabetes (T2D), insulin-induced weight gain may stem from a reduction in resting energy expenditure (REE). We sought to determine the early effects of insulin introduction on REE in 20 poorly controlled T2D patients.MethodsAfter improving the glycaemia, REE was measured on Day 0 and Day 4 during two treatment regimens: bedtime insulin (n = 10, group 1); and one off (3-day) intravenous insulin infusion (n = 10, group 2).ResultsBoth groups were similar in age, gender, BMI, C-peptide, HbA_1c and initial REE. By Day 4, fasting glycaemia had similarly improved in both groups: group 1: −5.3 ± 2.7 mmol/L vs group 2: −5.8 ± 4.2 mmol/L. In group 2, the second REE was measured 12 h after stopping the intravenous insulin infusion, whereas subcutaneous insulin was maintained in group 1. REE did not change in group 2 (−1.3 ± 6.5%), whereas it decreased significantly in group 1 (−8.0 ± 7.0%; P < 0.05).ConclusionBedtime insulin led to an early and specific reduction in REE.     

呼吸睡眠暂停低通气患者脑功能的功能性MRI诊断

目的:探讨静息状态下呼吸睡眠暂停低通气((SAHS)患者脑功能是否发生异常及经持续正压通气(CAPP)治疗后脑功能的改变情况.方法:搜集25例中重度OSAHS患者(患者组)及25例健康志愿者(对照组).患者CAPP治疗前后及健康志愿者均进行MRI平扫及血氧依赖水平功能性磁共振成像(BOLD-fMRI)检查.结果:与对照组相比,患者组治疗前右侧额中回等脑区的局部一致性减低,右侧岛叶等脑区的低频振幅(ALFF)值减低,右侧额下回等脑区的低频振幅比率(fALFF)值减低.与对照组相比,患者组治疗后右侧小脑半球等脑区的局部一致性减低,右侧楔叶等脑区的ALFF减低,右侧额下回等脑区的fALFF减低.与治疗前相比.患者组治疗后右侧颞下回等脑区的局部一致性明显增高,右侧小脑半球等脑区的ALFF增高,左侧枕中回等脑区的fALFF增高.结论:静息状态下OSAHS患者脑功能状态存在异常,经CAPP治疗后脑功能异常的脑区可部分逆转.功能性MRI对呼吸睡眠暂停低通气患者脑功能的诊断具有重要价值.     

畲族人群静息心率与动脉硬化的关系研究

目的探讨中国福建畲族人静息心率与动脉硬化的关系及其影响因素。 方法于2009年采用整体抽样法抽取中国福建省福安市畲族聚集地6个自然村511人为调查对象,最终样本量325例,进行颈-股动脉脉搏波传导速度（CF-PWV）、颈-桡动脉脉搏波传导速度（CR-PWV）、心踝血管指数（CAVI）、踝臂指数（ABI）检查,超声检测颈动脉内-中膜厚度（CIMT）,静息心率（RHR）为动脉硬化设备VS-1000和超声设备三次测量的平均值,并检测血液血脂、血糖、尿酸、超敏C反应蛋白,调查人群一般资料（如年龄、性别、家族史和既往史等）。 结果不同四分位数RHR水平,RHR₁≤61次/分,RHR₂为62~69次/分,RHR₃为70~77次/分,RHR₄≥78次/分,其收缩压（SBP）、舒张压（DBP）、总胆固醇/高密度脂蛋白胆固醇（TC/HDL-C）、HDL-C、CF-PWV、CR-PWV显著不同（P＜0.05）,RHR₄组甘油三酯（TG）、TC/HDL-C、DBP、CF-PWV、CR-PWV显著高于RHR₁组,而HDL-C显著低于RHR₁组（P＜0.05）;而CAVI、ABI、CIMT水平差异无统计学意义（P＞0.05）。非条件Logistic回归分析提示在未调整其他危险因素的模型中,与RHR₁组相比,RHR₄组的动脉硬化OR值为3.074（95%置信区间为1.555~6.075,P=0.001）;而进一步调整了年龄和性别之后,RHR₄组的OR值增加为4.542（95%置信区间为2.078~9.928,P＜0.001）;再一次调整了年龄、性别、血压、血脂、血糖及超敏C反应蛋白等传统危险因素之后,RHR₄组的OR值更进一步增加为5.336（95%置信区间为1.512~18.831,P=0.009）。 结论在中国福建畲族自然人群中,较高的静息心率水平伴随有较高的血压、血脂和动脉硬化水平。此外,较高的静息心率与动脉硬化密切相关,且独立于传统的血管疾病危险因素。     

Fatigability as a function of physical activity energy expenditure in older adults

Increased fatigue is a predictor of morbidity and mortality in older adults. Fatigability defines a change in performance or self-reported fatigue in response to physical activity (PA). However, the relationship of fatigability to PA-related energy expenditure (PAEE) is unknown. Changes in performance, fatigue, and energy expenditure were measured simultaneously in 17 adults (11 females, 74–94 years old) performing eight standardized PA tasks with various energy expenditure requirements in a whole-room indirect calorimeter. Change in performance was objectively measured using a PA movement monitor and change in fatigue was self-reported on a seven-point scale for each task. Performance and perceived fatigability severity scores were calculated as a ratio of change in performance and fatigue, respectively, and PAEE. We found that change in both objective performance and self-reported fatigue were associated with energy expenditure (Spearman rho = −0.72 and −0.68, respectively, p < 0.001) on a task requiring relatively high level of energy expenditure. The performance and perceived fatigability severity scores were significantly correlated (rho = 0.77, p < 0.001) on this task. In summary, results of this proof of concept pilot study show that both perceived and performance fatigability severity scores are related to PAEE-induced fatigue on a task requiring relatively high level of energy expenditure. We conclude that fatigability severity is a valid measure of PAEE-induced fatigue in older adults.