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Tricho–dento–osseous (TDO) syndrome, an autosomal‐dominant disorder, affects the morphological appearance of the tooth enamel, hair, and bone. Previous studies have confirmed that mutations in the DLX3 gene are responsible for TDO. In this study, we describe a Chinese patient with the typical traits of TDO – kinky hair, enamel hypoplasia, skull and jaw bones thickening, and sclerosis. Unfortunately, as a result of excessive attrition, we were unable to assess taurodontism. Examination of the tooth ground section showed a thin layer of enamel with no rods on the patient's tooth and abnormalities in Tomes' granular layer and the dentinal tubules. Scanning electron microscopy and energy‐dispersive X‐ray spectroscopy of the tooth enamel showed significant differences between the patient and the control individuals. A hair sample from the patient observed under a laser‐scanning microscope showed longitudinal grooves in the hair shaft. Dual‐energy X‐ray absorptiometry measurement showed that the bone mineral density values of the patient's bones was much higher than normal. Finally, genetic analysis revealed a novel de novo missense mutation c.533A>G (p.Q178R) in the conserved homeodomain of the DLX3 gene. This DLX3 mutation is the sixth causative mutation for TDO to be identified so far.  相似文献   
64.
目的分析创新管理理念下医院管理的方法与实践工作。方法对创新管理理念组和常规管理组进行回顾性分析,比较两组的医疗服务效果。结果创新管理理念组在医疗质量、医技水平、服务水平、就医环境、医风医德的总体评分以及医护人员满意度上均优于常规管理组。结论切合医院实际创新的内部管理理念,能够提升医院的服务质量,提高医院的竞争力。  相似文献   
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A graphene coated hexagonal ZnO (HZO@Gr) with enhanced activity in photocatalysis was synthesized. However, the photoinduced charge transfer behavior and the beneficial role of graphene in promoting photocatalytic reactions have not been sufficiently investigated experimentally. In this paper, the surface potentials of the ±(0001)-polar plane of HZO (Zn-polar plane and O-polar plane), graphene, graphene/Zn-polar plane and graphene/O-polar plane were measured using Kelvin probe force microscopy (KPFM). On the basis of the KPFM results, the respective Fermi levels were calculated and the internal electric field (IEF) of HZO was confirmed. Taking the IEF of HZO into consideration, the three-dimensional band diagrams of the HZO@Gr composites in methyl blue (MB) solution in the dark and under UV-visible irradiation after equilibrium were proposed. Accordingly, it is found that there could emerge different interactions between graphene and HZO at the ±(0001)-polar plane of HZO. Furthermore, the photogenerated holes and electrons tend to migrate to opposite directions. With the participation of graphene and IEF, the composites show a decrease in possibility of charge recombination. As a result, the active groups, namely ˙OH and ˙O2 radicals, could be mainly generated at/near the O-polar plane and Zn-polar plane, respectively. This work can serve as a supplemental explanation of the charge transfer during the photocatalytic process at the polar ZnO/graphene composite surface.

The Fermi levels and three-dimensional band diagrams of the synthesized HZO@Gr composites in methyl blue (MB) solution before and after equilibrium were assumed.  相似文献   
67.
目的 探讨浙江省非超重成年人血脂异常流行特征及影响因素。方法 选择参加2010年浙江省代谢综合征流行病学调查的10 868名年龄≥18周岁非超重/肥胖居民(BMI<24.0 kg/m2)为研究对象,进行问卷调查、体检和血脂检测,采用多因素logistic回归模型分析影响因素。结果 该人群血脂异常患病率为41.38%,男性(43.19%)显著高于女性(39.84%)(χ2=12.53,P<0.001);随年龄增长,男性血脂异常患病率降低(趋势χ2=47.61,P<0.001),女性患病率升高(趋势χ2=3.88,P<0.05),<50岁男性患病率明显高于女性;农村人群患病率(41.49%)略高于城市(41.21%),但差异无统计学意义(χ2=0.08,P=0.774);多因素logistic回归分析显示,性别、慢性病家族史、现在吸烟、现在饮酒、高肉蛋类饮食、烹调使用动物油、体力活动、中心性肥胖和BMI是非超重成年人血脂异常的影响因素。结论 浙江省非超重成年人血脂异常患病率较高,家族史、吸烟、高脂饮食、体力活动不足、中心性肥胖等是主要影响因素。  相似文献   
68.

Purpose

Recent clinical data suggest that terlipressin, a vasopressin analogue, may be more beneficial in septic shock patients than catecholamines. However, terlipressin’s effect on mortality is unknown. We set out to ascertain the efficacy and safety of continuous terlipressin infusion compared with norepinephrine (NE) in patients with septic shock.

Methods

In this multicentre, randomised, double-blinded trial, patients with septic shock recruited from 21 intensive care units in 11 provinces of China were randomised (1:1) to receive either terlipressin (20–160 µg/h with maximum infusion rate of 4 mg/day) or NE (4–30 µg/min) before open-label vasopressors. The primary endpoint was mortality 28 days after the start of infusion. Primary efficacy endpoint analysis and safety analysis were performed on the data from a modified intention-to-treat population.

Results

Between 1 January 2013 and 28 February 2016, 617 patients were randomised (312 to the terlipressin group, 305 to the NE group). The modified intention-to-treat population comprised 526 (85.3%) patients (260 in the terlipressin group and 266 in the NE group). There was no significant difference in 28-day mortality rate between the terlipressin group (40%) and the NE group (38%) (odds ratio 0.93 [95% CI 0.55–1.56]; p?=?0.80). Change in SOFA score on day 7 was similar between the two groups: ??7 (IQR ??11 to 3) in the terlipressin group and ??6 (IQR ??10 to 5) in the NE group. There was no difference between the groups in the number of days alive and free of vasopressors. Overall, serious adverse events were more common in the terlipressin group than in the NE group (30% vs 12%; p?<?0.001).

Conclusions

In this multicentre, randomised, double-blinded trial, we observed no difference in mortality between terlipressin and NE infusion in patients with septic shock. Patients in the terlipressin group had a higher number of serious adverse events.

Trial registration

This trial is registered at ClinicalTrials.gov: ID NCT01697410.
  相似文献   
69.
Highly efficient solar light absorption capabilities and quantum yields in photocatalysts are key to their application in photocatalytic fields. Towards this end, TiO2/InVO4 nanofibers (NFs) have been designed and fabricated successfully by a one-pot electrospinning process. The resulting TiO2/InVO4 NFs display excellent visible-light photocatalytic activity, owing to their prominent visible-light absorption and electron–hole separation properties. Time-resolved transient PL spectroscopy demonstrated that the TiO2/InVO4 NFs display longer emission decay times (22.0 ns) compared with TiO2 NFs (15.5 ns), implying that the heterojunction can remarkably suppress the electron–hole recombination and promote the carrier transfer efficiency. With tailored heterostructure features, TiO2/InVO4 NFs exhibit superior visible-light photodegradation activity, and after 80 min of visible-light irradiation, almost 95% of RhB molecules can be decomposed by TiO2/InVO4 NFs, while only 18% of RhB molecules can be decomposed by pure TiO2 NFs.

TiO2/InVO4 nanofibers have been designed and fabricated successfully by one-pot electrospinning process, which display longer carrier lifetime (22 ns) and enhanced visible-light photocatalytic activity.  相似文献   
70.
目的 探讨食管静脉曲张(EV)破裂出血的独立危险因素.方法 69例乙肝肝硬化患者分为出血组(31例)和非出血组(38例),采用超声测量门静脉内径和血流速度、脾静脉内径(Dsv)和血流速度、脾厚径和脾长径、胃左静脉内径(DLGv)和血流方向.将是否发生EV出血作为因变量,上述超声参数作为自变量,用Logistic回归分析筛选出EV出血的独立危险因素,用卡方检验比较两组DLGv>5.95 mm和Dsv>10.55 mm的发生率.结果 Logistic回归分析显示,DLGv和Dsv是EV出血的独立危险因素优势比(OR)=0.458,P<0.01、OR=1.425(P<0.05).出血组DLGv>5.95 mm者占83.9%,高于非出血组28.9%(P<0.01);出血组Dsv>10.55 mm者占74.2%,高于非出血组的28.9%(P<0.01).结论 DLGv和Dsv是EV出血的独立危险因素.  相似文献   
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