Sodium phenylbutyrate improved the clinical state in an adult patient with arginase 1 deficiency

An adult female patient was diagnosed with arginase 1 deficiency (ARG1-D) at 4 years of age, and had been managed with protein restriction combined with sodium benzoate therapy. Though the treatment was successful in ameliorating hyperammonemia, hyperargininemia persisted. After being under control with a strict restriction of dietary protein, severe fall of serum albumin levels appeared and her condition became strikingly worsened. However, after sodium phenylbutyrate (NaPB) therapy was initiated, the clinical condition and metabolic stability was greatly improved.Current management of ARG1-D is aimed at lowering plasma arginine levels. The nitrogen scavengers, such as NaPB can excrete the waste nitrogen not through the urea cycle but via the alternative pathway. The removal of nitrogen via alternative pathway lowers the flux of arginine in the urea cycle. Thereby, the clinical complications due to insufficient amount of protein intake can be prevented. Thus, NaPB therapy can be expected as a useful therapeutic option, particularly in patients with ARG1-D.     

飞行员鼻息肉手术对飞行评定的影响

目的分析鼻息肉手术对飞行员飞行的影响。方法用鼻内窥镜手术切除鼻息肉,并扩大窦口;临床治愈后行飞行评定。结果 13例飞行员在地面观察3～6月后,100%实现临床痊愈;飞行评定时,全部达到合格标准。结论患鼻息肉的飞行员经过成功的治疗后仍然可达到飞行评定合格的标准。     

Enhanced electrocatalytic performance of nickel diselenide grown on graphene toward the reduction of triiodide redox couples

The promising activity of nickel diselenide (NiSe₂) towards electrocatalysis has made it especially attractive in energy conversion fields. However, NiSe₂ with high electrocatalytic performance always requires complicated fabrication or expensive conductive polymers, resulting in the scale-up still being challenging. Herein, we introduce a simple and cost-effective synthesis of NiSe₂ dispersed on the surface of graphene (NiSe₂/RGO NPs). NiSe₂/RGO NPs exhibited enhanced electrocatalytic performance and long-term stability for the reduction reaction of triiodide redox couples in dye-sensitized solar cells (DSSCs). Leveraging the advantageous features, the DSSC fabricated with NiSe₂/RGO NPs as CE had a smaller charge-transfer resistance (R_ct) value and higher short-circuit current density and fill factor than naked NiSe₂ NPs. Additionally, NiSe₂/RGO NPs achieved a PCE of 7.76%, higher than that of pure NiSe₂ (6.51%) and even exceeding that of Pt (7.56%). These prominent features demonstrated that the NiSe₂/RGO NPs in this work are a promising cheap and efficient electrocatalyst to replace state-of-the-art Pt.

The promising activity of nickel diselenide (NiSe₂) towards electrocatalysis has made it especially attractive in energy conversion fields.     

Skeletal class II malocclusion caused by mouth breathing in a pediatric patient undergoing treatment by interceptive guidance of occlusion

A 7-year 10-month-old boy was evaluated for mouth breathing and snoring habits. Examination revealed soft convex tissues, maxillary protrusion, mandibular retrusion, and a class II sagittal osteofascial pattern. The patient failed a water holding test. He was clinically diagnosed with skeletal class II malocclusion caused by mouth breathing. Under interceptive guidance of occlusion (iGo), the malocclusion improved with fixed maxillary expansion using functional appliances and interventional treatment of mouth breathing by lip closure exercises. These treatments enabled the patient to gradually return to nasal breathing and guided him to develop physiological occlusion for a coordinated jaw-to-jaw relation. At the 5-year 2-month post-correction follow-up visit (at the age of 13 years), the patient had stable occlusion, a coordinated osteofascial pattern, and normal dentition, periodontium, and temporomandibular joints.     

动脉自旋标记技术在脑部疾病中的临床应用

动脉自旋标记(ASL)技术是一种基于内源性对比剂的灌注功能磁共振成像技术,可以无损测量脑血流量(CBF)。与正电子发射断层摄影术(PET)、单光子发射计算机体层投影术(SPECT)、CT以及采用对比剂的磁共振灌注成像相比,ASL技术具有无损、简单易行、成本低等优点,适合长期随访研究。下面就当前ASL技术在诸如脑血管疾病、脑肿瘤、阿尔茨海默病以及癫痫等脑部疾病的临床应用进行综述。     

民航飞行人员同型半胱氨酸与高血压病的关系及其与血脂的相关性

目的 分析同型半胱氨酸（homocysteine，Hcy）与原发性高血压（高血压病）的关系及其Hcy与血脂的相关性。 方法 28例健康体检者为对照组，38例高血压病患者为高血压病组，高血压病组又分为调脂组20例和未调脂组18例。分别测定各组受试对象血浆Hcy及血脂指标。 结果 与对照组比较，高血压组Hcy水平显著升高（P<0.01）；与未调脂组比较，调脂组总胆固醇(TC)、低密度脂蛋白胆固醇(LDL-C)、载脂蛋白B(ApoB)显著降低（P <0.01），但Hcy水平下降不显著；Pearson相关性分析显示:调脂组Hcy与三项血脂指标TC、LDL-C、ApoB均无相关性。 结论 高Hcy血症（HHcy）是民航飞行员高血压患者的危险因素；Hcy与TC、LDL-C水平无相关性，调脂治疗并不能够改善血浆Hcy水平。     

Microglial activation induced by traumatic brain injury is suppressed by postinjury treatment with hyperbaric oxygen therapy

BackgroundThe mechanisms underlying the protective effects of hyperbaric oxygen (HBO) therapy on traumatic brain injury (TBI) are unclear. TBI initiates a neuroinflammatory cascade characterized by activation of microglia and increased production of proinflammatory cytokines. In this study, we attempted to ascertain whether the occurrence of neuroinflammation exhibited during TBI can be reduced by HBO.MethodsTBI was produced by the fluid percussion technique in rats. HBO (100% O₂ at 2.0 absolute atmospheres) was then used at 1 h (HBO I) or 8 h (HBO II) after TBI. Neurobehavior was evaluated by the inclined plane test on the 72 h after TBI and then the rats were killed. The infarction area was evaluated by Triphenyltetrazolium chloride. Immunofluorescence staining was used to evaluate neuronal apoptosis (TUNEL + NeuN), microglial cell aggregation count (OX42 + DAPI), and tumor necrosis factor-alpha (TNF-α) expression in microglia cell (OX42 + TNF-α).ResultsThe maximum grasp angle in the inclined plane test and cerebral infarction of the rats after TBI were significantly attenuated by HBO therapy regardless of whether the rats were treated with HBO 1 or 8 h after TBI compared with the controls. TBI-induced microglial activation, TNF-α expression, and neuronal apoptosis were also significantly reduced by HBO therapy.ConclusionsOur results demonstrate that treatment of TBI during the acute phase of injury can attenuate microgliosis and proinflammatory cytokine TNF-α expression resulting in a neuroprotective effect. Even treating TBI with HBO after 8 h had a therapeutic effect.     

丹参酮IIA通过介导PI3K/Akt-eNOS信号通路改善野百合碱所致大鼠肺动脉高压

目的 探究丹参酮IIA治疗野百合碱所致大鼠肺动脉高压（PAH）的作用机制。方法 选取100只SD雄性大鼠,按随机数字表法分为5组（20只/组）：模型组（MCT）、丹参酮IIA组（TIIA）、丹参酮IIA+PI3K抑制剂组（TP）、PI3K抑制剂组（PI）、对照组。除对照组颈背部注射等体积生理盐水外,剩余大鼠均颈背部注射野百合碱（MCT）诱导肺动脉高压模型。2周后,丹参酮IIA组腹腔注射丹参酮IIA10 mg/kg进行治疗,丹参酮IIA+PI3K抑制剂组注射丹参酮IIA10 mg/kg+PI3K抑制剂1 mg/kg,PI3K抑制剂组注射PI3K抑制剂1 mg/kg,对照组和模型组均腹腔注射等体积生理盐水。治疗2周后,采用苏木精-伊红（HE）染色实验和α-SMA免疫荧光染色实验评估大鼠肺血管形态;Western blot实验评估大鼠肺组织中磷脂酰肌醇3激酶（PI3K）、蛋白激酶B（PKB/Akt）、内皮型一氧化氮合酶（eNOS）的磷酸化水平;ELISA法测定eNOS、NO水平。结果 HE染色实验和α-SMA免疫荧光染色证实丹参酮IIA能有效抑制PAH大鼠肺血管中膜厚度和血管肌化水平（P<0.01）;Western blot实验结果显示丹参酮IIA能够显著提高PAH大鼠肺组织中PI3K、Akt和eNOS蛋白的磷酸化水平;ELISA 实验结果显示模型组eNOS、NO水平降低（P<0.01）。结论 丹参酮IIA能够通过介导PI3K/Akt-eNOS信号通路改善野百合碱所致大鼠肺动脉高压。     

中学生元认知与自我意识的相关研究

目的探讨中学生元认知与自我意识的关系。方法通过元认知问卷和自我意识量表对250名中学生进行调查研究。结果相关分析显示,元认知知识与内向性和外向性自我意识均呈显著正相关;元认知体验仅与内向性自我意识呈显著正相关。回归分析显示,元认知知识对内向性自我意识和外向性自我意识均具有显著的回归效应,元认知体验仅对内向性自我意识具有显著的回归效应。结论自我意识与元认知之间存在关联,元认知知识能够预测内、外向性自我意识,而元认知体验仅能够预测内向性自我意识。