Primary hyperoxaluria in an adult presenting with end-stage renal failure together with hypercalcemia and hypothyroidism

Primary hyperoxaluria (PH) is a rare genetic disorder characterized by overproduction of oxalate due to specific enzyme deficiencies in glyoxylate metabolism. The primary clinical presentation is in the form of recurrent urolithiasis, progressive nephrocalcinosis, end-stage renal disease, and systemic oxalosis. Herein, we present a case of PH who was diagnosed at 47 years of age after 6 years on hemodialysis. He presented with fatigue, anorexia, weight loss, and was found to have cachexia, diffuse edema, hepatomegaly, ascites, hypercalcemia, hyperphosphatemia, hypoalbuminemia, low parathyroid hormone levels, lytic and resorptive areas in the vertebrae, diffusely increased echogenity of the liver, multiple renal stones, and bilateral nephrocalcinosis. Bone marrow biopsy showed calcium oxalate crystals and crystal granulomas. The liver biopsy could not be performed. The absence of an identifiable reason for secondary forms, the severity of the clinical presentation, and pathological findings led to the diagnosis of PH2. He died while waiting for a potential liver and kidney donor. The presented case is consistent with the literature as he had renal stone disease in the third decade and end-stage renal disease in the fifth decade. Hypercalcemia was thought to be due to osteoclast-stimulating activity of macrophages constituting the granuloma. Erythropoietin-resistant anemia and hypothyroidism were thought to be due to accumulation of oxalate in the bone marrow and thyroid gland, respectively. It is very important to keep in mind the possibility of PH when faced with a patient with nephrocalcinosis and oxalate stone disease.     

Plasma vitamin C levels in ESRD patients and occurrence of hypochromic erythrocytes

Introduction: The achievement of erythropoiesis in hemodialysis (HD) patients is typically managed with erythropoiesis‐stimulating‐agents (ESA's) and intravenous iron (IV‐iron). Using this treatment strategy, HD patients frequently show an elevated fraction of red blood cells (RBC) with hemoglobin (Hb) content per cell that is below the normal range, called hypochromic RBC. The low Hb content per RBC is the result of the clinical challenge of providing sufficient iron content to the bone marrow during erythropoiesis. Vitamin C supplements have been used to increase Hb levels in HD patients with refractory anemia, which supports the hypothesis that vitamin C mobilizes iron needed for Hb synthesis. Methods: We conducted a cross‐sectional survey in 149 prevalent HD patients of the percent hypochromic RBC, defined as RBC with Hb < 300 ng/uL of packed RBC, in relation to plasma vitamin C levels. We also measured high‐sensitivity CRP, (hs‐CRP), iron, and ferritin levels. and calculated ESA dose. Findings: High plasma levels of vitamin C were negatively associated with hypochromic RBC (P < 0.003), and high ESA doses were positively associated (P < 0.001). There was no significant association of hs‐CRP with percent hypochromic RBC. Discussion: This finding supports the hypothesis that vitamin C mobilizes iron stores, improves iron delivery to the bone marrow, and increase the fraction of RBC with normal Hb content. Further research is warranted on development of protocols for safe and effective use of supplemental vitamin C for management of renal anemia.     

Renal involvement of mantle cell lymphona leading to end stage renal disease

Mantle cell lymphoma (MCL), owing to its insensitivity to chemotherapy, has a poor prognosis, with a median survival of 3 years to 4 years. MCL frequently infiltrates other organs. However, reports involving kidney in living patients are rare. Here, we report a case of MCL with renal involvement leading to end stage renal disease that required renal replacement therapy. A 69-year-old man diagnosed with MCL 3 years earlier was admitted to our emergency room due to uremic symptoms. After eight cycles of chemotherapy, he had displayed complete remission, but experienced a recurrence 1.5 years later; after refusing chemotherapy, the patient was lost on follow-up in the final 10 months. On presentation at the emergency room, the patient's serum blood urea nitrogen was 109.5 mg/dL, and creatinine was 11.1 mg/dL. All serological markers for secondary glomerulonephritis were negative. Renal biopsy revealed 50% sclerosis of the glomerulus and small dense lymphocyte infiltration of the tubulo-interstitium. Similar cells were found on the gastric mucosa. Despite our recommendation for chemotherapy, he refused all treatments except for hemodialysis, which was maintained for 12 months until his death. This patient represents the first case report of the renal involvement of MCL leading to end stage renal disease.     

Rationale and study design of a three‐period, 58‐week trial of ferric citrate as a phosphate binder in patients with ESRD on dialysis

Chronic kidney disease associated mineral and bone disorders arise as a result of aberrant bone mineral metabolism in patients with advancing levels of renal dysfunction and end‐stage renal disease. One of the cornerstones of treatment is the use of phosphate‐binding agents. We describe the rationale and study design for a clinical trial to assess the safety and efficacy of ferric citrate as a phosphate binder. This trial is a three‐period, international, multicenter, randomized, controlled clinical trial to assess the safety and efficacy of ferric citrate as a phosphate binder, consisting of a 2‐week washout period, a 52‐week safety assessment period in which subjects are randomized to ferric citrate or active control, and a 4‐week efficacy assessment period in which subjects randomized to ferric citrate in the safety assessment period are randomized to ferric citrate or placebo. Eligible subjects include end‐stage renal disease patients who have been treated with thrice‐weekly hemodialysis or peritoneal dialysis for at least 3 months in dialysis clinics in the United States and Israel. Primary outcome measure will be the effect of ferric citrate vs. placebo on the change in serum phosphorus. Safety assessments will be performed by monitoring adverse events, concomitant medication use, and sequential blood chemistries (including iron parameters, phosphorus, and calcium). This three‐period trial will assess the efficacy of ferric citrate as a phosphate binder. If proven safe and efficacious, ferric citrate will likely provide an additional phosphate binder to treat chronic kidney disease associated mineral and bone disorders.     

Heparin leak from a hemodialysis catheter causing major bleeding,ultimately leading to transplant rejection and death

Leakage of hemodialysis catheter‐locking solutions into the circulation has been reported in in vitro and in vivo studies, although there have been few reports of serious clinical adverse events. We describe a case of heparin leak from a hemodialysis catheter, which caused significant clinical bleeding requiring multiple transfusions and may have ultimately been responsible for the patient's death after transplantation.     

ITS－90（13．8～273．16K）国家温度基准组和工作基准组的建立

本文介绍了ＩＴＳ－９０（１３．８～２７３．１６Ｋ）国家温度基准组和工作基准组的建立、误差传递的研究、以及基准组和工作基准组的非唯一性的实验研究。实验表明，该基准组和工作基准组在全温区（１３．８～２７３．１６Ｋ）的总不确定度为２．７ｍＫ。研究了基准组在全温区的误差分布。     

Primary explosives: electrostatic discharge initiation, additive effect and its relation to thermal and explosive characteristics

All explosives, under all conditions must be considered vulnerable to generation, accumulation and discharge of static charge. The low energy static hazards of the order as low as 2-3 mJ need to be guarded against in case of highly sensitive compounds namely primary explosives. The hazard is normally associated with manufacturing and filling operations due to discharge of static charge accumulated on a person supplying energy up to 20 mJ. To reduce the risk associated with static initiation hazard in the processing and handling of the explosives, the electrostatic sensitivity tests can provide an important input regarding electrostatic hazards. This paper presents electrostatic sensitivity data in terms of zero ignition probability data (E(SE0)) of some of the initiatory explosives such as nickel/cobalt hydrazinium nitrate, silver azide, lead azide and mercury salt of 5-nitro tetrazole. Similar data has also been presented for samples coated with polyvinyl pyrrolidone to study its effect on electrostatic sensitivity. The electrostatic spark sensitivity of some conventional and novel made to explain the increased spark sensitivity behavior on the basis of the possible primary explosives has been studied. The electrostatic spark sensitivity of primary explosives decreased in the order of AgN3 = NHN > PbN6 > MNT > CoHN > BNCP. A possible correlation of spark energy with approximation and assumption has been drawn with thermal, detonation and mechanical properties. The polyvinyl pyrrolidone coated samples followed the same order but interestingly with increased spark sensitivity. An attempt has been reasoning of dielectric nature of the materials or exothermic effects of decomposition products of PVP. The present work also reports the electrostatic spark sensitivity of cap compositions.     

13.803 3～273.16 K温度国家基准的不确定度评定

本依据国家质量技术监督局发布的中华人民共和国计量技术规范ＪＪＦ１０５９－１９９９《测量不确定度评定与表示》,对１９９２年完成的１３．８０３－２７３．１６Ｋ温度国家基重新作不确定度评定。     

基层医院开展超声BI-RADS分级对乳腺良恶性结节诊断价值

目的:探讨超声BI-RADS分级在乳腺良恶性结节中的诊断价值。方法:选取2017年7月-2019年3月在我院行乳腺超声检查BI-RADS分级诊断为3-5级的患者95例,结节127个进行分析,随访患者穿刺活检或手术病理检查结果,将其作为诊断金标准进行对比分析。结果:和病理检查相比,超声BI-RADS分级对乳腺结节诊断的敏感度为88.2%(30/34),特异度为95.3%(81/85),准确度为87.4%(111/127),阳性预测值为71.4%(30/42),阴性预测值为87.1%(81/93)。结论:应用超声BI-RADS分级可有效鉴别乳腺结节的良恶性。     

毫米波WR-15（50~75 GHz）国家功率基准的研制

介绍了一种基于热敏电阻功率座的对称双线结构的WR-15（50~75 GHz）矩形波导微量热计的设计及传递标准有效效率计算方法。给出了功率基准热电堆测量曲线及该频段毫米波功率传递标准量值结果。该微量热计将作为我国50~75 GHz毫米波功率基准,将我国的毫米波功率测量能力提高到75 GHz。     

中枢神经系统原发性黑色素肿瘤的MRI诊断分析

目的:探究中枢神经系统原发性黑色素肿瘤的MRI特征,总结诊断经验。方法:以某院收治并行手术病理证实的原发性中枢神经系统黑色素肿瘤患者共14例作为研究对象,对其MRI诊断结果进行分析。结果:14例患者弥漫1例,局限者13例;累及全脑脊膜1例,椎管内颈段软脊膜1例,颅内肿瘤12例;颅内肿瘤12例中肿瘤位于颅脑外2例,颅底脑实质6例。10例实性,2例为囊实性。6例MRI表现典型,T1WI信号高、T2WI信号低,肿瘤内部表现为散点状T1、T2长信号,增强扫描后表现为均匀强化;8例患者MRI表现不典型,6例T1、T2信号较短或短信号分布不均,增强扫描后仍呈现明显不均匀特征;2例呈现囊实性特征,信号分布不均匀、长短不一,血肿边缘呈现局部实性化表现为T1信号长于T2信号。结论:中枢神经系统原发性黑色素肿瘤MRI不典型比例较高,以恶性肿瘤为主,对于黑色素聚集密集区域,T1与T2信号混杂应提高警惕。     

Primary Recrystallization of Grain Oriented Silicon Steel Strip Rolled by CSR and Annealed in Magnetic Field

The magnetic properties and textures of grain oriented silicon steel with different thickness rolled by cross shear rolling (CSR) of different mismatched speed ratio (MSR) and annealed in magnetic field under hydrogen were presented. Effects of the factors such as thickness and mismatched speed ratio on the magnetic properties and recrystallization texture were analyzed and the recrystallization principles in magnetic field annealing were discussed. The study would provide a new route for mass production of high quality ultra-thin grain oriented silicon steel strip.     

Development and evaluation of an intervention to reduce rip current related beach drowning

The objective of this research was to evaluate a campaign to improve beachgoer recognition of calm-looking rip currents, known to contribute to surf drowning. Posters, postcards, and brochures conveying the message “Don’t get sucked in by the rip” were distributed in an intervention area. Beachgoers were interviewed in this and a similar control area one year before and immediately after the intervention (respective response rates: 69.9% and 82.3%), Consenting respondents were sent follow-up questionnaires after approximately 6 months and 55% responded. In the intervention area, 28.8% of post-intervention, and 57.2% of follow-up respondents, had seen our campaign. At post-intervention, intervention respondents demonstrated improvement (relative to baseline) in intentions to swim away from a calm-looking rip, ability and confidence in identifying a rip, intention never to swim at unpatrolled beaches, and responses to being caught in a rip, compared to the control respondents. Similar improvements were observed post-intervention for respondents in the intervention area who had seen our campaign (relative to those who had not), and at 6 month follow-up for intervention respondents (relative to control respondents). The relatively brief print-based campaign was effective in warning beachgoers about calm-looking rips.     

Primary and secondary resonance analysis of FG/lipid nanoplate with considering porosity distribution based on a nonlinear elastic medium

Abstract

In this study, the nonlinear vibration analysis of the new generation nanostructures is investigated. The composite nanoplate is fabricated from the functional-graded (FG) core and two lipid layers on top and bottom of the FG core as face sheets. The nonlinear vibration analysis is studied in the presence of the external harmonic excitation force. The porosity effect on the free and force vibration analysis of the composite nanoplate is investigated. The nonlocal elasticity theory is utilized to obtain the nonlinear differential governing equation. The Kelvin–Voigt model is used to model the viscoelastic effect of the lipid layers. The Hamilton's principle is utilized to obtain the differential governing equation. The Galerkin's method is used to discrete the nonlinear partial differential governing equation to a nonlinear ordinary differential equation. The multiple scale method is used to solve the ordinary differential equation. The numerical results are compared with the reported results in the literature. A comparison between the presented numerical results and the Runge–Kutta results is done and good agreement is obtained. In the presence and absence of the porosity, the system vibration behavior is studied in the primary and secondary resonance cases. The results show that the porosity distribution types play an important role in the mechanical behavior of the composite nanoplate. Also, the numerical results show that the nonlinear frequency of the system decreases by passing time. This study can be useful to product the sensors and devices at the nanoscale with high biocompatibility.     

Age and sex differences in the risk of causing vehicle collisions in Spain, 1990 to 1999

This retrospective, paired case-control study was designed to estimate crude and adjusted effects of age and sex on the risk of causing collisions between vehicles with four or more wheels in Spain during the period from 1990 to 1999. We selected all 220284 collisions registered from 1990 to 1999 in the Spanish Dirección General de Tráfico (DGT) traffic crash database in which only one driver committed any infraction. Information was collected about age, sex and several confounding factors for both the responsible and paired-by-collision nonresponsible drivers. Crude and adjusted odds ratios (aORs) were calculated for each age and sex category. For men, the lowest risk was seen for drivers aged 25-49 years. Below the age of 35 years the crude odds ratio (cOR) was highest in the 18-24-year-old group (1.61; CI: 1.57-1.65). The risk increased significantly and exponentially after the age of 50 years, to a maximum odds ratio of 3.71 (3.43-4.00) for drivers aged >74 years. In women, the lowest risk values were found for the 25-44-year-old age group. In older women the risk increased significantly with age to a maximum odds ratio of 3.02 (2.31-3.97) in the oldest age group. aOR estimates tended to be lower than crude estimates for drivers younger than 40 years of age, but the opposite was seen for drivers 40 years old and older. Regarding sex differences, among younger drivers crude and aORs for men were higher than for women. Our results suggest that the risk of causing a collision between vehicles with four or more wheels is directly dependent on the driver's age.     

Creep behaviour and creep microstructures of a high-temperature titanium alloy Ti–5.8Al–4.0Sn–3.5Zr–0.7Nb–0.35Si–0.06C (Timetal 834): Part I. Primary and steady-state creep

The tensile creep behaviour of the high-temperature near -Ti alloy Ti–5.8Al–4.0Sn–3.5Zr–0.7Nb–0.35Si–0.06C (Timetal 834) with a duplex microstructure has been extensively investigated in the temperature range from 500°C to 625°C and the stress range from 100 to 550 MPa. Both primary and secondary creep are being considered. The results of the primary creep are analysed in terms of the dependencies of stress on strain (strain hardening) and on strain rate (strain rate sensitivity). It is shown that the strain-hardening exponent depends on temperature, and takes values between 0.5 for 500°C and 0.33 for higher temperatures; this would give a dependence of the primary creep strain of σ² and σ³. The strain rate exponents obtained in both primary and secondary creep have been found to be similar; this is also the case for the activation energies. It is thought that, in the stress and temperature range investigated, creep is controlled by bow-out and climb of dislocation segments pinned at lath boundaries and second-phase particle. Analysis of the dislocation substructure is presented to give some support for this mechanism.     

Design spaces,measures and metrics for evaluating quality of time operators and consequences leading to improved algorithms by design—illustration to structural dynamics

For the first time, for time discretized operators, we describe and articulate the importance and notion of design spaces and algorithmic measures that not only can provide new avenues for improved algorithms by design, but also can distinguish in general, the quality of computational algorithms for time‐dependent problems; the particular emphasis is on structural dynamics applications for the purpose of illustration and demonstration of the basic concepts (the underlying concepts can be extended to other disciplines as well). For further developments in time discretized operators and/or for evaluating existing methods, from the established measures for computational algorithms, the conclusion that the most effective (in the sense of convergence, namely, the stability and accuracy, and complexity, namely, the algorithmic formulation and algorithmic structure) computational algorithm should appear in a certain algorithmic structure of the design space amongst comparable algorithms is drawn. With this conclusion, and also with the notion of providing new avenues leading to improved algorithms by design, as an illustration, a novel computational algorithm which departs from the traditional paradigm (in the sense of LMS methods with which we are mostly familiar with and widely used in commercial software) is particularly designed into the perspective design space representation of comparable algorithms, and is termed here as the forward displacement non‐linearly explicit L‐stable (FDEL) algorithm which is unconditionally consistent and does not require non‐linear iterations within each time step. From the established measures for comparable algorithms, simply for illustration purposes, the resulting design of the FDEL formulation is then compared with the commonly advocated explicit central difference method and the implicit Newmark average acceleration method (alternately, the same conclusion holds true against controllable numerically dissipative algorithms) which pertain to the class of linear multi‐step (LMS) methods for assessing both linear and non‐linear dynamic cases. The conclusions that the proposed new design of the FDEL algorithm which is a direct consequence of the present notion of design spaces and measures, is the most effective algorithm to‐date to our knowledge in comparison to the class of second‐order accurate algorithms pertaining to LMS methods for routine and general non‐linear dynamic situations is finally drawn through rigorous numerical experiments. Copyright © 2005 John Wiley & Sons, Ltd.     

影响GCr15钢M／B复相组织强韧性的主次因素初探

通过正交设计试验和方差分析方法讨论了奥氏体化温度，形成贝氏体的等温温度及贝氏体的相对量对ＧＣｒ１５钢Ｍ／Ｂ复相组织的冲击韧性与硬度的影响。结果表明：贝氏体的相对量显著影响复相组织的冲击韧性及硬度，而奥氏体化温度和开成贝氏体的等温温度则对其影响不大。