Citrullinemia: management and clinical course. Apropos of a familial case

The authors report two cases of citrullinemia in siblings which add to 68 observations from the literature. They overview the clinical presentation, diagnosis and therapeutic management of the disease. The prognosis of severe neonatal form remains poor but an early adequate management may contribute to an acceptable outcome.     

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The diagnostic capacity of serum amyloid A protein for early recognition of kidney allograft rejection

Chitosan derivatives, sulfated N-acyl-chitosan (S-Cn-chitosan) possessing various lengths of alkyl chain, were prepared, and the properties of their aqueous solutions were examined. The 1H-NMR spectrum of D2O solutions of S-C12-chitosan showed broadening of the proton signals caused by aggregation of the alkyl chain. The solubility of a hydrophobic compounds, azobenzene, was small in the aqueous solutions of S-Cn-chitosan with shorter alkyl chains, but increased with increasing length of the chains above C10, showing that micelles had been formed. The ESR spectrum of a spin probe, TEMPO, in an S-C14-chitosan solution showed the existence of a hydrophobic region in the solution, but this region did not exist in the S-C2-chitosan solution. The rigidity of this region was examined by using a spin probe, 16-doxyl-stearic acid. From these results, it was revealed that S-Cn-chitosan with longer alkyl chains formed a novel type of micelle called a "polymer micelle," which was more stable than the ordinary micelles formed from low-molecular-weight surfactants.     

Gait function in spastic hemiparetic patients walking barefoot, with firm shoes, and with ankle-foot orthosis

Cortical spreading depression is a wave of electrical and biochemical changes that spreads across the cerebral cortex. It has been hypothesized to be an important underlying cause of the visual disturbances occurring during the migraine aura, but this is difficult to test in animals or humans. We created a computational model of cortical spreading depression and found that during the wave of biochemical changes the spatial pattern of neural activity broke up into irregular patterns of lines and small patches of highly activated elements. The corresponding visual disturbances that would be produced by these patterns of neural activity resemble the hallucinations reported during the migraine aura, providing strong support for the cortical spreading depression hypothesis of migraine. The model also makes the testable prediction that these hallucinations move at an exponentially increasing speed across the visual field.     

Input and central processing expressed in ERP and heart rate changes to rare target and rare nontarget stimuli

Whether late positive components of event-related potentials (ERPs) parallel changes in heart rate (HR) indicative of attention/orienting to rare stimuli has been debated. In the present study, a three-stimulus design was used, with rare target, rare nontarget, and frequent standard stimuli delivered under identical conditions except that instructions to subjects described the targets to which subjects should respond but did not describe the nontargets. In Experiment 1, stimuli varied among modalities; in Experiment 2, auditory stimuli were employed. Both ERPs and HR were consistent with automatic processing preceding two stages of controlled processing. Rare stimuli evoked larger parietal P300 and initial HR deceleration than standards. Presumably because of load-reducing effects of long interstimulus intervals, targets and nontargets were not distinguished before a late slow wave and a late phase of HR acceleration. Neither rare stimulus elicited a recognizable frontal P3a.     

Pd-Decorated Tungsten as Pt-Free Bimetallic Catalysts for Hydrogen Oxidation Reaction in Alkaline Electrolyte

Developing Pt-free catalysts for hydrogen oxidation reaction (HOR) in alkaline solution is becoming a key challenge in the development of anion exchange membrane fuel cells and electrochemical reactors. Herein, we present the preparation, HOR activity, and stability of Pd-decorated tungsten (Pd-d-W) catalysts. The Pd-d-W catalysts were prepared by the chemically activated surface of tungsten nanoparticles by Pd ions. The resultant bimetallic catalysts consisted of crystalline phases of both Pd and W nanoparticles. The CO stripping voltammograms and H-desorption (H_des) peak potential of hydrogen desorption in Pd suggests that the enhancement of HOR catalytic activity observed in Pd-d-W catalyst can be ascribed to the modification of electronic property of Pd and availability of OH_ad near-surface Pd atoms.     

A Preliminary Study of the Toxicological Properties of Selected Polymer–Ferrocene Conjugates

Prompted by early observations of the cytotoxic and antineoplastic properties of certain ferrocene and ferricenium derivatives, efforts in this laboratory were focused on the synthesis of carrier-bound ferrocene compounds. Subsequent cell culture tests carried out with selected conjugates obtained in that program showed these polymers to be highly active antiproliferative agents. In the present project toxicological work has been performed in vivo on several ferrocene conjugates in an effort to assess their toxic effects in experimental animals (CD-1 mice). The conjugates, all based on an ,-DL-polyaspartamide backbone structure, comprise the ferrocene drug model as a terminal on short side chains containing biofissionable amide or ester links for intracellular drug release. The polymers, dissolved in phosphate-buffered saline, have been injected in predetermined concentrations into the vein of the mice, and the maximum tolerated dose (MTD) levels have been determined, the latter referring to the highest dose levels administered that would allow long-term survival of the test animals. For the five conjugates tested, MTD levels range from about 3 to 30 mg Fe/kg or 0.05–0.66 mmol Fe/kg. Compared on a molar metal-to-metal basis with similarly structured platinum conjugates tested previously (MTD, 0.14–2.66 mmol Pt/kg), these values are indicative of comparatively high toxicity of the ferrocene polymers. Some implications of these findings are discussed.