We describe a childhood mitochondrial disorder in which the clinical symptoms began and remained confined to the gastrointestinal (GI) system during the first 4 y. Seizures heralded the onset of progressive encephalopathy at age 7. Peripheral neuropathy, retinitis pigmentosa, and neural deafness developed subsequently. Laboratory investigations disclosed elevated levels of plasma lactate, and a muscle biopsy revealed ragged red fibers lacking cytochrome c oxidase activity and diminished levels of respiratory chain enzyme complexes. Molecular genetic tests failed to show any of the previously reported pathogenic mitochondrial DNA (mtDNA) mutations. We therefore screened the whole mitochondrial genome by coupling restriction digestions with single-strand conformational polymorphism (SSCP) patterns. We identified a unique SSCP in the segment that encompassed the tRNA(Lys) gene, and direct sequencing of this segment revealed a G-->A transition at an evolutionarily conserved nucleotide at mtDNA position 8313. This G8313A transition was heteroplasmic in muscle and fibroblasts of the patient, but was absent in the white blood cells and platelets from his maternal relatives. This report illustrates how GI symptoms can be the initial manifestation in a mitochondrial disorder and suggests that mitochondrial dysfunction should be considered in differentials of unexplained chronic GI symptoms, especially when lactic acidosis or other unrelated clinical signs or symptoms are present. 相似文献
Stadium Innsbruck “Tivoli Neu” extended for the European Football Championship 2008. Austria and Switzerland are the hosts of the European Football Championship EURO 2008. Each of both countries needs four stadiums with at least 30000 spectator seats. Innsbruck had a facility with a capacity of nearly 15000 seats. This existing stadium “Tivoli Neu” has therefore to be extended. The community of Innsbruck requests, that after the Championship the stadium has to be reconstructed into the original state. 相似文献
A catalyst system consisting of the palladium(0) complex Pd2(dba)3 and tri(p‐tolyl)phosphine was found to efficiently promote the decarboxylation of allyl benzoates with formation of allylarenes. This catalytic C O activation followed by extrusion of carbon dioxide and C C bond formation represents a sustainable alternative to traditional waste‐intensive cross‐couplings. The scope of the transformation includes allyl and cinnamyl esters of various ortho‐substituted benzoic acids. For particularly activated substrates, the palladium catalyst can optionally be replaced by an inexpensive nickel complex.
Summary: The powder blend of poly(acrylic acid) (PAA) with zinc oxide or zinc oxide/magnesium oxide was applied successfully to produce zinc polycarboxylate during the 3D dispensing of an aqueous ink by means of 3D printers (Z402? from ZCorporation and Deskmodeler? from bmt Company). The layer‐by‐layer inkjet printing afforded zinc ionomer 3D objects with excellent water resistance, no inherent color formation and high mechanical and dimensional stabilities. In contrast to the 3D printing of conventional powders such as starch/cellulose/dextrose blends, poly(vinyl alcohol) (PVA) or plaster, no postprinting treatments were required. Excellent dimensional accuracy of the models, as evidenced by very small deviation of the dimensions from those of the corresponding CAD data, was achieved. The mechanical properties improved with increasing PAA content, ink amount (saturation value), decreasing particle size of the sintered zinc oxide ceramic. At high PAA content >7 wt.‐%, post‐treatment with aqueous zinc acetate solution improved the mechanical properties. The setting time of the zinc cements shows no significant effect on the mechanical properties, but on the water resistance of the models. The porosity of the 3D objects, measured by means of X‐ray microtomography (μ‐CT), had profound impact on the variations of the mechanical properties of the 3D objects prepared by 3D printing processes.
Comparison of three‐point‐bending tests of commercially available powders with selected zinc cements (ZC1, ZC2, ZC4, ZC19). Commercially available powders printed with recommended values of manufacturer. Zinc cements printed with saturation value 1.14 (Z402?). 相似文献
The influence of coherent optical pumping in semiconductor lasers is investigated theoretically. In particular the mathematical conditions under which an optically pumped system behaves like an electrically (incoherently) pumped system are derived. We show that it is practically impossible to reach the interesting regime where coherent effects are important because of the inherent constraints to absorb photons at the pump frequency and to reach threshold gain at the lasing frequency. The effects of changing the temperature and of reduced dimensionality are discussed 相似文献
In this study, we investigated the involvement of matrix metalloproteinases (MMPs) in the pathophysiology of bacterial meningitis. By using an enzyme immunoassay, high concentrations of MMP-9 were detected in the cerebrospinal fluid (CSF) of adult patients with bacterial meningitis but not in controls, and in patients with Guillain-Barré syndrome. Moreover, we observed significantly elevated concentrations of the tissue inhibitor of metalloproteinase-1 (TIMP-1) in the CSF of patients with bacterial meningitis, compared with controls. In a rat model of meningococcal meningitis, intracisternal injection of heat-killed meningococci caused a disruption of the blood-brain barrier (BBB), an increase in intracranial pressure, and CSF pleocytosis paralleled by the occurrence of MMP-9 activity in the CSF 6 hours after meningococcal challenge. The MMP inhibitor batimastat (BB-94) significantly reduced the BBB disruption and the increase in intracranial pressure irrespective of the time of batimastat administration (15 minutes before and 3 hours after meningococcal challenge) but failed to significantly reduce CSF white blood cell counts. In conclusion, our results suggest that MMPs are involved in the alterations of BBB permeability during experimental meningococcal meningitis. 相似文献