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  • 1
    Electronic Resource
    Electronic Resource
    s.l. : American Chemical Society
    Inorganic chemistry 28 (1989), S. 2234-2236 
    ISSN: 1520-510X
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1520-510X
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    s.l. : American Chemical Society
    Chemistry of materials 4 (1992), S. 1433-1437 
    ISSN: 1520-5002
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1520-5827
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    s.l. : American Chemical Society
    Chemical reviews 90 (1990), S. 459-479 
    ISSN: 1520-6890
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 0992-7689
    Keywords: Hydrology (evapotranspiration; soil moisture; water-energy interactions)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences , Physics
    Notes: Abstract The MUREX (monitoring the usable soil reservoir experimentally) experiment was designed to provide continuous time series of field data over a long period, in order to improve and validate the Soil-vegetation-Atmosphere Transfer (SVAT) parameterisations employed in meteorological models. Intensive measurements were performed for more than three years over fallow farmland in southwestern France. To capture the main processes controlling land-atmosphere exchanges, the local climate was fully characterised, and surface water and energy fluxes, vegetation biomass, soil moisture profiles, surface soil moisture and surface and soil temperature were monitored. Additional physiological measurements were carried out during selected periods to describe the biological control of the fluxes. The MUREX data of 1995, 1996, and 1997 are presented. Four SVAT models are applied to the annual cycle of 1995. In general, they succeed in simulating the main features of the fallow functioning, although some shortcomings are revealed.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1106
    Keywords: Inferior colliculus ; Efferent discharges
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Résumé Des résultats antérieurs avaient montré: 1. que les décharges efférentes spinales que peut susciter un son chez l'animal en anesthésie profonde au chloralose ne sont pas supprimées par la néodécortication totale. Ces activités efférentes ≪phasiques ≫ seraient donc élaborées dans un ou plusieurs centres réflexes sous-corticaux et emprunteraient des voies descendantes non pyramidales. 2. que malgré cette indépendance vis-à-vis de l'écorce, ces décharges sont soumises de la part des aires acoustiques corticales, à un contrôle instantané facilitateur et spécifique de leur amplitude. De telles données suggéraient l'existence d'un centre sous-cortical où convergeraient d'une part une voie courte directement issue du trajet sensoriel ascendant, d'autre part une voie descendant de l'écorce primaire correspondante (≪boucle corticale≫). Dans le présent travail, on s'est efforcé d'identifier un de ces centres élaborateurs, pour la modalité acoustique, au colliculus inférieur (tubercule quadrijumeau posterieur), en particulier à sa région latérale et latéro-basale. 1. le colliculus inférieur répond spécifiquement d'une part à la stimulation sonore, ce qui est évident, mais également à la stimulation électrique de l'écorce acoustique. Il reçoit donc bien des fibres efférentes de ce territoire. 2. recueillie dans la partie latéro-basale du colliculus, la réponse au son comporte en général deux composantes; lorsque par strychninisation ou blocage local du cortex acoustique ipsilatéral, on modifie l'amplitude de la volée descendante corticifuge au son, on altère parallèlement l'amplitude de la seconde composante et celle de la décharge spinale au son. Ainsi cette seconde composante serait-elle due partiellement à des activités corticifuges. 3. le blocage colliculaire supprime électivement les décharges spinales à la stimulation du cortex acoustique, ainsi que celles à la stimulation sonore. Les décharges d'autre provenance, sensorielle ou corticale, ne sont pas ou sont peu affectées. 4. la strychninisation locale du colliculus (par injection) peut amplifier considérablement les décharges spinales au son. Dans ces conditions d'hyperexcitabilité du centre sous-cortical, l'écorce acoustique cesse d'exercer son contrôle. Cette région pourrait ainsi jouer le rôled 'un centre réflexe acoustique, élaborateur d'activités motrices ≪phasiques ≫ déclenchées par la stimulation sonore.
    Notes: Summary The short bursts of spinal efferent discharges elicited by clicks in chloralose-anaesthetized cats are not abolished by complete bilateral neodecortication, which indicates that they can originate from subcortical “reflex centers”. Although the cortex is not essential for these click-evoked discharges, the primary cortical acoustic area exerts a specific facilitatory control upon their amplitude. These previous data suggested that subcortical reflex center(s) may receive both afferent impulses from the ascending pathway and descending impulses from the specific primary cortex (“cortical loop”). The observations presented here suggest that one of these reflex centers for the acoustic modality is located in the lateral and latero-basal part of the inferior colliculus. Results 1. Short latency responses are elicited in inferior colliculus by single electric shocks delivered to the acoustic cortex, thus confirming the existence of a cortico-collicular pathway. 2. Sound evoked responses recorded from this part of the inferior colliculus often consist of two successive components; only the second component is altered when the amplitude of the initial phase of the evoked potential to sound in the ipsilateral auditory cortex is modified experimentally (by slight strychninization or local depression); these procedures are likely to modify the local input-to-output “transfer” function of the cortex. These alterations in the collicular response are accompanied by similar modifications of the amplitude of spinal sound-evoked discharges; thus the second collicular component may be partly due to impulses from the cortical loop. 3. Reversible blocking of this collicular area produces selective suppression of spinal discharges elicited either by sound or by electrical stimulation of the acoustic cortex. Discharges of other origins, sensory or cortical, are little affected. 4. A local injection of a small amount of strychnine into this collicular area can increase sound-evoked spinal discharges. These results are discussed together with previous data suggesting that the inferior colliculus can act as a center for “sound-evoked movements”.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1076
    Keywords: Key words Inborn errors of metabolism ; Liver transplantation ; Urea cycle disorders ; AbbreviationsASL argininosuccinate lyase (argininosuccinic aciduria) ; ASL argininosuccinate layse (citrullinaemia) ; CPS carbamoyl phosphate synthase ; OLT orthotopic liver transplantation ; OTC ornithine transcarbamylase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report here our experience in the long-term management of 28 patients with citrullinaemia, 13 patients with carbamoyl phosphate synthase deficiency and 15 patients with argininosuccinic aciduria. In addition, we report a national French survey of 119 patients with ornithine transcarbamylase (OTC) deficiency enzymatically characterized in our laboratory. We also include in this report four personal patients (two with OTC and two with citrullinaemia) who were liver transplanted, and one OTC patient from the National French survey. Although this retrospective series is not really representative of the modern treatment combining low protein diet and arginine, sodium benzoate and sodium phenylbutyrate, it is obvious that the long-term outcome of all urea cycle disorders remains very guarded. We highlight the severity of the neonatal forms of such disorders, and mostly for OTC-deficient males. According to this evidence, our policy is not to treat such severely affected patients in the neonatal period who die anyway spontaneously within 2 to 3 days. At the present time, we only have three patients with neonatal citrullinaemia, aged 1, 6 and 10 years respectively, who are still doing well. One of them has been successfully liver transplanted at 5 years. Another transplanted patient died in the post-surgical phase. We emphasize the unexpected severity of argininosuccinic aciduria in which there is no one patient doing well. This is a rather surprising finding as this disorder is easy to manage and rarely presents with recurrent attacks of hyperammonaemia when it is treated by arginine supplementation. This consideration would suggest to extend the indication of orthotopic liver transplantation in this disorder. Finally, the most difficult indication is in the late onset symptomatic female OTC group. In this last group, despite a significant residual activity due to heterozygote status, even with a variable lyonisation, only seven girls are still mentally and neurologically normal. Interestingly, three of these seven were liver-transplanted before the constitution of irreversible neurological damage. These three girls and their family declare their well-being, their feeling to be cured and enjoy their normal life.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1076
    Keywords: Key words PropionylCoA carboxylase ; Management of inborn errors of metabolism ; Propionic acidaemia ; Liver transplantation ; AbbreviationsOLT orthotopic liver transplantation ; PA propionic acidaemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Despite the improvement in dietary therapy during the past 20 years, the overall outcome of severe forms of propionic acidaemia (PA) remains often disappointing. Good results can be obtained at a very high price in terms of medical attention, family burden and high cost. In most early onset forms of PA, the intake of natural protein must be rigidly restricted to 8–12 g/day for the first 3 years of life, and then slowly increased to 15–20 g/day by the age of 6–8 years. Supplementation with a precursor-free aminoacid mixture to provide 1.5 g/kg protein per day is generally recommended, although remains controversial. From the age of 1 year onward, these children are often severely anorectic and most of the diet must be delivered by nocturnal gastric drip feeding or gastrostomy. Metronidazole is very effective in reducing the excretion of propionate metabolites derived from the gut. L-carnitine (50 to 100 mg/kg) is systematically given to promote propionylcarnitine synthesis and excretion. We report here a retrospective study of 33 patients with PA diagnosed during the last 20 years in our hospital. Of them, 2 have been liver transplanted. In these two patients who presented frequent severe and unexpected metabolic decompensations despite good compliance with the dietary therapy, orthotopic liver transplantation (OLT) was done at 7 and 9 years respectively. One child died 15 months after transplantation due to a severe lymphoproliferative disorder; the other child now aged 13.5 years is doing well. Despite a persistent methylcitrate excretion, she is under normal moderate daily protein intake (40–50 g/day) and still on carnitine supplementation. Interestingly, another patient who filled the criteria for OLT (very frequent and severe decompensations leading to frequent admissions to the intensive care unit despite excellent dietary management) was also placed on the list for OLT. From the time he was registered onward, he experienced no further episodes of metabolic decompensation, there was almost no interruption in his daily intake and he gained height and weight and developed well. He was finally removed from the list and is still doing very well 2 years thereafter. Correction of propionylCoA carboxylase deficiency restricted to hepatic tissues seems to induce a change towards clinical normalisation and a milder biochemical phenotype. Liver transplanted PA patients still require slight protein restriction and carnitine treatment. We consider that at the moment OLT should only be performed in severe forms of PA, mostly characterised by frequent and unexpected episodes of metabolic decompensation despite good dietary therapy. However, a strict appreciation of these criteria is difficult. A more generalised indication for OLT in PA will require more information about the long-term outcome of transplanted patients. We should also await other alternatives like auxiliary partial OLT from living donors or transplantation of isolated allogenic hepatocytes, genetically modified or not.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1471-0528
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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