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  • 1
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Chromatography B: Biomedical Sciences and Applications 310 (1984), S. 390-395 
    ISSN: 0378-4347
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: The existence of a mechanism by which the ester- and ether-linked aliphatic chains of the major phospholipids are retailored during their axonal transport and sorted to specific membrane systems along the optic nerve and tract was investigated. A mixture of [I-14C]hexadecanol and [3H]arachidonic acid was injected into the vitreous body of albino rabbits. At 24 h and 8 days later, the distribution (as measured by the 3H/14C ratio) and the positioning (as monitored by hydrolytic procedures) of radioactivity in the various phospholipid classes of retina, purified axons, and myelin of the optic nerve and tract were determined. At the two intervals after labeling, the 3H/14C ratios of each diradyl type of phosphatidylethanolamine and phosphatidylcholine were (a) substantially unchanged all along the axons within the optic nerve and tract and (b) markedly modified in comparison with those found in the retina and axons for molecular species selectively restricted to myelin sheath. Evidence is thus available that intraxonally moving ethanolamine and choline glycerophospholipids, among others, are added to axonal membranes most likely without extensive modifications. In contrast, they are transferred into myelin after retailoring. Through these two processes, the sorting and targeting of newly synthesized phospholipids to their correct membrane domains. such as axoplasmic organelles, axolemma, or periaxonal myelin, could be controlled.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    FEBS Letters 330 (1993), S. 129-132 
    ISSN: 0014-5793
    Keywords: Fluorescence quenching ; Lipid bilayer ; Tryptophan
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Neurochemical research 6 (1981), S. 633-647 
    ISSN: 1573-6903
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The uptake of different labeled precursors, their incorporation into lipids, and transport along the rabbit optic pathway [ipsilateral retina and optic nerve (ON), and contralateral optic tract (OT), lateral geniculate body (LGB), and superior colliculus (SC)] were investigated. Albino rabbits were used. The following radioactive precursors, either combined or separately, dissolved in 50 μl of saline containing 15% BSA, were injected into vitreous body: [2-3H]glycerol (50 μCi), [1-14C]palmitate (15 μCi), and [1-14C]linoleate (7.5 μCi). Animals were killed at different time intervals from 1 hr up to 24 days. The radioactivity of total lipids and of different phospholipid classes from total tissue was measured. One hour after the administration of precursors, the radioactivity into the retina was high and the incorporation of [3H]glycerol and [14C]palmitate increased until 12 hr and 24 hr, respectively. The incorporation of [14C]linoleate reached a maximum on the second day. The phospholipids of LGB and SC were intensively labeled after 4–8 hr, and their radioactivity increased up to the 10th day after injection, independent of the precursor employed. The results obtained indicate that the labeled hydrophilic and hydrophobic precursors used were actively incorporated into the retina. The phospholipids were later transported at a rapid rate along the optic pathway.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1590-3478
    Keywords: Optic nerve hypoplasia ; pupillary light-near dissociation ; hereditary ataxias and paraplegias ; peripheral neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Viene riportato un caso di un paziente di 12 anni che presenta l'associazione di ipoplasia bilaterale del nervo ottico con areflessia pupillare alla luce, paraparesi spastica, ipotrofia muscolare distale prevalente agli arti inferiori, piede cavo bilaterale e neuropatia periferica prevalentemente motoria; inoltre sono presenti rinolalia, ipotrofia con fascicolazioni della lingua e cardiopatia. Viene discusso il possible inquadramento di tale sindrome nell'ambito delle forme degenerative con paraparesi: vengono richiamati in particolare i caratteri differenziali dalla paraparesi spastica familiare di Strümpell e dalla sclerosi laterale amiotrofica.
    Notes: Abstract We report a case of bilateral optic nerve hypoplasia in a patient with pupillary light-near dissociation, spastic paraparesis with deep hyperreflexia, mild distal hypotrophy especially of lower limbs, bilateral pes cavus and signs of motor peripheral neuropathy. Other associated signs were rhinolalia, hypotrophy and fasciculations of the tongue and cardiomyopathy. We differentiate the condition from a number of hereditary ataxias and paraplegias. The clinical features of Strümpell-Lorrain spastic paraplegia and amyotrophic lateral sclerosis seem to resemble most closely that of our patient.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 7 (1993), S. 105-118 
    ISSN: 1432-198X
    Keywords: Uric acid ; 2,8-Dihydroxyadenine ; Xanthine ; Adenine phosphoribosyltransferase ; Hypoxanthineguanine phosphoribosyltransferase ; Familial juvenile gouty nephropathy ; Allopurinol
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Although gout and hyperuricaemia are usually thought of as conditions of indulgent male middle age, in addition to the well-known uricosuria of the newborn, there is much of importance for the paediatric nephrologist in this field. Children and infants may present chronically with stones or acutely with renal failure from crystal nephropathy, as a result of inherited deficiencies of the purine salvage enzymes hypoxanthine-guanine phosphoribosyltransferase (HPRT) and adenine phosphoribosyltransferase (APRT) or of the catabolic enzyme xanthine dehydrogenase (XDH). Genetic purine overproduction in phosphoribosylpyrophosphate synthetase superactivity, or secondary to glycogen storage disease, can also present in infancy with renal complications. Children with APRT deficiency may be difficult to distinguish from those with HPRT deficiency because the insoluble product excreted, 2,8-dihydroxyadenine (2,8-DHA), is chemically very similar to uric acid. Moreover, because of the high uric acid clearance prior to puberty, hyperuricosuria rather than hyperuricaemia may provide the only clue to purine overproduction in childbood. Hyperuricaemic renal failure may be seen also in treated childhood leukaemia and lymphoma, and iatrogenic xanthine nephropathy is a potential complication of allopurinol therapy in these conditions. The latter is also an under-recognised complication of treatment in the Lesch-Nyhan syndrome or partial HPRT deficiency. The possibility of renal complications in these three situations is enhanced by infection, the use of uricosuric antibiotics and dehydration consequent upon fever, vomiting or diarrhoea. Disorders of urate transport in the renal tubule may also present in childhood. A kindred with X-linked hereditary nephrolithiasis, renal urate wasting and renal failure has been identified, but in general, the various rare types of net tubular wasting of urate into the urine are recessive and relatively benign, being found incidentally or presenting as colic from crystalluria. However, the opposite condition of a dominantly inherited increase in net urate reabsorption is far from benign, presenting as familial renal failure, with hyperuricaemia either preceding renal dysfunction or disproportionate to it. Paediatricians need to be aware of the lower plasma urate concentrations in children compared with adults when assessing plasma urate concentrations in childhood and infancy, so that early hyperuricosuria is not missed. This is of importance because most of the conditions mentioned above can be treated successfully using carefully controlled doses of allopurinol or means to render urate more soluble in the urine. Xanthine and 2,8-DHA are extremely insoluble at any pH. Whilst 2,8-DHA formation can also be controlled by allopurinol, alkali is contraindicated. A high fluid, low purine intake is the only possible therapy for XDH deficiency.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Graefe's archive for clinical and experimental ophthalmology 209 (1979), S. 225-230 
    ISSN: 1435-702X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Chemical sympathectomy of the anterior segment of the eye was performed in rabbits with 6-hydroxydopamine (6-OHDA) intravitreously injected. The response of the pupillary diameter (PD) and intraocular pressure (IOP) to NE, E and Isoproterenol was determined through instillation of the drugs in the pretreated and control eyes. The results were as follows: 1) Both PD and IOP at different intervals of time from the injection show a sharp decrease in the treated eye. 2) About four weeks after the injection, the instillation of NE and E increases the PD only in the 6-OHDA pretreated eyes, while IP administration is not effective on both control and pretreated eyes. 3) The instillation of the three drugs, while decreasing IOP in the control eyes, does not modify the already low IOP in the pretreated eyes. The authors suggest the presence of a postdenervation supersensitivity affecting only the α receptors, since the β stimulant IP is inactive in both treated and untreated eyes.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Documenta ophthalmologica 14 (1960), S. 383-398 
    ISSN: 1573-2622
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary 1. Besides the forms of Leptospiral Uveitis with clinical evidence, there are others of slight degree and clinically silent, or almost so, involving the anterior segment (Iris and Ciliary body), whose identification is possible only by means of a very accurate Slit-lamp examination. It follows from the above-stated that the occurrence of Leptospiral Uveitis becomes strikingly higher than generally admitted (80–85% of the cases). 2. The appearance of the process, with respect to the onset of the general disease, is variable; however it most frequently manifest itself during the late part of the localization phase. Sometimes the Uveitis becomes evident even much later. In such cases it is debatable whether it represents a primary manifestation occurring late or an aggravation or a relapse of a previously established uveitis with a discret and inapparent course. In these cases a useful indicative criterion is given by a gonioscopic examination. 3. In the Author's series the serotype by far most frequent causing the process was the Leptospira ictero-hemorrh., followed by L. pomona, L. canicola, L. Bataviae. From a practical point of view it may be stated that all serotypes of Leptospira can produce a uveitis. It is still under discussion however if certain serotypes determine such conditions more frequently and with more severe course rather than others. 4. Leptospiral Uveitis is very frequently bilateral. The more common clinical forms are represented by: A) Anterior Uveitis; a) Serous Uveitis and Serofibrinous Uveitis under the form of Iritis or Iridocyclitis; b) Silent Cyclitis; B) Panuveitis. 5. The anterior type of Uveitis may recover quickly and in a definitive way or become chronic with the possible extension to involve the optic nerve and retina, thus creating forms of Neuro-Uveo-retinitis. Sometimes one can observe relapsing types. Forms have been described which were complicated by hypertension, cataract or panophthalmitis leading to eye enucleation. 6. One may also observe panuveitis characterized by a very slow and torpid course and with marked alterations of the vitreous body, which can be hardly differentiated from tuberculous Uveitis. 7. The diagnosis of Leptospiral Uveitis is based on: the existence of an associated or previously present general infectious process, the clinical characteristics, the serological tests on blood and aqueous humour, the demonstration (bacteriological and biological) of the Leptospira in the aqueous humour. 8. Therapy consists of symptomatic and antibiotic treatment, the latter not constantly effective. Aureomycin, alone or associated with penicillin, seems to be most efficacious. Sometimes paracenthesis is useful.
    Abstract: Zusammenfassung 1. Die Augenbeteiligung an einer Leptospirose vollzicht sich nicht immer unter dem klinischen Bild einer Uveitis; symptomarme Zustände, deren Diagnose sorgfältigste Spaltlampenuntersuchungen voraussetzen, sind nicht selten. Eine Augenbeteiligung an Leptospirosen erscheint unter Berücksichtigung der erwähnten symptomarmen Formen außerordentlich häufig: in etwa 85% der Fälle! 2. Die Uveitis entwickelt sich meist am Ende der Lokalisationsphase des gesamten Krankheitsbildes, mitunter jedoch auch sehr viel später. Bei Spätmanifestationen ist noch zu klären, ob es sich nicht etwa um das Aufflackern einer vorher nicht diagnostizierten symptomarmen Erkrankung handelt. 3. Wahrscheinlich können alle Leptospiren eine Krankheit mit Augenbeteiligung auslösen. In den meisten Fällen handelt es sich um Infektionen mit der L. icterohämorrhagiae; weniger häufig sind Infektionen mit L. pomona, L. canicola, L. Bataviae. 4. Die Uveitis bei Leptospirose betrifft oft beide Augen. Die häufigsten klinischen Formen sind: 5. Die Uveitis bei Leptospirose kann sowohl schnell und für die Dauer abheilen als auch zur Beteiligung von Sehnerv und Netzhaut führen (Uveo-neuro-Retinitis). Recidive sind häufig; Komplikationen (Sekundärglaukon, Cataract, Panophthalmie) werden zuweilen beobachtet. 6. In manchen Fällen ist der Glaskörper erheblich mitbeteiligt; die differentialdiagnostische Abgrenzung gegen tuberkulöse Erkrankungen ist dabei oft schwierig. 7. Die Diagnose einer durch Leptospiren hervorgerufenen Uveitis stützt zich auf Symptome der Allgemeininfektion, serologische Blut- und Kammerwasserbefunde und den Erregernachweis. 8. Neben einer symptomatischen Therapie kommt die Verabfolgung von Aureomycin (u. U. kombiniert mit Penicillin) in Betracht; die Wirkung der Antibiotica ist allerdings individuell sehr verschieden. Manchmal ist die Paracentese nützlich. A) Vordere Uveitis; a) seröse und serofibrinöse Iritis bzw. Iridocyclitis; b) symptomarme Cyclitis B) Panuveitis.
    Notes: Résumé 1. A coté des formes d'uvéite leptospirosique cliniquement manifestes, il y a des formes extrêmement légères et cliniquement muettes, ou presque, qui intéressent exclusivement le segment antérieur de l'uvée (iris et corps ciliaire). Ces formes peuvent être identifiées presque seulement par un examen très soigneux à la lampe à fente. En tenant compte de l'existence de ces manifestations discrètes, la fréquence des uvéites leptospirosiques devient beaucoup plus élévée qu'on ne croit couramment (80–85% des cas de leptospirose environs). 2. L'apparition de l'uvéite par rapport au début de la maladie générale est variable; on observe cependant que l'uvéite survient fréquemment à la fin de la phase de localisation. Quelque fois l'uvéite apparaît beaucoup plus tardivement. Dans cas on peut discuter s'il s'agit d'une première manifestation réellement tardive, d'une aggravation ou d'une récidive d'une uvéite passée initialement inaperçue. L'examen gonioscopique pourrait nous fournir dans ces cas douteux des indications utiles. 3. Dans la statistique de l'autéur le sérotype infectant prédominant est la L. ictero-haemorrhagiae, ensuite, avec un fort écart, la L. pomona, L. canicola, L. Bataviae. En pratique on peut supposer que tous les sérotypes de leptospires peuvent donner lieu à l'uvéite; reste encore à établir, si certains sérotypes peuvent donner des uvéites plus fréquentes et plus graves par rapport à d'autres. 4. L'uvéite leptospirosique est très souvent bilatérale; les formes cliniques plus communes sont: A) Uvéite antérieure a) uvéite séreuse et sérofibrineuse sous forme d'iritis on iridocyclite; b) cyclite silencieuse; B) Panuvéite. 5. Les formes d'uvéite antérieure peuvent guérir rapidement et définitivement, passer à l'état chronique ou s'étendre au nerf optique et à la rétine et donner lieu à des formes d'uvéo-neuro-rétinite. Quelques fois on peut observer des formes à récidives. On a aussi signalé des formes compliquées avec hypertension, cataracte, panophtalmie. 6. On peut rencontrer des panuvéites caractérisées par une allure très torpide et une profonde altération de la charpente vitréenne; elles posent un problème de diagnostic différentiel délicat avec des formes d'uvéite tuberculeuse. 7. Le diagnostic d'uvéite leptospirosique ressort de l'existence d'une infection générale, des caractères cliniques, de l'examen sérologique du sang et de l'humeur aqueuse, de la démonstration de la leptospire dans l'humeur aqueuse. 7. La thérapie se base sur le traitement symptomatique et sur le traitement général avec les antibiotiques. L'auréomycine, seule ou associée avec la penicilline, paraît la plus efficace. La paracentèse peut parfois être utile.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of inherited metabolic disease 20 (1997), S. 351-353 
    ISSN: 1573-2665
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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