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  • 1
    Online Resource
    Online Resource
    Berlin, Heidelberg : Springer Berlin Heidelberg
    Keywords: Medicine ; Radiology, Medical ; Ophthalmology ; Medicine & Public Health ; Ophthalmology ; Radiology, Medical ; Medicine ; Retinal Diseases diagnosis ; Diagnostic Imaging ; Atlases ; Atlas ; Augenhintergrund ; Fluoreszenzangiographie ; Augenhintergrund ; Fluoreszenzangiographie
    Description / Table of Contents: This lavishly illustrated unique atlas provides a comprehensive and up-to-date overview of FAF imaging in retinal diseases. It also compares FAF findings with other imaging techniques such as fundus photograph, fluorescein- and ICG angiography as well as optical coherence tomography.General ophthalmologists as well as retina specialists will find this a very useful guide which illustrates typical FAF characteristics of various retinal diseases.
    Type of Medium: Online Resource
    Pages: Online-Ressource (XIV, 342p. 132 illus. in color, digital)
    ISBN: 9783540719946
    Series Statement: SpringerLink
    RVK:
    RVK:
    Language: English
    Note: Includes bibliographical references and index , Front Matter; Lipofuscin of the Retinal Pigment Epithelium; Origin of Fundus Autofluorescence; Fundus Autofluorescence Imaging with the Confocal Scanning Laser Ophthalmoscope; How To Obtain the Optimal Fundus Autofluorescence Image with the Confocal Scanning Laser Ophthalmoscope; Autofluorescence Imaging with the Fundus Camera; Macular Pigment Measurement-Theoretical Background; Macular Pigment Measurement -Clinical Applications; Evaluation of Fundus Autofluorescence Images; Macular and Retinal Dystrophies , Discrete Lines of Increased Fundus Autofluorescence in Various Forms of Retinal DystrophiesAge-Related Macular Degeneration I-Early Manifestation; Age-Related Macular Degeneration II-Geographic Atrophy; Age-Related Macular Degeneration III-Pigment Epithelium Detachment; Age-Related Macular Degeneration IV-Choroidal Neovascularization (CNV); Idiopathic Macular Telangiectasia; Chorioretinal Inflammatory Disorders; Autofluorescence from the Outer Retina and Subretinal Space; Miscellaneous; Perspectives in Imaging Technologies; Back Matter
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  • 2
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] The gene RPGR was previously identified in the RP3 region of Xp21.1 and shown to be mutated in 10–20% of patients with the progressive retinal degeneration X-linked retinitis pigmentosa (XLRP). The mutations predominantly affected a domain homologous to RCC1, a guanine nucleotide exchange ...
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Retinitis pigmentosa (RP) is the term applied to a clinically and genetically heterogeneous group of retinal degenerations that primarily affects the rod photoreceptors and has a prevalence of approximately 1 in 3,000. RP is characterized by progressive loss of vision, initially manifesting as ...
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Autosomal dominant retinitis pigmentosa (adRP) is known to result from mutations in two different retinal genes—rhodopsin and peripherin—while a third locus has been implicated by linkage data. However, families have been reported in which all three known loci have been excluded. We ...
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Malattia Leventinese (ML) and Doyne honeycomb retinal dystrophy (DHRD) refer to two autosomal dominant diseases characterized by yellow-white deposits known as drusen that accumulate beneath the retinal pigment epithelium (RPE). Both loci were mapped to chromosome 2p16-21 (Refs 5,6) and this ...
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  • 6
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract Doyne honeycomb retinal dystrophy (DHRD) is a late-onset autosomal dominant disorder that causes degeneration of the retina and can lead to blindness. We have previously assigned DHRD to a 5-cM region of chromosome 2p16 between marker loci D2S2739 and D2S378. Using sequence-tagged sites (STSs), expressed sequence tags (ESTs) and polymorphic markers within the DHRD region, we have identified 18 yeast artificial chromosomes (YACs) encompassing the DHRD locus, spanning approximately 3 Mb. The YAC contig was constructed by STS content mapping of these YACs and incorporates 13 STSs, including four genes and six polymorphic marker loci. We also report the genetic mapping of two families with a dominant drusen phenotype to the DHRD locus, and genetic refinement of the disease locus to a critical interval flanked by microsatellite marker loci D2S2352 and D2S2251, a distance of approximately 700 kb. These studies exclude a number of candidate genes and provide a resource for construction of a transcriptional map of the region, as a prerequisite to identification of the DHRD disease-causing gene and genes for other diseases mapping in the region, such as Malattia leventinese and Carney complex.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Graefe's archive for clinical and experimental ophthalmology 237 (1999), S. 1-9 
    ISSN: 1435-702X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  · Background: Recently a technique of imaging the retinal pigment epithelium (RPE) has been developed that takes advantages of its intrinsic fluorescence derived from lipofuscin. The purpose of this study was to document the distribution of fundus autofluorescence in patients with various retinal diseases and its change over time. · Methods: The intensity and spatial distribution of fundus autofluorescence was documented in 318 eyes from 159 patients with various retinal diseases using a confocal Laser Scanning Ophthalmoscope. Thirty patients with macular dystrophies and 30 with age-related macular disease underwent serial examinations over a period of 1–3 years in order to monitor the changes over time of fundus autofluorescence. · Results: Absent autofluorescence corresponded well spatially with outer retinal atrophy in eyes with retinitis pigmentosa and rod-cone dystrophy. Abnormally high background autofluorescence was seen in the macular region in some patients with dominant and recessive retinitis pigmentosa and rod-cone dystrophies. In areas of macular edema fundus autofluorescence was abnormal. Fundus autofluorescence showed changes over time in most of the eyes with retinal diseases studied. · Conclusion: Fundus autofluorescence allows documentation of areas of photoreceptor cell loss in eyes with retinitis pigmentosa and rod-cone dystrophies. If abnormal high background autofluorescence in the surviving areas occurs only in some patients with retinitis pigmentosa, the technique may serve to distinguish the regional from the diffuse type of disease. Over time, fundus autofluorescence may demonstrate change or may remain stable.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1433-0423
    Keywords: Key words Lipofuscin • Laser scanning ophthalmoscope • Fundus autofluorescence • Central serous chorioretinopathy ; Schlüsselwörter Lipofuszin • Autofluoreszenz • Chorioretinopathia centralis serosa • Retinales Pigmentepithel
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Hintergrund: Der Mangel an histopathologischem Material begrenzte bisher den Wissensstand über die strukturellen Veränderungen bei Chorioretinopathia centralis serosa (CRCS). Ziel dieser Arbeit war es, anhand der Methode der bildlichen Darstellung der Fundus-Autofluoreszenz Erkenntnisse über die pathologischen Veränderungen des retinalen Pigmentepithels (RPE) bei CRCS zu gewinnen. Patienten und Methoden: Das Verteilungsmuster von Lipofuszin im RPE wurde mit einem konfokalen Laser Scanning Ophthalmoskop (LSO, Zeiss, Oberkochen; Exzitationswellenlänge: 488 nm, Leistung: 250 μW, Filter ab 521 nm, Bildauflösung: 768 × 572 Pixel) an 62 Augen von 44 Patienten mit CRCS untersucht. Ergebnisse: Die Areale mit subretinaler Flüssigkeitsansammlung entsprachen im Frühstadium einer vermehrten Fundus-Autofluoreszenz, im fortgeschrittenen Stadium einem gemischten Bild von vermehrter und verminderter Autofluoreszenz. Schlußfolgerungen: Die Veränderungen der Autofluoreszenz im RPE, ein Index der Stoffwechselaktivität, treten bei CRCS fokal auf. Der Nachweis vorhandener, reduzierter oder fehlender Stoffwechselaktivität des RPE könnte zur Indikationsstellung einer Lasertherapie beitragen.
    Notes: Purpose: The lack of histopathological material has placed limitations on our knowledge on lipofuscin in central serous chorio-retinopathy (CSCR). This study was designed to document the pathological changes of the retinal pigment epithelium (RPE) in CSCR using in vivo recording of fundus autofluorescence. Methods: Fundus autofluorescence was documented in 62 eyes of 44 subjects with CSCR using a laser scanning ophthalmoscope (Zeiss, Oberkochen; excitation wavelength: 488 nm, barrier filter at 521 nm). Images were compared to the respective fundus appearance and fluorescein angiograms. Results: Neurososensory retinal detachments showed diffuse increased autofluorescence corresponding to the detached area. Long-standing lesions showed very irregular autofluorescence with regions greater and less than the background levels of autofluorescence. Conclusion: Focal accumulation of autofluorescent material occurs at the level of the RPE in patients with CSCR, relating to variation in metabolic activity of the RPE. This technique may be useful in selecting patients for laser photocoagulation.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1433-0423
    Keywords: Key words Lipofuscin • Laser scanning ophthalmoscope • Age-related macular degeneration • Tapeto-retinal dystrophies ; Schlüsselwörter Lipofuszin • Laser Scanning Ophthalmoskop • Altersbedingte Makuladegeneration • Vererbte Netzhautdystrophien • Tapetoretinale Dystrophien
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Hintergrund: Die Technik zur in vivo-Darstellung der Lipofuszinverteilung im retinalen Pigmentepithel (RPE) wurde so modifiziert, daß eine detailliertere Bildauflösung (768 × 572 Pixel) erreicht wurde. Erste Ergebnisse werden vorgestellt. Patienten und Methoden: Das Verteilungsmuster von Lipofuszin im RPE wurde mit einem konfokalen Scanning Laser Ophthalmoskop-Prototyp (Zeiss, Oberkochen; Exzitationswellenlänge: 488 nm, Leistung: 250 μW, Filter ab 521 nm) an 286 Augen von 143 Patienten mit Retinitis pigmentosa, vererbten Makuladystrophien und altersbedingter Makuladegeneration untersucht. Ergebnisse: Die Fundus-Autofluoreszenz war bei allen untersuchten Augen mit Netzhauterkrankungen im Vergleich zu den Normwerten verändert, jeder untersuchten Netzhauterkrankung konnte ein spezifisches Autofluoreszenzverteilungsmuster zugeordnet werden. Schlußfolgerungen: Die modifizierte Technik zur Bilderfassung der Fundus-Autofluoreszenz ermöglicht Untersuchungen des Lipofuszinverteilungsmusters im RPE in vivo mit bisher unerreichter Bildauflösung. Studien zur Einlagerung und des Abbaus von Lipofuszin in seiner Dynamik bei Patienten mit tapetoretinalen Dystrophien und altersbedingter Makuladegeneration könnten zur Klärung der Pathogenese dieser Erkrankungen beitragen.
    Notes: Background: The fundus autofluorescence imaging technique has been modified allowing improved image resolution (768 × 572 pixel). We present results of fundus autofluorescence studies using this technique. Materials and methods: Fundus autofluorescence was studied in 286 eyes of 143 patients with retinitis pigmentosa, macular dystrophies and age-related macular degeneration using a confocal laser scanning ophthalmoscope prototype (Zeiss, Oberkochen; excitation wavelength: 488 nm, cut-off filter at 521 nm). Results: The spatial distribution of autofluorescence was different in all diseased eyes investigated compared to the normal pattern of fundus autofluorescence. Each disorder showed a specific fundus autofluorescence appearance. Conclusions: The advanced technique of imaging fundus autofluorescence allows detailed studies of the lipofuscin distribution. In vivo analysis of the dynamics of accumulation and degradation of lipofuscin in eyes with tapeto-retinal dystrophies and age-related macular disease may contribute to elucidation of the pathogenesis of these disorders.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Graefe's archive for clinical and experimental ophthalmology 230 (1992), S. 362-365 
    ISSN: 1435-702X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Facioscapulohumeral (FSH) muscular dystrophy is known to be associated with retinal telangiectasis. However, there are only few reports of severe visual loss due to exudative complications, so the risk to vision has not been established. Because of the possible therapeutic implications, we have described two cases of young girls who developed FSH muscular dystrophy and exudative retinal detachment due to telángiectasis. In the first patient, the severity of the disease precluded visual recovery despite extensive photo- and cryotherapy. In the other, visual acuity in both affected eyes was retained after treatment. Fundus examinations in young children at risk of having the gene for FSH muscular dystrophy may be justified so that retinal vascular disease can be detected before it becomes untreatable.
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