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  • 1
    Electronic Resource
    Electronic Resource
    Molecular basis of variegate porphyria: a de novo insertion mutation in the protoporphyrinogen oxidase gene (1996)
    Springer
    Human genetics 〈Berlin〉 99 (1996), S. 126-129 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract The porphyrias are disorders that result from the inherited or acquired dysregulation of one of the eight enzymes in the heme biosynthetic pathway. Variegate porphyria (VP) is characterized by deficiencies in protoporphyrinogen oxidase (PPO) and has recently been genetically linked (Z = 6.62) to the PPO gene on chromosome 1q21. In this study, we have identified two sequence variants in the PPO gene in a family with VP. The first is a neutral polymorphism at the -47 position of intron 2; this polymorphism is present in the general population and is unlikely to underlie the VP phenotype. The second is a mutation in the PPO gene in a patient with VP; the mutation consists of an apparently de novo 2-bp insertion in exon 3 of PPO and results in a frameshift and downstream premature termination codon. These data establish that a frameshift mutation in PPO is the underlying mutation in this patient with VP and explain the sporadic occurrence of the phenotype in this family.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004390050325
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  • 2
    Electronic Resource
    Electronic Resource
    Niedrig dosierte Cyclosporin-A-Therapie bei Pyoderma gangraenosum Erfahrungen bei 6 Patienten (1995)
    Springer
    Der Hautarzt 46 (1995), S. 697-701 
    Details
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Pyoderma gangraenosum ; Cyclosporin A ; Key words Pyoderma gangraenosum ; Cyclosporine A
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Pyoderma gangraenosum can cause great therapeutic problems. High dosed corticosteroids are the treatment of choice. However, recalcitrant pyoderma gangraenosum or side effects from corticosteroid treatment may require therapeutic alternatives. Pyoderma gangraenosum responds excellently to treatment with cyclosporine A. Because of side effects and drug interactions, the patients must be selected and carefully and closely monitored. Six patients with pyoderma gangraenosum, unresponsive to various topical and systemic therapies, were treated with oral cyclosporine A at mean daily doses of approximately 3 mg/kg. Marked improvement of the skin lesions and complete healing occurred in all patients over a period of 3–6 months. Only one patient suffered a relapse after discontinuation of the treatment. No severe irreversible side effects occurred. The results show that low-dose cyclosporine A treatment can be considered a first-line treatment of pyoderma gangraenosum.
    Notes: Zusammenfassung Das Pyoderma gangraenosum bereitet oft große therapeutische Probleme. Behandlung der Wahl ist eine hochdosierte Glukokortikoidtherapie, die häufig zur Abheilung führt. Therapierefraktäre Fälle oder schwere Steroidnebenwirkungen erfordern jedoch therapeutische Alternativen. Das Pyoderma gangraenosum gehört zu den Dermatosen, die hervorragend auf Cyclosporin A ansprechen. Nebenwirkungen und die Interaktionen mit anderen Medikamenten erfordern eine sorgfältige Auswahl und Überwachung der Patienten. Die Ergebnisse der Behandlung von 6 Patienten mit therapierefraktärem Pyoderma gangraenosum werden dargestellt. Alle Patienten sprachen innerhalb eines Zeitraums von 3–6 Monaten gut auf die Therapie an. Eine Cyclosporin-A-Dosis von 3 mg/kg KG oder weniger war bei allen Patienten ausreichend. Lediglich bei einer Patientin kam es nach Absetzen des Cyclosporin A zum Rezidiv. Schwere irreversible Nebenwirkungen wurden nicht beobachtet. Unsere Ergebnisse zeigen, daß niedrig dosiertes Cyclosporin A als Therapie der ersten Wahl beim Pyoderma gangraenosum in Erwägung gezogen werden sollte.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001050050324
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  • 3
    Electronic Resource
    Electronic Resource
    Modellierung , Simulation, Visualisierung: Zu aktuellen Aufgaben der Informatik (1998)
    Springer
    Informatik-Spektrum 21 (1998), S. 265-272 
    Details
    ISSN: 1432-122X
    Keywords: Schlüsselwörter  Modellierung ; Simulation ; Visualisierung ; Methodologie ; Key words  Modelling ; Simulation ; Visualisation ; Methodology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Computer Science
    Description / Table of Contents: Summary   In many applications of Computer Science specifications and implementations of process models are designed requiring detailed knowledges of basic modelling procedures. In this paper the authors develop a specific meta-terminology on a high level of abstraction by using the background of various disciplines. Different model types and their particular applications (simulation and visualization) are outlined. Some illustrative examples are used to explain the basic methods.
    Notes: Zusammenfassung   Zahlreiche Anwendungen der Informatik erfordern die Erstellung oder Bearbeitung von Modellen konkreter Abläufe oder Prozesse, zu deren erfolgreicher Durchführung ein Einblick in die grundlegenden Verfahrensschritte der Modellierung unabdingbar ist. Die Verfasser versuchen, im vorliegenden Text über eine genaue Beschreibung der Aufgaben auf einem hohen Abstraktionsniveau zu einer einschlägigen (Meta-)Terminologie zu gelangen und zu zeigen, unter welchen Bedingungen sich unterschiedliche Modelltypen auch für nachfolgende Verarbeitungsschritte (Simulation und Visualisierung) nutzen lassen. Die zentralen Methoden werden mit einigen einfachen Beispielen illustriert.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002870050104
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  • 4
    Electronic Resource
    Electronic Resource
    Contact dermatitis from UV-A and UV-B filters in a patient with erythropoietic protoporphyria (1993)
    Oxford, UK : Blackwell Publishing Ltd
    Contact dermatitis 28 (1993), S. 0 
    Details
    ISSN: 1600-0536
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-0536.1993.tb03443.x
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  • 5
    Electronic Resource
    Electronic Resource
    Lipid composition and synthesis of HaCaT cells, an immortalized human keratinocyte line, in comparison with normal human adult keratinocytes (1993)
    Oxford, UK : Blackwell Publishing Ltd
    Experimental dermatology 2 (1993), S. 0 
    Details
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract Cultured keratinocytes are frequently employed for studies of epidermal lipid metabolism. Interpretation of experimental data may be complicated by donor to donor variability, the relatively short culture lifetime and variations between passages, problems thai are not encountered to the same extent with immortalized cell lines. The present study was undertaken to compare the lipid composition and synthesis of normal human adult keratinocytes (NHAK) with HaCaT cells, a long-lived. spontaneously immortalized human keratinocyte line, in relation to proliferation and differentiation. No differences between the two cell types were observed: a) in total lipid content; b) in the distribution of major lipid classes during growth at 50%, 75% and 100% confluence: c) in cultures grown at 0.6 mM calcium, at which differentiation is retarded, or at 1.6 mM calcium, at which some differentiation takes place; d) in the incorporation of [14C] acetate into cellular lipids at confluence, or e) in the fatty acid composition of major cellular lipid classes. At 100% confluence NHAK and HaCaT cells differ in their cholesterol metabolism. At all stages of growth, cholesterol synthesis in HaCaT cells is more LDL-dependent than in NHAK. Furthermore, NHAK become less LDL-dependent at confluence whereas HaCaT cells do not. HaCaT cells also revealed a significantly larger fraction of phosphatidyl-ethanolamine, -serine and -inositol at 0.6 mM calcium concentration than NHAK. These findings suggest that HaCaT cells do not differentiate as well as NHAK in vitro and may therefore serve as a model for the study of lipid metabolism in cells defective in terminal differentiation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-0625.1993.tb00030.x
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  • 6
    Electronic Resource
    Electronic Resource
    UVA irradiation stimulates the synthesis of various matrix-metalloproteinases (MMPs) in cultured human fibroblasts (1993)
    Oxford, UK : Blackwell Publishing Ltd
    Experimental dermatology 2 (1993), S. 0 
    Details
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract UVA irradiation leads to photoaging including clinical features such as wrinkle formation, reduced recoil capacity and blister formation of the skin. Besides synthesis of the extracellular matrix, its regulated degradation by various matrix-metalloproteinases (MMPs) determines the amount and the composition of the extracellular matrix within the dermis and the basement membrane of the dermo-epidermal junction. In this study we therefore ascertained whether UV irradiation could modulate the synthesis of MMPs with substrate specifities for dermal (collagen I, III, V) and basement membrane compounds (collagen IV, VII, proteoglycans, laminin) and whether synthesis of the counteracting tissue inhibitor of metalloproteinases (TIMP-I) was also affected. Following UVA irradiation specific mRNAs of MMPs 1, 2 and 3 were induced concomitantly up to 5-fold compared to mock irradiated controls. In contrast, TIMP-1 mRNA levels remained unaltered. Immunoprecipitation indicated that after UVA irradiation synthesis and secretion of MMPs 1, 2 and 3 into the supernatant increased. Taken together, our data show that UVA irradiation coordinately induced MMPs 1, 2 and 3 implying similar mechanisms in their regulatory pathways, while TIMP-I synthesis was not altered. Hence, unbalanced synthesis of MMPs potentially contributes to the dissolution of dermal and basement membrane compounds finally leading to blister formation and cutaneous photoaging.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-0625.1993.tb00015.x
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  • 7
    Electronic Resource
    Electronic Resource
    Dapsone in the treatment of lupus erythematosus (1981)
    Oxford, UK : Blackwell Publishing Ltd
    British journal of dermatology 104 (1981), S. 0 
    Details
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Dapsone yielded excellent therapeutic results in certain forms of lupus erythematosus (LE), whereas discoid lesions and the maculo-papular rash of the systemic and disseminated chronic forms of discoid LE remained uninfluenced by the drug. On the basis of these observations, we suggest the following indications for dapsone treatment in LE:〈list xml:id="l1" style="custom"〉1Vasculitic urticaria2Oral ulceratino3Non-scarring form of chronic LE4Chloroquine intolerance.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2133.1981.tb01711.x
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  • 8
    Electronic Resource
    Electronic Resource
    3 cases of contact dermatitis from alkylammonium amidobenzoate (Osmaron B®) (1992)
    Oxford, UK : Blackwell Publishing Ltd
    Contact dermatitis 27 (1992), S. 0 
    Details
    ISSN: 1600-0536
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-0536.1992.tb03301.x
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  • 9
    Electronic Resource
    Electronic Resource
    Porphyrin concentrations in various human tissues (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Experimental dermatology 4 (1995), S. 0 
    Details
    ISSN: 1600-0625
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract We measured the concentrations of total porphyrins and their metabolites (uro-, hepta-, hexa-, penta-, copro- and protoporphyrin) in various human tissues: liver, erythrocytes, skin, adipose tissue, and mammary gland. The porphyrin concentrations varied within minor limits, e.g., 3.1 ±2.3 nmol porphyrins/g liver and 0.50±0.10 nmol/g erythrocytes. No significant differences were detectable in other tissues in comparison with liver. In all tissues, the predominant metabolite was protoporphyrin, followed by coproporphyrin, whereas only low concentrations of higher carboxylatcd porphyrins such as uroporphyrin were detectable. It is concluded that porphyrin metabolism and its regulation is similar in all human tissues, perhaps with some small differences in the erythrocytes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1600-0625.1995.tb00248.x
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  • 10
    Electronic Resource
    Electronic Resource
    Extrakorporale Photopherese bei therapieresistentem disseminiert diskoidem Lupus erythematodes (DDLE) (1998)
    Springer
    Der Hautarzt 49 (1998), S. 487-491 
    Details
    ISSN: 1432-1173
    Keywords: Schlüsselwörter Extrakorporale Photopherese ; Diskoider Lupus erythematodes ; Kutaner Lupus erythematodes ; Photochemotherapie ; Key words Extracorporeal photopheresis ; Discoid lupus erythematosus ; Cutaneous lupus erytheamatosus ; Photochemotherapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations. Discoid LE (DLE) is the most common form of cutaneous LE; the disseminated form of DLE is rare. We report an encouraging response to treatment with extracorporeal photopheresis (ECP) in a single patient with disseminated DLE who did not respond to conventional therapy. To the best of our knowledge this is the first successful use of ECP in the management of such a patient. Extracorporeal photopheresis is a therapeutic modality that has been under investigation for more than 12 years. Although originally developed for the treatment of cutaneous T-cell lymphoma, ECP has recently been used for the management of autoimmune diseases including systemic scleroderma, pemphigus vulgaris and SLE, as well as prevent organ rejection in patients with cardiac or kidney transplants and graft versus host disease after bone marrow transplantation.
    Notes: Zusammenfassung Der Lupus erythematodes (LE) ist eine Erkrankung, die ein breites Spektrum von kutanen und systemischen Manifestationen zeigt. Der diskoide LE (DLE) wird am häufisten beobachtet, seltener ist die disseminierte Variante des DLE. Wir berichten über eine Patientin mit disseminiert diskoidem Lupus erythematodes (DDLE), die auf alle angewendeten konventionellen Therapien nicht ansprach; ein erstmals bei diesem Krankheitsbild durchgeführter Behandlungsversuch mit extrakorporaler Photopherese (ECP) führte zu einer raschen und deutlichen Besserung der Hautveränderungen. Die ECP stellt seit über 12 Jahren ein inzwischen erprobtes Behandlungsverfahren dar. Obwohl zunächst zur Behandlung des kutanen T-Zellymphom entwickelt, kommt die ECP heute zunehmend erfolgreich bei der Therapie von Autoimmun- und entzündlichen Erkrankungen zum Einsatz, z.B. systemische Sklerodermie, systemischer Lupus erythematodes (SLE), Pemphigus vulgaris, Abstoßungsreaktionen nach Herz- oder Nierentransplantation und „graft-versus-host-disease” (GvHD), nach Knochenmarktransplantation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001050050775
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