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  • 1
    Electronic Resource
    Electronic Resource
    Oxford BSL : Blackwell Science Ltd
    British journal of dermatology 140 (1999), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford : Blackwell Science Ltd
    Anaesthesia 54 (1999), S. 0 
    ISSN: 1365-2044
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Graefe's archive for clinical and experimental ophthalmology 206 (1978), S. 79-88 
    ISSN: 1435-702X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem normal bis überdurchschnittlich intelligenten 6-jährigen Mädchen mit enzymatisch gesicherter Niemann-Pickscher Krankheit (Restaktivität der Sphingomyelinase ca.10 % der Norm) werden Augenhintergrundsveränderungen beschrieben, die denjenigen des klassischen kirschroten Flecks bei Neurolipidosen ähnlich, jedoch quantitativ davon abgrenzbar sind. Es handelt sich um eine ringförmige retinale Opacität in der unmittelbaren Umgebung der bräunlich-rot erscheinenden Foveola mit zusätzlichen punktförmigen weißen Einlagerungen (Lipide?). Dem Fehlen jeglicher sonstiger neurologischer Symptomatik stehen eine extreme Hepatosplenomegalie, Speicherzellen im Knochenmark, Lungeninfiltration, Untergewicht und Minderwuchs entgegen. Daher wird der Fall dem Typ B der Niemann-Pickschen Erkrankung zugerechnet, obwohl zu diesem Typ definitionsgemäß keine zentralnervöse und bisher speziell auch keine oculo-neurale Beteiligung gehörte. In der Literatur wurde ein vergleichbarer Fall vorgefunden, der allerdings ohne enzymatische Sicherung blieb. Die Definition des Typs B der NiemannPickschen Erkrankung sollte künftig die Möglichkeit der oculo-neuralen Beteiligung einschließen.
    Notes: Summary In a 6-year-old girl with normal to outstanding intelligence Niemann-Pick disease was diagnosed by the demonstration of an about 90 % deficient sphingomyelinase activity. Abnormalities of the eye fundi are described which are comparable to but quantitatively deviate from the classic cherry-red spot as known from neurolipidoses. The brownish-red foveola was surrounded by a relatively thin opaque ring around which punctate white deposits (lipids?) could be detected. The absence of any other neurologic symptom was in contrast with an extreme hepatosplenomegaly, foam cells in the bone marrow, lung infiltration, underweight, and undergrowth. Therefore, the case was classed with the type B of Niemann-Pick disease, although the common definition of this type excludes cerebral as well as oculoneural involvement. In the literature only one comparable case could be found which, however, had not been enzymatically confirmed. In the future the definition of type B of Niemann-Pick diesease should include the possibility of oculoneural involvement.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Graefe's archive for clinical and experimental ophthalmology 215 (1980), S. 137-143 
    ISSN: 1435-702X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Vom Januar 1977 bis Mai 1978 wurden bei 101 Patienten mit einer Uveitis anterior aus dem südwestdeutschen Raum die HLA-Antigene bestimmt. Davon zeigten 75 (21 Patienten unter dem 20. Lebensjahr, 54 Patienten über dem 20. Lebensjahr) keine nachweisliche Begleit- oder Systemerkrankung. In der Gesamtserie von 75 Patienten ohne nachweisliche Begleiterkrankungen fand sich eine B27-Häufigkeit von 33,3% (Vergleichskollektiv 8,9%), eine Cwl-Häufigkeit in 16,0% (Vergleichskollektiv 5,8%) sowie die Kopplung B27-Cwl in 13,3% (Vergleichskollektiv 1,9%). Eine signifikante Assoziation mit HLA-Antigenen wiesen auf: 1. die adulte Form der Uveitis anterior (Erkrankungsalter über dem 20. Lebensjahr): B27-Antigen 38,9%; Cwl-Antigen 18,5%; Kopplung B27-Cwl 16,7%; 2. männliche Uveitis anterior-Patienten ohne Begleiterkrankung: B27-Antigen 46,2%; Cwl-Antigen 17,9%; Kopplung B27-Cwl 17,9%. Keine signifikante Assoziation mit HLA-Antigenen zeigten: 1. die juvenile Form der Uveitis anterior (Erkrankungsalter unter dem 20. Lebensjahr); 2. weibliche Uveitis anterior-Patienten ohne Begleiterkrankung.
    Notes: Abstract From January 1977 to May 1978, 101 patients with anterior uveitis were typed for HLA antigens. All came from the southwestern area of Germany, and 75 of these patients exhibited no signs of associated systemic disease (21 under 20 years of age, 54 over 20). In this group of 75 patients without associated systemic disease, a B27 frequency of 33.3% (8.9% in a random population) and a Cwl frequency of 16.0% (5.8% in a random population) was found as well as a B27-Cwl linkage of 13.3% (1.9% in a random population). A significant association with HLA antigens was found in (1) the adult form of anterior uveitis (disease onset over 20 years of age), namely, B27 antigen 38.9%, Cwl antigen 18.5%, B27-Cwl linkage 16.7%; and (2) male patients with anterior uveitis but without associated systemic disease, namely, B27 antigen 46.2%, Cwl antigen 17.9%, and B27-Cwl linkage 17.9%. No significant association with HLA antigens was found in (1) the juvenile form of anterior uveitis (disease onset under 20 years of age) and (2) female patients with anterior uveitis but without associated systemic disease.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1440
    Keywords: Hypertension ; Microcirculation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In this prospective cross-sectional study blood fluidity and peripheral microcirculation were measured in patients suffering from essential hypertension with and without macroangiopathy. The cutaneous microcirculation was evaluated by intravital microscopy and the intramuscular by pO2 needle electrode. Disorders in the microcirculation without macroangiopathy in the system of the feeding arteries are defined as primary microangiopathy. Disturbed microcirculation with macroangiopathy in the feeding arteries in one area but no detectable microcirculatory disorder in another region is defined as a secondary microcirculatory disorder. Of the 57 patients in this study 27 had a primary microcirculatory disorder. It was remarkable that all 27 hypertension patients had a microcirculatory disorder in the area of the skin. Intramuscular microcirculatory disorder on its own without affection of the skin was not detected in any case. An exclusively secondary microcirculatory disorder occurred in 16 patients. This study shows that 93% of the patients with long-term essential arterial hypertension have microcirculatory disorders. It is most interesting that about one-half of these hypertension patients had a primary microcirculatory disorder, i.e., no indication of a hemodynamically active stenosis was found in the large vessels.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-055X
    Keywords: Schlüsselwörter Orale Prämedikation ; Midazolam ; kognitive Funktionen ; Retrobulbäranästhesie ; ambulante Chirurgie ; Key words Oral premedication ; Midazolam ; Cognitive functions ; Retrobulbar anaesthesia ; Day surgery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Abstract The number of surgical procedures performed as day surgery has significantly increased in recent years. Therefore, a safe and short postoperative recovery period has become increasingly important. The aim of the present study was to investigate perioperative cognitive and physiological function after oral premedication with low-dose midazolam (3.75 mg), especially during the postoperative period. Methods. Forty-seven men (age 〉60 years, weight 50–90 kg) scheduled for elective cataract surgery under retrobulbar anaesthesia (RBA) were included in the study. The patients were randomly assigned to either group 1 (n=28), receiving 3.75 mg midazolam p.o. (Dormicum®), or group 2 (n=19), receiving a placebo orally 30 min before RBA. We measured the following parameters: sedation (modified Glasgow coma scale); anxiety (visual analogue scale); numerical and verbal memory (digit span and reproduction of previously presented words); concentration (Revisionstest of Stender/Marschner). To identify depression of ventilation, pulse oximetry and nasal end-tidal PCO2 were monitored intraoperatively. Results. After premedication with 3.75 mg midazolam, patients were significantly more sedated (P〈0.01) and systolic blood pressures were significantly reduced (P〈0.05); 30 min after midazolam premedication only concentration was significantly (P〈0.05) decreased. The results of the other cognitive functions did not differ. No differences in cognitive and physiological functions between the groups could be found 2 h after the operation (293±41 min after premedication). Intraoperatively, there were no significant differences in end-tidal PCO2 and oxygenation between the groups. In both groups anxiety and blood pressure were significantly higher pre- than postopertively. Conclusion. Oral administration of low-dose midazolam (0.049±0.006 mg/kg) seems to be appropriate for premedication before ambulatory surgical procedures in elderly patients. In the interest of patient safety, standardised oral premedication with 3.75 mg midazolam may not be sufficient for some of the patients.
    Notes: Zusammenfassung Insbesondere unter dem Aspekt ambulanter Chirurgie sollte überprüft werden, inwieweit eine orale Prämedikation mit 3,75 mg Midazolam bei Männern über 60 Jahre perioperativ kognitive und physiologische Funktionen verändert. In einer randomisierten, doppelt maskierten und plazebokontrollierten Studie wurden insgesamt 47 Männer (Alter 〉60 Jahre, Gewicht 50 bis 90 kg) untersucht, bei denen ein intraokularer Eingriff in Retrobulbäranästhesie durchgeführt wurde. Folgende Meßgrößen wurden bestimmt: Sedierung (Rangskalierung mit 5 Stufen), Ängstlichkeit (visuelle Analogskala), Merkfähigkeit (Zahlennachsprechen), Konzentrationsfähigkeit (Revisionstest nach Stender u. Marschner), Langzeitgedächtnis (Wortmerktest) und Schmerzempfindung (visuelle Analogskala). Zur Erfassung einer intraoperativen Atemdepression wurden intraoperativ pulsoxymetrische Sauerstoffsättigung und endexspiratorischer pCO 2 (nasal) kontrolliert. Eine halbe Stunde nach Midazolam-Prämedikation war die Sedierung signifikant stärker (p〈0,01) ausgeprägt und der systolische Blutdruck signifikant niedriger (p〈 0,05) als in der Plazebogruppe. Bei den kognitiven Funktionstests fanden sich nur 1 / 2 h nach Midazolam-Prämedikation signifikante Unterschiede im Konzentrationstest nach Stender u. Marschner (p〈0,05). 2 h nach Operation (293±41 min nach Prämedikation) zeigten sich keine Veränderungen der physiologischen und kognitiven Funktionen. Da kognitive und physiologische Funktionen unmittelbar nach der Prämedikation geringfügig eingeschränkt sind, postoperativ aber keine Veränderungen dieser Funktionen nachzuweisen sind, erscheint eine orale Prämedikation mit durchschnittlich 0,049±0,006 mg/kg Midazolam für ambulante Eingriffe bei älteren Patienten geeignet zu sein. Zugunsten der Sicherheit des Patienten ist bei routinemäßigem Einsatz von 3,75 mg Midazolam die Prämedikationsdosis bei einem Teil der Patienten vermutlich zu niedrig.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Human genetics 〈Berlin〉 80 (1988), S. 135-139 
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary In extension of a previous study, spontaneous and clastogen-induced chromosome damage was analyzed in cultures of peripheral blood lymphocytes from six further patients with Werner syndrome (WS) and six healthy controls. In addition, sister chromatid exchange (SCE) was estimated in four of these cases. Lymphocytes of patients with various other diseases were used for another series of control experiments. Diepoxybutane (DEB), 4-nitroquinoline-1-oxide (NQO), and bleomycin (BLM) were the standard clastogens throughout the study. While the spontaneous frequency of chromosomal breakage was significantly higher in lymphocytes from all the patients than in the control cells, the basis SCE rate was un-affected in WS cells. Sensitivity of WS cells to the chromosome-damaging action of BLM did not differ from that of control cells, and their sensitivity to DEB was slightly greater than that of control lymphocytes. However, NQO induced a more distinct increase of both break and interchange aberrations in the WS cells than in control cells or cells from patients with other diseases. This effect was not found for the SCE rate. Our data demonstrate the exceptional cytogenetic features of this syndrome: Although the spontaneous and the DEB- and NQO-induced chromosomal breakage rate would suggest that WS is like a classic chromosomal instability syndromes, the lack of sensitivity of WS cells to bleomycin and their stable SCE frequency compared with that of control cells clearly delimitate this syndrome from other entities.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Human genetics 〈Berlin〉 70 (1985), S. 324-327 
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Chromosome studies were performed on peripheral lymphocytes from two patients with Werner syndrome and two healthy control individuals to detect spontaneous and/or mutagen-induced chromosomal instability of this disease. Diepoxybutane, isonicotinic acid hydrazide, 4-nitro-quinoline-1-oxide, and bleomycin were used as standard clastogens. While the spontaneous frequency of chromosomal breakage was much higher in lymphocytes from both patients than in the control cells, the basic rate of sister chromatid exchange (SCE) was found to be in the control range. The sensitivity to clastogens of the patients' cells, however, was not substantially increased as compared with the controls if the degree of multiplication of the spontaneous breakage rate or SCE frequency was taken as the basis for comparison. No indication of a greater inhibition of proliferation by the clastogens in the patients' cells than in normal cells was observed using BrdU-labelled lymphocytes. Thus, the lymphocytes from both patients of the present study lacked essential features of the classical chromosome instability syndromes.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European archives of psychiatry and clinical neuroscience 230 (1981), S. 129-139 
    ISSN: 1433-8491
    Keywords: Carotid stenosis ; Ocular symptoms ; Ischemic ophthalmopathy ; Ischemic optic neuropathy ; Retinal embolism ; Carotisstenose ; Augensymptome ; Ischämische Ophthalmopathie ; Opticusneuropathie ; Retinale Embolien
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 46 Patienten mit einer durch direkte Dopplersonographie gesicherten Stenose oder einem Verschluß der A. carotis interna erfolgte eine ophthalmologische Untersuchung. Neun Patienten wiesen dabei eine ischämische Ophthalmopathie auf. Acht litten unter Amaurosis fugax, sechs unter einer ischämischen Opticusneuropathie, fünf unter retinaler Embolie und drei unter Gesichtsfelddefekten. Zwei der obengenannten Patienten hatten zwei verschiedene oculäre Manifestationen. Sieben Patienten waren ophthalmologisch unauffällig. Zehn Patienten wiesen Erkrankungen der Augen auf, die nicht offensichtlich in Beziehung zu dem Verschlußprozeß der extrakraniellen Arterien standen. Eine ischämische Ophthalmopathie, gemäß augenärztlicher Primärdiagnose, war immer die Folge einer hochgradigen Stenose oder eines Verschlusses der A. carotis interna.
    Notes: Summary Forty-six patients with stenosis or occlusion of the internal carotid artery were evaluated by direct Doppler sonography and ophthalmologic examination. A retrospective analysis showed the following secondary ocular manifestations of vascular disease: ischemic ophthalmopathy (9), amaurosis fugax (8), ischemic optic neuropathy (6), and retinal embolism ((5). Two patients had two ocular manifestations; three showed homonymous visual field defects. Results of the ophthalmologic examination were normal in seven patients. Eye changes not obviously related to extracranial vascular disease were present in ten patients. Ischemic ophthalmopathy was invariably due to an occlusive disease of the internal carotid artery.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of orofacial orthopedics 46 (1985), S. 498-498 
    ISSN: 1615-6714
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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