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  • 1
    ISSN: 1432-1076
    Keywords: Key words Lyme arthritis ; Diagnosis ; Clinical score
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Diagnosis of Lyme arthritis (LA) in children and adolescents may be difficult due to non-specific clinical manifestations and unreliable serological tests for antibodies to Borrelia burgdorferi. In a national prospective study, 186 children with arthritis were examined in whom the attending physicians had considered the diagnosis of LA. Ultimately, LA was confirmed in 87 patients and these were compared with the remaining 99 children in whom arthritis was attributable to other causes. In comparison to patients with other causes of arthritis, patients with LA had a higher frequency of episodic arthritis and initial knee joint arthritis, reported tick bites more frequently, were older, had a lower frequency of initial arthralgias, and there were fewer large joints involved. A score was developed in a group of these patients and tested in a second group. It enabled patients with LA to be distinguished from those with other causes of arthritis: within a range from 12 to −7 points, a score of 2.5 or less excluded LA whereas 6 or more points were highly indicative of LA. If only those children with a score result between 2.5 and 6 had been tested for antibodies to B. burgdorferi, the number of tests would have been reduced by 63%. Conclusion Careful analysis of clinical presentation and use of a clinical score may help in distinguishing LA from other causes of arthritis and thus reduce unnecessary and expensive testing and uninterpretable test results.
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Der Onkologe 4 (1998), S. 791-797 
    ISSN: 1433-0415
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Bei den akuten myeloischen Leukämien (AML) handelt es sich um heterogene, maligne Erkrankungen des hämatopoetischen Systems, denen die maligne Transformation einer hämatopoetischen Vorläuferzelle zugrunde liegt [9]. Folge dieser Transformation ist ein Verlust oder die Einschränkung des Differenzierungspotentials der Zellen mit einem Ausreifungsstop auf einem frühen Stadium der Myelopoese. Bei erhaltener Proliferationskapazität kommt es zur klonalen Expansion der malignen Zellen mit sukzessiver „Verdrängung” der normalen Hämatopoese, an deren Folgen der Patient unbehandelt innerhalb weniger Wochen verstirbt. Haupttodesursachen sind Blutungen und /oder Infektionen.
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  • 3
    ISSN: 1433-8491
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 4
    ISSN: 1433-0415
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Der vorgestellte Fallbericht beschreibt den klinischen Verlauf einer Patientin mit einer Reihe benigner Erkrankungen sowie einem Nierenzellkarzinom beidseits, einem Osteosarkom des Beckenknochens und einem Mammakarzinom. Der mögliche Kausalzusammenhang zwischen multiplen Erkrankungen der Patientin und der intravenösen Behandlung mit einem radioaktiven Medikament im Kindesalter wird dargestellt.
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  • 5
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Juvenile chronische Arthritis ; Oligoartikuläre Spätform ; Sulfasalazin ; HLA B27 ; Key words Juvenile rheumatoid arthritis ; Late onset pauciarthritis ; Sulphasalazine ; HLA B27
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Objective: To prove the response and side effects of sulphasalazine in early and late onset juvenile pauciarthritis, also the therapeutic effect dependent on the marker HLA B27. Methods: 48 patients between the age of 3 to 16 years were included in the retrospective study, median age 11,8 years. Mean time of follow-up was 64 weeks. 8 children had early onset pauciarthritis, 40 had late onset pauciarthritis. The dosage of sulphasalazine was 30–50 mg/kg/d. Clinical and laboratory disease activity were documented. Results: Partial and complete remission were observed in 3 of 8 patients with early onset pauciarthritis and in 27 of 40 patients with late onset pauciarthritis. The best response was noted in children with HLA B27 associated late onset pauciarthritis in 21 of 27 cases, in HLA B27 negative late onset pauciarthritis only 6/13 responded (p〈0.05). Side effects were seen in 7 out of 48 patients and had been reversible. Conclusion: The best response in the treatment with sulphasalazine was noted in HLA B27 associated late onset pauciarthritis. In general sulphsalazine is well tolerated, documented side effects had been reversible.
    Notes: Zusammenfassung Fragestellung: Geprüft werden sollen die Wirksamkeit und die Verträglichkeit von Sulfasalazin bei der Früh- und Spätform der juvenilen chronischen Oligoarthritis sowie der Therapieeffekt in Abhängigkeit vom Merkmal HLA B27. Methode: Die retrospektive Untersuchung umfaßte 48 Patienten im Alter von 3–16 Jahren, Median 11,8 Jahre, über einen mittleren Beobachtungszeitraum von 64 Wochen. 8 Kinder sind an einer Frühform, 40 an einer Spätform der Oligoarthritis erkrankt. Die Dosis von Sulfasalazin lag bei 30–50 mg/kg und Tag. Untersucht wurden klinische und laborchemische Aktivitätskriterien. Ergebnisse: Teil- und Vollremissionen zeigten 3 von 8 Kinder mit Frühform und 27 von 40 Patienten mit Spätform der Oligoarthritis. Den größten Therapieerfolg verzeichnete die Gruppe der HLA-B27-assoziierten Spätform mit 21 von 27 Patienten, gegenüber 6 von 13 Patienten mit HLA-B27-negativer oligoartikulärer Spätform (p〈0,05). Reversible Nebenwirkungen wurden bei 7 der 48 Patienten beobachtet. Schlußfolgerung: Die Therapie mit Sulfasalazin ist bei HLA-B27-assoziierter oligoartikulärer Spätform am wirksamsten. Sulfasalazin ist gut verträglich, die Nebenwirkungen waren reversibel.
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  • 6
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Systemische Sklerodermie ; Morbus Sharp ; 24-h-pH-Metrie ; Gastroösophagealer Reflux ; Key words Systemic scleroderma ; Mixed connective tissue disease ; 24-hours pH-metry ; Gastro-esophageal reflux
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Objectives: Esophageal motility disorders and gastroesophageal refluxes are the most frequent gastrointestinal manifestations of progressive systemic sclerosis and Morbus Sharp (”mixed connective tissue disease”). The prognosis of the internal involvements of this uncommon disorders in childhood and adolescence is not well known. Methods: Four patients with systemic sclerosis and two with Morbus Sharp were investigated with two times ambulatory 24-hour esophageal pH-metry for existence and/or degeree of gastro-esophageal reflux. The interval between the two investigations averaged 13,5 months. During this time, none of the patients had symptoms and therefore no H2-antagonists or prokinetic drugs. Results: At both examinations 3/6 patients had an increased percentage of pH〈4. One patient developed retrosternal pain before the second examination. Three patients had an increase of their reflux index. We documented an increase of reflux events lasting for more than 5 min; this result documents a decrease of the esophageal clearance function. Conclusion: In patients with systemic scleroderma or Morbus Sharp a lack of symptoms does not exclude gastroesophageal involvement. In the course of the diseases some of the patients develope a deterioration of the esophageal motility, inspite of immunosuppresive or antiinflammatory therapy. Further studies have to prove the effects of a pre-symptomatic treatment with H2-antagonists or prokinetic drugs on the prognosis of esophageal involvement in systemic scleroderma or Morbus Sharp during childhood.
    Notes: Zusammenfassung Fragestellung:Ösophagusmotilitätsstörungen und gastroösophagealer Reflux sind die häufigsten gastrointestinalen Manifestationen der systemischen Sklerodermie und des Morbus Sharp (mixed connective tissue disease). Über den Spontanverlauf dieser internen Organmanifestationen liegen für das Kindes- und Jugendalter keine Daten vor. Wir untersuchten diese Fragestellung bei pädiatrischen Patienten, die an dieser im Kindesalter seltenen Erkrankung litten, im Rahmen der klinischen Verlaufskontrollen. Patienten und Methode: Es wurden bei 4 Patienten mit systemischer Sklerodermie und 2 Patienten mit Morbus Sharp in 2 24-h-pH-Metrien das Vorliegen und das Ausmaß eines gastroösophagealen Refluxes gemessen. Der durchschnittliche Zeitabstand betrug 13,5 Monate. Während des Beobachtungsintervalls erhielten die Patienten aufgrund fehlender klinischer Symptome keine Säureblocker oder Prokinetika. Ergebnisse: Bei 3/6 Patienten wurde zu beiden Meßzeitpunkten ein erhöhter Refluxindex nachgewiesen. Während nur 1 Patient im Beobachtungszeitraum bis zum Zeitpunkt der Wiedervorstellung und Zweitmessung ein Sodbrennen entwickelte, konnte bei 3 Patienten eine z.T. deutliche Zunahme des gastroösophagealen Refluxes dokumentiert werden. Dabei fand sich insbesondere eine Zunahme der längeren Refluxereignisse als Hinweis auf eine gestörte Clearance-Funktion des distalen Ösophagus. Schlußfolgerung: Das Fehlen klinischer Symptome schließt eine gastroösophageale Beteiligung bei pädiatrischen Patienten mit Sklerodermie und Morbus Sharp nicht aus. Im Spontanverlauf kommt es bei einem Teil der Patienten trotz immunsupressiver und antientzündlicher Therapie der rheumatologischen Grunderkrankung zu einer Progredienz der Ösophagusmotilitätsstörung. Es ist in weiteren Studien zu prüfen, ob eine frühzeitige Therapie mit Prokinetika und H2-Rezeptor-Antagonisten sich im Kindes- und Jugendalter vorteilhaft auf die ösophagealen Komplikationen der systemischen Sklerodermie und des Morbus Sharp auswirkt.
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Annals of hematology 45 (1982), S. 351-357 
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 8
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 9
    ISSN: 1432-0584
    Keywords: Myelofibrosis ; Chromosomal aberrations ; Circulating hemopoietic precursor cells ; Human placenta-conditioned medium
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Cytogenetic studies were performed on nine patients with myelofibrosis. Mononuclear cells from the peripheral blood were cultured for two days in the presence of human placenta-conditioned medium (HPCM) prior to the preparation of metaphases. Two patients showed abnormal clones. In one of them the Y chromosome was missing. In the other patient the abnormal clone was characterized by the involvement of the chromosomes 7 and 9 (46,XX,7q-,−9,+mar). The significance of cytogenetically abnormal circulating hemopoietic precursor cells in patients with myelofibrosis is discussed.
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  • 10
    ISSN: 1432-0584
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To define the contribution of T-lymphocyte subsets in the development of aplastic anemia (AA), T-cell subpopulations including αβT cells, γδT cells, and δTCS1-positive γδT cells, were analyzed by cytophotometry in the peripheral blood (PB) and bone marrow (BM) of patients with AA before and after 6 weeks of therapy with anti-lymphocyte globulin (ALG), methylprednisolone, and cyclosporin A (CSA). In nine patients with AA a significant decrease of PB- and BM-derived T cells was observed after 6 weeks of therapy as compared with normal controls. At diagnosis, the CD4/CD8 ratio in PB and BM of the patients did not differ from the ratio in the control population; however, a reversed ratio (〈 1) was present in PB as well as in BM after weeks of therapy. Interestingly, lymphocytes expressing the γδT-cell receptor (TCRτδ) were significantly decreased both before (PB 1.2±0.1%; BM 0.8±0.1%) and after 6 weeks of therapy (PB 0.7±0.1%; BM 0.7±0.1%) as compared with healthy controls (PB 2.4±0.2%; BM 2.3±0.2%). However, the proportion of the γδ-T-cell subpopulation expressing the δTCS1 phenotype was markedly increased before (PB 42±3.5%; BM 31±3%) and especially after 42 days of therapy (PB 77±12%; BM 45±2%) as compared with that in normal subjects (PB 19±2%; BM 9.7±0.8%). At present, follow-up is under evaluation to correlate these findings with hematological response. The pathophysiological significance of the observed alterations within the T-cell subsets and especially the γδT-cell populations will require further functional analyses, in particular since δTCS1-positive γδT cells exhibit autoimmunological capacity.
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