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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
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    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: 1433-0415
    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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  • 4
    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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  • 5
    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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  • 6
    ISSN: 1432-0584
    Keywords: Itraconazole ; Vinca alkaloids ; Neurotoxicity ; MDR ; Cytochrome P-450
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In four of 14 patients with acute lymphoblastic leukemia (ALL) who received induction chemotherapy containing weekly injections of vincristine and simultaneous antifungal prophylaxis with itraconazole, unusually severe and early vincristine-induced neurotoxicity was observed. In these patients (three female, one male) paresthesia and muscle weakness of the upper/lower extremities and paralytic ileus occurred after the first or second vincristine injection. In one patient a laryngeal nerve paresis required mechanical ventilation. The neurotoxic complications were more serious than those seen in a previous series of 460 ALL patients under the identical cytostatic regimen but without itraconazole prophylaxis. The underlying mechanism is unclear. Interaction with the cytochrome P-450 system, reversal of multidrug resistance, and influence of estrogens are to be considered.
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  • 7
    ISSN: 1432-0584
    Keywords: Key words Acute myelocytic leukemia ; Endometriosis ; Acute abdomen ; Ultrasound
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Acute abdomial pain is a frequent diagnostic and therapeutic challenge in hematologic patients. We report on the very rare case of organ endometriosis with acute abdominal symptoms in a 43-year-old female patient with AML-M5, starting 4 days after induction chemotherapy with idarubicin, ara-C, and etoposide. The patient presented with an acute abdomen with clinical findings of acute cholecystitis, subileus, and local pain in the right upper abdomen accompanied by severe diarrhea. Probably due to impaired intestinal resorption, menstrual bleeding occurred despite regular administration of lynestrenol. Ultrasound examination of the abdomen disclosed a tumor with poor echoes in the pouch of Douglas, a subcapsular splenic hemorrhage, and a thickened gallbladder wall with surrounding edema. A cystic adnex tumor was confirmed by endovaginal ultrasound. Based on history and the findings on ultrasound, an endometriosis was diagnosed, and the LHRH agonist (nafarelin) was administered nasally in combination with lynestrenol. Following this medication the abdominal pain ceased, supporting the diagnosis of endometriosis. Nasal administration of an LHRH agonist in the following cycles of chemotherapy was effective in preventing further abdominal discomfort and vaginal bleeding. LHRH agonists should be given to patients with known endometriosis before starting myeloablative chemotherapy to prevent painful hemorrhage from endometriosis.
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  • 8
    ISSN: 1432-0584
    Keywords: Key words CD54 ; ALL ; TNFα ; IFNγ
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  CD54 (intercellular adhesion molecule-1, ICAM-1) surface expression on the blasts in ten cases of acute lymphoblastic leukemia (ALL) and its up-regulation by human recombinant interferon-γ (IFNγ) and/or tumor necrosis factor-α (TNFα) were studied in a serum-free culture system. In addition, the function of CD54 was assessed by a cellular aggregation assay. Prior to in vitro culture, only 3/10 ALL cases had more than 20% CD54-positive blasts. A 24-h incubation in serum-free medium alone induced CD54 positivity in another two cases. In these two ALLs, stimulation with IFNγ and/or TNFα further enhanced CD54 positivity. In addition, TNFα induced CD54 expression in one further case. In the remaining four cases no CD54 expression was induced by either cytokine. Of the six cases with constitutive or inducible CD54 expression, only five displayed CD54-dependent cellular aggregation. Taken together, the ALLs studied were heterogeneous with respect to their constitutive and cytokine-driven CD54 expression, while TNFα seemed to be more effective than IFNγ. In most cases, the CD54 molecule was functionally active, in that CD54 expression was paralleled by CD54-dependent homotypic aggregation of the blasts.
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  • 9
    ISSN: 1432-0584
    Keywords: Key words CML ; Chemotherapy ; PBSC mobilization
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  High-dose chemotherapy with autologous transplantation of in vivo purged PBSC is a novel investigational approach to treating chronic myelogenous leukemia (CML) patients not responsive to conventional therapy with interferon-α (IFN-α) and not eligible for allogeneic transplantation. PBSC mobilization using either '5+2/7+3'-type chemotherapy or 'mini-ICE/ICE' chemotherapy was investigated in 43 patients with advanced phases of Philadelphia (Ph)-positive CML. Thirty patients were in late chronic phase (〉12 months post diagnosis) and 13 patients in accelerated phase (AP) or blast crisis (BC). Contamination with Ph-positive cells was evaluated in harvests from 37/43 patients. The outcome of PBSC mobilization was dependent on the type of chemotherapy administered: a complete or major cytogenetic response (〈35% Ph-positive metaphases) in leukapheresis collections was obtained in ten of 15 patients treated with 'mini-ICE/ICE' but in only three of 28 patients treated with '5+2/7+3' chemotherapy. One patient (1/43) in blast crisis died during mobilization therapy (2%). Twenty-five patients underwent PBSC transplantation and all of them engrafted successfully. Transplantation-related mortality was 0%. The data show that in advanced phases of CML the chance of harvesting Ph-negative peripheral blood stem cells depends on the type of chemotherapy used for mobilization.
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  • 10
    ISSN: 1432-0584
    Keywords: Key words Myelodysplastic syndrome ; All-trans retinoic acid ; Interferon alpha ; Granulocyte colony-stimulating factor ; Differentiation therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Used as single agents, ATRA, G-CSF, and IFN-α have shown a moderate benefit in patients with low-risk MDS, with a response rate of 10%. The aim of the present study was to evaluate the efficacy of a combination of these agents. The effect on hemoglobin (Hb), platelets, and absolute neutrophil count (ANC), as well as on transfusion frequency, was examined in 25 patients with MDS (11 RA, four RARS, eight RAEB, two CMML). The median age was 61 years (range 44–81), and the male/female ratio was 14/11. Treatment consisted of ATRA at 25 mg/m2/day p.o. for months 1, 3, 5, 7, 9, and 11, IFN-α at 1.5 MIU twice a week s.c. for 52 weeks, and, in patients with initial ANC 〈500/μl, G-CSF at 100–480 μg daily s.c. according to the degree of ANC. The duration of therapy was scheduled for 12 months. Two patients achieved ongoing CR (+19 months; +16 months), one patient with RA after 3 months and one with CMML after 7 months of treatment. In all patients, the mean ANC increased significantly from 1400±200/μl before the start of therapy to 3500±600/μl at the end of treatment (p=0.025). In two patients an increase of Hb was observed, and one patient ceased to require transfusions. In an additional patient with RA and 5q-syndrome, the platelet count normalized following administration of ATRA/IFN-α, increasing from 89,000/μl to 293,000/μl. The eight RAEB patients were nonresponders. We conclude that therapy with ATRA, IFNα, and G-CSF is effective in approximately 35% of low-risk MDS patients (in this study: six of 17) and may induce complete remission in individual cases.
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