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  • Seckel syndrome  (2)
  • Sonographie  (2)
  • Clinical trial  (1)
  • 1
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Nephroblastom ; Klinische Studie ; Präoperative Chemotherapie ; Diagnostische Sicherheit ; Therapiereduktion ; Key words Nephroblastoma ; Clinical trial ; Pre-operative chemotherapy ; Diagnostic accuracy ; Reduction of therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: In a new study the intensity of treatment used in the former Wilms' Tumor Study of the „Gesellschaft für Pädiatrische Onkologie und Hämatologie“ (GPOH) since 1980 had to be reduced maintaining the good results. Methods: 1989 the strategy of the International Society of Pediatric Oncology (SIOP) with pre-operative chemotherapy after diagnostic imaging was adopted. The pretreatment should reduce the tumorsize to allow complete tumor resection in most patients. Results: Of 505 patients enrolled in the study over 5.25 years from 78 participating centers, 486 had a nephroblastoma, 14 another malignant tumor and 5 a benign renal lesion. Of 438 patients with nephroblastoma aged between 0.5 and 16 years 85.4 % received pre-operative chemotherapy. Compared to the prior study the percentage of patients with irradiation (22.8 %) was lowered and the cumulative dose of Adriamycin for higher stages and unfavourable histology reduced. The 91 % probability of crude survival after 3 years for all nephroblastoma patients was similiar to the former study. Conclusions: The concept of general pre-operative chemotherapy for nephroblastoma was accepted by GPOH with evident reduction of treatment intensity compared to the prior study.
    Notes: Zusammenfassung Hintergrund: Die intensive Therapie der von der Gesellschaft für Pädiatrische Onkologie und Hämatologie (GPOH) ab 1980 durchgeführten Wilmstumorstudie sollte in der Folgestudie unter Wahrung der guten Ergebnisse reduziert werden. Methode: 1989 wurde das Therapiekonzept der International Society of Pediatric Oncology (SIOP) mit präoperativer Chemotherapie nach bildgebender Diagnose übernommen, wobei der Tumor durch die Vorbehandlung verkleinert wird, damit er bei einem Großteil der Kinder vollständig entfernbar ist. Ergebnisse: Bei 505 in 5,25 Jahren aus 78 Kliniken gemeldeten Patienten hatten 486 ein Nephroblastom, 14 ein anderes Malignom und 5 eine benigne renale Läsion. Von 438 gemeldeten Nephroblastompatienten zwischen 0,5 und 16 Jahren erhielten 85,4 % präoperativ eine Chemotherapie. Der Anteil bestrahlter Nephroblastompatienten (22,8 %) und die kumulative Adriamycindosis bei hohen Stadien bzw. hoher Malignität wurden im Vergleich zur Vorstudie reduziert bei ähnlicher Überlebenswahrscheinlichkeit von 91 % nach 3 Jahren. Schlußfolgerung: Das Konzept einer generellen präoperativen Chemotherapie zur Nephroblastombehandlung ist in der GPOH mit gutem Ergebnis und deutlicher Therapiereduktion im Vergleich zur Vorstudie durchzuführen.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Der Radiologe 38 (1998), S. 417-419 
    ISSN: 1432-2102
    Keywords: Schlüsselwörter Ultraschall ; Sonographie ; Pädiatrie ; Fortschritt ; Technologie ; Übersichtsbild ; Key words Ultrasound ; Sonography ; Pediatrics ; Progress ; Technology ; Extended field of view imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The extended field of view imaging procedure named ”SieScape” provides, for the first time, a mode of establishing in real time a sonographic picture up to 60 cm in length. This allows the display of large organs or tumors as well as large areas of the body in a single view, without having to assemble several pictures. Furthermore, pathological find-ings can be shown in full size in their surroundings. This method makes it considerably easier to convey sonographic results to the therapist who is not present at the investigation and to control a finding, for instance a tumor, during therapy.
    Notes: Zusammenfassung Das Panoramabildverfahren „Siescape” erlaubt erstmals im Echtzeitverfahren die Erstellung eines sonographischen Schnitt-bildes von bis zu 60 cm Länge. Dadurch lassen sich große Organe oder Tumoren sowie ganze Körperareale auf einem Bild ohne Montage darstellen. Außerdem können pathologische Befunde in ihrer Umgebung großflächig dargestellt werden. Die Übermittlung des sonographischen Befundes an den nicht bei der Untersuchung anwesenden Therapeuten und die Kontrolle eines Befundes, z.B. eines Tumors, unter Therapie, werden dadurch erheblich verbessert.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Der Radiologe 37 (1997), S. 62-73 
    ISSN: 1432-2102
    Keywords: Schlüsselwörter Maligne Lymphome im Kindesalter ; Sonographie ; Indikationen ; Befunde ; Differentialdiagnose ; Key words Malignant lymphoma in childhood ; Ultrasound ; Indications ; Findings ; Differential diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The sonographic diagnosis of malignant lymphoma in childhood is described. Malignant lymphomas are sonographically relatively uniform: initial enlargement of the lymph nodes and organs involved and disturbance of normal echo texture by mainly hypoechoic lesions can be found. Generally, four sonographic patterns of infiltration are described: diffuse, small nodular, large nodular and bulky type. Secondary, tumor-related or inflammatory complications (e. g. dislocation or compression of vessels, thoracic inlet syndrome, venous thrombosis, ileus, urinary retention, abscess and effusion) can be sonographically evaluated. Response to therapy correlates with normalization of size and echo texture and recovery from tumor-related complications. Differential diagnosis with ultrasound is based on the topographic distribution and echo pattern of infiltration and, with certain restrictions, on the echogenicity of lesions and perfusion feasible with Doppler sonography. The primary diagnosis has to be established histologically.
    Notes: Zusammenfassung Die Ultraschalldiagnostik maligner Lymphome im Kindesalter wird beschrieben. Lymphatische Sytemerkrankungen des Kindes sind sonographisch relativ einheitlich: initial finden sich Lymphknoten- und Organvergrößerungen, sowie Störung der normalen Echotextur meist in Form von hypoechogenen Läsionen. An den parenchymatösen Organen werden sonographisch im wesentlichen 4 Infiltrationsmuster beobachtet: diffus, klein-nodulär, groß-nodulär und der „Bulcky-Typ“. Sekundäre, tumor- oder entzündungsbedingte Komplikationen (Gefäßkompression, Einflußstauung, Thrombose, Ileus, Harntransportstörung, Abszesse, Ergüsse) sind sonographisch erfaßbar. Ein Therapieerfolg ist im Ultraschall an der Normalisierung der Lymphknoten- und Organgröße, der Rückkehr der normalen Organechogenität und der Rückbildung tumor- bzw. entzündungsbedingter Komplikationen erkennbar. Differentialdiagnostische Hinweise ergeben sich aus dem topographischen Verteilungsmuster und dem sonographischen Erscheinungsbild der Läsionen, sowie unter gewissem Vorbehalt anhand der Echogenität und der dopplersonographisch analysierbaren Durchblutungsverhätnisse. Die primäre Diagnose ist immer histologisch zu sichern.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. 796-799 
    ISSN: 1432-1076
    Keywords: Bird-headed dwarfism ; Craniosynostosis ; Microcephalic osteodysplastic primordial dwarfism ; Osteodysplastic primordial dwarfism ; Seckel syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Abstract We report on a 13-month old boy with microcephalic osteodysplastic primordial dwarfism (MOPD), whose radiographic signs correspond with type II of this entity. Some of his clinical signs, such as the anomalies of the external genitalia and the urinary tract, are common to this subgroup of MOPD, but he also shows unusual clinical signs including bilateral knee dislocation and hypoplasia of the anterior corpus callosum. His clinical course was unusual with several episodes of breathing difficulties and increased intracranial pressure secondary to craniosynostosis at the age of 16 months. After fronto-orbital advancement for the treatment of brachycephaly, his psychomotor development improved remarkably. Conclusion MOPD type II may have a wider range of expression than previously delineated.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 155 (1996), S. 796-799 
    ISSN: 1432-1076
    Keywords: Key words Bird-headed dwarfism ; Craniosynostosis ; Microcephalic ; osteodysplastic primordial dwarfism ; Osteodysplastic primordial ; dwarfism ; Seckel syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report on a 13-month old boy with microcephalic osteodysplastic primordial dwarfism (MOPD), whose radiographic signs correspond with type II of this entity. Some of his clinical signs, such as the anomalies of the external genitalia and the urinary tract, are common to this subgroup of MOPD, but he also shows unusual clinical signs including bilateral knee dislocation and hypoplasia of the anterior corpus callosum. His clinical course was unusual with several episodes of breathing difficulties and increased intracranial pressure secondary to craniosynostosis at the age of 16 months. After fronto-orbital advancement for the treatment of brachycephaly, his psychomotor development improved remarkably. Conclusion MOPD type II may have a wider range of expression than previously delineated.
    Type of Medium: Electronic Resource
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