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  • Craniosynostosis  (2)
  • Asymptomatic bacteriuria  (1)
  • Clinical trial  (1)
  • 1
    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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  • 2
    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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  • 3
    ISSN: 1432-1440
    Keywords: Vesico-renal reflux ; Renal parenchymal scar ; Urinary tract infection ; Asymptomatic bacteriuria ; Uroepithelial cell ; Vesico-renaler Reflux ; Nierenparenchymnarbe ; Harnwegsinfektion ; Asymptomatische Bakteriurie ; Uroepithelzelle
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung 62 Patienten (14 Knaben, 48 Mädchen), bei denen an insgesamt 85 Niereneinheiten ein vesico-renaler Reflux (Grad 2–4) erfolgreich operiert worden war, wurden im Durchschnitt 9,3 Jahre postoperativ auf das Auftreten von Harnwegsinfektionen (HWI) und von neuen Nierenparenchymnarben (NN) verfolgt. Bei den männlichen Patienten traten postoperativ bis auf einen Knaben keine HWI bzw. NN auf. Ähnliches galt auch für etwa 45% der Mädchen. Beide Gruppen von Patienten wiesen vor Operation hohe Refluxgrade auf. Bei den übrigen Mädchen, deren präoperative Reflux-Grade im Durchschnitt relativ niedrig lagen, traten jedoch postoperativ über Jahre noch HWI auf. NN entwickelten sich vorwiegend in dieser Zeit. Untersuchungen der Fähigkeit von Uroepithelzellen (UEZ), das Bakterienwachstum zu hemmen, zeigten, daß die UEZ dieser Patienten das Bakterienwachstum nicht supprimieren konnten. Somit entsprachen diese Mädchen bezüglich ihrer UEZ-Funktion Patientinnen mit asymptomatischer Bakteriurie. HWI und NN bei Reflux scheinen somit durch unterschiedliche Ursachen ausgelöst zu werden.
    Notes: Summary 62 patients (14 boys, 48 girls) representing 85 refluxive renal units (Grade 2–4) were investigated after successful operation for the development of further urinary tract infections (UTI) and renal scars (RS). The mean follow-up was 9.3 years. With the exception of one boy, none of the male patients developed any UTI or new RS. A similar result was obtained for about 45% of the girls. These two groups of patients presented with high-grade reflux before surgery. The remaining female patients (about 55%), however, presenting with lower-grade reflux before surgical treatment, developed further UTI as well as new RS despite surgical correction of their reflux. Investigations on the capacity of uroepithelial cells (UEC) to suppress bacterial growth revealed a deficient antibacterial effect of UEC in these patients. Such an UEC defect has also been shown in patients with asymptomatic bacteriuria. In conclusion, different reasons seem to be responsible for recurrent UTI and the development of RS in patients with reflux.
    Type of Medium: Electronic Resource
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  • 4
    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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