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  • 1
    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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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 145 (1986), S. 330-336 
    ISSN: 1432-1076
    Keywords: UTI ; Renal scars ; Virulence factors ; Host factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Nephrogenic diabetes insipidus ; Intracerebral calcification ; Dwarfism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two siblings are reported with a unique association of nephrogenic diabetes insipidus, intracerebral calcifications, defective psychomotor development, dwarfism and peculiar facial appearance. The disorder is to be differentiated from similar disorders, notably the Cockayne syndrome.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 147 (1988), S. 579-581 
    ISSN: 1432-1076
    Keywords: Nephrolithiasis ; Extracorporeal shock-wave lithotripsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Non-surgical removal of renal and ureteral stones has proven successful in adults. In this study, 21 paediatric patients have been treated with the first generation extracorporeal shock-wave lithotriptor and an additional 13 children with a second generation local shock-wave lithotriptor. A total of 47 stones was treated. Treatment-related complications such as colics (17%/18.8%) or fever (8.3%/6.2%) were minimal. Stone passage occurred in 93% and 100%, respectively of each group. Open surgery is still the treatment of choice for large staghorn calculi. Extracorporeal shock-wave lithotripsy can be performed successfully in paediatric nephrolithiasis with stones of limited size.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 153 (1994), S. 257-259 
    ISSN: 1432-1076
    Keywords: Lymphocyte phenotyping ; Neonatal lymphocytes Flow cytometry ; Normal values
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Using flow cytometric analysis we investigated the distribution of major lymphocyte surface antigens in newborn infants. A total of 221 newborns entered the study, of whom 53 fullfilled our criteria of healthy mature neonates. Percentages of immunofluorescent-positive cells were as follows (median and range from 25th to 75th percentiles given): for CD1 3.8%; 2.3%–5.8%. CD2 60.9%; 52.4%–66.8%. CD3 57.5%; 50.5%–63.3%. TcRaß 57.7%; 48.1%–60.0%. CD4 36.3%; 28.0%–42.6%. CD8 23.0%; 20.0%–27.4%. CD11a 56.3%; 46.3%–68.5%. CD19 12.1%; 8.6%–14.8%. CD20 10.9%; 8.4%–12.9%. CD25 2.6%; 2.1%–4.5%. CDw52 61.0%; 51.2%–76.1%. CD71 5.2%; 3.1%–9.3%. While the ranges for the percentage of immunofluorescent-positive cells were rather small, there was a wide variation in the absolute numbers of marker immunofluorescent-positive cells.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Beckwith-Wiedemann syndrome ; Congenital hemihypertrophy ; Medullary sponge kidney ; Wilms tumour
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The occurrence of a Wilms tumour in a 4-year-old girl with bilateral medullary sponge kidney, Beckwith-Wiedemann syndrome and congenital hemihypertrophy demonstrates the close relationship between these disorders. Another six cases from the literature with congenital hemihypertrophy and with medullary sponge kidney are discussed, two of them also developed intra-abdominal neoplasm.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Urinary tract infection ; Bladder defence mechanism ; Uroepithelial cells ; Buccal epithelial cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The effect of buccal epithelial cells (BEC) on bacterial growth was investigated in healthy subjects as well as in patients with recurrent urinary tract infections (UTI) and compared to the antibacterial capacity of uroepithelial cells (UEC) of the same individuals. Epithelial cells were obtained from the following groups: healthy female controls; females without further UTI after reflux operation; females with asymptomatic bacteriuria (ABU); females with further UTI despite successful reflux operation; and patients with meningomyelocele (MMC) and recurrent UTI due to significant residual urinary volume. Cocultivation of Escherichia coli with BEC as well as UEC from healthy females or patients with MMC resulted in significant suppression of bacterial growth. However, neither type of epithelial cell showed an antibacterial effect when they were obtained from patients with recurrent UTI in the absence of urological abnormalities (ABU patients; reflux-corrected patients with further UTI). From these results it is concluded that a generalised epithelial defence defect is one important pathogeneic factor for recurrent idiopathic UTI.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 150 (1991), S. 599-600 
    ISSN: 1432-1076
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1076
    Keywords: Key words Niemann-Pick disease type C ; Genetic heterogeneity ; Complementation studies ; Pulmonary form
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An infant with an unusual clinical presentation and course of Niemann-Pick disease type C (NPC) is described. The baby presented with severe pulmonary involvement and hepatosplenomegaly at the age of 3 months and died of respiratory failure at the age of 7 months. Cell hybridization studies revealed that the infant belonged to the rare genetic complementation group 2. To our knowledge, this is the third reported case with early-lethal pulmonary involvement in NPC. All three reported patients belong to the minor complementation group 2. An elder brother of the present case had also died at 6 weeks of age from NPC with severe pulmonary involvement. The two complementation groups cannot be distinguished from each other by clinical, cellular and biochemical criteria except for severe pulmonary involvement which may be characteristic of the second group. Conclusion NPC may present in early infancy with severe pulmonary involvement mimicking interstitial pneumonia. Such manifestation may be characteristic of the rare genetic complementation group 2.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 153 (1994), S. 257-259 
    ISSN: 1432-1076
    Keywords: Key words: Lymphocyte phenotyping – Neonatal lymphocytes – Flow cytometry – Normal values
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. Using flow cytometric analysis we investigated the distribution of major lymphocyte surface antigens in newborn infants. A total of 221 newborns entered the study, of whom 53 fullfilled our criteria of healthy mature neonates. Percentages of immunofluorescent-positive cells were as follows (median and range from 25th to 75th percentiles given): for CD1 3.8%; 2.3% – 5.8%. CD2 60.9%; 52.4% – 66.8%. CD3 57.5%; 50.5% – 63.3%. TcRAβ 57.7%; 48.1% – 60.0%. CD4 36.3%; 28.0% – 42.6%. CD8 23.0%; 20.0% – 27.4%. CD11a 56.3%; 46.3% – 68.5%. CD19 12.1%; 8.6% – 14.8%. CD20 10.9%; 8.4% – 12.9%. CD25 2.6%; 2.1% – 4.5%. CDw 52 61.0%; 51.2% – 76.1%. CD71 5.2%; 3.1% – 9.3%. While the ranges for the percentage of immunofluorescent-positive cells were rather small, there was a wide variation in the absolute numbers of marker immunofluorescent-positive cells.
    Type of Medium: Electronic Resource
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