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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric radiology 9 (1980), S. 19-26 
    ISSN: 1432-1998
    Keywords: Nephrocalcinosis ; Radiographs ; Computed tomography ; Sonography ; Histology ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 12 patients with nephrocalcinosis, radiographs were compared with computed tomography, sonography and histology. Computed tomography detects nephrocalcinosis at a very early stage of the disease, gives a better picture of the density and exten of nephrocalcinosis and may detect other findings (e. g. cysts). Radiographs may show many more details than computed tomography; they seem to be more suitable for observing the course of the disease and have a lower radiation dose. Sonography has not been helpful in the diagnosis of nephrocalcinosis. Histology correlates only moderately with the degree of nephrocalcinosis demonstrated in radiographs and computed tomography.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 59 (1981), S. 1149-1157 
    ISSN: 1432-1440
    Keywords: Hemodialysis ; Foreign body reaction ; Silicone ; Hepatosplenomegaly ; NonA, non-B Hepatitis ; Hämodialyse ; Fremdkörperreaktion ; Silikon ; Hepatosplenomegalie ; Non-A, non-B Hepatitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung In der vorliegenden Untersuchung wurde das Sektionsgut von 47 Patienten überprüft, die zwischen 1965 und 1980 unter Verwendung von Silikonschläuchen dialysiert wurden. Ferner wurde das Sektionsgut von zehn Patienten aus zwei Dialysezentren untersucht, in denen routinemäßig PVC Schläuche verwandt wurden. Bei 40 Patienten aus dem Zentrum mit Silikonschläuchen, bei denen die Dialysezeit unter 53 Monaten lag, konnte kein Fremdmaterial nachgewiesen werden. Bei sieben Patienten, die zwischen 52 und 165 Monaten dialysiert worden waren, zeigten sich bei der licht- und elektronenmikroskopischen Untersuchung Fremdkörpereinschlüsse in Makrophagen und Riesenzellen in folgenden Organen: Lunge, Leber, Milz, Knochenmark, Haut, thorakale und abdominale Lymphknoten. Das Material konnte in Hirn, Herz, Niere und endokrinen Organen nicht nachgewiesen werden. Fibrose, Nekrose oder Epitheloidzellreaktionen wurden nicht beobachtet. Das Fremdmaterial war nicht-isotrop, nicht kristallin und färbte sich mit Routine-Färbeverfahren nicht an. Die Elektronenmikroskopie zeigte, daß es von lysosomalen Membranen umschlossen war. Bei Patienten mit wesentlich kürzerer Dialysezeit konnte das Material in vivo durch Leberbiopsie eindeutig nachgewiesen werden. Hingegen wurde das Fremdkörpermaterial nicht gefunden bei den zehn Langzeithämodialyse-Patienten aus den zwei Zentren, die nicht routinemäßig Silikonschläuche verwandten. Alle Patienten mit positivem Nachweis wiesen eine Hepatosplenemegalie auf, viele Patienten hatten Transaminasen-Erhöhungen, ein Patient hatte Hypersplenismus mit Panzytopenie und ein Patient hatte ein Gallengangskarzinom. Durch Gaschromatographie und Massenspektrometrie konnten in der Leber von Dialysepatienten in hoher Menge Phthalatweichmacher nachgewiesen werden (5–11 ppm); daneben fanden sich bei einem Patienten in beträchtlicher Menge normalerweise nicht vorkommende, cholesterinähnliche Steroide. Durch Rasterelektronenmikroskopie und Röntgenfluoreszenz-Mikroanalyse ließen sich in Leber und Milz zahlreiche Partikel von mehreren μ-Größe in Makrophagen nachweisen. Entsprechende Einschlüsse konnten bei nichtdialysierten Kontrollindividuen nie gefunden werden. Das Energiespektrum der Partikel zeigte einen scharfen Si-Peak mit geringer Beimengung von Al und Cl. Die Partikel entsprachen in Form und Energiespektrum dem Abrieb von Silikonschläuchen nach Einwirkung von Rollerpumpen. Partikel mit reinem Cl-Peak als Hinweis auf PVC-haltige Partikel konnten bei Patienten mit Silikoneinschlußkörpern nicht nachgewiesen werden. Wegen der beobachteten klinischen Komplikationen müssen aus den vorgelegten Ergebnissen Konsequenzen für die Herstellung von Dialysematerial gezogen werden.
    Notes: Summary We studied autopsy material of 47 patients who were dialysed between 1969 and 1980 in one center in which silicone tubing was used. In addition, we examined ten patients from two other centers where silicone tubing was not routinely used. In 40 patients with exposure to silicone tubing who were dialysed for up to 53 months, no foreign material was demonstrable. In seven patients who were dialysed for periods between 52 and 165 months, light microscopy and electron microscopy showed varying amounts of foreign material in macrophages and giant cells of lung, liver, spleen, bone marrow, skin, thoracic and abdominal lymph nodes. No such material was found in brain, heart, kidney or endocrine organs. No epitheloid cell reaction, necrosis or fibrosis was observed. The material was non-isotropic, non-crystalline, and did not take up routine stains. Electron microscopy showed its presence within lysosomal membranes. In addition, in some patients who had been dialysed for much shorter periods of time, similar material could be demonstrated in liver biopsies obtained in vivo. No such material was observed in ten long-term dialysis patients of two centers where silicone tubing was not routinely used. All positive patients had hepatosplenomegaly, some had elevated transaminases, one patient had hypersplenism with pancytopenia and one patient had bile duct carcinoma. Gaschromatography with mass spectrometry of livers of dialysed patients showed high concentrations of phthalate plasticizer (5–10 ppm) and in one patient cholesterol-like steroids which were not found in normal livers. Surface scanning electron microscopy with non-dispersive X-ray fluorescence analysis revealed the presence of numerous particles up to 10 µ, in liver and spleen of dialysed patients but of not control patients. The particles showed a characteristic Si peak with a concomitant small contribution of Al and Cl. The particles were identical with filing of silicone tubing exposed to roller pumps. No material exhibiting a pure Cl peak (as evidence for PVC) was demonstrable in patients with positive silicone inclusions. Because of its clinical consequences, the observation will have implications for manufacturing of dialysis devices.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 61 (1983), S. 911-915 
    ISSN: 1432-1440
    Keywords: Mesangial IgA-glomerulonephritis ; IgA1 ; Polymeric IgA
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary IgA deposits in kidney and skin biopsies from patients with idiopathic mesangial IgA-glomerulonephritis were characterized with immunofluorescence microscopy using monoclonal antibodies against the IgA subclasses IgA1 and IgA2. IgA1 was the major constituent in all biopsy specimens. Double immunofluorescence microscopy showed that IgA deposits were constantly associated with J-chain. Secretory component was never found in the deposited material. In vitro fixation of free secretory component, however, was observed in some biopsies. These findings indicate that most if not all of the deposited IgA in patients with idiopathic IgA-glomerulonephritis is polymeric in nature.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 394 (1982), S. 235-254 
    ISSN: 1432-2307
    Keywords: Nephronophthisis ; Chronic sclerosing tubulo-interstitial nephropathy ; Associated defect
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinical and morphological findings are described in 27 children with nephronophthisis. Seventeen children were considered as sporadic cases. In 10 familial cases the presumed mode of inheritance was autosomal recessive. The clinical picture was rather uniform: polyuria-polydipsia, hyposthenuria, anemia, growth retardation, and azotemia with progressive renal failure. Six patients presented with tapeto-retinal degeneration. In a further seven children other ocular changes were detected. Two female siblings showed additional non-renal manifestations: mental retardation, pulmonary emphysema, skeletal anomalies, and congenital hepatic fibrosis. Renal histology displayed a chronic sclerosing tubulo-interstitial nephropathy with extensive tubular atrophy and dedifferentiation. Medullary cysts were frequently found in end-stage kidneys. Immunofluorescence was either non-specific or completely negative. On electron microscopy, the tubular basement membrane changes predominated: thickening, lamellation, splitting, and deposition of microfibrils within the increased basement membrane substance. Detailed light- and electron microscopic findings were non-specific but the overall morphologic picture was characteristic and even diagnostic in conjunction with the clinical presentation. A recurrence of nephronophthisis in transplanted kidneys has not been observed. The pathogenesis of nephronophthisis is obscure but with respect to the morphologic findings a primary or secondary tubular basement membrane defect seems very likely. Our experience suggests that nephronophthisis is a frequent cause of chronic renal failure in children and commonly associated with non-renal abnormalities. To avoid the separation of different syndromes presenting with a uniform renal disease but various non-renal manifestations, we suggest that the term “nephronopthisis complex” be used.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 397 (1982), S. 193-202 
    ISSN: 1432-2307
    Keywords: Minimal change nephrotic syndrome ; Children ; Morphometry ; Mesangial hypercellularity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Renal biopsies from 25 children with steroid-sensitive minimal change nephrotic syndrome were evaluated retrospectively to determine whether there is any relation between the morphological changes and the frequency of relapses. Biopsy material was examined by light-, immunofluorescence-, and electron microscopy, and by morphometric methods. The patients were divided in a group of 15 children with frequent relapses (FR) and another group of 10 children with an absence of, or only infrequent, relapses (NR/IR). Semiquantitative evaluation of biopsy specimens disclosed no significant differences between groups, but morphometric measurements performed on toluidine stained semithin sections showed a significant increase of mesangial nuclei in FR compared with NR/IR (P〈0.01). Furthermore, the mean area of mesangial nuclei was decreased and the relative frequency of smaller nuclear profiles was higher in patients with FR compared to NR/IR (p〈0.01). These findings suggest mesangial cell activation in FR which may be related to a longer course of the disease prior to renal biopsy (mean 4.0 years in FR vs. 1.4 years in NR/IR). In our opinion, morphometric assessment of discrete mesangial alterations is a promising method for exploring clinicopathological correlations in minimal change nephrotic syndrome.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 134 (1980), S. 13-22 
    ISSN: 1432-1076
    Keywords: IgA-Glomerulonephritis ; Haematuria ; Proteinuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Nineteen out of 83 children with asymptomatic haematuria were classified as having IgA glomerulonephritis, characterized by diffuse mesangial deposition of IgA in the absence of systematic disease. Different histological patterns were observed: i.e., minor glomerular lesions (5 cases), focal and segmental proliferative glomerulonephritis (8 cases) and diffuse proliferative glomerulonephritis (6 cases). Recurrent gross haematuria is the clinical hallmark of the disease. Proteinuria was present in 14 children and exceeded 1 g/m2/day in 3 patients. Clinico-pathological correlations showed a close relation between the degree of proteinuria and the histological lesions. Serum IgA levels were elevated in 3 children. Glomerular filtration rate remained above 80 ml/min/1.73 m2 in a 1 to 9 year follow-up.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 138 (1982), S. 354-357 
    ISSN: 1432-1076
    Keywords: Adolescent cystinosis ; Renal function ; Renal morphology ; Renal transport of amino acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Morphological and functional investigations of the kidney were performed in an adolescent with cystinosis. The late onset and the gradual development of moderate complex tubular dysfunction was documented. Whereas in patients with infantile cystinosis tubulo-intersitial lesions and tubular dysfunction are pronounced, this patient with adolescent cystinosis show a predominance of glomerular lesions and glomerular dysfunction.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Langenbeck's archives of surgery 361 (1983), S. 401-403 
    ISSN: 1435-2451
    Keywords: Pericardium ; Congenital wall defects ; Perikard ; Congenitale Bauchwanddefekte
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Lyophilisierte Humandura hat sich zur operativen Behandlung angeborener Bauchwanddefekte als biologischer Gewebeersatz bewährt. Nachteilige Adhäsionen mit den Intestinalorganen müssen dabei in Kauf genommen werden. Im Tierexperiment wurde Pferdeperikard auf seine Eignung zum heteroplastischen Bauchwandverschluß getestet und mit Dura verglichen. Makroskopisch zeichnete sich das Perikard durch bessere Flexibilität, höhere Reißfestigkeit und geringere Adhäsionsbildung aus. Histologisch fand sich im Gegensatz zur Dura ein Neoperitoneum aus Mesothelzellen.
    Notes: Summary Lyophilized human dura is commonly used as a biological tissue prosthesis for closing connatal wall defects. Unfortunately, adhesions with the intestine often occur. In an animal model we therefore compared dura with equine pericardium for heteroplastic closure of experimental abdominal wall defects. The pericardium resulted macroscopically in a better flexibility, higher tensile strength and less adhesions. In addition, histological findings indicated contrary to dura a neoperitoneal covering with mesothelial cells.
    Type of Medium: Electronic Resource
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