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  • 1
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochemical and Biophysical Research Communications 136 (1986), S. 665-671 
    ISSN: 0006-291X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-2307
    Keywords: Hypertension ; Diabetes mellitus ; Cardiac hypertrophy ; Myocardial ultrastructure ; Morphometry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The effects of combined renovascular hypertension and diabetes mellitus on the rat heart were investigated in order to detect possible synergistic effects of the two conditions. Hypertensive diabetic and hypertensive nondiabetic young male Wistar rats were compared with diabetic and nondiabetic controls. Since the normal body weight increase of the diabetic animals was markedly suppressed a weight-matched nondiabetic control group was introduced in addition. Hypertension was established for eight weeks by a surgical stenosis of the left renal artery, diabetes mellitus was maintained for four weeks after a single intraperitoneal injection of 75 mg/kg streptozotocin. Light and electron microscopic stereological parameters were obtained for the left ventricular papillary muscles. The whole hearts were also investigated histologically. Qualitative morphology failed to substantiate synergistic effects in the hypertensive diabetic rats. Vascular abnormalities were not observed. The stereological parameters, however, revealed microstructural reactions which were observed exclusively in the hypertensive diabetic group: the volume ratio of mitochondria-to-myofibrils was decreased, the surface-to-volume ratio of mitochondria was increased (reduction of mitochondrial size) and the mean cross sectional area of capillaries was decreased. Similar quantitative mitochondrial changes have been frequently described in long-standing hypertension, but in the present investigation, they were not found in the nondiabetic hypertensive group. It is therefore concluded that diabetes mellitus potentiates the effects of chronic pressure overload on myocardial cells. However, the myocardial fibrosis which has been found by other groups at later stages of hypertension and/or diabetes mellitus was not detected in the present study. The reduced mean cross sectional area of capillaries in hypertensive-diabetic rats may be correlated with early molecular changes of the myocardial interstitium or with early abnormalities of small arteries. Thus our stereological results support the hypothesis that a non-coronary hypertensive diabetic cardiomyopathy occurs in mammalian hearts.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-2307
    Keywords: Bartter's syndrome ; Hyperprostaglandin E-syndrome ; Juxtaglomerular apparatus ; Renin-angiotensin system ; Immunocytochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A comparative immunocytochemical and electron microscopic study was performed on renal biopsies from two children with classical Bartter's syndrome (BS) and three children with a recently described variant, the so-called hyperprostaglandin E-syndrome (HES). Compared to age-matched controls, kidney specimens from patients with BS and HES disclosed a marked hypertrophy and hyperplasia of the juxtaglomerular apparatus (JGA). In addition, in HES focal tubular and interstitial calcifications accompanied by interstitial fibrosis and tubular atrophy were noted. On immunocytochemistry, chronic stimulation of the JGA in BS and HES was characterized by an increase in the number of renin-positive cells, particularly in the media of afferent arterioles, but also in efferent arterioles and in the glomerular stalk. The length of the renin-positive portion of the preglomerular arterioles was significantly increased when compared to controls (100±32 vs. 49±17 µm;p〈0.001). In addition, the immunoreactivity of individual renin-positive cells was markedly enhanced. On electron microscopy, “hypertrophy” of the RER and of Golgi complexes with paracrystalline deposits in dilated RER cisterns and protogranules indicated an increased renin synthesis. Renin could be identified in mature secretory granules as well as protogranules by immune electron microscopy. Angiotensinogen was present in hypertrophied epithelial cells of Bowman's capsule. Converting-enzyme reactivity was observed in controls as well as in BS and HES in the brush border of the proximal tubule. In contrast to previous reports, Angiotensin II was completely negative in control as well as in diseased kidneys. We conclude from our results that both BS and HES are characterized by a marked activation of the JGA and severe stimulation of the renin-angiotensin system. Since activation of this system, however, leads - independently of the primary stimulus - to qualitatively very similar morphological reactions, these results do not implicate a common pathogenetic mechanism to both conditions.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 415 (1989), S. 317-321 
    ISSN: 1432-2307
    Keywords: Gastric stromal tumours ; Myogenic tumours ; Nucleolar organizer regions ; Gastric leiomyosarcoma
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Silver staining of nucleolar organizer regions (AgNOR) was studied in 26 primary benign and malignant gastric stromal tumours of myogenic origin. The absolute number of AgNOR per nucleus and the size of AgNOR were compared with histomorphologic features of the tumours. The total number of AgNOR per nucleus in epithelioid and spindle cell leiomyosarcomas significantly (p〈0.001) exceeded that in leiomyomas, cellular leiomyomas and epithelioid leiomyoblastomas. The mean number or the size of AgNOR did not correlate with the number of mitoses or the tumour size. In addition, large and bizarre AgNOR were seen predominantly in histologically malignant tumours. Only one exceptional epithelioid leiomyoblastoma recurred despite a lack of conventional characteristics of malignancy and a low AgNOR count. Therefore, quantitative determination of the number of AgNOR is a new independent variable in myogenic gastric tumours. It provides additional information for the histopathological evaluation of this heterogenous group of mesenchymal tumours.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 379 (1978), S. 169-179 
    ISSN: 1432-2307
    Keywords: Progressive systemic sclerosis in childhood ; Segmental membranoproliferative glomerulonephritis ; Immune complex disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The renal lesions of a 5-year-old girl with progressive systemic sclerosis are described. The nephropathy was clinically characterised by moderate proteinuria, microscopic hematuria and transient hypertension. Light microscopy showed membranoproliferative glomerulonephritis of segmental character. On electron microscopy intramesangial, subendothelial and extramembranous glomerular deposits were observed. By immunofluorescence microscopy deposits of IgG, Clq, C4, C3, C5, C8 und C9 in a predominantly subendothelial location were found in all glomeruli. Vascular lesions were of minor degree. Histological and immunohistological findings are compatible with an immune complex disease.
    Type of Medium: Electronic Resource
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  • 6
    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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  • 7
    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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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 368 (1975), S. 73-86 
    ISSN: 1432-2307
    Keywords: Oligomeganephronie ; Hypertrophy ; Morphometric studies ; Progressive secondary sclerosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bioptisch gewonnenes Nierengewebe von 3 Patienten mit einer besonderen Variante kongenitaler, bilateraler Nierenhypoplasie, der bilateralen Nierenhypoplasie mit Oligomeganephronie wurden morphometrisch, licht- und elektronenmikroskopisch untersucht. Die morphometrischen Ergebnisse wurden mit denen gleichaltriger Kontrollfälle verglichen. Bei Oligomeganephronie lag eine Hypertrophie der Glomeruli ohne anteilige quantitative Unterschiede der einzelnen Glomerulusstrukturen vor. Alle gemessenen Strukturanteile (Einzelvolumen der Glomeruli, Volumen der Kapillarlumina, Volumen des Bowmanschen Kapselraumes und Oberfläche der Kapillaren pro Glomerulus) waren im gleichen Verhältnis vergrößert (5,4fach). Die Hypertrophie des proximalen Tubulus (2,4fach) zeigte sich jedoch disproportional stärker ausgeprägt als die Hypertrophie des distalen Tubulus (1,3fach). Die Gesamtzahl der Glomeruli war stärker vermindert, als bisher in der Literatur angegeben worden war. Der sekundäre progressive Sklerosierungsprozeß der Glomeruli wird als Folge einer chronischen Überlastung der Nephrome diskutiert.
    Notes: Summary Kidney biopsy specimens of three patients with a special form of hypoplasia, the oligomeganephronic hypoplasia (Oligomeganephronie) were studied with light- and electronmicroscopy as well as morphometric methods. The morphometric measurements demonstrated that there were no quantitative differences of the glomerular components between normal and oligomeganephronic glomeruli of patients of the same age. All of the structures measured — glomerular capillary surface, glomerular volume, volume of the glomerular capillary lumina and volume of Bowman's capsular space — were increased about 5.4 times. The volume of the proximal and distal tubules per μm tubular length in oligomeganephronic patients was disproportionally increased. The total number of glomeruli was more diminuished than mentionned in the literature until this time. The secondary glomerular sclerosis, initially focal, was progressiv and supposed to be caused by “chronic overwork”.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 408 (1985), S. 15-27 
    ISSN: 1432-2307
    Keywords: Human kidney ; Renal cell carcinoma ; Intermediate-sized filaments ; Co-expression ; Immunocytochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The expression of intermediate-sized filaments (IF) was examined by immunocytochemical methods in 40 primary renal cell carcinomas and compared with the IF distribution in the normal adult human kidney. All tumours stained positively with cytokeratin IF antibodies. Co-expression of cytokeratins and vimentin was observed in 21/40 (52,5%) renal carcinomas. Double immunofluorescence labelling demonstrated that in most of these cases tumour cells contained both cytokeratin and vimentin type IF. In normal human kidneys, cells of the various tubular segments disclosed a positive reaction with cytokeratin antibodies in a different intensity and intracellular localization. Co-expression of cytokeratin and vimentin IF in normal adult human kidneys has never been observed. From a histogenetic point of view, co-expression of cytokeratins and vimentin in renal cell carcinoma obviously represents an atavistic phenomenon since vimentin is re-expressed by these tumour cells during neoplastic transformation. This finding indicates the metanephric origin of the renal parenchyma. In surgical pathology the possibility of very rare co-expression of cytokeratin and vimentin IF within tumour cells should be considered, particularly in the differential diagnosis of clear cell carcinomas.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 13 (1999), S. 816-823 
    ISSN: 1432-198X
    Keywords: Key words Schönlein-Henoch purpura ; Glomerulonephritis ; Hypertension ; Proteinuria ; Renal failure ; Plasma exchange
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  We studied the long-term outcome of 64 children with biopsy-proven Schönlein-Henoch purpura (SHP) nephritis over 1–23 years of follow-up. Overall renal survival 10 years after onset was 73%. Multivariate logistic regression analysis identified initial renal insufficiency (P=0.004), nephrotic syndrome (P=0.037), and the severity of histological alterations, as defined by the proportion of glomerular crescents (P=0.051), as significant independent predictors of progressive renal failure. Four patients followed for more than 19 years showed glomerular damage after transient recovery. Eight children with crescentic nephritis associated with a rapidly progressive course and/or persistent nephrotic syndrome were treated by at least seven sessions of plasma exchange (PE) within 16 weeks of onset of purpura. During treatment serum creatinine levels dropped in each patient from a mean of 2.3 to 1.1 mg/dl, followed by a rebound increase. Repeated courses of PE in 5 patients produced comparable responses. Four patients undergoing PE reached end-stage renal disease at 1.2.–3.7 years after onset, whilst 3 finally were in preterminal renal failure (creatinine 3.2–6.1 mg/dl after 7–13.5 years), and 1 patient reached a normal glomerular filtration rate. Our experience suggests that initial renal insufficiency is the best single predictor of the further clinical course in children with SHP nephritis. Early PE appears to delay the progression in some patients with severe, rapidly progressive forms of the disease.
    Type of Medium: Electronic Resource
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