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  • 1
    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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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 8 (1994), S. 407-407 
    ISSN: 1432-198X
    Keywords: Bartter's syndrome ; Hyperprostaglandin E2 syndrome ; Pathophysiology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 147 (1988), S. 341-349 
    ISSN: 1432-1076
    Keywords: Eiosanoids ; Prostaglandins ; Modulation ; Mediation ; Early life
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Prostanoids are unsaturated cyclic fatty acids, are synthesized primarily from arachidonic acid, and, like the leukotrienes, belong to the growing family of eicosanoids. As tissue hormones, prostanoids act on specific receptors near their site of synthesis and degradation. Prostanoids operate as modulators and mediators in a large spectrum of physiological processes. They are involved in the regulation of maternal and fetal circulation, patency of the ductus arteriosus, plateletvessel wall interaction and kidney function. Besides their physiological function in protecting organ perfusion under stress conditions, they are also involved in diseases as described in the hyperprostaglandin E2-syndrome or — together with leukotrienes-in inflammatory processes. More specific pharmacological tools than the nonsteroidal antiinflammatory drugs, such as receptor antagonists, selective synthesis inhibitors, and eicosanoid analogues offer the prospect of enriching our arsenal of pharmacotherapeutic interventions in a variety of diseases. Before active intervention, however, more and specific biochemical analyses are required to identify the pathophysiological role of eicosanoid.
    Type of Medium: Electronic Resource
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