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  • 1
    ISSN: 1432-2277
    Schlagwort(e): Pediatric renal transplantation ; Cyclosporin, low-dose, in children ; Growth, cyclosporin, in kidney transplantation
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Fifty-one pediatric patients undergoing a first cadaveric kidney transplantation were followed for at least 2 years after grafting. They were divided into two groups: those treated with methylprednisolone plus azathioprine (AZA) and those treated with methylprednisolone plus low-dose cyclosporin A (CyA; median dose 109 mg/m2 per day ≙ 3.4 mg/kg per day after 1 year). The steroid dosage given was significantly lower in the second group. The 4-year graft survival rate was 68% for the AZA group and 78% for the CyA group. Renal function did not differ significantly in the two groups; after 1, 2, and 3 years, the median 24-h creatinine clearance was 79, 69, and 51 ml/min/1.73 m2, respectively, for the AZA group and 78, 63, and 68 ml/min/1.73 m2, respectively, for the CyA group. Linear growth was similar in the two groups. We conclude that in pediatric patients the results of low-dose CyA immunosuppression do not differ significantly from those obtained with AZA in terms of graft survival, renal function, or growth.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Springer
    Virchows Archiv 368 (1975), S. 73-86 
    ISSN: 1432-2307
    Schlagwort(e): Oligomeganephronie ; Hypertrophy ; Morphometric studies ; Progressive secondary sclerosis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: 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.
    Notizen: 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”.
    Materialart: Digitale Medien
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  • 3
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric nephrology 1 (1987), S. 428-435 
    ISSN: 1432-198X
    Schlagwort(e): Glomerular basement membrane ; Type IV collagen ; Alport's syndrome
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Urinary excretion of glomerular basement membrane (GBM)-related peptides was analysed in 72 patients with a variety of renal diseases by immunoblotting using polyclonal antibodies against either collagenase or pepsin digests of human GBM. The specificity of the antibodies was verified by elution of antibodies bound to urinary GBM-related peptides on nitrocellulose blots and demonstration of reactivity of the eluted antibodies with the respective GBM digests. Furthermore, six mice immunized with urinary GBM-related peptides all developed focal linear deposits of mouse IgG along their GBM, linear and mesangial deposits of C3 in the glomeruli and serum antibodies reactive with human GBM. Monoclonal antibodies against urinary GBM-related peptides of one of the mice reacted with different peptides of the non-collagenous and collagenous domains of type IV collagen, the major structural protein of GBM. In the majority of the 75 patients' urines tested, excretion of GBM-related peptides with molecular weights of 33, 50, 80 and 150 kilodaltons (kD) was detectable. Patients with a diminished glomerular filtration rate (GFR) demonstrated excretion of the 33 kD peptide more frequently (91%) and never of the 80 kD peptide as compared with patients with normal GFR (33 kD [42%] 80 kD [87%]). The pattern of urinary GBM-related peptides was not specific for the underlying renal disease as in Alport's syndrome.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    Digitale Medien
    Digitale Medien
    Springer
    Pediatric nephrology 3 (1989), S. 397-400 
    ISSN: 1432-198X
    Schlagwort(e): Aldosterone ; Atrial natriuretic peptide ; Chronic renal failure ; Dopamine ; Noradrenaline ; Sodium homeostasis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract In order to evaluate the possible role of vasoactive hormones in the mechanism of exaggerated sodium loss due to reduced renal mass we measured plasma concentration of atrial natriuretic peptide (ANP), aldosterone, plasma renin activity (PRA), plasma noradrenaline, and dopamine, in 12 children with advanced chronic renal failure (mean CIn17.8-2.6,x± SEM, CPAH93.5±17 ml/min per 1.73 m2, FENa7.0±0.95%). No patient had clinical signs of volume overload. Plasma concentrations of ANP were not significantly different from those of healthy agematched controls (29.2±7.2 vs 23.2±3.1 fmol/ml) and did not correlate with urinary sodium excretion. Plasma concentrations of aldosterone, PRA and noradrenaline, were also within the physiological range, while plasma dopamine levels were elevated (260±36 vs 98±11 pg/ml, 〈0.001). Our data do not support the notion that ANP or the renin-aldosterone axis play a major role in the adaptation of remaining nephrons to maintain long-term sodium balance in normotensive children with chronic renal failure.
    Materialart: Digitale Medien
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  • 5
    Digitale Medien
    Digitale Medien
    Springer
    Journal of molecular medicine 54 (1976), S. 405-413 
    ISSN: 1432-1440
    Schlagwort(e): Uremia ; slipped epiphysis ; renal osteodystrophy ; vitamin D ; parathyreoidectomy ; growth ; Urämie ; Epiphysenlösung ; renale Osteodystrophie ; Vitamin D ; Parathyreoidektomie ; Wachstum
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Zusammenfassung Die urämische Epiphyseolyse unterscheidet sich grundlegend von der juvenilen Epiphyseolyse. Anhand eigener Erfahrungen bei der Behandlung von 8 Kindern mit urämischer Epiphysenlösung wurden Indikation und Durchführung der konservativen sowie der chirurgischen aktiven Behandlung besprochen. Eine Stabilisierung der abgerutschten Epiphysen wurde ohne chirurgische Maßnahmen und meist ohne Parathyreoidektomie allein durch Vitamin D3-Therapie innerhalb weniger Wochen erreicht. Die Initialdosis betrug 10 000–30 000 E Vitamin D3/die, die kurative Gesamtdosis 1,8–5,6 Mill. E Vitamin D3. Längerdauernde Immobilisation durch Bettruhe war unnötig. Auf den Wert der Urin-Calciumausscheidung zur Früherkennung der Vitamin D-Intoxikation auch bei fortgeschrittener Niereninsuffizienz wurde hingewiesen. Stärkste metaphysäre Verbiegungen an distalem Femur und distaler Tibia machten bei einem Patienten eine operative Korrektur notwendig, um die Gehfähigkeit des Kindes sicherzustellen. — Als allgemeine Behandlungs-richtlinie ist anzustreben, zunächst die metabolische Skeleterkrankung durch Vitamin D-Therapie mit oder ohne Parathyreoidektomie zu beseitigen. Erst anschließend sollen notwendige Osteotomien zur Beseitigung metaphysärer Deformierungen oder einer Coxa vara epiphysarea durchgeführt werden.
    Notizen: Summary Epiphyseal slipping in uraemia differs strikingly from juvenile epiphyseal slipping with respect to pathology and therapy. Based on our own experience with the treatment of 8 uraemic children with epiphyseal slipping, an effort was made to establish the respective indications for conservative and surgical treatment. Mechanical stabilization of slipped epiphyses was achieved within a few weeks without any surgery and usually without parathyreoidectomy by vitamin D3 alone. The initial dose was 10,000 to 30,000 I.U./day, the total curative dose 1.8 to 5.6 millions I.U. Prolonged immobilization was unnecessary. Rising urinary calcium excretion was a valuable indicator of vitamin D intoxication even in advanced renal failure. In one case, pronounced metaphyseal deformations (distal femur, distal tibia) required surgical correction before the ability to walk normally was restored. — The following therapeutical approach is recommended: metabolic bone disease must be cured by vitamin D therapy with or without parathyreoidectomy. Osteotomy to correct metaphyseal deformities or coxa vara epiphysaria never should be performed before metabolic bone disease is healed.
    Materialart: Digitale Medien
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  • 6
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 120 (1975), S. 111-120 
    ISSN: 1432-1076
    Schlagwort(e): Infantile nephrotic syndrome ; Mesangial sclerosis ; Immunohistology ; Pathogenesis of nephrotic syndrome
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Zusammenfassung Bei einem Säugling, der im Alter von 10 Monaten an einem steroid-resistenten nephrotischen Syndrom erkrankte, wurde bei der Nierenbiopsie eine diffuse mesangiale Sklerose der Glomeruli festgestellt. Nach dem Tod an interstitieller Pneumonie 3 Monate nach Krankheitsbeginn wurde der Nierenbefund bestätigt. Der Prozeß betraf sämtliche Glomeruli, war aber segmental verschieden stark ausgeprägt. Immunhistologisch wurden granuläre Ablagerungen von IgM und Komplement (C3) im Mesangium und subendothelial an der Basalmembran beobachtet. Die Befunde sprechen dafür, daß bei der Pathogenese der diffusen mesangialen Sklerose möglicherweise die Bildung und glomeruläre Ablagerung von Immunkomplexen eine Rolle spielen.
    Notizen: Abstract A 10-month-old infant developed a steroid resistant nephrotic syndrome. The renal biopsy revealed diffuse mesangial sclerosis of the glomerula. The child died from interstitial pneumonia 3 months after onset of the renal symptoms. Post mortem, the glomerular changes were diffuse but prominent in certain segments of the glomeruli. The immunohistological examination showed granular deposits of IgM and C3 in the mesangium and in the subendothelial region of the basement membrane. These findings are compatible with the hypo. thesis that diffuse mesangial sclerosis is caused by glomerular deposition of immune complexes.
    Materialart: Digitale Medien
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  • 7
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 145 (1986), S. 565-568 
    ISSN: 1432-1076
    Schlagwort(e): Pyelonephritis ; xanthogranulomatous ; Amyloidosis ; Chronic renal failure
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Unilateral nephrectomy in an 8-year-old male with chronic renal failure and urosepsis revealed a combined histologic lesion of xanthogranulomatous pyelonephritis and amyloidosis. Amyloid deposits were found also in the rectum and liver. On clinical grounds xanthogranulomatous pyelonephritis was also suspected in the remaining kidney. Common causes of systemic amyloidosis were excluded. After operation the signs of amyloidosis diminished, probably due to removal of the infected kidney, but renal function deteriorated rapidly.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 8
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 128 (1978), S. 103-111 
    ISSN: 1432-1076
    Schlagwort(e): 2,3-diphosphoglycerate ; Adenosinetriphosphate ; Chronic renal failure ; Hemodialysis ; Anemia
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Erythrocyte 2,3-diphosphoglycerate (2,3-DPG) and adenosinetriphosphate (ATP) levels were determined in 43 children with chronic renal failure on conservative treatment (CT), and 12 children on regular hemodialysis (HD) immediately before and after a HD session. The results were compared to non-anemic and anemic controls. In spite of anemia, erythrocyte 2,3-DPG in renal failure was similar to non-anemic controls at normal blood pH, but rose during dialysis as a result of alkalosis. In contrast, ATP levels were high already at a normal blood pH. 2,3-DPG correlated with packed cell volume (PCV) in children with renal failure but at lower concentrations compared to controls. Both organic phosphates in the erythrocytes showed a significant correlation with blood pH. The poor increase of 2,3-DPG, in combination with elevated ATP levels, suggests uremia-induced inhibition of 2,3-DPG synthesis.
    Materialart: Digitale Medien
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  • 9
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 147 (1988), S. 233-238 
    ISSN: 1432-1076
    Schlagwort(e): Nephrotic syndrome ; Lipoproteins ; Apolipo-proteins ; Atherosclerosis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract We investigated lipoprotein profiles in 24 children with normal renal function at different stages of the idiopathic nephrotic syndrome (NS). Four groups of patients were studied: (I) steriod-resistant NS with persistent proteinuria; (II) untreated steroid-sensitive NS during a relapse; (III) steroid-sensitive NS in remission induced by steroid-treatment; (IV) steroid-sensitive NS in long-term remission with-out therapy. Triglycerides (TG), cholesterol (CHOL), and phospholipids (PLP) were measured in plasma as well as in the lipoprotein fractions of very low (VLDL), intermediate (IDL), low (LDL) and high density (HDL). Apoproteins (Apo) AI, AII, B and C-apoproteins were measured in patients of groups I and IV. Results were compared to those obtained in 24 healthy control subjects. All patients with active NS (groups I–III) had significantly elevated CHOL levels. TG and CHOL in the VLDL, IDL, LDL, and CHOL in HDL2, but not HDL3 were inversely correlated with the serum albumin level. Patients with active NS had increased concentrations of TG and CHOL in lipoprotein fractions of lower density. Total and fractionated HDL-CHOL was not significantly different from control levels in any group. Patients in group I had significantly reduced Apo AI levels, whereas an increase of Apo AI and Apo AII in HDL3 and of most C-apoproteins in both HDL fractions was observed in patients of group IV. While changes in HDL apoprotein composition during longterm remission are of yet unknown clinical significance, our data indicate an increased risk of atherosclerosis only in those paediatric patients with persistent steroid-resistant NS.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 10
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 128 (1978), S. 67-79 
    ISSN: 1432-1076
    Schlagwort(e): Nephrocalcinosis ; Renal tubular acidosis ; Chronic renal failure ; Calcium ; Magnesium ; Citrate
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Polyuria, hyposthenuria, hypomagnesemia, hypercalciuria, advanced nephrocalcinosis, low citrate excretion and low glomerular filtration rates were observed in two female siblings who were followed over 10 years. Acid loading revealed incomplete distal tubular acidosis. Hypomagnesemia was due to renal magnesium wasting. It is suggested that the defect in tubular transport of magnesium is an important factor in the pathogenesis of this syndrome.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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