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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 394 (1982), S. 235-254 
    ISSN: 1432-2307
    Schlagwort(e): Nephronophthisis ; Chronic sclerosing tubulo-interstitial nephropathy ; Associated defect
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    Digitale Medien
    Digitale Medien
    Springer
    Virchows Archiv 397 (1982), S. 193-202 
    ISSN: 1432-2307
    Schlagwort(e): Minimal change nephrotic syndrome ; Children ; Morphometry ; Mesangial hypercellularity
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    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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  • 5
    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
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 6
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 138 (1982), S. 354-357 
    ISSN: 1432-1076
    Schlagwort(e): Adolescent cystinosis ; Renal function ; Renal morphology ; Renal transport of amino acids
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 7
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 141 (1983), S. 63-65 
    ISSN: 1432-1076
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 8
    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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  • 9
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 134 (1980), S. 13-22 
    ISSN: 1432-1076
    Schlagwort(e): IgA-Glomerulonephritis ; Haematuria ; Proteinuria
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: 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.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 10
    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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